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It has previously been shown that 2,5-hexanedione (2,5-HD) and its 3,4-dimethyl derivative (3,4-DMHD) induce neurofilamentous accumulations at prenodal sites in distal and proximal, respectively, regions of peripheral axons. For 2,5-HD, neurofilament (NF) transport is accelerated and this is thought to be directly related to the appearance of the axonal(More)
Dysplastic gangliocytoma of the cerebellum is reported in a newborn. It is characterized by large ganglion cells in the molecular layer and premature attenuation of the external granule-cell layer. The internal granule-cell layer appears rarefied but otherwise normally organized. It appears that in this disease the migrating granule cells mature too early(More)
We are presenting evidence of subclinical extrapyramidal hemosiderosis in chronic cystic fibrosis (CF) by histological staining and quantitative analysis. Comparison was made between age-matched control (eight cases) and CF brains (14 cases). None of the CF patients had extrapyramidal or other focal CNS symptoms but showed an increased iron pigment, and(More)
A homosexual man with a history of sexually transmitted infections including recent giardiasis and high cytomegalovirus (CMV) titer was admitted with generalized weakness, headache, and depression. He rapidly became comatose and developed signs of increased intracranial pressure. CT scan revealed a right cerebral lesion. Pathologic examination disclosed an(More)
The chronic exposure to gamma-diketones results in the formation of giant neurofilament (NF)-containing axonal enlargements, followed by axonal degeneration in peripheral axons. Based on the specific ability of gamma-diketones to react with primary amino groups to form pyrroles, and the observation of such reaction with NF protein in vitro and with other(More)
Postmortem examination of the central nervous system (CNS) of a dicephalus thoracopagus tetrapus revealed two separate brains and spinal cords. On the conjoined side the spinal cord showed hypoplasia of the anterior horns and dorsal funiculi at the lower cervical and upper thoracic levels. Hypoplasia of the peripheral nerves, anterior horn cells, and dorsal(More)
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