Oxford Handbook of Neurology (2 edn)
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Status epilepticus
Definition
Continuous seizures.
Two or more seizures with incomplete recovery inbetween.
Duration > 30 minutes.
Presentation
More common in those with mental handicaps or structural lesions, especially children. In established epilepsy, recent medication reduction/withdrawal, intercurrent illness, metabolic derangement, or progressive disease should be considered. Ensure any withdrawn/reduced AEDs are restarted. If no history of epilepsy consider the following:
Febrile illness (children).
Cerebral infections (e.g. encephalitis, meningitis).
Space-occupying lesion (e.g. tumour, haematoma).
Subarachnoid haemorrhage.
Cerebrovascular disease—haemorrhagic/ischaemic infarcts.
Metabolic derangement: ↓ glucose, ↓ Na, ↓↑Ca++.
Alcohol intoxication/withdrawal.
Toxicity (e.g. cocaine, carbon monoxide, tricyclic antidepressants).
Pseudo-status epilepticus—may have a previous history/normal EEG.
Note: mortality 10–20%.
Complications and management
| Cerebral | Cardiorespiratory | Systemic |
|---|---|---|
Cerebral oedema + ↑ ICP | Hyper/hypotension | Dehydration |
Cerebral damage secondary to hypoxia, seizure, or metabolic derangement | Cardiac arrhythmias | Electrolyte derangement (especially ↓ glucose, ↓ Na, ↓ Mg, ↑ K) |
Cerebral venous thrombosis | Cardiogenic shock | Metabolic acidosis |
Cerebral haemorrhage and infarction | Cardiac arrest | Hyperthermia |
Hypoxia (often severe) | Rhabdomyolysis | |
Aspiration pneumonia | Pancreatitis | |
Pulmonary oedema | Acute renal failure (often acute tubular necrosis) | |
Pulmonary embolism | Acute hepatic failure | |
Respiratory failure | Disseminated intravascular coagulation | |
Fractures |
| Cerebral | Cardiorespiratory | Systemic |
|---|---|---|
Cerebral oedema + ↑ ICP | Hyper/hypotension | Dehydration |
Cerebral damage secondary to hypoxia, seizure, or metabolic derangement | Cardiac arrhythmias | Electrolyte derangement (especially ↓ glucose, ↓ Na, ↓ Mg, ↑ K) |
Cerebral venous thrombosis | Cardiogenic shock | Metabolic acidosis |
Cerebral haemorrhage and infarction | Cardiac arrest | Hyperthermia |
Hypoxia (often severe) | Rhabdomyolysis | |
Aspiration pneumonia | Pancreatitis | |
Pulmonary oedema | Acute renal failure (often acute tubular necrosis) | |
Pulmonary embolism | Acute hepatic failure | |
Respiratory failure | Disseminated intravascular coagulation | |
Fractures |
Management of status epilepticus.
Further reading
Meierkord H, Boon P, Engelsen B, et al. (
Acute neuromuscular weakness
Acute flaccid paralysis may be due to disorders of:
nerve;
muscle;
neuromuscular junction.
In the early stages of an acute myelopathy due to trauma, an intraspinal haemorrhage or myelitis due to inflammatory or infectious causes, clinical signs may resemble those of a peripheral rather than a central disorder.
Clinical features
The tempo of progression will give a clue to aetiology. For example, sudden-onset paraparesis is most likely to be due to a vascular insult to the spinal cord such as anterior spinal artery (ASA) thrombosis.
Most of the neuromuscular causes tend to have a subacute course, progressing over a few days.
An exception are the periodic paralyses (both hyperkalaemic and hypokalaemic) which may be recurrent. Key finding is depressed or absent reflexes which will also be found in weakness due to secondary hypokalaemia. In the periodic paralyses attacks may last for minutes or hours in hyperKPP and hours/days in hypoKPP.
Significant sensory deficit is unusual in GBS, whereas a pure motor deficit without sensory loss is unusual in vasculitic neuropathy.
Sensory level and sphincter dysfunction implies a spinal cord disorder. Spinal cord compression without pain and a sensory level is unusual.
Back pain may be a feature of GBS.
Autonomic dysfunction occurs in GBS, but pupillary dilatation and hypersalivation are found in botulism. Persistent hypertension and tachycardia in association with pure motor weakness occurs in porphyria.
Differential diagnosis
See Table 3.2.
| Disorder | Clinical features | Investigations |
|---|---|---|
Peripheral nerve disorders | ||
Guillain–Barré syndrome | Subacute onset but may be sudden; few sensory signs; no sphincter involvement. Vascular autonomic dysfunction; no sensory level | NCT shows slowing but may be normal. CSF protein ↑; few cells, 10–20 mm3 |
Vasculitic neuropathy | Patchy motor and sensory loss; pain and dysaesthesia. Underlying primary vasculitic or rheumatological syndrome | NCT may reveal clinically asymptomatic lesions. Nerve ± muscle biopsy |
Acute intermittent porphyria | Distal motor neuropathy hypertension, and tachycardia | Blood and urine analysis |
Diphtheria | Oropharyngeal weakness at onset. Pharyngeal membrane | NCT—axonal neuropathy; serology |
Heavy metal poisoning, e.g. lead | Motor neuropathy, blue gum line, Mees lines, abdominal pain | Serum lead level |
Neuromuscular junction disorders | ||
Myasthenia gravis | Fluctuating muscle weakness, ocular, bulbar, respiratory involvement. Reflexes intact | Tensilon test, ACh receptor antibodies. EMG studies show decrement. Single fibre—jitter |
Lambert–Eaton syndrome | Variable muscle weakness. Ocular muscles spared. Underlying carcinoma | Voltage-gated calcium-channel antibodies. EMG shows potentiation |
Botulism | Muscle weakness; ophthalmoplegia with pupillary and autonomic changes | Isolation of organism from wound; serology |
Muscle disorders | ||
Inflammatory myopathy | Muscle pain and weakness, usually proximal. Rhabdomyolysis | CPK ↑, EMG myopathic; muscle biopsy |
Hypokalaemic periodic paralysis | Autosomal dominant. Duration: hours to days. Triggers: rest after exercise, carbohydrate meal, stress | Short exercise EMG; mutation in CACNA1S gene |
Hyperkalaemic periodic paralysis | Autosomal dominant. Duration: minutes to hours. Triggers: rest after exercise, K+-containing foods | Short exercise EMG; mutation in SCN4A gene |
Anterior horn cell disorder | ||
Due to poliovirus or other enteroviruses | Acute lower motor neuron syndrome | Stool culture; CSF PCR |
Myelopathic disorders | ||
Acute transverse myelitis | Initially, flaccid rather than spastic. Sphincter involvement, sensory level. May be first episode of demyelination or viral, e.g. herpes varicella zoster | MRI spine ± brain; CSF for oligoclonal bands; PCR |
Anterior spinal artery syndrome | Acute flaccid paralysis with sensory level. Sparing of posterior columns | MRI thoracic spine; cardiac, thrombophilia, vasculitic screen. Consider spinal AV malformation (usually thoracolumbar) |
Functional disorders | Bizarre gait, Hoover’s sign, non-organic sensory level, e.g. anterior not posterior chest. | MRI and CSF to exclude organic disorder |
| Disorder | Clinical features | Investigations |
|---|---|---|
Peripheral nerve disorders | ||
Guillain–Barré syndrome | Subacute onset but may be sudden; few sensory signs; no sphincter involvement. Vascular autonomic dysfunction; no sensory level | NCT shows slowing but may be normal. CSF protein ↑; few cells, 10–20 mm3 |
Vasculitic neuropathy | Patchy motor and sensory loss; pain and dysaesthesia. Underlying primary vasculitic or rheumatological syndrome | NCT may reveal clinically asymptomatic lesions. Nerve ± muscle biopsy |
Acute intermittent porphyria | Distal motor neuropathy hypertension, and tachycardia | Blood and urine analysis |
Diphtheria | Oropharyngeal weakness at onset. Pharyngeal membrane | NCT—axonal neuropathy; serology |
Heavy metal poisoning, e.g. lead | Motor neuropathy, blue gum line, Mees lines, abdominal pain | Serum lead level |
Neuromuscular junction disorders | ||
Myasthenia gravis | Fluctuating muscle weakness, ocular, bulbar, respiratory involvement. Reflexes intact | Tensilon test, ACh receptor antibodies. EMG studies show decrement. Single fibre—jitter |
Lambert–Eaton syndrome | Variable muscle weakness. Ocular muscles spared. Underlying carcinoma | Voltage-gated calcium-channel antibodies. EMG shows potentiation |
Botulism | Muscle weakness; ophthalmoplegia with pupillary and autonomic changes | Isolation of organism from wound; serology |
Muscle disorders | ||
Inflammatory myopathy | Muscle pain and weakness, usually proximal. Rhabdomyolysis | CPK ↑, EMG myopathic; muscle biopsy |
Hypokalaemic periodic paralysis | Autosomal dominant. Duration: hours to days. Triggers: rest after exercise, carbohydrate meal, stress | Short exercise EMG; mutation in CACNA1S gene |
Hyperkalaemic periodic paralysis | Autosomal dominant. Duration: minutes to hours. Triggers: rest after exercise, K+-containing foods | Short exercise EMG; mutation in SCN4A gene |
Anterior horn cell disorder | ||
Due to poliovirus or other enteroviruses | Acute lower motor neuron syndrome | Stool culture; CSF PCR |
Myelopathic disorders | ||
Acute transverse myelitis | Initially, flaccid rather than spastic. Sphincter involvement, sensory level. May be first episode of demyelination or viral, e.g. herpes varicella zoster | MRI spine ± brain; CSF for oligoclonal bands; PCR |
Anterior spinal artery syndrome | Acute flaccid paralysis with sensory level. Sparing of posterior columns | MRI thoracic spine; cardiac, thrombophilia, vasculitic screen. Consider spinal AV malformation (usually thoracolumbar) |
Functional disorders | Bizarre gait, Hoover’s sign, non-organic sensory level, e.g. anterior not posterior chest. | MRI and CSF to exclude organic disorder |
Guillain–Barré syndrome (GBS)
Epidemiology
Most common cause of acute neuromuscular paralysis. Annual incidence: 1–2/100 000. Occurs sporadically but epidemics occur in Northern China (AMAN).
Pathophysiology
Two-thirds preceded by a GI or URT infection. Most common are:
Campylobacter jejuni;
cytomegalovirus (CMV);
Epstein–Barr virus;
Haemophilus influenzae;
Mycoplasma pneumoniae.
75% cases due to an acute inflammatory demyelinating neuropathy (AIDP) with cellular and antibody mechanisms playing a role. In cases preceded by C. jejuni infection, molecular mimicry results in ganglioside antibodies (GM1). Significance of antibodies more apparent in the Miller Fisher variant (GQ1b antibody) and acute motor axonal neuropathy (AMAN) with the GD1a antibody.
Clinical features
Onset is with progressive, usually ascending, weakness with or without paraesthesia. By definition nadir is reached in 4 weeks.
Severe back pain may occasionally be a feature.
Cranial nerve involvement involves the facial and bulbar musculature.
Tendon reflexes are gradually lost.
Up to 25% have respiratory muscle weakness that may require ventilation.
Autonomic involvement (cardiac arrhythmia, hypertension, hypotension).
Regional variants
Miller Fisher syndrome (ophthalmoplegia, ataxia, and areflexia) strongly associated with the GQ1b antibody.
Pharyngo-cervico-brachial pattern.
Acute oropharyngeal palsy (similar to diphtheria).
Flaccid paraparesis variant.
Pure sensory variant.
Acute pandysautonomia.
Investigations
Check immunoglobulin levels as patients with IgA deficiency may develop anaphylaxis with IV immunoglobulin.
CSF examination: usually ↑ protein level but may be normal in the first week. WCC is usually normal (< 5 cells/mm3) (cytoalbuminaemic dissociation). If ↑ consider HIV infection (seroconversion), Lyme disease, or malignant infiltration (e.g. lymphoma).
Antibody measurements have little role to play in diagnosis but may have a prognostic role (GD1a).
NCT may be normal in the early stages (see Chapter 8).
Focal conduction block is a diagnostic hallmark but occurs proximally and may be difficult to demonstrate.
‘F’ waves may be prolonged indicative of a proximal demyelination.
Acute axonal degeneration occurs in AMAN or AMSAN but in AIDP may be due to secondary axonal damage associated with a poor outcome in terms of residual deficit.
Management
Disease-modifying treatment
Intravenous immunoglobulin (IV Ig) has become the treatment of choice. Similar efficacy to plasma exchange (PE). Dose: 0.4 g/kg/day for 5 days.
PE effective compared with supportive treatment alone. Four exchanges sufficient for moderate to severe disease. In mild disease (able to stand but not run), two exchanges may be adequate.
Combining PE and IV Ig does not confer additional benefit.
Although there are no data, in patients who show no response after 2 weeks (especially if there is still evidence of conduction block):
consider repeat course IV Ig or
consider PE.
If there is a relapse after a course of IV Ig, a repeat course may be reasonable.
Corticosteroids have not been shown to be useful in GBS.
General supportive management
Warn ITU and anaesthetist about a patient with GBS in hospital.
Respiratory: failure to recognize this insidious complication is one cause of mortality. Regular monitoring of vital capacity (VC), not peak flow, is essential. If this falls below 20 mL/kg (1.5 L for an average adult), transfer to the ITU. By the time O2 saturation or the PO2 falls, it is too late.
Swallowing: need SALT assessment. If compromised consider NG tube or PEG.
Cardiac: brady-and tachyarrhythmias as well as fluctuations in blood pressure occur as a result of autonomic involvement. ECG monitoring essential on severely affected patients at least until they are recovering.
Thromboembolic: all patients should be on low molecular weight heparin + TED stocking for DVT prevention.
Neuropathic pain is common: treat with gabapentin, carbamazepine, or analgesics such as tramadol. Amitriptyline should be avoided especially in the early stages because of its potential cardiac side effects.
Depression needs to be anticipated and treated if necessary.
Bowel functioning needs regulation—constipation occurs due to immobility and drug side effects.
Physiotherapy: essential in the early stages to prevent contractures and later during rehabilitation.
Outcome
Mortality is 5%. At 1 year 15% unable to walk unaided. Poor outcome associated with:
older age;
preceding diarrhoeal illness;
severity and rapid rate of deterioration;
electrically inexcitable nerves, and muscle wasting.
Further reading
van Doorn PA, Ruts L, Jacobs BC (
Acute headache (thunderclap headache)
2% of visits to A&E department are due to headache.
In patients with ‘worst ever’ headache and a normal neurological examination, 12% may have a subarachnoid haemorrhage (SAH). If neurological exam is abnormal, this becomes 25%. The diagnosis of SAH is missed initially in up to 32%.
‘Thunderclap headache’ may be defined as an abrupt-onset, often a ‘worst ever’, headache that is maximal in seconds but may develop in minutes.
Differential diagnoses
Vascular causes
SAH.
Carotid and vertebral artery dissection.
Cerebral venous thrombosis.
Arterial hypertension.
Non-vascular causes
Meningoencephalitis.
Intermittent hydrocephalus (colloid cyst of the third ventricle).
Spontaneous intracranial hypotension.
Primary headache syndromes
Coital cephalgia (headache associated with sexual activity). Note: first ever episode—exclude SAH.
Crash migraine.
Benign cough and exertional headache.
Ice-pick or idiopathic stabbing headache.
Exploding head syndrome.
Clinical features
The ‘red flags’ in a patient with such a presentation include:
worst ever headache;
onset with exertion (20% of SAH occur with exertion, e.g. sexual intercourse);
impaired alertness or conscious level, neck stiffness, progressive neurological deterioration;
abnormal neurological examination (third or sixth nerve palsy, papilloedema, subhyaloid haemorrhage, hemiparesis, or diplegia (anterior communicating aneurysm)).
A first episode of headache cannot be classified as tension-type headache (IHS criteria for diagnosis requires at least 9 similar episodes) or migraine (4 previous episodes required for diagnosis) without aura.
Investigations
All patients should have a CT scan and, if that is negative, a lumbar puncture.
CT scans become less sensitive to the detection of blood with time:
day 1 95%
day 3 74%
day 7 50%
day 14 30%
day 21 almost 0%.
Therefore 5% of scans in patients with SAH are normal initially. Technical factor: thin cuts (< 10 mm) are more sensitive than thicker cuts; if the haemoglobin is < 10g/L, blood appears isodense. Expertise in reading CT scans is essential.
If the CT scan is negative, an LP should be performed provided that there are no contraindications such as signs of ↑ICP.
Always measure OP—elevated in 60% of SAH, and in cerebral venous thrombosis.
Sample should be centrifuged immediately and the CSF compared with plain water in a glass tube against a white background.
In SAH, usually > 100 000 RBC + 1–2 WBC per 1000 RBC. If there are a lot more white cells consider meningitis complicated by a traumatic tap.
Alternatively, a few days after a SAH, a meningitic reaction may occur. In SAH protein is usually elevated.
Xanthochromia (resulting from breakdown of haemoglobin to oxyhaemoglobin (pink) and bilirubin (yellow)) may take at least 12 hours to develop; hence the recommendations to delay LP until 12 hours after ictus unless meningitis is a strong possibility. This may disappear after 14 days.
Although spectrophotometry is more sensitive than visual inspection in looking for xanthochromia, it is not widely available.
Other causes of xanthochromia: jaundice, elevated CSF protein (> 1.5g/L), malignant melanoma, and rifampicin.
If CT positive or there is persistently bloody CSF and/or xanthochromia by visual inspection, CT angiogram or formal cerebral angiography + neurosurgical opinion.
SAH versus traumatic tap
OP elevated in SAH.
Use three-tube test against a white background for xanthochromia.
WBC—in SAH, 1 per 1000 RBC. After 3–5 days, polymorphs and lymphocytes.
Subarachnoid haemorrhage (SAH)
SAH occurs in 1/10 000 of the population per year in the UK.
Clinical presentation
Clinical severity varies widely.
Headache—worst ever headache; ‘hit on the back of the head’. May occur during strenuous activity such as sexual intercourse. Associated with vomiting.
Coma.
Sudden death.
Examination may reveal:
typical signs of meningism (neck stiffness, photophobia, positive Kernig’s sign);
presence of subhyaloid haemorrhages on fundoscopy;
signs of ↑ICP (bradycardia, hypertension);
(late) papilloedema.
| Grade | Glasgow Coma Scale(GCS) score | Motor deficit |
|---|---|---|
1 | 15 | Absent |
2 | 14–13 | Absent |
3 | 14–13 | Present |
4 | 12–7 | Present or absent |
5 | 6–3 | Present or absent |
| Grade | Glasgow Coma Scale(GCS) score | Motor deficit |
|---|---|---|
1 | 15 | Absent |
2 | 14–13 | Absent |
3 | 14–13 | Present |
4 | 12–7 | Present or absent |
5 | 6–3 | Present or absent |
| Grade | Blood on CT |
|---|---|
1 | No blood detected (SAH diagnosed on LP) |
2 | Diffuse/vertical layers < 1 mm thick |
3 | Localized clot or layers > 1 mm thick |
4 | Intracerebral or intraventricular clot with diffuse or no SAH |
| Grade | Blood on CT |
|---|---|
1 | No blood detected (SAH diagnosed on LP) |
2 | Diffuse/vertical layers < 1 mm thick |
3 | Localized clot or layers > 1 mm thick |
4 | Intracerebral or intraventricular clot with diffuse or no SAH |
Causes
Berry aneurysm.
Traumatic and infectious aneurysms.
Clotting disorder, e.g. warfarin.
Dural AVM.
Diagnosis
CT scan positive in 95% in first 24 hours. If negative proceed to:
LP—measuring opening pressure and looking for evidence of blood and/or xanthochromia;
check clotting screen.
If CT scan positive or LP positive → CT angiogram or digital subtraction angiography (formal catheter angiogram) (
see Chapter 7).
Management
See Fig. 3.2 for flowchart.
Management flowchart for SAH.
Cerebral vasospasm
Focal cerebral ischaemia as a result of cerebral artery vasospasm is the greatest cause of neurological morbidity.
Vasospasm is maximal from 5 to 10 days post-SAH.
Standard prevention and treatment
Calcium antagonist nimodipine 60 mg 4-hourly has been shown to decrease the rate of development of vasospasm-induced ischaemic deficits from > 25% to < 20%.
Hydration with normal saline.
‘Triple H’ therapy = hypertension (with inotropic drugs), haemodilution, and hypervolaemia; used in established vasospasm.
Use of colloid solutions such as albumin, dextran, or hexastarch (to improve flow and rheology viscosity).
Chemical (papaverine) or balloon angioplasties to physically open up the cerebral arteries are also used, but with mixed results. Appears most useful around the time of endovascular (coil) or neurosurgical (clip) interventions, but the effects are probably not sustained.
Investigations
Many units utilize transcranial Doppler to monitor cerebral arterial flow as a surrogate marker of vasospasm.
Xenon-CT and diffusion–perfusion MRI are also used when available to study deficits in regional cerebral perfusion.
Securing the aneurysm to prevent rebleeding
Timing of the definitive treatment of cerebral aneurysms (coiling or clipping) will depend on:
patient’s general and neurological condition;
extent of angiographically defined vasospasm;
the ethos of the neurosurgical unit as to what degree the patients are treated ‘early’ or ‘late’.
However, emergency treatment is only advocated in those with large ICHs secondary to middle cerebral artery aneurysms.
Hydrocephalus
Outcome and prognosis
Angiogram-negative SAH. Following SAH, cerebral angiography is negative in 15–20% of cases, typically associated with prepontine (perimesencephalic) blood on CT. Has a typically benign course. Patient may have headaches for several weeks but no further haemorrhages. Small risk for development of hydrocephalus.
Patients with SAH due to an aneurysm:
30% die, usually out of hospital;
30% recover completely;
30% recover with some disability.
Further reading
Kassell NF, Torner JC, Haley EC Jr, Jane JA, Adams HP, Kongable GL (
Kassell NF, Torner JC, Jane JA, Haley EC Jr, Adams HP (
Molyneux A, Kerr R, Stratton I, et al. (
Imaging of SAH: examples
See Fig. 3.3 for acute subarachnoid haemorrhage with acute hyperdense blood within the basal cisterns, Sylvian fissures, and anterior interhemispheric fissure. A small amount of sulcal blood is also shown (small black arrow). Note the mild degree of communicating hydrocephalus.
Subarachnoid haemorrhage (SAH) (non-enhanced CT).
See Fig. 3.4 for acute SAH in the suprachiasmatic cistern and fourth ventricle with a focal haematoma in the inferior aspect of the anterior interhemispheric fissure at the site of the anterior communicating artery (ACom). Note the surrounding bilateral inferior frontal parenchymal low attenuation representing early ischaemia. Catheter angiography confirmed the presence of an irregular small aneurysm (black arrow) arising from the junction of the A1 and A2 segments of the left anterior cerebral artery (open black arrow). There is marked vasospasm and slower flow in the proximal left anterior cerebral artery (black arrowheads) with reduced opacification of the distal vessels in the ACA territory.
Subarachnoid haemorrhage (SAH); anterior communicating artery aneurysm. (a) Non-enhanced CT; (b) digital subtraction angiography.
See Fig. 3.5a,b, Fig. 3.5c,d for extensive acute subarachnoid haemorrhage which is shown within the anterior interhemispheric fissure, fourth ventricle, and Sylvian fissures, prominently on the right. Communicating hydrocephalus. The distribution is suggestive of a right MCA aneurysm which is shown on subsequent MRI as a rounded signal flow void (black arrow).
Right middle cerebral artery (MCA) aneurysm and acute SAH. (a) Non-enhanced CT; (b) axial T2-weighted; (c) 3-dimensional TOF MRA of the circle of Willis; and (d) MRA maximum intensity projection (MIP) MRI.
Right middle cerebral artery (MCA) aneurysm and acute SAH. (c) MRA and (d) rotated MIP image demonstrate the aneurysm arising at the bifurcation of the right MCA. The linear flow void in (b) (black arrowheads) represents the M1 segment of the right MCA.
Figure 3.6 shows an ill-defined hyperdensity within the right Sylvian fissure proximally (open white arrowheads) in keeping with acute SAH. The fundus of the large PCom artery aneurysm (white arrowheads) is directed posterolaterally and the neck arises from the communicating segment of the right internal carotid artery (white arrows). This is confirmed on digital subtraction angiogram following selective catheterization of the right internal carotid artery. In (b) the aneurysm (open black arrowheads) arises via a relatively narrow neck (black arrow) from the communicating segment of the right ICA. Note contrast within the right posterior cerebral artery (closed black arrowheads) indicating the presence of a prominent persistent posterior communicating artery.
Right posterior communicating artery (PCom) aneurysm and SAH. (a) Axial multiplanar reformation (MPR) from CT angiogram; (b), (c), (d) digital subtraction angiogram and coil embolization.
Figures 3.6 (c) and (d) depict the post-endovascular coil embolization of the aneurysm with the coil ball in (c) (black arrowheads) subtracted from the image in (d). The aneurysm is completely excluded and the posterior communicating artery is preserved with continued flow within the posterior cerebral artery (black arrowheads in (d)).
(c), (d) Post-endovascular coil embolization of the aneurysm.
Figure 3.7 shows hyperdense subarachnoid blood within the left cerebellar pontine angle extending into the fourth ventricle. The CT angiogram demonstrates a small rounded aneurysm arising at the origin of the left PICA (black arrow).
SAH: left posterior inferior cerebellar artery (PICA) aneurysm. (a) Non-enhanced CT; (b) axial image from CT angiogram.
Acute focal neurological syndromes
In patients who present with acute focal neurological deficit, the history and examination should point to the site of pathology and the possible pathological mechanism(s).
Clinical notes
Onset of symptoms
Sudden onset of focal neurological dysfunction without warning suggests a vascular aetiology.
Slow progression (‘march’) of symptoms over a few seconds suggests an ictal phenomenon.
Progression over minutes or hours points to a migrainous diathesis.
Exceptions to these rules occur since occasionally a stroke may progress in a stepwise manner over hours or days.
Gradual development of focal neurological deficit over days or weeks and months indicates a space-occupying lesion such as a tumour.
Duration of symptoms
The only factor distinguishing a TIA from a stroke is that the duration of TIA is < 24 hours, although most episodes last only a few minutes.
Nature of symptoms
Cerebrovascular events cause negative symptoms and signs, i.e. loss of sensory, motor, language, or visual function.
Ictal events generally cause positive phenomena such as tingling in an arm or leg.
Migraine may cause both positive and negative symptoms and signs—tingling marching up the arm and dysphasia.
Space-occupying lesions will result in a progressive loss of function or may trigger positive ictal symptoms.
Additional symptoms and signs
Associated throbbing unilateral headache during or after the development of neurological symptoms points to migraine, but headache occurs in 15% of patients with TIAs, 25% of patients with acute ischaemic stroke, and all cases of SAH.
Carotid and vertebral artery dissection may both cause focal neurological deficits in association with head, face, neck, or ocular pain.
In an elderly patient with monocular visual loss temporal arteritis must be excluded.
Subdural haematoma may present with an acute onset with or without headache.
Partial seizures may progress rapidly to generalized tonic–clonic seizures.
2% of patients presenting with an acute stroke may have a seizure, either partial or generalized, at onset.
Meningoencephalitis may present with symptoms and signs such as headache, neck stiffness, and photophobia, as well as focal signs due to an associated vasculitis.
Loss of consciousness
TIA and ischaemic stroke patients very rarely present with loss of consciousness.
If this does occur, the most likely causes are SAH, a large brainstem stroke, or a massive hemispheric intracerebral haemorrhage.
Large hemispheric ischaemic strokes may progress to coma after a few days (secondary haemorrhage).
Following a seizure, some patients may present with Todd’s paresis.
Causes of acute focal neurological symptoms and signs
Transient ischaemic attack (TIA)/stroke.
Migraine aura.
Partial (focal) seizure.
Intracranial structural lesions:
tumour;
subdural haematoma;
AVM;
giant aneurysm.
Multiple sclerosis and inflammatory CNS disorders.
Metabolic disorders:
hypoglycaemia;
hypo- and hypercalcaemia;
Wernicke’s encephalopathy.
Meningoencephalitis:
cerebral abscess;
associated vasculitis;
specific organisms, e.g. herpes simplex and temporal lobes, Listeria monocytogenes and brainstem involvement.
Other disorders:
myasthenia gravis;
hyperventilation and panic attacks;
somatization disorders.
Management of acute ischaemic stroke
| Patient Identification. ___ ___-___ ___- ___ ___ | Pt. Date of Birth ___ /___ /___ |
Hospital ____________(___-___) | Date of Exam ___ /___/___ | |
Interval: [ ] Baseline [ ] 2 hours post treatment | [ ] 24 hours post onset of symptoms ±20 minutes [ ] | 7–10 days [ ] 3 months [ ] |
Other ________________________________(___ ___) | ||
Time: ___ ___:___ ___ [ ]am [ ]pm Person Administering Scale _________________________________ | ||
Administer stroke scale items in the order listed. Record performance in each category after each subscale exam. Do not go back and change scores. Follow directions provided for each exam technique. Scores should reflect what the patient does, not what the clinician thinks the patient can do. The clinician should record answers while administering the exam and work quickly. Except where indicated, the patient should not be coached (i.e., repeated requests to patient to make a special effort). | ||
Instructions | Scale Definition | Score |
1a. Level of Consciousness: The investigator must choose a response if a full evaluation is prevented by such obstacles as an endotracheal tube, language barrier, orotracheal trauma/bandages. A 3 is scored only if the patient makes no movement (other than reflexive posturing) in response to noxious stimulation. | 0 = Alert; keenly responsive. 1 = Not alert; but arousable by minor stimulation to obey, answer, or respond. 2 = Not alert; requires repeated stimulation to attend, or is obtunded and requires strong or painful stimulation to make movements (not stereotyped). 3 = Responds only with reflex motor or autonomic effects or totally unresponsive, flaccid, and areflexic. | _____ |
1b. LOC Questions: The patient is asked the month and his/her age. The answer must be correct - there is no partial credit for being close. Aphasic and stuporous patients who do not comprehend the questions will score 2. Patients unable to speak because of endotracheal intubation, orotracheal trauma, severe dysarthria from any cause, language barrier, or any other problem not secondary to aphasia are given a 1. It is important that only the initial answer be graded and that the examiner not “help” the patient with verbal or non-verbal cues. | 0 = Answers both questions correctly. 1 = Answers one question correctly. 2 = Answers neither question correctly. | _____ |
1c. LOC Commands: The patient is asked to open and close the eyes and then to grip and release the non-paretic hand. Substitute another one step command if the hands cannot be used. Credit is given if an unequivocal attempt is made but not completed due to weakness. If the patient does not respond to command, the task should be demonstrated to him or her (pantomime), and the result scored (i.e., follows none, one or two commands). Patients with trauma, amputation, or other physical impediments should be given suitable one-step commands. Only the first attempt is scored. | 0 = Performs both tasks correctly. 1 = Performs one task correctly. 2 = Performs neither task correctly. | _____ |
2. Best Gaze: Only horizontal eye movements will be tested. Voluntary or reflexive (oculocephalic) eye movements will be scored, but caloric testing is not done. If the patient has a conjugate deviation of the eyes that can be overcome by voluntary or reflexive activity, the score will be 1. If a patient has an isolated peripheral nerve paresis (CN III, IV or VI), score a 1. Gaze is testable in all aphasic patients. Patients with ocular trauma, bandages, pre-existing blindness, or other disorder of visual acuity or fields should be tested with reflexive movements, and a choice made by the investigator. Establishing eye contact and then moving about the patient from side to side will occasionally clarify the presence of a partial gaze palsy. | 0 = Normal. 1 = Partial gaze palsy; gaze is abnormal in one or both eyes, but forced deviation or total gaze paresis is not present. 2 = Forced deviation, or total gaze paresis not overcome by the oculocephalic maneuver. | _____ |
3. Visual: Visual fields (upper and lower quadrants) are tested by confrontation, using finger counting or visual threat, as appropriate. Patients may be encouraged, but if they look at the side of the moving fingers appropriately, this can be scored as normal. If there is unilateral blindness or enucleation, visual fields in the remaining eye are scored. Score 1 only if a clear-cut asymmetry, including quadrantanopia, is found. If patient is blind from any cause, score 3. Double simultaneous stimulation is performed at this point. If there is extinction, patient receives a 1, and the results are used to respond to item 11. | 0 = No visual loss. 1 = Partial hemianopia. 2 = Complete hemianopia. 3 = Bilateral hemianopia (blind including cortical blindness). | _____ |
4. Facial Palsy: Ask – or use pantomime to encourage – the patient to show teeth or raise eyebrows and close eyes. Score symmetry of grimace in response to noxious stimuli in the poorly responsive or non-comprehending patient. If facial trauma/bandages, orotracheal tube, tape or other physical barriers obscure the face, these should be removed to the extent possible. | 0 = Normal symmetrical movements. 1 = Minor paralysis (flattened nasolabial fold, asymmetry on smiling). 2 = Partial paralysis (total or near-total paralysis of lower face). 3 = Complete paralysis of one or both sides (absence of facial movement in the upper and lower face). | _____ |
5. Motor Arm: The limb is placed in the appropriate position: extend the arms (palms down) 90 degrees (if sitting) or 45 degrees (if supine). Drift is scored if the arm falls before 10 seconds. The aphasic patient is encouraged using urgency in the voice and pantomime, but not noxious stimulation. Each limb is tested in turn, beginning with the non-paretic arm. Only in the case of amputation or joint fusion at the shoulder, the examiner should record the score as untestable (UN), and clearly write the explanation for this choice. | 0 = No drift; limb holds 90 (or 45) degrees for full 10 seconds. 1 = Drift; limb holds 90 (or 45) degrees, but drifts down before full 10 seconds; does not hit bed or other support. 2 = Some effort against gravity; limb cannot get to or maintain (if cued) 90 (or 45) degrees, drifts down to bed, but has some effort against gravity. 3 = No effort against gravity; limb falls. 4 = No movement.UN = Amputation or joint fusion, explain: _______________ 5a. Left Arm 5b. Right Arm | _____ _____ |
6. Motor Leg: The limb is placed in the appropriate position: hold the leg at 30 degrees (always tested supine). Drift is scored if the leg falls before 5 seconds. The aphasic patient is encouraged using urgency in the voice and pantomime, but not noxious stimulation. Each limb is tested in turn, beginning with the non-paretic leg. Only in the case of amputation or joint fusion at the hip, the examiner should record the score as untestable (UN), and clearly write the explanation for this choice. | 0 = No drift; leg holds 30-degree position for full 5 seconds. 1 = Drift; leg falls by the end of the 5-second period but does not hit bed. 2 = Some effort against gravity; leg falls to bed by 5 seconds, but has some effort against gravity. 3 = No effort against gravity; leg falls to bed immediately. 4 = No movement.UN = Amputation or joint fusion, explain: ______________ 6a. Left Leg 6b. Right Leg | _____ |
7. Limb Ataxia: This item is aimed at finding evidence of a unilateral cerebellar lesion. Test with eyes open. In case of visual defect, ensure testing is done in intact visual field. The finger-nose-finger and heel-shin tests are performed on both sides, and ataxia is scored only if present out of proportion to weakness. Ataxia is absent in the patient who cannot understand or is paralyzed. Only in the case of amputation or joint fusion, the examiner should record the score as untestable (UN), and clearly write the explanation for this choice. In case of blindness, test by having the patient touch nose from extended arm position. | 0 = Absent. 1 = Present in one limb. 2 = Present in two limbs. UN = Amputation or joint fusion, explain: ________________ | _____ |
8. Sensory: Sensation or grimace to pinprick when tested, or withdrawal from noxious stimulus in the obtunded or aphasic patient. Only sensory loss attributed to stroke is scored as abnormal and the examiner should test as many body areas (arms [not hands], legs, trunk, face) as needed to accurately check for hemisensory loss. A score of 2, “severe or total sensory loss,” should only be given when a severe or total loss of sensation can be clearly demonstrated. Stuporous and aphasic patients will, therefore, probably score 1 or 0. The patient with brainstem stroke who has bilateral loss of sensation is scored 2. If the patient does not respond and is quadriplegic, score 2. Patients in a coma (item 1a=3) are automatically given a 2 on this item. | 0 = Normal; no sensory loss. 1 = Mild-to-moderate sensory loss; patient feels pinprick is less sharp or is dull on the affected side; or there is a loss of superficial pain with pinprick, but patient is aware of being touched. 2 = Severe to total sensory loss; patient is not aware of being touched in the face, arm, and leg. | _____ |
9. Best Language: A great deal of information about comprehension will be obtained during the preceding sections of the examination. For this scale item, the patient is asked to describe what is happening in the attached picture, to name the items on the attached naming sheet and to read from the attached list of sentences. Comprehension is judged from responses here, as well as to all of the commands in the preceding general neurological exam. If visual loss interferes with the tests, ask the patient to identify objects placed in the hand, repeat, and produce speech. The intubated patient should be asked to write. The patient in a coma (item 1a=3) will automatically score 3 on this item. The examiner must choose a score for the patient with stupor or limited cooperation, but a score of 3 should be used only if the patient is mute and follows no one-step commands. | 0 = No aphasia; normal. 1 = Mild-to-moderate aphasia; some obvious loss of fluency or facility of comprehension, without significant limitation on ideas expressed or form of expression. Reduction of speech and/or comprehension, however, makes conversation about provided materials difficult or impossible. For example, in conversation about provided materials, examiner can identify picture or naming card content from patient’s response. 2 = Severe aphasia; all communication is through fragmentary expression; great need for inference, questioning, and guessing by the listener. Range of information that can be exchanged is limited; listener carries burden of communication. Examiner cannot identify materials provided from patient response. 3 = Mute, global aphasia; no usable speech or auditory comprehension. | _____ |
10. Dysarthria: If patient is thought to be normal, an adequate sample of speech must be obtained by asking patient to read or repeat words from the attached list. If the patient has severe aphasia, the clarity of articulation of spontaneous speech can be rated. Only if the patient is intubated or has other physical barriers to producing speech, the examiner should record the score as untestable (UN), and clearly write an explanation for this choice. Do not tell the patient why he or she is being tested. | 0 = Normal. 1 = Mild-to-moderate dysarthria; patient slurs at least some words and, at worst, can be understood with some difficulty. 2 = Severe dysarthria; patient’s speech is so slurred as to be unintelligible in the absence of or out of proportion to any dysphasia, or is mute/anarthric. UN = Intubated or other physical barrier, explain:_____________________________ | _____ |
11. Extinction and Inattention (formerly Neglect): Sufficient information to identify neglect may be obtained during the prior testing. If the patient has a severe visual loss preventing visual double simultaneous stimulation, and the cutaneous stimuli are normal, the score is normal. If the patient has aphasia but does appear to attend to both sides, the score is normal. The presence of visual spatial neglect or anosagnosia may also be taken as evidence of abnormality. Since the abnormality is scored only if present, the item is never untestable. | 0 = No abnormality. 1 = Visual, tactile, auditory, spatial, or personal inattention or extinction to bilateral simultaneous stimulation in one of the sensory modalities. 2 = Profound hemi-inattention or extinction to more than one modality; does not recognize own hand or orients to only one side of space. | _____ |
| Patient Identification. ___ ___-___ ___- ___ ___ | Pt. Date of Birth ___ /___ /___ |
Hospital ____________(___-___) | Date of Exam ___ /___/___ | |
Interval: [ ] Baseline [ ] 2 hours post treatment | [ ] 24 hours post onset of symptoms ±20 minutes [ ] | 7–10 days [ ] 3 months [ ] |
Other ________________________________(___ ___) | ||
Time: ___ ___:___ ___ [ ]am [ ]pm Person Administering Scale _________________________________ | ||
Administer stroke scale items in the order listed. Record performance in each category after each subscale exam. Do not go back and change scores. Follow directions provided for each exam technique. Scores should reflect what the patient does, not what the clinician thinks the patient can do. The clinician should record answers while administering the exam and work quickly. Except where indicated, the patient should not be coached (i.e., repeated requests to patient to make a special effort). | ||
Instructions | Scale Definition | Score |
1a. Level of Consciousness: The investigator must choose a response if a full evaluation is prevented by such obstacles as an endotracheal tube, language barrier, orotracheal trauma/bandages. A 3 is scored only if the patient makes no movement (other than reflexive posturing) in response to noxious stimulation. | 0 = Alert; keenly responsive. 1 = Not alert; but arousable by minor stimulation to obey, answer, or respond. 2 = Not alert; requires repeated stimulation to attend, or is obtunded and requires strong or painful stimulation to make movements (not stereotyped). 3 = Responds only with reflex motor or autonomic effects or totally unresponsive, flaccid, and areflexic. | _____ |
1b. LOC Questions: The patient is asked the month and his/her age. The answer must be correct - there is no partial credit for being close. Aphasic and stuporous patients who do not comprehend the questions will score 2. Patients unable to speak because of endotracheal intubation, orotracheal trauma, severe dysarthria from any cause, language barrier, or any other problem not secondary to aphasia are given a 1. It is important that only the initial answer be graded and that the examiner not “help” the patient with verbal or non-verbal cues. | 0 = Answers both questions correctly. 1 = Answers one question correctly. 2 = Answers neither question correctly. | _____ |
1c. LOC Commands: The patient is asked to open and close the eyes and then to grip and release the non-paretic hand. Substitute another one step command if the hands cannot be used. Credit is given if an unequivocal attempt is made but not completed due to weakness. If the patient does not respond to command, the task should be demonstrated to him or her (pantomime), and the result scored (i.e., follows none, one or two commands). Patients with trauma, amputation, or other physical impediments should be given suitable one-step commands. Only the first attempt is scored. | 0 = Performs both tasks correctly. 1 = Performs one task correctly. 2 = Performs neither task correctly. | _____ |
2. Best Gaze: Only horizontal eye movements will be tested. Voluntary or reflexive (oculocephalic) eye movements will be scored, but caloric testing is not done. If the patient has a conjugate deviation of the eyes that can be overcome by voluntary or reflexive activity, the score will be 1. If a patient has an isolated peripheral nerve paresis (CN III, IV or VI), score a 1. Gaze is testable in all aphasic patients. Patients with ocular trauma, bandages, pre-existing blindness, or other disorder of visual acuity or fields should be tested with reflexive movements, and a choice made by the investigator. Establishing eye contact and then moving about the patient from side to side will occasionally clarify the presence of a partial gaze palsy. | 0 = Normal. 1 = Partial gaze palsy; gaze is abnormal in one or both eyes, but forced deviation or total gaze paresis is not present. 2 = Forced deviation, or total gaze paresis not overcome by the oculocephalic maneuver. | _____ |
3. Visual: Visual fields (upper and lower quadrants) are tested by confrontation, using finger counting or visual threat, as appropriate. Patients may be encouraged, but if they look at the side of the moving fingers appropriately, this can be scored as normal. If there is unilateral blindness or enucleation, visual fields in the remaining eye are scored. Score 1 only if a clear-cut asymmetry, including quadrantanopia, is found. If patient is blind from any cause, score 3. Double simultaneous stimulation is performed at this point. If there is extinction, patient receives a 1, and the results are used to respond to item 11. | 0 = No visual loss. 1 = Partial hemianopia. 2 = Complete hemianopia. 3 = Bilateral hemianopia (blind including cortical blindness). | _____ |
4. Facial Palsy: Ask – or use pantomime to encourage – the patient to show teeth or raise eyebrows and close eyes. Score symmetry of grimace in response to noxious stimuli in the poorly responsive or non-comprehending patient. If facial trauma/bandages, orotracheal tube, tape or other physical barriers obscure the face, these should be removed to the extent possible. | 0 = Normal symmetrical movements. 1 = Minor paralysis (flattened nasolabial fold, asymmetry on smiling). 2 = Partial paralysis (total or near-total paralysis of lower face). 3 = Complete paralysis of one or both sides (absence of facial movement in the upper and lower face). | _____ |
5. Motor Arm: The limb is placed in the appropriate position: extend the arms (palms down) 90 degrees (if sitting) or 45 degrees (if supine). Drift is scored if the arm falls before 10 seconds. The aphasic patient is encouraged using urgency in the voice and pantomime, but not noxious stimulation. Each limb is tested in turn, beginning with the non-paretic arm. Only in the case of amputation or joint fusion at the shoulder, the examiner should record the score as untestable (UN), and clearly write the explanation for this choice. | 0 = No drift; limb holds 90 (or 45) degrees for full 10 seconds. 1 = Drift; limb holds 90 (or 45) degrees, but drifts down before full 10 seconds; does not hit bed or other support. 2 = Some effort against gravity; limb cannot get to or maintain (if cued) 90 (or 45) degrees, drifts down to bed, but has some effort against gravity. 3 = No effort against gravity; limb falls. 4 = No movement.UN = Amputation or joint fusion, explain: _______________ 5a. Left Arm 5b. Right Arm | _____ _____ |
6. Motor Leg: The limb is placed in the appropriate position: hold the leg at 30 degrees (always tested supine). Drift is scored if the leg falls before 5 seconds. The aphasic patient is encouraged using urgency in the voice and pantomime, but not noxious stimulation. Each limb is tested in turn, beginning with the non-paretic leg. Only in the case of amputation or joint fusion at the hip, the examiner should record the score as untestable (UN), and clearly write the explanation for this choice. | 0 = No drift; leg holds 30-degree position for full 5 seconds. 1 = Drift; leg falls by the end of the 5-second period but does not hit bed. 2 = Some effort against gravity; leg falls to bed by 5 seconds, but has some effort against gravity. 3 = No effort against gravity; leg falls to bed immediately. 4 = No movement.UN = Amputation or joint fusion, explain: ______________ 6a. Left Leg 6b. Right Leg | _____ |
7. Limb Ataxia: This item is aimed at finding evidence of a unilateral cerebellar lesion. Test with eyes open. In case of visual defect, ensure testing is done in intact visual field. The finger-nose-finger and heel-shin tests are performed on both sides, and ataxia is scored only if present out of proportion to weakness. Ataxia is absent in the patient who cannot understand or is paralyzed. Only in the case of amputation or joint fusion, the examiner should record the score as untestable (UN), and clearly write the explanation for this choice. In case of blindness, test by having the patient touch nose from extended arm position. | 0 = Absent. 1 = Present in one limb. 2 = Present in two limbs. UN = Amputation or joint fusion, explain: ________________ | _____ |
8. Sensory: Sensation or grimace to pinprick when tested, or withdrawal from noxious stimulus in the obtunded or aphasic patient. Only sensory loss attributed to stroke is scored as abnormal and the examiner should test as many body areas (arms [not hands], legs, trunk, face) as needed to accurately check for hemisensory loss. A score of 2, “severe or total sensory loss,” should only be given when a severe or total loss of sensation can be clearly demonstrated. Stuporous and aphasic patients will, therefore, probably score 1 or 0. The patient with brainstem stroke who has bilateral loss of sensation is scored 2. If the patient does not respond and is quadriplegic, score 2. Patients in a coma (item 1a=3) are automatically given a 2 on this item. | 0 = Normal; no sensory loss. 1 = Mild-to-moderate sensory loss; patient feels pinprick is less sharp or is dull on the affected side; or there is a loss of superficial pain with pinprick, but patient is aware of being touched. 2 = Severe to total sensory loss; patient is not aware of being touched in the face, arm, and leg. | _____ |
9. Best Language: A great deal of information about comprehension will be obtained during the preceding sections of the examination. For this scale item, the patient is asked to describe what is happening in the attached picture, to name the items on the attached naming sheet and to read from the attached list of sentences. Comprehension is judged from responses here, as well as to all of the commands in the preceding general neurological exam. If visual loss interferes with the tests, ask the patient to identify objects placed in the hand, repeat, and produce speech. The intubated patient should be asked to write. The patient in a coma (item 1a=3) will automatically score 3 on this item. The examiner must choose a score for the patient with stupor or limited cooperation, but a score of 3 should be used only if the patient is mute and follows no one-step commands. | 0 = No aphasia; normal. 1 = Mild-to-moderate aphasia; some obvious loss of fluency or facility of comprehension, without significant limitation on ideas expressed or form of expression. Reduction of speech and/or comprehension, however, makes conversation about provided materials difficult or impossible. For example, in conversation about provided materials, examiner can identify picture or naming card content from patient’s response. 2 = Severe aphasia; all communication is through fragmentary expression; great need for inference, questioning, and guessing by the listener. Range of information that can be exchanged is limited; listener carries burden of communication. Examiner cannot identify materials provided from patient response. 3 = Mute, global aphasia; no usable speech or auditory comprehension. | _____ |
10. Dysarthria: If patient is thought to be normal, an adequate sample of speech must be obtained by asking patient to read or repeat words from the attached list. If the patient has severe aphasia, the clarity of articulation of spontaneous speech can be rated. Only if the patient is intubated or has other physical barriers to producing speech, the examiner should record the score as untestable (UN), and clearly write an explanation for this choice. Do not tell the patient why he or she is being tested. | 0 = Normal. 1 = Mild-to-moderate dysarthria; patient slurs at least some words and, at worst, can be understood with some difficulty. 2 = Severe dysarthria; patient’s speech is so slurred as to be unintelligible in the absence of or out of proportion to any dysphasia, or is mute/anarthric. UN = Intubated or other physical barrier, explain:_____________________________ | _____ |
11. Extinction and Inattention (formerly Neglect): Sufficient information to identify neglect may be obtained during the prior testing. If the patient has a severe visual loss preventing visual double simultaneous stimulation, and the cutaneous stimuli are normal, the score is normal. If the patient has aphasia but does appear to attend to both sides, the score is normal. The presence of visual spatial neglect or anosagnosia may also be taken as evidence of abnormality. Since the abnormality is scored only if present, the item is never untestable. | 0 = No abnormality. 1 = Visual, tactile, auditory, spatial, or personal inattention or extinction to bilateral simultaneous stimulation in one of the sensory modalities. 2 = Profound hemi-inattention or extinction to more than one modality; does not recognize own hand or orients to only one side of space. | _____ |
Reproduced from National Institute of Neurological Disorders and Stroke (<http://www.nihstrokescale.org>), with permission. <http://www.nihstrokescale.org/docs/HospitalStrokeScale.pdf>
Flowchart for management of acute ischaemic stroke.
Contraindications to use of thrombolysis in acute ischaemic stroke
Age < 18 and > 80 years
Symptoms onset > 4.5 hours or unclear time of onset (patients waking up from sleep cannot be considered unless they went to sleep within the last 4.5 hours and well at the time)
Neurological deficit minor/rapidly resolving (NIHSS < 5)
Evidence of intracranial haemorrhage
Very severe stroke (NIHSS > 25) or CT > 1/3 MCA territory ischaemic changes
Seizure activity at stroke onset
SAH suspected
Evidence of internal bleeding
Stroke, head trauma, major surgery in last 3 months
Prior stroke and diabetes
History of intracranial haemorrhage
History of CNS damage (neoplasm, aneurism)
History of intracranial or spinal surgery ever
AVM or aneurysm
Known bleeding diathesis
GI or urinary tract bleeding in last 3 weeks
Recent GI ulcer in last 3 months
Oesophageal varices
Severe liver disease
Endocarditis or pericarditis
Acute pancreatitis
Pregnant or recent delivery (10 days)
Recent puncture of non-compressible vessel (10 days)
Lumbar puncture (in last 7 days)
Recent CPR (< 10 days)
History of haemorrhagic diabetic retinopathy
Further reading
Al-Mahdy H (
Intercollegiate Stroke Working Party (
McArthur KS, Quinn TJ, Dawson J, Walters M (
NICE (
Spontaneous intracranial haemorrhage (ICH)
Spontaneous ICH is a common cause of morbidity.
20% of strokes are caused by cerebral haemorrhage (75% ICH and 25% SAH).
Risk factors for ICH are similar to those with ischaemic stroke:
age;
male gender;
hypertension;
smoking;
diabetes;
excess alcohol.
Aetiology
Haemorrhage is due to rupture of small vessels and microaneurysms in perforating vessels.
Underlying vascular conditions should be considered:
AVM;
aneurysm;
cavernoma;
amyloid angiopathy;
cerebral venous thrombosis.
Haemostatic factors:
anticoagulant drugs;
antiplatelet drugs;
coagulation disorders;
thrombolytic therapy.
Other aetiologies:
drug abuse (cocaine);
moyamoya syndrome;
haemorrhage into a tumour (metastatic malignant melanoma, renal, thyroid, and lung carcinoma, choriocarcinoma, oligodendroglioma, and ependymoma).
Clues to the aetiology may come from the site:
basal ganglia in hypertensive bleeds;
Sylvian fissure in MCA aneurysms;
lobar bleeds in amyloid angiopathy.
Clinical features
Sudden ictus as a stroke.
± signs and symptoms of ↑ICP—severe headache and vomiting.
Seizures and meningism.
Imaging features
CT scan is sensitive diagnostically.
MRI may help to differentiate hypertensive haemorrhage from other causes.
Management
Standard medical support.
Stop antiplatelet drugs, reverse anticoagulation.
Surgical evacuation of the haematoma depends on location, age, and premorbid performance status of the patient. Recent STICH trials suggest no benefit for surgery versus conservative management in the early stages. Infratentorial haematomas are special cases—may warrant surgical intervention for evacuation or shunt insertion for hydrocephalus.
Further reading
Gupta RK, Jamjoom AA, Nikkar-Esfahani A, Jamjoom DZ (
Imaging of ICH: examples
Figure 3.9 shows acute right frontoparietal intraparenchymal haematoma with high-density elements anteriorly and slightly lower attenuation components posteriorly (white arrowheads), indicating less acute blood. Note the small rim of surrounding low attenuation and associated mass effect with ipsilateral sulcal effacement. Figure 3.10 shows a typical ganglionic haematoma with acute haemorrhage involving the right lentiform nucleus.
Acute primary intracerebral haematoma (non-enhanced CT).
Primary hypertensive haemorrhage (non-enhanced CT).
Figure 3.11 (a) shows a large area of ill-defined hyperdensity with little associated mass effect (black arrows). (b) Following contrast, multiple serpiginous enhancing structures are demonstrated in the centre of the hyperdense area, with surrounding parenchyma in keeping with the nidus of an AVM. (c) Right occipital AVM with multiple focal and serpiginous flow voids (white arrowheads). There is little associated mass effect. Note the large draining vein entering the vein of Galen (white arrow).
Arteriovenous malformation: (a) non-enhanced CT; (b) contrast-enhanced CT; (c) T2-weighted MRI.
Figure 3.12 was obtained from an elderly patient. Bilateral posteriorly distributed peripheral haemosiderin staining indicates previous lobar haemorrhage (white arrows), with multiple widely distributed foci of haemosiderin in both cerebral hemispheres (black arrowheads).
Amyloid angiopathy (axial gradient echo T2* MRI).
Figure 3.13 shows a large right inferomedial parietal cavernoma with a hypointense ring of haemosiderin surrounding more recent haemorrhage (predominantly extracellular methaemoglobin). Note the absence of surrounding white matter signal change, suggesting no recent extralesional haemorrhage.
Cavernous angioma: (a) axial gradient echo T2* and (b) sagittal T1W MRI.
Delirium
Delirium is defined as a non-specific organic brain syndrome or acute brain dysfunction or acute brain failure within the setting of physical illness—medical or surgical.
Epidemiology
Common in general medicine, especially in the elderly.
Pathophysiology
Not well understood, but evidence for cholinergic underactivity and dopaminergic overactivity. ↑ activity in the hypothalamic–pituitary axis with ↑ cortisol levels.
Aetiology
Predisposing factors
Old age (↓ cognitive reserve).
Premorbid cognitive impairment (↓ cognitive reserve).
Sensory (visual or auditory) impairment.
Advanced physical disease.
Malnutrition.
Precipitating factors
Metabolic derangement.
Hypoxia.
Infection.
Dehydration.
Constipation (e.g. opiate induced).
Pain.
Drugs:
opioids;
benzodiazepines—paradoxical reaction in elderly;
anticholinergic side effect of drugs (e.g. oxybutinin);
antihistamines:
anti-emetics (e.g. cyclizine, cinnarizine, prochlorperazine)
sleeping tablets (e.g. promethazine)
anti-allergics (e.g. cetirizine, chlorphenamine);
antidepressants (e.g. tricyclics);
corticosteroids.
Clinical features
Acute onset with diurnal fluctuation of:
Activity
Psychomotor—↑ arousal, distractibility, restlessness, and wandering. However, ↓ activity is as common, but is under-recognized and diagnosed as depressed or tired.
Sleep–wake cycle disturbance.
Behaviour
Emotions: anxiety, fear, anger, mood changes (depression > mania).
Psychosis: hallucinations (usually visual) or delusions.
Cognition
Look for signs of infection, dehydration, constipation.
Look at drug chart carefully.
Investigations
First line:
blood tests—FBC, ESR, CRP, U&E, LFT, Ca, PO4, glucose, blood cultures;
urine: dipstick, MSU;
CXR.
Second line:
CT/MRI;
US abdomen;
LP;
EEG (in case of complex partial status).
Management
Non-pharmacological:
treat infections;
rationalize drug input;
optimize hydration;
ensure adequate pain control;
remove unnecessary cannulae and catheters;
minimize changes to surroundings (e.g. change of bed or ward);
optimize sensory input (e.g. hearing aid battery);
normalize sleep–wake cycles by discouraging sleep during the day by providing stimulation; consider sleeping tablets for the short term;
non-confrontational approach, but these patients are challenging.
Pharmacological—indications for treatment:
uncontrollable agitation despite non-pharmacological interventions;
danger to self, other patients, or staff;
in order to perform investigations or other treatments.
Medication
Typical antipsychotics, e.g. haloperidol (< 3 mg/day).
Atypical antipsychotics, e.g. olanzapine (PO or IM), risperidone (PO), or quetiapine (PO).
Benzodiazepines (for alcohol withdrawal): short-acting, e.g. lorazepam (PO, IM, IV), midazolam (PO, IM, IV); longer-acting, e.g. chlordiazepoxide (PO), diazepam (PO, PR, IV), or clonazepam (PO).
Currently no evidence for the use of cholinesterase inhibitors.
Note: Concerns that atypical antipsychotics:
↑ risk of stroke in those with dementia and the elderly—still controversial;
↓ extrapyramidal side effects;
↓ hypotension side effects;
↑ expensive than the typical antipsychotics.
Further reading
Inouye, SK, Westendorp RGJ, Saczynski JS (
Head injury (HI)
Trauma is a leading cause of death in adults < 45 years.
Head injury accounts for > 50% of these deaths.
Alcohol is a significant factor in > 50% of these deaths.
Mortality for patients undergoing neurosurgery for post-trauma complications is 40%.
In the UK, 1500 per 100 000 attend A&E with HI, 300 per 100 000 are admitted, 15 per 100 000 are transferred to a neurosurgical unit, and 9 per 100 000 die every year.
Pathophysiology
Diffuse or primary brain injury applies to structural and functional damage sustained at the time of injury.
Mass lesions include haematomas (extradural, subdural, intracerebral) or intracerebral contusions that affect the frontal and temporal lobes at the site of injury (coup) or opposite the injury (contre-coup).
Secondary insults relate to subsequent events to which the injured brain is acutely susceptible—hypoxia, hypoperfusion, hyperthermia, ↑ ICP, and metabolic derangements.
Assessment
Initial assessment according to ATLS protocols. Avoid hypoxia (O2 sat. < 90%) and hypotension (systolic BP < 90 mmHg).
Assessment of conscious level using the Glasgow Coma Scale (see Table 3.6).
If GCS not depressed, detailed assessment of limb power, sensory assessment, cranial nerve function including pupillary responses, corneal reflexes, gag and cough reflexes.
Observe respiratory pattern and rate.
Check pulse and BP.
Check for scalp lacerations, rhinorrhoea, otorrhoea, haemotympanum, and extracranial injuries. Bruising associated with base of skull injuries includes Battle’s sign and racoon eyes.
If possible determine retrograde and anterograde amnesia.
| Score | Eye opening | Motor response | Verbal response |
|---|---|---|---|
1 | None | None | None |
2 | To pain | Extension | Sounds |
3 | To speech | Abnormal flexion | Words |
4 | Spontaneously | Flexion | Confused speech |
5 | Localizes | Orientated | |
6 | Obeys commands |
| Score | Eye opening | Motor response | Verbal response |
|---|---|---|---|
1 | None | None | None |
2 | To pain | Extension | Sounds |
3 | To speech | Abnormal flexion | Words |
4 | Spontaneously | Flexion | Confused speech |
5 | Localizes | Orientated | |
6 | Obeys commands |
Note that the minimum GCS score is 3.
Head injuries are strongly associated with cervical injury.
Classification
Severity
Mild, GCS 13–15 after resuscitation.
Moderate, GCS 9–12.
Severe, GCS 3–8.
Post-traumatic amnesia
Very mild, < 5min.
Mild, 5–60min.
Moderate, 1–24 hours.
Severe, 1–7 days.
Very severe, 1–4 weeks.
Extremely severe, > 4 weeks.
Management
See also Chapter 7.
Indications for urgent CT
Depression of conscious level after resuscitation.
Focal neurological signs.
Epileptic seizure.
CSF rhinorrhoea or otorrhoea.
Basal skull fracture.
Potential penetrating injury.
Difficulty in assessment due to alcohol and drugs.
Uncertain diagnosis.
Primary phase management
Regular neurological observation to detect any deterioration.
Sedation, intubation, and ventilation indicated for:
patients in coma with GCS < 9 or deteriorating GCS;
poor airway protection;
abnormal respiratory pattern;
PaO2 < 9 kPa on air or < 13 kPa on O2; Paco2 > 6 kPa or < 3kPa on O2;
confused and/or agitated patients before CT;
significant maxillofacial injuries or oropharyngeal haemorrhage.
Mannitol 20% 1 g/kg: 200 mL for average adult may help lower ICP.
Corticosteroids have no place in the management of head injuries.
Referral to a neurosurgical centre and image transfer if possible of: all moderate to severe HI, abnormal CT scan, depressed GCS with normal CT scan, all penetrating injuries, uncertain CT findings due to lack of expertise.
Physiological parameters for transfer
Assume ICP to be 30 mmHg and maintain cerebral perfusion pressure (CPP) (mean arterial pressure – ICP) at > 70 mmHg (with inotropes if necessary).
Maintain Pao2 > 15 kPa and Paco2 at 4–4.5 kPa.
Secondary phase management
Respiratory management
Aim for target arterial CO2 of 4–4.5 kPa. If prolonged ventilation, consider tracheostomy.
ICP
Aim for an ICP < 25 mmHg and CPP > 70 mmHg. ICP is monitored via an intraventricular or intracerebral bolt placed close to the most affected region.
Stage 1:
nurse head up tilt 10–15°;
SaO2 > 97%;
PaO2 > 11 kPa;
PaCO2 at 4.5 kPa;
SjvO2 > 55%;
temperature < 37°C.
Stage 2:
mannitol, inotropes as necessary;
↓ PaCO2 to 4.0 kPa;
maintain SjvO2 > 55%;
maintain temperature < 35–36°C (note: role of hypothermia is controversial);
consider external ventricular drain.
Stage 3:
temperature 33°C;
consider decompressive craniectomy.
Stage 4:
thiopental.
Management of specific head injuries
Space-occupying lesions
Expanding mass lesions due to extradural and subdural haematomas need to be detected early.
Initially, cerebral hemisphere compression causes contralateral focal signs, followed by deteriorating conscious level (GCS), and finally herniation of the ipsilateral uncus through the tentorial hiatus causes an ipsilateral third nerve palsy.
Continued expansion leads to bilateral herniation and brainstem compression.
Present with decerebrate posturing and Cushing’s response (bradycardia and hypertension) followed by hypotension and diabetes insipidus.
Rarely, a mass lesion causes ipsilateral hemiparesis through brainstem shift impacting the contralateral free edge of the tentorium (Kernohan’s notch).
Extradural haematoma (EDH)
Classically after a HI (e.g. cricket ball): instant LOC, followed by a lucid interval and later by a progressive decline in GCS.
Haemorrhage is arterial (usually posterior branch of middle meningeal artery is torn at site of skull fracture).
Bleeding is extradural and strips the dura mater from the inner aspect of the skull, compressing the brain.
Imaging
CT
Biconvex high-density extra-axial mass.
Some have low-attenuation components, ‘swirl sign’ indicative of hyperacute bleeding.
Does not cross suture lines.
20% can develop or enlarge after a delay of 36 hours.
50% associated with other lesions, e.g. contre-coup contusions, SDH, and SAH.
Management
True neurosurgical emergency: if necessary resuscitate during transfer.
Surgical procedure: burr hole over pterion (to ensure that further haemorrhage escapes instead of expanding the clot further) followed by craniotomy and evacuation of the haematoma.
Outcome
Depends on preoperative status.
Patients with bilateral fixed dilated pupils may still recover if surgery is immediate.
If preoperative GCS ≥ 8, 90–100% recovery. If GCS < 8, mortality rate 30%; good outcome 50–60%.
Acute subdural haematoma (ASDH)
Occurs after high impact injury, e.g. fall from a height or RTA.
Highest mortality rate among post-traumatic mass lesions.
Immediate LOC with progressive decline in GCS.
Haemorrhage is arterial and venous from contused cerebral cortex, cerebral arteries, and veins.
Haemorrhage occurs between dura and brain with additional brain damage.
Imaging
CT: crescentic hyperdense mass. May cross sutures and extend into the interhemispheric fissure and over tentorium. Hyperacute or active bleeding can be low density.
Anaemia and coagulopathy can cause isodense acute haemorrhages.
Management
Emergency trauma craniotomy with a large flap to expose entire haematoma and affected cortex for evacuation and haemostasis.
Cerebral swelling is common and may require frontal or temporal lobectomy for decompression and bone flap removal.
Patients usually require postoperative ventilation and ICP monitoring.
Outcome
Depends on:
conscious level;
extent of underlying brain injury;
degree of secondary swelling.
Mortality, 50–70%. Good outcome in 20–40%.
Chronic subdural haematoma
Late sequela of minor/moderate HI, usually in the elderly.
A history of a low-velocity HI 4–8 weeks previously is often forgotten or ignored.
There is a gradual evolution of:
headaches;
cognitive decline;
ataxia;
hemiparesis;
impaired conscious level.
Imaging
CT: low density or isodense mass that may be loculated.
Management
Consider dexamethasone 2 mg TDS if treatment non-surgical.
Cortical compression, midline shift, and contralateral hydrocephalus (due to obstruction of third ventricle) indicate need for surgery.
Burr-hole drainage ± subdural drain.
Reoperation required in 10–15% and further surgery in 5%.
Complications, usually in the elderly: subdural empyema, < 1%.
Intracerebral haematoma
Develops from major contusions or vascular injury.
Management depends on clinical condition and extent of mass effect.
Surgical evacuation via craniotomy is indicated when focal signs are evident or ↓ GCS.
Other complications
Cortical contusions due to impact of the brain against corrugated bone or dura. Most common sites are the anterior-inferior temporal and frontal lobes.
Parasagittal and dorsal brainstem lesions less common.
May be multiple and bilateral.
Frequently associated with ASDH and EDH.
These may develop into mass lesions as the contusions mature.
25% develop diffuse brain swelling.
Craniotomy with evacuation and/or lobectomy may be necessary to manage mass effect.
Traumatic SAH
Most common cause of SAH and indicative of a severe brain injury.
Blood is usually in the sulci adjacent to the contusions and SDH rather than in the basal cisterns.
Vasospasm risk is low, but nimodipine 60 mg 4-hourly PO/via NG for 10 days is recommended.
Hydrocephalus is rare.
Penetrating head injuries
History of injury may be unclear or the patient may be unaware of a penetration.
Mortality from gunshot wounds 50–70%.
Should be suspected if:
intracranial air is seen on CT;
evidence of indriven bone.
There is a particular risk of:
infection (meningitis, cerebritis, and abscess);
cortical injury;
ICH;
neurovascular injury (carotid artery, sagittal sinus);
injury to the optic nerve.
Angiography is mandatory for deep penetrating injuries.
Management
Close any CSF fistulae.
↑ risk of infection: use prophylactic antibiotics.
↓ epilepsy rate by removing bone spicules.
Depressed fractures are locked in place and a circumferential craniectomy is performed.
Haematoma, contused brain tissue, and implanted bone are removed.
Elevation and debridement indicated for depression of > 1 cm with dural breach within 24 hours of injury.
However, contraindicted when delayed, eloquent areas of the brain affected, or venous sinus involvement.
Diffuse axonal injury
Results from shearing of axons within brain matter in a closed brain injury.
Usually with immediate LOC and is a common cause of post-traumatic persistent coma.
Risk of diffuse brain swelling.
Imaging
CT:
normal initially in 50–80%;
later development of petechial haemorrhages.
MRI more sensitive:
multiple hyperintense lesions on T2W imaging and FLAIR, especially in corpus callosum and at the grey–white matter interface;
hypointense on T2* if haemorrhagic.
Maintain a low threshold for re-imaging as appearances evolve.
Management
Sedation, intubation, and ventilation.
ICP monitoring and control.
Basal skull fractures
Most are undisplaced and do not require surgery.
Displaced fractures may compress cranial nerves (e.g. optic nerve) and require decompression.
Unstable maxillofacial fractures require elective fixation.
CSF leaks usually cease spontaneously within 7–10 days.
Continued leakage requires surgical closure.
Antibiotic prophylaxis not required for CSF leaks or base-of-skull fractures.
Avoid NG tube in base-of-skull fractures; use orogastric tubes.
Seizures
Occur commonly in the context of HI, especially if there is a depressed skull fracture.
Treat with phenytoin, which can be given IV/orally/via NG tube.
Infection
Antibiotics are only prescribed in the presence of infection—not as prophylaxis.
Aspiration pneumonia and MRSA are common complications.
Delayed complications
Vascular:
chronic subdural haematoma;
carotid dissection;
traumatic aneurysms;
carotid–cavernous fistula.
Infection:
cerebral abscess;
meningitis;
subdural empyema.
Epilepsy
Cranial nerve deficits:
olfactory;
trigeminal;
facial;
vestibulo-cochlear (e.g. BPPV).
Psychological: behavioural disturbance, depression.
Imaging of head injuries: examples
Figure 3.14 shows blunt trauma to the right frontal region with extracranial soft tissue swelling (open white arrowheads) and right frontal fracture (closed white arrow). There is an extensive underlying parenchymal contusion comprising low-attenuation components (closed white arrowheads) and central haemorrhagic change (open white arrows). There is an associated mass effect with ipsilateral sulcal and ventricular effacement and minor distortion of the midline. Note also the small right frontal extradural haematoma (black arrow).
Direct impact (non-enhanced CT).
Figure 3.15 shows blunt trauma to the left temporoparietal region (white arrows) with sudden cranial deceleration and angular rotation resulting in shear–strain forces causing large haemorrhagic contre-coup contusions in the inferior aspects of both frontal and right temporal lobes (open white arrowheads). Note also smaller foci of parenchymal haemorrhage in the occipital lobe bilaterally (closed white arrowheads), intraventricular and subarachnoid blood (black arrows), and an extensive tentorial subdural haematoma (black arrowheads).
Indirect impact (non-enhanced CT).
Large acute subdural haematoma with crescentic configuration overlying left cerebral convexity with minor extension into interhemispheric fissure is shown in Fig. 3.16 (white arrowheads). There is marked associated mass effect with ipsilateral sulcal and ventricular effacement and severe midline shift. Note the indirect site of impact over right parietal bone (white arrow). In contrast, note CSF clefts (black arrowheads) associated with bifrontal extradural haematoma in (b) which has a biconvex configuration. Frontal horns of the lateral ventricles are grossly effaced.
Acute extra-axial haematoma (non-enhanced CT).
The large subdural haematoma over the left cerebral convexity and extending into the interhemispheric fissure shown in Fig. 3.17(a) is hyperdense in keeping with an acute haematoma. Figure 3.17(b) shows a subacute right-sided subdural haematoma with isodense to mildly hyperdense material overlying the right cerebral convexity (white asterisk) resulting in effacement and obscuration of ipsilateral cerebral sulci compared with the contralateral side (black arrowheads) and midline distortion.
Subdural haematoma: (a) and (b) non-enhanced CT.
The left frontal chronic subdural haematoma shown in Fig. 3.17(c) is predominantly hypodense but also demonstrates some mass effect with effacement of sulci in the underlying left frontal lobe. There is also a minor alteration in the configuration of the left frontal horn due to mass effect. Figure 3.17(d) shows a hyperacute right frontal subdural haematoma with mass effect. The hyperdense components represent acute haemorrhage; the low-attenuation material reflects active bleeding and unclotted oxygenated blood.
Subdural haematoma: (c) and (d) non-enhanced CT.
Figure 3.18 shows a severe shear–strain injury resulting in multiple foci of acute petechial haemorrhage involving the splenium and posterior aspects of the corpus callosum and frontal parenchyma predominantly at the grey–white matter interface.
Diffuse axonal injury (DAI): non-enhanced CT.
In Fig. 3.19 there is a reversal of the normal grey–white matter pattern with low-density change involving the cortex with generalized cerebral swelling indicative of hypoxic/anoxic brain injury.
Severe secondary brain injury: non-enhanced CT.
Spinal injuries
These are often associated with multiple injuries and head trauma.
Early detection and immobilization are vital to avoid secondary insults.
Spinal level is defined by the affected vertebral level and the most cephalad cord segment involved.
Completeness: the prognosis and management are dictated by whether the lesion is complete or not. Incomplete lesions (including sacral sparing, i.e. sensation and control of anal sphincter) may recover to a variable extent and may benefit from decompression.
Spinal shock refers to both the haemodynamic effects of cord injury and the flaccid phase (first 1–2 weeks after cord injury).
Spinal stability
Instability is defined as the loss of ability of the spine to maintain normal anatomical alignment under normal physiological loads. Instability refers to the increased likelihood of further spinal damage.
Spinal stability is classifed according to the Denis three-column model of the spine:
anterior column = the anterior half of the vertebral body and annulus fibrosus and anterior longitudinal ligament (ALL);
middle column = the posterior half of the vertebral body and annulus fibrosus and posterior longitudinal ligament (PLL);
posterior column = pedicles, laminae, spinous processes, and ligaments.
The spine is unstable if ≥ 2 columns are disrupted.
Acute cord injury
Management
Resuscitation and airway protection.
Immobilization of the neck and log rolling during assessment and resuscitation.
Treatment of life-threatening injuries and bleeding.
Urinary catheter.
Full neurological examination to determine level and completeness lesion.
Palpate spine for any ‘step’.
Note any autonomic dysfunction, e.g. ileus, priapism.
IV methylprednisolone may improve outcome when administered within 8 hours of injury (30 mg/kg over 15 min bolus and maintenance 5.4 mg/kg/hour for 23 hours).
C1 and C2 fracture
C1 and C2 are rings. Fracture in two places is typical.
Atlanto-occipital dislocation
Distance between the anterior margin of the foramen magnum (basion) and dens > 12.5 cm. Usually fatal.
C1 fracture (Jefferson)
Caused by disruption of the C1 ring due to compression or a burst fracture.
Clinical features
Rarely have a neurological deficit as the spinal canal is wide and fragments burst outwards.
Imaging
Open-mouth view X-ray: lateral displacement of C1 lateral masses relative to C2 lateral masses (overhang on C2 ≥ 7 mm).
Lateral X-ray: fractures of anterior and posterior arch of C1.
Management
This is an unstable fracture.
Requires halo immobilization for 3 months (rigid orthosis using a ring (halo) attached to outer table of skull by four screws attached by vertical side bars joining a rigid jacket strapped to the chest).
C2 fractures
Odontoid peg fractures
Frequently associated with multiple injuries with high force impact.
Classification
Type 1: upper dens fracture (10%).
Type 2: base of neck of peg (60%).
Type 3: transverse fracture through C2 vertebral body (30%).
Clinical features
Neurological deficit in 20% of type 2 fractures. Unusual in type 3 fractures.
Imaging
High-resolution CT from occiput to C3; MRI cervical cord.
Management
All unstable fractures.
Majority treated by halo immobilization. Surgical treatment with fixation is indicated in the following:
displacement of fracture > 4 mm;
persistent movement in halo;
non-union including fibrous union after 3 months;
comminuted type 2 fracture.
Hangman’s fracture
Usually caused by high impact axial loading injury. Due to bilateral fractures of pars articularis of C2.
Clinical features
Majority are neurologically intact.
Complain of neck pain and a sensation of instability.
May walk into A&E holding head!
Imaging
Usually apparent on lateral cervical spine X-ray.
MRI is indicated if neurological signs are present.
Management
Minimally displaced fractures are treated in a SOMI (sterno- occipito-mandibular immobilizer) brace if compliant or halo jacket.
If fractures displaced > 4 mm, halo is mandatory.
Indications for surgical treatment
Displacement not reduced with judicious neck extension.
Persistent movement in halo.
Associated C2/3 disc disruption.
Non-union after halo treatment for 3 months.
Subaxial (C3–C7) fractures
Commonly associated with head injuries and severe neurological deficit.
Flexion injuries are more severe.
Fractures through vertebral body, wedge fractures, teardrop fractures (anterior portion of vertebral body), and avulsion fragments.
Clinical features
Neck pain.
Radiculopathy.
Myelopathy.
Tetraplegia.
Imaging
AP and lateral cervical X-ray reveal majority of fractures.
Flexion/extension lateral cervical spine views only to exclude occult instability and only in patients who are fully conscious.
Increased anterior soft tissue shadow: (C1–4 normal = half vertebral body; C5–7 normal = whole vertebral body) if > requires further investigation with CT or MRI.
MRI necessary in all patients with abnormal neurological signs.
Management
Complete neurological deficit: further management aimed at avoiding secondary damage and maximizing rehabilitation.
Unstable fractures treated with halo or internal fixation to allow early mobilization and avoid respiratory complications.
Incomplete neurological injury with cord compression requires decompression and fixation (internal or external halo).
Unstable injury without a deficit or stable deficit managed with a halo if minor displacement; otherwise internal fixation and fusion.
Cervical facet dislocation
Hyperflexion injury resulting in superior facet ‘jumping’ inferior facet and becoming trapped in dislocation by rim of facet.
Flexion alone results in bilateral facet dislocation accompanied by disc and ligament disruption.
Flexion with rotation leads to unilateral facet dislocation.
Clinical features
Usually severe cord injury.
Unilateral facet dislocation: 25% are intact neurologically; 25% incomplete cord injury; 40% root injury; 10% complete cord injury.
Imaging
Lateral cervical spine X-ray shows anterior transposition of upper vertebra by 25% vertebral body width in unifacet dislocation and 50% vertebral body width in bilateral facet dislocations.
Management
Skull traction with muscle relaxant, e.g. diazepam, commencing at 3 times upper vertebral levels in pounds, increasing by 4–10 pounds every 15 minutes until relocated using image intensifer. Cease at 10 pounds per vertebral level or if there is any evidence of overdistraction (any disc height > 10 mm).
Open reduction if traction fails.
Majority require internal fixation with interspinous wiring, lateral mass plates, and bone graft to maintain position.
Thoracolumbar fractures
Caused by high force and associated with multiple trauma.
Comprise wedge fractures (anterior ± posterior column), burst fractures (anterior and middle column), or fracture dislocations (all columns).
Unstable if wedge > 75% vertebral height or three adjacent vertebrae wedged.
Clinical features
High proportion have a significant neurological deficit.
Imaging
Plain films followed by high-resolution CT or MRI.
Management
Complete neurological deficit. Avoid secondary complications and maximize rehabilitation. If unstable, fractures are treated with prolonged bed rest or internal fixation to allow early rehabilitation.
Incomplete neurological injury with cord compression requires early decompression and internal fixation.
Unstable injury with no or stable deficit managed with bed rest, corset, or internal fixation. All others require internal fixation and fusion.
Rehabilitation of spinal cord injury
Survivors of spinal cord injury require expert management to avoid the complications of:
DVT;
bed sores;
infections (e.g. UTI);
respiratory failure;
contractures;
osteoporosis;
psychological problems.
Imaging spinal injuries: examples
Figure 3.20 shows a slightly displaced fracture through the body of C2 in the coronal plane ((a) white arrowheads; (b) black arrowheads). There is slight anterior subluxation of C2 upon C3.
Coronal fracture of body of C2 hangman’s fracture: (a) CT sagittal MPR; (b) axial CT.
Figure 3.21 shows severe spinal injury with Grade 2 spondylolisthesis (anterior subluxation) of C4 upon C5, C4/5 intervertebral discal injury with hyperintensity and posterior bulge into vertebral canal (closed white arrows), and posterior ligamentous injury (black arrow). There is a shallow epidural haematoma posterior to the C4 vertebral body (open white arrow) which has elevated and posteriorly displaced the dura (black arrowheads). The spinal cord is distorted and displaced, although no cord contusion is evident at this stage. Note the shallow haematoma in the pre-vertebral soft tissue compartment (open white arrowheads).
Bilateral cervical facet subluxation: sagittal T2W MRI.
Figure 3.22 shows Grade 2 spondylolisthesis of C6 upon C7 with large disc protrusion ((a) white arrow), epidural haematoma ((b) open white arrowheads), and rupture of the posterior ligamentous structures ((a) open black arrow). Discontinuity of the anterior longitudinal ligament indicates probable injury ((b) closed white arrow). The spinal cord is compressed and intramedullary signal change, in keeping with oedema, extends from C6 to T1 ((a) closed black arrow). Note the absence of intramedullary hyperintensity on T1W imaging or hypointensity on T2W imaging, which would indicate spinal cord haemorrhage.
Bilateral facet dislocation: (a) sagittal T2W and (b) sagittal T1W MRI.
Dynamic plain X-rays demonstrate marked instability at the atlanto-axial joint as a result of a fracture through the odontoid peg (Fig. 3.23). On flexion, there is marked anterior subluxation of the C1 ring and fracture fragment of C2 (dotted line) in relation to the inferior portion of C2 (dashed line) with consequent reduction in the calibre of the vertebral canal at this level.
Type 2 odontoid peg fracture: (a) flexion and (b) extension plain X-rays.
Further evaluation with MRI (Fig. 3.23(c)) confirms a fracture through C2 with possible interposition of soft tissue between the fractured fragments (white arrow). Intramedullary signal change and spinal cord volume loss (black arrow) are in keeping with myelomalacia and long-standing instability and intermittent spinal cord compression. Note the anterior arch of C1 (white arrowhead) and the fracture fragment of C2 (white asterisk).
(c) Sagittal T2W MRI.
Meningitis
| Patient subgroup/features | Likely causative organism(s) |
|---|---|
Age > 50 years | Listeria monocytogenes |
Pregnancy, childbirth | Listeria monocytogenes |
Diabetes mellitus | Streptococcus pneumoniae |
Presence of seizures | Streptococcus pneumoniae, Haemophilus influenzae |
Skull fracture, middle or inner ear fistula, alcoholism | Streptococcus pneumoniae |
Penetrating skull trauma, CSF shunts | Staphylococcus, Gram-negative bacilli |
From TB endemic country/PMH of TB/insidious onset with weight loss, fevers, and focal deficits | Tuberculosis |
Splenic dysfunction (splenectomy/sickle cell disease) | Streptococcus pneumoniae |
T-lymphocyte dysfunction (HIV, chemotherapy, malignancy) | Listeria monocytogenes |
Immunosuppression (HIV, neutropenia) | Fungal (cryptococcus), TB, pseudomonas. |
| Patient subgroup/features | Likely causative organism(s) |
|---|---|
Age > 50 years | Listeria monocytogenes |
Pregnancy, childbirth | Listeria monocytogenes |
Diabetes mellitus | Streptococcus pneumoniae |
Presence of seizures | Streptococcus pneumoniae, Haemophilus influenzae |
Skull fracture, middle or inner ear fistula, alcoholism | Streptococcus pneumoniae |
Penetrating skull trauma, CSF shunts | Staphylococcus, Gram-negative bacilli |
From TB endemic country/PMH of TB/insidious onset with weight loss, fevers, and focal deficits | Tuberculosis |
Splenic dysfunction (splenectomy/sickle cell disease) | Streptococcus pneumoniae |
T-lymphocyte dysfunction (HIV, chemotherapy, malignancy) | Listeria monocytogenes |
Immunosuppression (HIV, neutropenia) | Fungal (cryptococcus), TB, pseudomonas. |
| Condition | CSF pressure (mmH2O) | WBC (/L) | Protein (g/L) | Glucose (mmol/L) |
|---|---|---|---|---|
Normal | 50–200 | > 5 lymphocytes | 0.2–0.45 | 75% blood glucose |
Bacterial meningitis | ↑ | 100–60 000, mainly neutrophils | 0.5–5 | < 40% blood glucose |
Tuberculous meningitis | ↑ | 10–500, neutrophils in early disease, lymphocytes later | 0.5–5 | ↓ < 40% of blood glucose |
Fungal meningitis | ↑ | 25–500, mainly lymphocytes | 0.5–5 | ↓ |
Viral meningitis | Normal or ↑ | ↑ lymphocytes | 0.5–2 | Normal |
| Condition | CSF pressure (mmH2O) | WBC (/L) | Protein (g/L) | Glucose (mmol/L) |
|---|---|---|---|---|
Normal | 50–200 | > 5 lymphocytes | 0.2–0.45 | 75% blood glucose |
Bacterial meningitis | ↑ | 100–60 000, mainly neutrophils | 0.5–5 | < 40% blood glucose |
Tuberculous meningitis | ↑ | 10–500, neutrophils in early disease, lymphocytes later | 0.5–5 | ↓ < 40% of blood glucose |
Fungal meningitis | ↑ | 25–500, mainly lymphocytes | 0.5–5 | ↓ |
Viral meningitis | Normal or ↑ | ↑ lymphocytes | 0.5–2 | Normal |
| Organism | Specific therapy |
|---|---|
N.meningitidis | Benzylpenicillin 2.4 g IV (4-hourly) or ampicillin 2 g (4-hourly) *If betalactam allergy give chloramphenicol 25 mg/kg (6-hourly) |
S.pneumoniae | Ceftriaxone or cefotaxime Add vancomycin or rifampicin (600 mg 12-hourly) if from penicillin-resistant area |
H.influenzae | Cefotaxime or ceftriaxone |
L.monocytogenes | Ampicillin 2 g (4-hourly) + gentamycin 5 mg/kg (divided into 8-hourly doses) |
Tuberculosis | Isoniazid 5–10 mg/kg 24-hourly + rifampicin 8–15 mg/kg 24-hourly + pyrazinamide 20–30 mg/kg 24-hourly + ethambutol 15 mg/kg + pyridoxine 10–25 mg. > 50 kg standard dose = rifampicin 600 mg, isoniazid 300 mg, pyrazinamide 2 g, ethambutol 15 mg/kg, pyridoxine 10–25 mg; < 50 kg = rifampicin 450 mg, isoniazid 300 mg, pyrazinamide 1.5 g, ethambutol and pyridoxine as above. |
| Organism | Specific therapy |
|---|---|
N.meningitidis | Benzylpenicillin 2.4 g IV (4-hourly) or ampicillin 2 g (4-hourly) *If betalactam allergy give chloramphenicol 25 mg/kg (6-hourly) |
S.pneumoniae | Ceftriaxone or cefotaxime Add vancomycin or rifampicin (600 mg 12-hourly) if from penicillin-resistant area |
H.influenzae | Cefotaxime or ceftriaxone |
L.monocytogenes | Ampicillin 2 g (4-hourly) + gentamycin 5 mg/kg (divided into 8-hourly doses) |
Tuberculosis | Isoniazid 5–10 mg/kg 24-hourly + rifampicin 8–15 mg/kg 24-hourly + pyrazinamide 20–30 mg/kg 24-hourly + ethambutol 15 mg/kg + pyridoxine 10–25 mg. > 50 kg standard dose = rifampicin 600 mg, isoniazid 300 mg, pyrazinamide 2 g, ethambutol 15 mg/kg, pyridoxine 10–25 mg; < 50 kg = rifampicin 450 mg, isoniazid 300 mg, pyrazinamide 1.5 g, ethambutol and pyridoxine as above. |
Meningitis management flowchart.
Incidence and microbiology
Annual incidence around 2–3/100 000 with peaks in infants and adolescence. Vaccination against Haemophilus influenzae type b and group C meningococcus has had significant impact.
Clinical features
Presenting features typically with headache, fever, photophobia, neck stiffness. In addition:
cranial nerve palsies (III, IV, VI, VII);
focal neurological deficits;
seizures (20–30%)—usually in S.pneumoniae and Haemophilus influenzae meningitis;
↑ ICP (altered conscious level, hypertension, bradycardia, abnormal respiratory pattern, papilloedema (late));
purpura or petechial haemorrhages (non-blanching with glass test)—Neisseria meningitidis;
septic shock: N.meningitidis;
tuberculous meningitis may be more insidious with gradual development of fever, weight loss, headache with progression to focal deficit, altered consciousness;
look for evidence of immunosuppression as may be the first presentation of HIV or lymphoproliferative disorder (e.g. oral candidiasis, lymphadenopathy).
Investigations
Blood culture as latex agglutination bacterial antigen tests or PCR can be performed and may remain positive even after antibiotics.
CXR for evidence of TB.
Lumbar puncture is contraindicated if:
signs of ↑ ICP;
↓ GCS;
coagulopathy;
focal symptoms, signs, or seizures (unless CT scan normal);
cardiovascular compromise;
infection of skin at LP site.
See Table 3.8 for lumbar puncture and blood findings in different forms of meningitis.
Management
Choice of antibiotic
Choice of antibiotic depends on age of patient and any other associated features, e.g. immunocompromised. CT or LP should not delay first dose of antibiotic. In adults likely organisms are:
S.pneumoniae;
N.meningitidis;
if > 50 years, L.monocytogenes.
Drug recommendation before identification of organism
Meningitis with typical meningococcal rash:
IV 2.4 g benzylpenicillin 4-hourly or IV cefotaxime 2 g 6-hourly (8 g total). If history of penicillin allergy, IV chloramphenicol 50 mg/kg/day given in four divided doses.
Meningitis without typical rash:
IV cefotaxime 2 g 6-hourly or IV ceftriaxone 2 g 12-hourly (total 4 g) plus
IV vancomycin (in suspected S.pneumoniae until sensitivities are known in case of resistance) 1 g 12-hourly plus
IV ampicillin 2 g 4-hourly if > 50 years to cover listeria.
If clear history of betalactam anaphylaxis:
chloramphenicol 25 mg/kg 6-hourly plus
vancomycin 500 mg 6-hourly;
if > 50 years add cotrimoxazole to cover listeria.
Therapy after identification from CSF or blood
N.meningitidis:
2.4 g benzylpenicillin IV 4-hourly or ampicillin 2 g 4-hourly;
if history of allergy to betalactams, chloramphenicol 25 mg/kg IV 6-hourly.
S.pneumoniae:
ceftriaxone or cefotaxime;
add vancomycin or rifampicin 600 mg 12-hourly if patient from penicillin-resistant area.
H.influenzae: cefotaxime or ceftriaxone.
L.monocytogenes: ampicillin 2g 4-hourly + gentamicin 5 mg/kg divided into 8-hourly doses;
tuberculosis meningitis: isoniazid 5–10 mg/kg 24-hourly + rifampicin 8–15 mg/kg 24-hourly + pyrazinamide 20–30 mg/kg 24-hourly + pyridoxine 25 mg.
↑ ICP
A medical emergency. Patient should be managed on ITU. Give mannitol 0.25 g/kg IV over 10 minutes. May require sedation, intubation, and ventilation to reduce PCO2 and controlled hypothermia.
Seizures
Should be treated initially with lorazepam 4 mg IV, followed by phenytoin 18 mg/kg as a loading dose under ECG monitoring followed by maintenance dose IV. If seizures continue, treat as for status epilepticus.
Corticosteroids
Shown to reduce morbidity in adults specifically in S.pneumoniae and tuberculous meningitis. Data do not include meningococcal meningitis but it is reasonable to consider at least until organism isolated—10 mg dexamethasone 6-hourly IV for 4 days with first dose given with first antibiotic dose.
Further reading
Chaudhuri A, Martinez-Martin P, Kennedy PG (
Spinal cord disorders
Signs/symptoms of spinal cord lesions.
Spinal cord compression diagnosis flowchart.
Raised intracranial pressure
| Herniation syndrome | Anatomy | Signs | Notes |
|---|---|---|---|
Cingulate | Cingulate gyrus herniates under the falx | Typically asymptomatic ACA at risk of kinking, causing bilateral frontal infarcts | Warning sign of impending central herniation |
Central | Diencephalon forced through tentorial incisura. Pituitary may be sheared causing DI. PCA may be compressed causing infarction | Early ↓ GCS Midbrain signs (dilated minimal/unreactive pupils and Cheyne–Stokes) poor prognostic sign Bilateral Babinski Decorticate/decerebrate | Usually due to more chronic causes (e.g. tumour) compared with uncal |
Uncal | Uncus or hippocampal gyrus is forced over edge of tentorium | Early confusion Early CN III palsy Late (but rapid) coma Contralateral weakness Ipsilateral weakness in Kernohan’s notch phenomena (compression of contralateral cerebral peduncle and CN VI—false localizing signs) Decorticate rare | Often due to rapidly expanding haematoma. Impaired consciousness a late sign with rapid deterioration |
Upward cerebellar | Cerebellar vermis ascends above tentorium. May cause compression of SCA, great vein of Galen, and cerebral aqueduct, leading to hydrocephalus | Ataxia Unequal fixed pupils (may be small) Bilateral Babinski. ↓ GCS. Central respirations | Associated with posterior fossa masses. May be exacerbated by ventricular damage |
Tonsillar | Cerebellar tonsils ‘cone’ through foramen magnum | Ataxia CN VI palsy Bilateral Babinski ↓ GCS Central respirations | Rapidly fatal. Occurs with both supra- and infratentorial masses. Can occur post-LP |
| Herniation syndrome | Anatomy | Signs | Notes |
|---|---|---|---|
Cingulate | Cingulate gyrus herniates under the falx | Typically asymptomatic ACA at risk of kinking, causing bilateral frontal infarcts | Warning sign of impending central herniation |
Central | Diencephalon forced through tentorial incisura. Pituitary may be sheared causing DI. PCA may be compressed causing infarction | Early ↓ GCS Midbrain signs (dilated minimal/unreactive pupils and Cheyne–Stokes) poor prognostic sign Bilateral Babinski Decorticate/decerebrate | Usually due to more chronic causes (e.g. tumour) compared with uncal |
Uncal | Uncus or hippocampal gyrus is forced over edge of tentorium | Early confusion Early CN III palsy Late (but rapid) coma Contralateral weakness Ipsilateral weakness in Kernohan’s notch phenomena (compression of contralateral cerebral peduncle and CN VI—false localizing signs) Decorticate rare | Often due to rapidly expanding haematoma. Impaired consciousness a late sign with rapid deterioration |
Upward cerebellar | Cerebellar vermis ascends above tentorium. May cause compression of SCA, great vein of Galen, and cerebral aqueduct, leading to hydrocephalus | Ataxia Unequal fixed pupils (may be small) Bilateral Babinski. ↓ GCS. Central respirations | Associated with posterior fossa masses. May be exacerbated by ventricular damage |
Tonsillar | Cerebellar tonsils ‘cone’ through foramen magnum | Ataxia CN VI palsy Bilateral Babinski ↓ GCS Central respirations | Rapidly fatal. Occurs with both supra- and infratentorial masses. Can occur post-LP |
Management flowchart for raised intracranial pressure.
Acute encephalitis (including limbic encephalitis)
Limbic system = hippocampus, amygdala, hypothalamus, insular and cingulate cortex.
Definition: cortical brain infection or inflammation, acute or subacute (days to months) with features which may include:
memory impairment;
seizures.
Differential diagnosis:
Autoimmune disorders (voltage gated K+ complex channel complex antibodies (usually associated with LG11)).
Tumour, e.g. lymphoma.
Vasculitis.
Wernicke–Korsakoff syndrome.
Infectious causes
Aetiology
Herpes simplex (HS) type 1 (70% in immunocompetent patients).
Immunocompromised host: herpes simplex type 2, human herpes virus 6 and 7, enterovirus.
Clinical features
Abrupt-onset confusion, memory impairment, seizures.
Fever (may be absent).
Investigations
CT/MRI:
Swelling, mass effect and high signal in mesial temporal structures.
CSF:
↑ WCC (lymphocytosis);
↑ protein;
sugar usually normal;
CSF PCR for HS sensitivity and specificity 95%.
Treatment
IV aciclovir (10 mg/kg tds) for 14–21 days—monitor renal function;
treat seizures with IV phenytoin;
If PCR negative—consider other causes, repeat CSF and PCR; if clinical picture convincing continue with aciclovir.
Voltage-gated potassium channel (VGKC) complex antibodies associated limbic encephalitis (LGI1 antibody)
Note: VGKC antibodies (CASPR2 antibody) also associated with:
cramp fasciculation syndrome;
acquired neuromyotonia (Isaac’s syndrome);
Morvan’s syndrome (neuromyotonia, sleep disorders, autonomic dysfunction, cognitive changes).
Clinical features:
Subacute amnestic syndrome.
Confusion, behavioural disturbance.
Seizures (brachio facial dystonic seizures).
↓ Na due to SIADH.
Investigations
VGKC antibodies in serum and/or CSF.
MRI: signal change in mesial temporal lobe structures.
EEG: diffuse slowing with or without temporal spikes.
CSF:
↑ WCC (lymphocytosis);
↑ protein (sometimes) matched oligoclonal bands.
Management
Variable combinations of PE, IV Ig, corticosteroids. Recent open-label study showed immunological and clinical remission with PE (50 mL/kg), followed by IV Ig (2 g/kg) and IV methylprednisolone 1 g × 3 days. Maintenance with oral prednisolone 1 mg/kg with slow taper.
Further reading
Kennedy PG, Steiner I (
Schott J (
Wong SH, Saunders MD, Larner AJ, Das K, Hart IK (
Anti-NMDA receptor encephalitis
Recently recognized syndrome affecting mainly women (80%). A multicentre population-based study of encephalitis in UK found 4% caused by anti-NMDA receptor encephalitis. Consider diagnosis in any case, especially < 50 years, with rapid change in behaviour, psychosis, abnormal movements, seizures, autonomic instability, hypoventilation.
Clinical features
Age: median 23 years (range 5–76 years).
Prodromal viral-like syndrome common.
Psychiatric symptoms:
anxiety, agitation;
delusional and paranoid ideation;
visual and/or auditory hallucination;
catatonia.
Seizures.
Memory loss.
Movement disorders:
orofacial dyskinesias;
choreoathetoid movements;
complex abdominal pelvic movements;
dystonic posturing.
Autonomic instability.
Central hypoventilation.
↓ conscious level.
Investigations
Blood: + NMDA receptor antibodies.
CSF:
↑ WCC (lymphocytosis);
Protein ↑ or normal;
Oligoclonal bands + or –;
NMDA receptor antibody +.
EEG: slow-wave activity ± focal spikes—extreme delta brush sign.
MRI: may be normal (up to 50%) or show abnormalities (FLAIR or ↑ T2) with enhancement of mesial temporal lobes, cerebral cortex, cerebellum, corpus callosum brainstem, basal ganglia.
Tumour search with US, MRI, CT: ovarian teratoma (may be bilateral) in 60%. Consider transvaginal US. Other tumours: SCC lung, testicular teratoma.
Management
May require ITU.
Tumour resection—better prognosis.
No controlled trial data. Treat with IV methylprednisolone + IV Ig and/or plasma exchange. Second-line treatment: cyclophosphamide and/or rituximab.
Prognosis
Early detection of tumour—better outcome.
Slow recovery.
75% recover; 25% severe deficits or death.
Relapses may occur.
Further reading
Dalmau J, Lancaster E, Martinez-Hernandez E, Rosenfeld MR, Balice-Gordon R (
