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Washington University Experience | NEURODEGENERATION | ALS | 8A1 ALS (Case 8) LS anterior horn 10X
Case 8 History ---- This 56 year old male was diagnosed with ALS in 1998. Electromyography (EMG) studies showed scattered denervation with fasciculations in the left arm and both legs, as well as the tongue. MRI showed cord changes consistent with ALS. He had progressive lower extremity weakness that progressed to quadriparesis. Multiple admissions to Barnes-Jewish Hospital (BJH) for treatment of respiratory difficulty and pneumonia. His respiratory difficulties were managed with BiPAP and, following his final admission to BJH in September 2003, he lived at home on portable BiPAP therapy and under hospice care. He was assessed during a follow-up visit to the neuromuscular division in October 2003 as having ALS with prominent lower motor neuron involvement. He died in his home in August 2004. ---- At autopsy the weight of the unfixed brain was 1550g. The sulcal/gyral pattern is normal and there is no appreciable atrophy of the precentral gyrus. Coronal sections of the cerebral hemisphere show a normal cortical ribbon and the ventricular system has a normal configuration. ---- 8A1,2 All sections of the spinal cord show significant neuronal loss in the anterior horns with reactive astrocytosis; there is mild variability from section to section.
