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Isaacs Syndrome

(Isaacs' Syndrome)

By Michael Rubin, MDCM, Professor of Clinical Neurology; Attending Neurologist and Director, Neuromuscular Service and EMG Laboratory, Weill Cornell Medical College; New York Presbyterian Hospital-Cornell Medical Center

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Isaacs syndrome causes neuromuscular manifestations, including myokymia.

Isaacs syndrome (neuromyotonia) is a peripheral nerve hyperexcitability syndrome, generally thought to be a voltage-gated potassium channelopathy; it sometimes occurs as a paraneoplastic syndrome. It may also accompany other disorders (eg, myasthenia gravis, thymoma, Hashimoto thyroiditis, vitamin B12 deficiency, celiac disease, connective tissue disorders) or can be inherited.

Cause of Isaacs syndrome is unknown. Abnormalities are thought to originate in a peripheral nerve because they are abolished by curare but usually persist after general anesthesia.

The limbs are most affected. The sine qua non is myokymia—continuous muscle twitching described as bag-of-worms movements. Other symptoms include fasciculations, carpopedal spasms, intermittent cramps, increased sweating, and pseudomyotonia (impaired relaxation after a strong muscle contraction but without the typical waxing-and-waning electromyography [EMG] abnormality of true myotonia).


  • Clinical evaluation

  • Results of nerve conduction and EMG studies

The diagnosis of Isaacs syndrome is based on the above clinical findings, and results of nerve conduction and EMG studies, which show characteristic abnormalities; these abnormalities include after-discharges on nerve conductions studies and, on needle EMG studies, fasciculation potentials, myokymic discharges, neuromyotonic discharges, fibrillation potentials, and cramp discharges, most prominent in distal limb muscles.

Laboratory testing should include tests for the striational, voltage-gated calcium channel, gliadin, glutamic acid decarboxylase (GAD), muscle acetylcholine receptor (AChR), and voltage-gated potassium channel antibodies.


  • Drugs to relieve symptoms

  • Plasma exchange or IV immune globuln (IVIG)

Drugs that may relieve symptoms of Isaac syndrome include carbamazepine, phenytoin, gabapentin, mexiletine (experience is limited), valproate, lamotrigine, and clonazepam.

Plasma exchange and, to a lesser degree, IVIG are usually beneficial and are often used with prednisone and azathioprine.