Abstract:
:Retarded mental and motor development was observed during the first year of life of a full-term female who had been delivered with difficulty. Generalized psychomotor seizures and dysplastic "acromegalic" facial changes began to develop when she was 8 years old, and generalized amyotrophy developed over the next several years. The course was generally progressive, and she died at age 31. A variety of clinical laboratory studies were nondiagnostic. Autopsy findings showed normal brain weight and normal-appearing cerebral hemispheres, a peculiar atrophy of the inferior portion of the cerebellar hemispheres, a demyelinating process in the dorsal columns of the cervical and thoracic spinal cord, and lateral column and motor neuron degeneration. The pathogenesis of the syndrome is undetermined.
journal_name
Neurologyjournal_title
Neurologyauthors
Landau WM,Torack RM,Guggenheim MAdoi
10.1212/wnl.26.9.869subject
Has Abstractpub_date
1976-09-01 00:00:00pages
869-73issue
9eissn
0028-3878issn
1526-632Xjournal_volume
26pub_type
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