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An 18-year-old Portuguese Caucasian man presented with a gradual onset of painless weakness and atrophy of the right hand and forearm, accompanied by a numb sensation in the hand that had been worsening over the past 12 months despite physiotherapy. There was no history of cold paresis. Neurologic examination revealed atrophy in all muscles of the forearm and intrinsic hand muscles, no fasciculations, right upper limb (UL) hypotonia, distal and extensor predominant motor deficit, right UL pathologic hyperreflexia, and right C6-C8 dermatomes hypalgesia. Electrophysiologic study demonstrated severe chronic denervation of right C7-T1 myotomes, mild chronic

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