Paraneoplastic Encephalomyelitis


Paraneoplastic encephalomyelitis is a multifocal disorder of the central nervous system associated with remote neoplasia. Frequently, the disorder is accompanied by other neuropathies (see other pages). The neurological manifestations precede cancer diagnosis in most cases.

Progression of the disease is spread over weeks to months, finally reaching a plateau of neurological impairment. The clinical presentation reflects the distribution of this multifocal condition.

Limbic encephalitis: amnesia, personality changes, neuropsychiatric disturbances, seizures, olfactory and gustatory hallucinations, sleep disturbances, altered level of consciousness

Peripheral encephalitis: seizure, focal neurological disturbances, aphasia, weakness, or numbness.

Brainstem encephalitis: diplopia, facial numbness, dysarthria, hearing loss, and dysphagia.

Although many malignancies have been reported, most cases are associated with small-cell lung carcinoma.

Neurological impairment is generally more debilitating than cancer. No effective treatment has been established.

An auto-immune reaction against nuclear antigens is most common (anti-Hu IgG antibody). Other antibodies have occasionally been found including anti-CV2, anti-Yo, anti-Ma1, anti-Ta or anti-Ma2, and several other atypical antibodies.

Recent reports have noted the detection of a prion-related protein and of herpes simplex virus by polymerase chain reaction in the cerebrospinal fluid of certain patients. The significance of these findings remains unclear.

References:

Gultekin SH, Rosenfeld MR, Voltz R: Paraneoplastic limbic encephalitis: neurological symptoms, immunological findings and tumour association in 50 patients. Brain 2000 Jul; 123 ( Pt 7): 1481-94

See also: E-medicine

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