FAMILIAL RECURRENT ENCEPHALOMYELITIS

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Abstract
Twelve episodes of encephalitic illness in 3 members of a family between the ages of 11 and 14 yrs. are described. Individual patients suffered from 2 to 6 encephalitic episodes. Ten of the 12 acute neurologic illnesses followed banal infections of the upper respiratory tract. The clinical syndrome comprised an onset with headache, vertigo and ataxia and drowsiness, proceeding to stupor with hypotonia and loss of deep reflexes. In the more severe episodes stupor deepened to coma, sphincter disturbances occurred and the plantar responses became extensor. Fever and meningism were inconstant and spinal fluid changes inconspicuous. Various focal neurologic symptoms became evident with the return of consciousness, but residual clinical and electroencephalographic changes were short-lived. Five such episodes were treated with ACTH; in every instance, unequivocal clinical improvement was evident within 12 hrs. of the first injn. and recovery was rapidly progressive. Reasons are adduced in favor of considering these illnesses to be episodes of acute disseminated encephalomyelitis arising on the basis of anaphylactic hypersensitivity, rather than instances of neuro-tropic virus infection or of primary metabolic disorder.