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Washington University Experience | PERIPHERAL NEUROPATHY | 11 VASCULITIS - VASCULOPATHY | 3 Vasculitis - NOS | 17A0 Case 17 History
Case 17 History ---- The patient is an 87-year-old man who initially presented with left upper extremity weakness. More recent symptoms include slurred speech without additional bulbar symptoms. There were no gait issues or falls. Examination showed fasciculations in the bilateral cervical myotomes as well as fasciculation over the right and left quadriceps muscles. There was diminished quadriceps bulk on the right > left and atrophy of the distal left upper extremity, and to a lesser degree the proximal bilateral upper extremities. The tongue had normal strength, but exam showed mild lingual dysarthria and slow speech. Forced vital capacity was >80% of predicted. EMG from January 2016 showed ongoing changes consistent with active and chronic denervation of the left C8/T1 myotomes. Subsequent electrodiagnostics showed active denervation in the right lumbosacral, thoracic, and cervical myotomes, bolstering the clinical suspicion for motor neuron disease. There was no ANNA-1 antibody positivity. Quantitative immunoglobulins were within normal limits but he had a small IgG monoclonal protein. A course of IVIg led to no improvement in his symptoms. CT of the chest, abdomen and pelvis showed no malignancy. Operative procedure: Right vastus lateralis and right sural nerve biopsy.
