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Washington University Experience | MISCELLANEOUS | Wolfram Syndrome | 3X Case 3 Denouement
Neuro Diagnosis Comment ---- The collective neuropathologic features are consistent with the patient's history of Wolfram syndrome. The changes observed are overall subtle and mostly represent sequela of the patient's blindness. We see neuronal loss and gliosis of the lateral geniculate nucleus, as well as pallor and gliosis of the white matter in the region of the striate radiations from the lateral geniculate nucleus. We also see nonspecific Purkinje cell abnormalities and white matter gliosis in the cerebellum. Additionally, the brain size is very small and there is suggestion of some global and hippocampal atrophy. Many of these neuropathologic findings have been previously reported in Wolfram syndrome. Additional findings include absence of the neurohypophysis of the pituitary (? sampling) as well as pontine and inferior olivary neuron loss and shrinkage
