Disease Information for Isaacs potassium channel myopathy syndrome (Clinical Manifestations)

Isaacs potassium channel myopathy syndrome

auto-immune response to potassium channels in lower limb muscles; caused by anti-bodies to K+ channels (KCNA6) antigen;known as Isaac syndrome; benign condition;----------------------------------.Neuromyotonia;Continuous Muscle Fiber Activity Syndrome ; Isaacs" Syndrome; Isaacs-Merten Syndrome; Quantal Squander; Neuromyotonia is a rare neuromuscular disorder characterized by abnormal nerve impulses from the peripheral nerves; These impulses cause continuous muscle fiber activity that may continue, even during sleep; The disorder, which has both inherited and acquired forms, is characterized by muscular stiffness and cramping, particularly in the limbs; Continuous fine vibrating muscle movements (myokymia) can be seen; Muscle weakness may also be present; Muscle relaxation may be difficult especially after physical activity involving the particular muscle(s); [NORD]----------------------------------------.Isaac"s syndrome is a disorder of unknown cause that produces predominantly limb symptoms; Abnormalities may originate in a peripheral nerve because they are abolished by curare but usually persist after general anesthesia; The sine qua non is myokymia--continuous muscle twitching described as "bag of worms" movements; Usually also present are carpopedal spasms, intermittent cramps, increased sweating, and pseudomyotonia (impaired relaxation after a strong muscle contraction but without "dive bombers"--thecharacteristic electromyographic abnormality of true myotonia); Carbamazepine or phenytoin relieves symptoms;[Merck Manual 17th]

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