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Neurological complications of systemic disease 

Neurological complications of systemic disease

Chapter:
Neurological complications of systemic disease
Author(s):

Neil Scolding

DOI:
10.1093/med/9780199204854.003.2420
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date: 30 March 2017

Primary neuroimmune disorders such as multiple sclerosis or the Guillain–Barré syndrome are well recognized (and described elsewhere in this section), but there are numerous diverse systemic inflammatory, infective, or immunological disorders that can affect the nervous system.

Autoimmune rheumatic and vasculitic disorders

Autoimmune rheumatic disorders—(1) Systemic lupus erythematosus—neurological manifestations include headache, acute or subacute encephalopathy, fits, myelitis, strokes and movement disorders (including chorea and other extrapyramidal disorders), ataxia and brainstem abnormalities, cranial and peripheral neuropathies, and psychiatric and cognitive disturbances. Risk of stroke is particularly associated with the lupus anticoagulant and the primary antiphospholipid syndrome. (2) Other conditions—(a) rheumatoid arthritis: mononeuritis, cervical cord compression; (b) Sjögren’s syndrome: sensory neuropathy, myositis, various central nervous system complications; (c) Reiter’s disease: polyneuritis, radiculitis, various central nervous system manifestations.

Vasculitides—neurological features include (1) mixed sensory and motor neuropathy—usually rapidly progressive and often painful; (2) central nervous system disease—protean manifestations reflect focal or multifocal infarction, or diffuse ischaemia. Conditions of particular note include (1) giant cell arteritis—anterior ischaemic optic neuropathy is a feared and common complication; (2) Behçet’s disease—cerebral venous sinus thrombosis is one of the more specific serious complications; (3) sarcoidosis—often manifests with optic and other cranial neuropathies.

Other autoimmune disorders

(1) Ulcerative colitis and Crohn’s disease—may be associated with cerebrovascular accidents, epileptic seizures and (rarely) slowly progressive myelopathy. (2) Whipple’s disease—a wide variety of neurological manifestations are recognized. (3) Coeliac disease—malabsorption may lead to neurological sequelae; progressive (spino)cerebellar degeneration is a recognized (but unexplained) complication.

Thyroid disease—hyperthyroidism and myxoedema both carry neurological complications (see Chapter 24.19), but thyroid disease may be associated with immunologically driven neurological complications including (1) dysthyroid eye disease—Graves’ ophthalmoplegia; (2) Hashimoto’s thyroiditis-associated encephalopathy.

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