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- Disease Information
- Disease Comparison
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Disease Processes ▼
- Auto Immune
- Vascular-Arteriosclerosis
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- Deficiency
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- Systemic
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Disease Information for Isaacs potassium channel myopathy syndrome
- Clinical Manifestations
- Signs & Symptoms
- Hyperextensable Joints Infant
- Myopathic facies/snarling smile
- Constipation Children
- Tongue Protrudes Infant
- Cramping in Extremities
- Delay Sitting Unsupported Infant
- Mouth Hangs Open Infant
- Muscle cramps/spasms
- Muscle Pain
- Muscle twitches
- Muscle weakness
- Muscles Soft/Doughy Infant
- Myalgias
- Myokimia/Muscle pulling sensation
- Myotonic like rigidity of muscles
- Rolling Over Delay Infant
- Stiff muscles/spasms legs/feet
- Thigh pain
- Babkin infant sign/Abnormal
- Galant Infant reflex/Abnormal
- Head Neck Floppy Infant Hypotonia Sign
- Hyporeflexia/DTRs decreased
- Infant Head Support Delay
- Moro reflex Poor/Absent Infant
- Palmar Grasp infant Reflex Abnormal
- Primitive infant reflexes/Abnormal
- Rooting infant sign/Abnormal
- Spasms in Both Legs
- Swimming infant reflex/Abnormal
- Tonic Neck Infant reflex/Abnormal
- Shallow Breathing Infant
- Snoring
- Weakness
- Disease Progression
- Course/Progressive
- Laboratory Tests
- Microbiology & Serology Findings
- Serum specific antibodies increased
- Abnormal Lab Findings (Non Measured)
- Autoimmune antibody present/increased (Lab)
- Abnormal Lab Findings - Increased
- Anti-Potassium Channel/KCNA6 antibodies (labs)
- Diagnostic Test Results
- Electrodiagnosis
- EMG/Abnormal findings
- EMG/Continuous activity
- Associated Diseases & Rule outs
- Rule Outs
- Eaton-Lambert syndrome
- Muscular dystrophy
- Myotonia atrophica
- Polymyositis
- Associated Disease & Complications
- Apnea
- Metabolic Myopathy
- Myopathy
- Neonatal Hypotonia/Floppy Baby Syndrome
- Disease Mechanism & Classification
- Class
- CLASS/Primary organ/system disorder (ex)
- CLASS/Motor-end plate disorder (ex)
- CLASS/Muscle disorder (ex)
- CLASS/Muscle/tendon/extremities (category)
- CLASS/Striated muscle disorder (ex)
- Pathophysiology
- Pathophysiology/Sodium/potassium memb pump inhibited
- Pathophysiology/Potassium channel muscle disorder
- Process
- PROCESS/Allergy/collagen/autoimmune (category)
- PROCESS/Autoimmune disorder (ex)
- PROCESS/INCIDENCE/Esoteric disease (example)
- Synonyms
- Synonym
- Isaacs potassium channel myopathy syndrome, Synonym/Isaacs potassium channel myopathy, Synonym/Isaacs-Mertens Syndrome, Synonym/Neuromyotonia (Isaacs)
- Treatment
- Drug Therapy - Indication
- RX/Carbamazepine (Tegretol)
- Definition
-
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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- External Links Related to Isaacs potassium channel myopathy syndrome
- Wikipedia
- Merck
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- PubMed (National Library of Medicine)
- NGC (National Guideline Clearinghouse)
- Medscape (eMedicine)
- Harrison's Online (accessmedicine)
- NEJM (The New England Journal of Medicine)