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Waves in Extremities: A Rare Report of Isolated Isaacs' Syndrome
Isaacs' syndrome is a rare neuromuscular hyperexcitable syndrome with myriad manifestations ranging from motor and sensory to autonomic presentations, leading to diagnostic challenges. Among the commonest forms, a tetrad of stiffness, myokymia (muscle twitching at rest), weakness, and psuedomyo...
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Cureus
2019
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Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6639064/ https://www.ncbi.nlm.nih.gov/pubmed/31338264 http://dx.doi.org/10.7759/cureus.4687 |
Sumario: | Isaacs' syndrome is a rare neuromuscular hyperexcitable syndrome with myriad manifestations ranging from motor and sensory to autonomic presentations, leading to diagnostic challenges. Among the commonest forms, a tetrad of stiffness, myokymia (muscle twitching at rest), weakness, and psuedomyotonia (delayed muscle relaxation) is almost always present. Herein, we report a case of a 16-year-old male who presented to the neurology consult service with intense wave-like pain in the lower extremities with desquamating rash and cold-induced allodynia. Investigations were significant for raised CK levels, positive autoimmune panel, and anti-voltage-gated potassium channel (anti-VGKC) antibody that is involved in 35% reported cases of Isaacs' syndrome, with electrodiagnostic studies peculiar for Isaacs syndrome with negative imaging results. He was followed up on a long cocktail course of immunosuppressive, anticonvulsive medications, and immunoadsorption plasmapheresis (IAP) for 11 months with complete remission. |
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