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Washington University Experience | METABOLIC | Neuronal Ceroid Lipofuscinosis (NCL) | 6A3 NCL (Case 6) 2

There is modest atrophy of the cerebral hemispheres and dilatation and blunting of the lateral ventricles. ---- Not shown (Not available): Sections of cerebral cortex showed numerous large pyramidal and small neurons ballooned by a granular orange-tinged material. Apical dendrites and axons of cortical pyramidal neurons did not exhibit large focal spheroidal axonal dilatations. Oil red O and sudan black positive inclusions were identified in many neurons and was intensely autofluorescent at a wavelength of approximately 500 nanometers. Electron microscopic examination showed the stored material was composed of masses of dark osmiophilic granular and occasionally lamellar material admixed with homogeneous lipid droplets. These are the histochemical features and ultrastructural appearance of lipopigment. Only occasional senile plaques and neurofibrillary tangles were seen in the hippocampus .

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