Case 11 History ---- The decedent was a 51-year-old male with ALS who was first noted to have left hand weakness and fasciculations in the summer of 2017. Electromyogram and nerve conduction studies revealed active denervation and physical exam showed relatively increased reflexes and left arm compare with the right. Given his family history of dementia with some features of frontotemporal dementia, C9orf72 genetic testing was performed but was shown to be negative. The decedent’s condition deteriorated over time to the point that he became wheelchair bound and required average volume-assured pressure support throughout the day. In June 2019 he presented to the hospital with shortness of breath and was found to have a lobar and segmental pulmonary embolism involving the right upper and middle lobes, for which he was started on a heparin drip. He was later enrolled in hospice care with end stage ALS. He ultimately made the decision with his family to wean from noninvasive ventilation. Time of death was in October 2019. ---- At autopsy the weight of the unfixed brain was 1490g. Coronal sections of the cerebral hemispheres show a generally well-preserved cortical ribbon
