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• W2017338364 abstract "We report the clinical, neurophysiological, and immunocramps, either at rest or on exercise, in limb and trunk logical findings in a novel case, presenting with neuromuscles. Blood chemistry, including serum electrolytes, myotonia (NMT) and essential thrombocythemia (ET). calcium, phosphorus, creatine kinase, and thyroid function A 68-year-old was in good health until March 1988, tests, was normal. Anti-voltage-gated potassium channels when he begun experiencing progressing muscle cramps. (VGKC) antibodies were negative. Electrophysiologic These at first affected only his lower limbs and were testing revealed normal motor and sensory nerve conducbrought on by exercise. Later, they spread to affect his tion studies of the median, ulnar, sural, tibial, and common hands, arms, abdomen and thorax muscles. The cramps peroneal nerves, but needle recordings from resting musoften occurred while he was resting, and were always cles showed continuous activity of the motor units, preincreased by exercise, and were a cause of major disability. senting as single potentials or bursts of high frequency The patient had no other neurological symptoms. motor unit potentials in different limb muscles (abductor On admission, at another institution, physical examinapollicis brevis, flexor digitorum profundus, quadriceps tion was normal except that muscle twitching could be femori, biceps femoralis, gastrocnemius, and tibialis anseen, either at rest or on exercise, in limb and trunk terior). Ischemic block of the median nerve, lasting 4 min, muscles. Tone and power were normal and tendon reflexes did not modify the continuous muscle activity recorded were present. Plantar responses were flexor. There were no from the right abductor pollicis brevis. To define the origin sensory signs. Percussion did not elicit myotonia. Routine of this spontaneous activity, anesthetic block of the left laboratory analysis was negative and an electrophysiologic tibial and common peroneal nerves by local infiltration of study was considered normal. The patient was discharged 10 ml of 0.5% bupivacaine and 40 ml of 1% bupivacaine and treated with hydroxiquinine 200 mg daily. However, at the fibular head induced a complete nerve conduction the cramps persisted. block. Nevertheless, the involuntary muscle discharges In February 1991, routine laboratory analysis revealed a were still present, demonstrating the peripheral origin of persistently elevated platelet count of between 669 and the hyperexcitability. 9 772310 / l, and a diagnosis of essential ET was made on Therapy with phenytoin, 200 mg daily, only controlled the basis of the Polycythemia Vera Study Group criteria the cramps partially. However, his symptoms almost [1]. Therapy with hydroxyurea controlled the platelet completely disappeared following therapy with carbamazcounts, but the cramps remained unmodified. epine at a total dose of 600 mg a day. He is currently In May 1993, he was admitted to the Mostoles General asymptomatic. Hospital for further evaluation. On admission, the findings NMT is an acquired disorder of peripheral nerve hyperwere similar to previous observations with myokymia and excitability, characterized clinically by myokymia, cramps, especially induced by muscle activity, impaired muscle relaxation, and sometimes muscle weakness; there is often *Corresponding author. Tel.: 134-91-390-8666; fax: 134-91-390increased sweating and a raised creatine kinase level. The 8666. E-mail address: jbenitol@meditex.es (J. Benito-Leon) electrophysiological hallmark is the spontaneous firing of" @default.
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• W2017338364 date "2000-02-01" @default.
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• W2017338364 title "Neuromyotonia in association with essential thrombocythemia" @default.
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• W2017338364 doi "https://doi.org/10.1016/s0022-510x(99)00302-0" @default.
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