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Hyperviscosity syndrome and the nervous system Hyperviscosity syndrome and the nervous system
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Introduction Introduction
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Epidemiology Epidemiology
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Pathogenesis Pathogenesis
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Viscosity Viscosity
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Paraprotein levels and hyperviscosity syndrome Paraprotein levels and hyperviscosity syndrome
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Whole blood hyperviscosity and hyperleucocytosis Whole blood hyperviscosity and hyperleucocytosis
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Clinical presentation of hyperviscosity Clinical presentation of hyperviscosity
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Treatment of hyperviscosity syndrome Treatment of hyperviscosity syndrome
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Plasmapheresis Plasmapheresis
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Chemotherapy Chemotherapy
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Bing–Neel syndrome Bing–Neel syndrome
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AL amyloidosis and the nervous system AL amyloidosis and the nervous system
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Introduction Introduction
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Background Background
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Neurological complications by entity Neurological complications by entity
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Monoclonal gammopathies Monoclonal gammopathies
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Waldenström’s disease Waldenström’s disease
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Multiple myeloma Multiple myeloma
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Epidemiology, frequency, and risk factors Epidemiology, frequency, and risk factors
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Aetiology/pathogenesis Aetiology/pathogenesis
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Clinical presentation Clinical presentation
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Cranial nerves Cranial nerves
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Nerve roots Nerve roots
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Nerve plexuses Nerve plexuses
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Peripheral neuropathy Peripheral neuropathy
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Mononeuropathy Mononeuropathy
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Myopathy Myopathy
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Smooth muscle Smooth muscle
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Autonomic nervous system Autonomic nervous system
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Musculoskeletal Musculoskeletal
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Focal amyloidoma Focal amyloidoma
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Central nervous system Central nervous system
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Giant cell arteritis Giant cell arteritis
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Lower motor neuron diseases Lower motor neuron diseases
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Diagnostics, including imaging Diagnostics, including imaging
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Biopsy Biopsy
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Other tests Other tests
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Prevention and treatment Prevention and treatment
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Conclusions Conclusions
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References References
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13 Neurological complications of white blood cell disorders
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Published:July 2024
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Abstract
Disorders of white blood cells can cause a variety of neurological complications. Hyperviscosity syndrome is caused by hyperviscous blood, plasma, or serum and occurs in several haematological disorders. Waldenström’s macroglobulinaemia is the most common cause of hyperviscosity syndrome, followed by immunoglobulin A (IgA) and immunoglobulin G3 (IgG3) multiple myeloma, with a serum paraprotein concentration of over 8 g/dl. Symptoms of hyperviscosity syndrome include mucocutaneous bleeding and ophthalmological and neurological symptoms, including headache, vertigo, nystagmus, dizziness, tinnitus, hearing loss, diplopia, and ataxia. Hyperviscosity syndrome rarely causes confusion, dementia, disturbances of consciousness, stroke, and coma. There is no correlation between measured blood or serum viscosity and clinical symptomatology. Management of hyperviscosity syndrome consists of plasmapheresis for symptomatic improvement followed by concurrent initiation of chemotherapy. Bing–Neel syndrome, a rare disease manifestation of Waldenström’s macroglobulinaemia, characterized by infiltration of lymphoplasmacytic cells into the central nervous system, should be considered in the differential diagnosis of hyperviscosity syndrome. Amyloid and amyloidosis comprise a wide range of conditions, affecting the peripheral nervous system and, to a lesser extent, the central nervous system. AL amyloidosis occurs in monoclonal gammopathies, multiple myeloma, plasmacytoma, Waldenström’s macroglobulinaemia, chronic lymphocytic lymphoma, and other low-grade lymphomas. The most frequent peripheral nervous system lesions are generalized neuropathy and carpal tunnel syndrome, often combined with autonomic dysfunction. Other focal lesions can affect cranial nerves, nerve roots, the plexus, and individual nerves. Amyloidoma and muscle involvement are rare. Local amyloidosis can mimic peripheral nerve lesions. Involvement of the musculoskeletal system, including joints and fascia, has been reported. The central nervous system is less frequently affected, although brain and spinal amyloidomas have been described.
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