Case 14 History ---- The patient was a 72 year old female with a past medical history of lumbar spinal stenosis, hypertension and gastroesophageal reflux disease, who presented to BJH in 9/2012 with progressive bilateral lower extremity weakness. The patient first noticed weakness nine months before. Starting six months before, she started having troubles with daily tasks. Two months before, she required a walker to walk. In late August, she presented to an outside hospital for evaluation of this weakness; a muscle biopsy reportedly showed changes consistent with denervation, and electromyography (EMG) reportedly showed radiculopathy. Magnetic resonance imaging (MRI) of the brain at that time showed mild atrophy and small-vessel disease without evidence of an acute process; lumbar and cervical disk bulges, also noted, were minimal and without stenosis. The patient was discharged to a rehabilitation facility. A week later, she was sent to a nursing home, where she developed increased weakness and shortness of breath, for which she was transferred to BJH. At BJH, MRI of the cervical, thoracic, and lumbar spine was unrevealing. Computed tomography of the chest, neck, abdomen and pelvis was negative for systemic malignancy. Results from laboratory studies of the cerebrospinal fluid were unremarkable. Results from a serum panel for myopathy and for HIV infection were also negative. EMG at BJH was consistent with motor neuron disease. She was also evaluated clinically by the neuromuscular consult team, who confirmed the probable diagnosis of amyotrophic lateral sclerosis with predominantly lower motor neuron signs. During her hospital stay, the patient was intubated in 9/2012. Because the patient wished not to be artificially sustained, goals of care were switched to comfort measures. The patient expired in 9/2012. ---- A t autopsy the weight of the unfixed brain was 1060g.
