关键词: Isaacs' syndrome autoimmune channelopathy dermatomyositis myokymia neuromyotonia

Mesh : Adult Autoantibodies / blood Autoimmunity Biomarkers / blood Biopsy Dermatomyositis / complications drug therapy immunology physiopathology Electromyography Humans Immunohistochemistry Immunosuppressive Agents / therapeutic use Isaacs Syndrome / drug therapy etiology immunology physiopathology Male Motor Activity Muscle Strength Neuromuscular Agents / therapeutic use Potassium Channels, Voltage-Gated / immunology Recovery of Function Severity of Illness Index Treatment Outcome

来  源:   DOI:10.1111/1756-185X.12881   PDF(Sci-hub)

Abstract:
This is a case report of Isaacs\' syndrome in dermatomyositis. The patient presented with proximal muscle weakness, rash, elevated muscle enzyme, myopathic electromyograph and typical muscle biopsy. Ultimately he developed typical symptoms of Isaacs\' syndrome which is an autoimmune channelopathy from voltage gated potassium channel antibody (anti-VGKC) leading to dysfunction of axonal discharge at neuromuscular junctions. It shares some similar characteristics with dermatomyositis such as autoimmunity, its association with malignancy and the response to treatment.
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