Case 18 History ---- The patient was a 70 year old man with history of hypertension, bladder cancer status post surgical resection in 1998, left knee arthroscopy, who was diagnosed with amyotrophic lateral sclerosis (ALS) in December of 2012. The patient was initially referred in October 2012 for significant pain and progressive weakness of the lower extremities and loss of weight. Electromyography showed "non-length dependent, motor predominant neurogenic process with acute and chronic denervation in three spinal regions, most consistent with a motor neuronopathy". Deltoid muscle biopsy showed "ongoing denervation with moderate early reinnervation". Brain and spinal cord MRI showed nonspecific white matter T2 hyperintensities in the brain and moderate degenerative changes throughout the cervical and lumbar spine. The diagnosis of ALS was made. The patient’s ALS progressed rapidly and he continued to have weight loss. The patient fell and hit his head on a rock in December 2012. He suffered a brief loss of consciousness and a scalp laceration. Head CT showed there was evidence of a small right temporal contusion and right frontal post traumatic subarachnoid blood. He was stabilized and discharged. On 3/1/2013 the patient presented to the neuromuscle clinic for routine follow up, where he was found to have shortness of breath and fatigue. He was then transferred to the ED for further evaluation, where there was concern for pulmonary embolism in the setting of acute respiratory decline. A chest x-ray showed clear lungs and a chest computed tomography scan showed no evidence of pulmonary embolism. Upon further discussion of potential interventions with the family, it was decided that they would prefer to pursue comfort care measures only. The patient was transferred to inpatient neurology service for comfort care and expired in March 2013. ---- At autopsy the weight of the unfixed brain was 1190g. There is minimal global atrophy. The ventral nerve roots appear to be slightly thinner in diameter than the dorsal roots along-with presence of subtle gelatinous changes.
