A 19-year-old man presented with 4 years of facial numbness and 3 years of progressive dysarthria and dysphagia. Additionally, 18 months prior to presentation, he also developed arm weakness and hand atrophy followed by leg weakness 1 year later.
On examination, dysarthria, bilateral peripheral facial paralysis (Figure, A), reduced facial touch and vibration sensitivity in the mandible, temporal muscle atrophy (Figure, B), and poor soft palate motility were observed. Corneal reflexes were bilaterally absent. Deltoid, calf, and distal muscle atrophy (Figure, C) and weakness (right extensor carpi, Medical Research Council [MRC] grade 0; neck flexion, MRC grade 4; proximal muscles, MRC grade 3; other arm distal muscles, MRC grade 2-3; leg distal muscles, MRC grades 2-4) were observed. Absence of the bilateral biceps and triceps reflexes, right patellar tendon hyperreflexia, discontinuous clonus in the left knee, a Babinski sign present on the right side, and an unstable and steppage gait were also observed.
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Liu Y, Luo J, Xiao F. Facial Numbness, Dysarthria, Muscle Atrophy, and Weakness in a Young Patient. JAMA Neurol. Published online July 12, 2021. doi:10.1001/jamaneurol.2021.2136
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