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Clinical descriptions of Isaacs' syndrome, or acquired neuromyotonia, usually emphasize motor phenomena including stiffness, myokymia, cramps, and delayed muscle relaxation. Electrophysiologic studies demonstrate signs of motor nerve hyperexcitability, including fasciculations, myokymia, and neuromyotonia. Symptoms of sensory nerve hyperexcitability are less well-recognized features. Three patients with Isaacs' syndrome presented with prominent sensory symptomatology creating diagnostic confusion with polyneuropathy or myelopathy.
Case reports.
Patient 1.
A 45-year-old woman was referred for possible polyneuropathy. She presented with 7 weeks of nocturnal leg numbness, resolving over a few seconds after shaking her legs. She continued with frequent brief episodes of transient numbness or tingling in various distributions, progressing to involve the arms, torso, and neck. These could occur without a clear precipitant, but were often related to brief compression or stretching. At about the same time she noted almost constant involuntary twitching of the hands with occasional painless spasms and difficulty with relaxation. She described an episode of total body trembling and sweating which lasted 5 minutes. The neurologic examination was normal except for prominent clinical myokymia in intrinsic hand muscles (see video clips on the Neurology Web site at WWW.neurology.org). Multiple Tinel's signs were noted. Nerve conduction studies (NCS) showed compound muscle action potential (CMAP) repetitive afterdischarges, without polyneuropathy. EMG revealed prominent myokymic and rarer neuromyotonic discharges restricted …
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