Case of the month: Isaacs' syndrome associated with chronic inflammatory demyelinating polyneuropathy
| Autor: | Shin J. Oh, Guillermo A. Herrera, Juan L. Joy, Zeki Odabasi, Gwendolyn C. Claussen |
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| Rok vydání: | 1996 |
| Předmět: |
Weakness
medicine.medical_specialty medicine.diagnostic_test Physiology business.industry Chronic inflammatory demyelinating polyneuropathy Motor conduction block Electromyography medicine.disease Nerve conduction velocity Surgery Fasciculation Cellular and Molecular Neuroscience Physiology (medical) medicine Neurology (clinical) Myokymia medicine.symptom business Polyneuropathy |
| Zdroj: | Muscle & Nerve. 19:210-215 |
| ISSN: | 1097-4598 0148-639X |
| DOI: | 10.1002/(sici)1097-4598(199602)19:2<210::aid-mus13>3.0.co;2-y |
| Popis: | We report the first case of Isaacs' syndrome in which an inflammatory demyelinating neuropathy was documented histologically. For 9 months, the patient developed slowly progressive weakness, muscle spasms and stiffness, fasciculations, and myokymia in the arms, which were unmodified by sleep. Nerve conduction studies showed multifocal motor conduction block, abnormal dispersion phenomenon, and abnormal sensory and mixed nerve conduction. Needle electromyogram showed continuous motor unit potentials at rest with bursts of rapid-firing discharges which were unaffected by spinal anesthesia but diminished by peripheral nerve block and completely abolished by local curarization. Sural nerve biopsy demonstrated an inflammatory demyelinating neuropathy. Muscle cramping, twitching, and stiffness responded to phenytoin. The patient's weakness gradually responded to prednisone and azathioprine. Over a 17-year period, the patient had three relapses which were well controlled with prednisone and azathioprine. At this time, the patient is symptom-free without any medication. |
| Databáze: | OpenAIRE |
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