Isaacs syndrome: A slow potassium channelopathy caused by autoantibodies?
نویسندگان
چکیده
منابع مشابه
Long QT syndrome - a genetic cardiac channelopathy.
INTRODUCTION Long QT-syndrome (LQTS) is a genetic cardiac channelopathy characterised by a prolonged QT interval on a surface electrocardiogram (ECG), syncope, T-wave abnormalities, ventricular tachycardia of the torsades de pointes (TdPVT) type (Fig. 1) and an increased risk of sudden death [1]. LQTS has variable clinical presentation and is genetically characterised by incomplete penetrance, ...
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Autoantibodies against voltage-gated potassium channels (VGKC-Abs) are associated with acquired neuromyotonia (Isaacs' syndrome) and related disorders such as Morvan's syndrome and some cases of limbic encephalitis. The mechanisms underlying the various phenotypes induced by VGKC-Abs are not fully understood. Recently, we reported a case of LE with VGKC-Abs accompanied by severe intestinal pseu...
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Autoimmune voltage-gated potassium channelopathies represent a wide and expanding spectrum of neurological conditions. We present a case demonstrating the phenotypic heterogeneity of antivoltage-gated potassium channels (VGKC)-associated disorders. Such cases may easily be dismissed as functional disorders at first presentation. We propose that there must remain a high index of suspicion for an...
متن کاملI-15 Neuromyotonia: Potassium channelopathy or non ionic disease?
The early pathophysiologic study showed increasing evidence that autoimmunity is implicated in the pathogenesis of neuromyotonia. Antibodies to voltage gated potassium channel were detected in the serum of patients who had peripherical nerves hyperexcitability and also Morvan’s disease or limbic encephalitis. These discoveries offered new approaches to treatments. Recently, antibodies previousl...
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ژورنال
عنوان ژورنال: Clinical Neurophysiology
سال: 2018
ISSN: 1388-2457
DOI: 10.1016/j.clinph.2018.01.069