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Case Reports
. 1992 Mar-Apr;8(2):145-7.
doi: 10.1016/0887-8994(92)90038-z.

Abnormal dendritic development in maple syrup urine disease

A Kamei  1 , S Takashima, F Chan, L E Becker
Affiliations
Case Reports

Abnormal dendritic development in maple syrup urine disease

A Kamei et al. Pediatr Neurol. 1992 Mar-Apr.

Abstract

The neuropathology of a 6-year-old boy with maple syrup urine disease revealed spongy appearance of the white matter and marked edema of the brainstem. Golgi studies demonstrated aberrant orientation of neurons together with abnormalities of dendrites and dendritic spines. Similar changes were observed in a patient with dihydropteridine reductase deficiency. Disorders of amino acid metabolism may be associated with alterations in the terminal stages of neuronal migration and maturation.

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