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87. Neuromyotonia Synonyms. Continuous Muscle Fiber Activity Syndrome; Isaacs Syndrome; QuantalSquander; IsaacsMerten Syndrome. Disorder Subdivisions. None http://www.bchealthguide.org/kbase/nord/nord758.htm

var hwPrint=1;var hwDocHWID="nord758";var hwDocTitle="Neuromyotonia";var hwRank="1";var hwSectionHWID="nord758-Header";var hwSource="en-caQ2_05";var hwDocType="Nord"; National Organization for Rare Disorders, Inc. Neuromyotonia Important It is possible that the main title of the report Neuromyotonia is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Continuous Muscle Fiber Activity Syndrome Isaacs' Syndrome Quantal Squander Isaacs-Merten Syndrome Disorder Subdivisions None General Discussion 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). Resources NIH/National Arthritis and Musculoskeletal and Skin Diseases Information Clearinghouse 1 AMS Circle Bethesda, MD 20892-3675

88. Baylor Neurology Case Of The Month The syndrome has also been called a variety of other names such as QuantalSquander , The syndrome of continuous motor activity , Pseudomyotonia and http://www.bcm.edu/neurology/challeng/pat2/summary.html

Summary and Discussion Amit Verma, M.B.B.S. Chief Resident, Department of Neurology Diagnosis: The patient was admitted to the hospital and received 6 courses of plasma exchange. He was subsequently started on oral prednisone and cyclophosphamide and during his hospital stay noticed a marked improvement in the myokymia and muscle strength. The myotonia improved as well. The patient was then discharged and was to be followed in clinic. A Review of Isaacs' Syndrome Epidemiology The condition is extremely rare and has therefore never been studied in a controlled fashion. A recent review of all reported cases by Jamieson and Katiriji in 1994 suggests an equal incidence in both sexes. The disease appears to present earlier in life with most patients being less than 40 years of age at the time of symptom onset. A study of 3 families by Ashizawa et al in 1983 suggested a dominant mode of inheritance in the familial form of the disease. Etiology and Pathogenesis The etiology of the disease remains unclear. Isaacs in 1961 was the first person to localize the disorder to the peripheral nerves. Pharmacological localization of the generator of the myokymia seems to confirm the initial observations of Isaacs. More exact localization has been less fruitful. The electrical activity appears to originate from multiple sites in the axon. There is some evidence to suggest the role of autoimmunity and, specifically, antibodies against the potassium channel in motor nerve terminals. The potential importance of circulating antibodies is supported by the fact that plasma exchange is effective in treatment of some patients with this disease. A recent case has been reported in which a patient met criteria for both Isaacs' syndrome and CIDP, another potential autoimmune disease.

89. Isaacs' Syndrome report. Synonyms. Continuous Muscle Fiber Activity Syndrome; Neuromyotonia; QuantalSquander; IsaacsMerten Syndrome. Disorder Subdivisions. http://hw.healthdialog.com/kbase/nord/nord758.htm

document.write(''); var hwPrint=1; var hwDocHWID="nord758"; var hwDocTitle="Isaacs' Syndrome"; var hwRank="1"; var hwSectionHWID="nord758"; var hwSectionTitle=""; var hwSource="us6.0"; var hwProdCfgSerNo="wsh_html_003_s"; var hwDocType="NORD"; National Organization for Rare Disorders, Inc. Isaacs' Syndrome Important It is possible that the main title of the report is not the name you expected. Please check the synonyms listing to find the alternate name(s) and disorder subdivision(s) covered by this report. Synonyms Continuous Muscle Fiber Activity Syndrome Neuromyotonia Quantal Squander Isaacs-Merten Syndrome Disorder Subdivisions Neuromytonia, Generalized, Sporadic Neuromyotonia, Generalized, Familial Neuromyotonia, Focal Related Disorders List Information on the following diseases can be found in the Related Disorders section of this report: Hallervorden-Spatz Disease Paraplegia, Hereditary Spastic Stiff Man Syndrome General Discussion Isaacs' Syndrome is a peripheral motor neuron disorder characterized by muscular stiffness and cramping, particularly in the limbs. Continuous fine vibrating muscle movements (myokymia) can be seen. Muscle relaxation may be difficult especially after physical activity involving the particular muscle(s). These symptoms persist even during sleep. Symptoms In Isaacs' Syndrome, involuntary continuous muscle fiber activity may cause stiffness and delayed relaxation in the affected muscles. Continuous fine vibrating muscle movements (myokymia) may occur along with these symptoms. Affected individuals may have episodes of the inabilitiy to coordinate voluntary muscle movement and difficulty walking (ataxia). Other symptoms may include staggering and reeling (titubation), stiffness, and lack of balance in response to starle. There may be diminished spontaneous gross motors activity.

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