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

Abstract

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.”