A KINDRED WITH GRISCELLI DISEASE - SPECTRUM OF NEUROLOGICAL INVOLVEMENT

We report four members of a highly consanguineous family with silver-g rey pigmentation of hair, two of whom had skin histology compatible wi th Griscelli disease. Unlike previously reported patients, they did no t suffer from recurrent infections. In addition, there was a spectrum of neurological involvement varying from mild cognitive delay with a c onvulsive disorder in one patient, to a fatal degenerative course in t hree others. One patient developed a prolonged febrile illness with hi stological evidence of florid lymphoid hyperplasia.