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A fulminant, often devastating, demyelinating disease that has a monophasic course and may be associated with antecedent immunization or infection. Signs of disseminated neurologic disease are consistently present (e.g., hemiparesis or quadriparesis, extensor plantar responses, lost or hyperactive tendon reflexes, sensory loss, and brainstem involvement). Fever, headache, meningismus, lethargy progressing to coma, and seizures may occur. CSF pleocytosis, generally 200 cells/µL, is common. MRI may reveal extensive gadolinium enhancement of white matter in brain and spinal cord. Initial treatment is with high-dose glucocorticoids. Pts who fail to respond may benefit from a course of plasma exchange or IVIg.

For a more detailed discussion, see Hauser SL, Goodin DS: Multiple Sclerosis and Other Demyelinating Diseases, Chap. 458, p. 2661, in HPIM-19.

Outline

Section 14. Neurology