Abnormal dendritic development in maple syrup urine disease
A Kamei, S Takashima, F Chan, LE Becker - Pediatric neurology, 1992 - Elsevier
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.
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.
