[Cavitating neutral fat XXXX with recurrent course].

@entity1 @entity410 @entity17 @entity508 @entity1887 @entity8431 @entity1662 @entity7622 @entity8019
A @entity1 with non-contributory family history, at the age of 7 years developed a recurrent @entity508 followed by @entity1662 and @entity410 . She died in a decerebrate state at the age of 10 9/12 years. Diffuse EEG abnormalities changed in accordance with the clinical course. CSF findings were always negative. Brain biopsy performed at the age of 9 5/12 years was non-diagnostic. No clinical signs of @entity1887 were present. Neuropathological examination disclosed severe @entity17 of the cerebral white matter with formation of large periventricular cavities, relative sparing of the subcortical arcuate fibers, and peripheral myelin breakdown of the orthochromatic (neutral fat) type. There was complete absence of inflammatory lesions, comparative preservation of axons and oligodendroglia with moderate astroglial reaction. The optic system and cerebellum were not involved. Additional postanoxic changes were seen in the thalamus and hippocampus. The sporadic case is suggested to represent a cavitating form of @entity8431 which is to be separated from Schilder's @entity7622 and @entity8019 . The etiology of the disorder is unknown.
