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Washington University Experience | NEURODEGENERATION | ALS | 9A1 ALS (Case 9) H&E 2 copy

Case 9 History ---- This patient was a 62 year old female first seen at BJH in July 2007 with a one year history of dysarthria with gradual progression to dysphagia and weakness in her arms and legs with wasting in the hands as well. She has an augmentative communication device and uses a motorized wheelchair for ambulation. There is severe weakness in all extremities but more in the arms than the legs. Reflexes are brisk. Sensation is normal. ---- At autopsy the fresh brain weighed 1320g. There was mild atrophy of the frontal, temporal, and parietal lobes and the precentral gyrus was unremarkable. Coronal slicing revealed no significant dilatation of the lateral ventricles. The anterior roots of the spinal cord were gray and thinned relative to the posterior roots. ---- 9A1,2 There is loss and atrophy of individual motor neurons. Noted in these two sections are large glassy axonal spheroids which often accumulate adjacent to the cell bodies (a pattern which is commonly seen in axonal dystrophy where they likely represent abnormal synapses. Rare chromatolytic neurons (arrowhead, 9A1) mimic the dystrophic axons but often have multiple branches off the cell body and peripheral marginated Nissl. Rare Bunina bodies are observed on H&E stained sections.

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