Encephalopathy with astrocytic residual bodies. Report of a case and review of the literature.
Biopsy and autopsy findings in a girl who died at 7 1/2 months after having suffered from progressive axial hypotonia, myoclonus, EEG changes and retarded psychomotor development. Inclusions consisting of lamellar profiles, situated in membrane-bound cytosomes were found mainly in astrocytes, but also in neurones and in axons of peripheral nerves. Lipofuscin bodies were also increased in number. The patient belongs in the same category as cases studied by Towfighi et al. (1975) and Martin et al. (1977). Etiology and pathogenesis of this syndrome remain unknown. It is suggested, however, that the pathological changes observed might have been caused by the administration soon after birth of anti-epileptic drugs (diphenylhydantoin, clonazepam and nitrazepam).
Figols
J
J
Institute of Neuropathology, Free University Berlin, FRG.
Cervós-Navarro
J
J
Wolman
M
M
D000927:Anticonvulsants / Q000009:adverse effects; D001253:Astrocytes / Q000473:pathology*; D001921:Brain / Q000473:pathology*; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008607:Intellectual Disability / Q000209:etiology* / Q000473:pathology; D008099:Liver / Q000473:pathology; D008168:Lung / Q000473:pathology; D009207:Myoclonus / Q000209:etiology* / Q000473:pathology; D009460:Neurologic Examination; D010525:Peripheral Nerves / Q000473:pathology; D012640:Seizures / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
D000927:Anticonvulsants