Table 2.9
Classification of disorders resulting in autonomic dysfunction (After Mathias and Bannister (1999), with modifications)

Primary (aetiology unknown)

 

Acute/subacute dysautonomias (Section 21.10.4)

 

  Pure cholinergic dysautonomia

 

  Pure pandysautonomia

 

  Pandysautonomia with neurological features

 

Chronic autonomic failure syndromes

 

  Pure autonomic failure (Section 21.11.7)

 

  Multiple system atrophy (Shy–Drager syndrome) (Section 40.3.8)

 

  Autonomic failure with Parkinson’s disease (Section 40.3.1)

Secondary

 

Congenital

 

  Nerve growth factor deficiency

 

Hereditary

 

  Familial amyloid neuropathy (Section 21.9.1)

 

  Porphyria (Section 21.8.6)

 

  Hereditary sensory and autonomic neuropathies (Section 21.6)

 

  Familial dysautonomia—Riley–Day syndrome (21.6.3)

 

  Dopamine β-hydroxylase deficiency

 

  Aromatic L-amino acid decarboxylase deficiency

 

  Anderson–Fabry disease (Section 21.8.5)

 

Metabolic diseases

 

  Diabetes mellitus (Section 21.17.5)

 

  Chronic renal failure (Section 21.18.1)

 

  Chronic liver disease

 

  Vitamin B12 deficiency (Section 21.22.4)

 

Inflammatory

 

  Bacterial—Tetanus (Section 42.5.14)

 

  Viral—HIV infection (Section 43.3.7)

 

  Parasitic—Trypanosomiasis Cruzi; Chagas’ disease (Section 42.11.8)

 

  Prion—fatal familial insomnia (Section 42.9.8)

 

Neoplasia

 

  Brain tumours—especially of third ventricle or posterior fossa

 

  Paraneoplastic autonomic neuropathy (Section 21.11.7)

 

  Lambert–Eaton syndrome (Section 24.10.2)

 

  Primary amyloidosis (Section 21.9.2)

 

Connective tissue disorders

 

  Rheumatoid arthritis

 

  Systemic lupus erythematosus

 

  Mixed connective tissue disease

 

Surgery

 

  Regional sympathectomy—upper limb, splanchnic

 

  Vagotomy and drainage procedures—‘dumping syndrome’

 

  Organ transplantation—heart, kidney

 

Trauma

 

  Spinal cord transection

Neurally mediated syncope

 

  Vasovagal syncope

 

  Carotid sinus hypersensitivity

 

  Micturition syncope

 

  Cough syncope

 

  Swallow syncope

 

  Associated with glossopharyngeal neuralgia

Drugs, chemicals, poisons, and toxins

 

  Alcohol-induced (Section 21.19.1)

Primary (aetiology unknown)

 

Acute/subacute dysautonomias (Section 21.10.4)

 

  Pure cholinergic dysautonomia

 

  Pure pandysautonomia

 

  Pandysautonomia with neurological features

 

Chronic autonomic failure syndromes

 

  Pure autonomic failure (Section 21.11.7)

 

  Multiple system atrophy (Shy–Drager syndrome) (Section 40.3.8)

 

  Autonomic failure with Parkinson’s disease (Section 40.3.1)

Secondary

 

Congenital

 

  Nerve growth factor deficiency

 

Hereditary

 

  Familial amyloid neuropathy (Section 21.9.1)

 

  Porphyria (Section 21.8.6)

 

  Hereditary sensory and autonomic neuropathies (Section 21.6)

 

  Familial dysautonomia—Riley–Day syndrome (21.6.3)

 

  Dopamine β-hydroxylase deficiency

 

  Aromatic L-amino acid decarboxylase deficiency

 

  Anderson–Fabry disease (Section 21.8.5)

 

Metabolic diseases

 

  Diabetes mellitus (Section 21.17.5)

 

  Chronic renal failure (Section 21.18.1)

 

  Chronic liver disease

 

  Vitamin B12 deficiency (Section 21.22.4)

 

Inflammatory

 

  Bacterial—Tetanus (Section 42.5.14)

 

  Viral—HIV infection (Section 43.3.7)

 

  Parasitic—Trypanosomiasis Cruzi; Chagas’ disease (Section 42.11.8)

 

  Prion—fatal familial insomnia (Section 42.9.8)

 

Neoplasia

 

  Brain tumours—especially of third ventricle or posterior fossa

 

  Paraneoplastic autonomic neuropathy (Section 21.11.7)

 

  Lambert–Eaton syndrome (Section 24.10.2)

 

  Primary amyloidosis (Section 21.9.2)

 

Connective tissue disorders

 

  Rheumatoid arthritis

 

  Systemic lupus erythematosus

 

  Mixed connective tissue disease

 

Surgery

 

  Regional sympathectomy—upper limb, splanchnic

 

  Vagotomy and drainage procedures—‘dumping syndrome’

 

  Organ transplantation—heart, kidney

 

Trauma

 

  Spinal cord transection

Neurally mediated syncope

 

  Vasovagal syncope

 

  Carotid sinus hypersensitivity

 

  Micturition syncope

 

  Cough syncope

 

  Swallow syncope

 

  Associated with glossopharyngeal neuralgia

Drugs, chemicals, poisons, and toxins

 

  Alcohol-induced (Section 21.19.1)

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