J. Honnorat et al., ADULT FORM OF ALEXANDERS-DISEASE AND DIFF USE CEREBRAL GLIOMATOSIS INTHE SAME FAMILY, Revue neurologique, 149(12), 1993, pp. 781-787
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.