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Acquired unilateral vitiligo and poliosis of the head and subacute encephalitis with partial recovery

From the Departments of Medicine (Division of Neurology) and Pediatrics, University of Colorado School of Medicine, Denver

SUMMARYThis report involves a single case of a girl who, in her fourth year of life, insidiously developed progressive loss of speech and right-sided hemiconvulsions and spasticity. At the same time the left scalp hair and eyebrow became streaked with grayish-white hair and patches of vitiligo appeared along the left side of her face and neck. Pneumoencephalography demonstrated progressive cerebral atrophy, principally involving the left hemisphere. CSF consistently has shown a markedly elevated gamma globulin content. Histologically, the findings of the left frontal brain biopsy were consistent with a viral encephalitis. Other attempts at demonstrating the virus proved negative; however, the brain tissue was not inoculated into primates. The process not only became arrested but there has been significant partial recovery of neurological functions and repigmentation of hair and skin.

The vitiliginous and encephalitic process, so closely linked anatomically, may be due to a virus interfering with an enzyme system common to both melanocytes and neurons. The need for confirmation of this unusual picture is stressed. Until a more definitive diagnosis can be established, the resemblance of the reversible poliosis and vitiligo in this child to virus-induced loss or "breaking" of pigment in plants suggests that this syndrome be named "color-break encephalitis."

Dr. Nellhaus' address is University of Colorado Medical Center, 4200 East Ninth Avenue, Denver, Colorado 80220.

This investigation was supported in part by U.S. Public Health Service training grant NB 05584 from the National Institute of Neurological Diseases and Stroke and U.S. Public Health Service grant FR 69.

Presented to the Section on Child Neurology at the meeting of the American Academy of Neurology, April 25, 1969

Submitted for publication Dec. 5, 1969; resubmitted Jan. 21, 1970; accepted Jan. 27, 1970.

Wyoming for their referral and continued cooperation in following the patient. Thanks are also due to Dr. John Stears for aid in the pneumoencephalography, to Dr. WOEM. Kirsch for performing the brain biopsy, to Dr. Stuart Schneck for its interpretation, to Dr. Vincent Fulginiti for some of the viral studies, to Dr. Guinter Kahn for the skin biopsy, to Dr. David B. Clark for his discussion of the case following its presentation, and to Dr. James H. Austin for review of the manuscript. Dermatologic studies were performed by Dr. Kahn and Dr. Kent F. Jacobs under US. Army Medical Research and Development Command contract DADA 17–69-C-9051.

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