GRISCELLI-SYNDROME - REPORT OF 3 CASES

The clinical features of three children with Griscelli syndrome and au topsy findings of two are presented. The patients were 5 years, 9 mont hs, and 3 months old, respectively. Clinical features included partial albinism, hepatosplenomegaly, and various neurological symptoms. Ligh t and electron microscopic studies of the skin were compatible with Gr iscelli syndrome. Postmortem examination of the viscera and central ne rvous system revealed lymphohistiocytic infiltration with erythrophago cytosis. Bilateral diffuse involvement of the central nervous system, cranial nerve, and spinal cord was detected in both cases.