ADULT FORM OF ALEXANDERS-DISEASE AND DIFF USE CEREBRAL GLIOMATOSIS INTHE SAME FAMILY

A 31-year old woman died after 10 years of progressive dysautonomia an d cerebellar and pyramidal symptoms. CT scan showed pontine, bulbar an d cerebellar atrophy Post-mortem examination revealed Rosenthal's fibe rs widespread throughout the CNS, but especially in the subependymal a nd perivascular regions. While matter cavitations involving peri-ventr icular regions, hilum of dentate nuclei and pens were observed leading to a diagnosis of adult form of Alexander's disease. At the age of 5, the patient had been operated Upon for a chiasmatic tumor. Microscopi c examination revealed a pilocytic astrocytoma without Rosenthal's fib ers. No complementary radiotherapy had been done. Her mother has been operated upon in 1972, for a high-grade glioma and is still alive 20 y ears later. This suggests diffuse cerebral gliomatosis. This family hi story may suggest a relation between these different diseases. They mi ght be the result of a transmissible astrocytic abnormality, with vary ing expression.