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Washington University Experience | NEURODEGENERATION | ALS | 12A1 ALS (Case 12) LS Anterior horn H&E WM
Case 12 History ---- The patient was a 66 year old male with a history of progressive upper and lower extremity weakness, starting in 2008. Muscle biopsy (left deltoid) in 03/2011 showed evidence of neurogenic atrophy. Studies in 07/2011 showed evidence of widespread denervation in four body regions, without evidence of re-innervation, consistent with motor neuron disease. These findings supported a diagnosis of ALS in 2011, and his disease progressed to quadriplegia by February 2014. Approximately one month prior to his death, he presented with acute onset shortness of breath and chest pain. He was found to have elevated troponins, pulmonary hypertension, bilateral lower lobe infiltrates, and restrictive pulmonary changes. After diuresis, the patient improved and was discharged home where he eventually died. ---- The weight of the unfixed brain is 1540g. ---- 12A1-3 Multiple sections of the spinal cord, particularly lumbosacral, show severe loss of anterior horn motor neurons, with many remaining motor neurons appearing atrophic. In portions of the thoracic and lumbosacral spinal cord, the anterior horn shows dramatic parenchymal rarefaction, and several associated nerve roots show marked loss of myelinated axons (visible on Bielschowsky silver and Luxol Fast Blue - Periodic Acid Schiff (LFB-PAS) stained sections.
