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Published on: February 19, 2020
Course and outcome of a voltage-gated potassium channel antibody negative Morvan's syndrome
Carlo Rinaldi1, Cinzia Valeria Russo, Alessandro Filla
1Department of Neurological Sciences, Federico II University, Via Pansini, 5, 80131, Naples, Italy. rinaldi.ca@gmail.com
Abstract:
Morvan's syndrome is a rare disease characterized by peripheral nerve hyperexcitability, associated with CNS and autonomic systems involvement. High serum voltage-gated potassium channel (VGKC) antibody titers have been reported, and, till now, Morvan's syndrome has been considered as a VGKC antibody associated disease. We describe a patient with Morvan's syndrome associated with myasthenia gravis and a thymoma in his previous history, with surprisingly undetectable levels of VGKC antibodies. The clinical course is similar to those cases of Morvan's syndrome with VGKC-Ab, except for the lack of response to plasma exchange, previously considered as the first choice treatment. Nevertheless, the good response to corticosteroids therapy and the association with myasthenia confirm an autoimmune origin of the disease.
Insights
Morvan's syndrome can occur without voltage-gated potassium channel (VGKC) antibodies. This case study highlights a patient with Morvan's syndrome, myasthenia gravis, and thymoma, showing an autoimmune origin despite undetectable VGKC antibodies.
Area of Science:
- Neurology
- Immunology
Background:
- Morvan's syndrome is a rare autoimmune disorder affecting peripheral nerves, central nervous system, and autonomic systems.
- It is typically associated with high serum voltage-gated potassium channel (VGKC) antibody titers.
- Previously, VGKC antibody positivity was considered a hallmark of Morvan's syndrome.
Observation:
- This report details a patient with Morvan's syndrome, myasthenia gravis, and a history of thymoma.
- The patient presented with clinical features consistent with Morvan's syndrome.
- Notably, serum VGKC antibody levels were undetectable in this patient.
Findings:
- The clinical presentation mimicked VGKC antibody-associated Morvan's syndrome.
- The patient did not respond to plasma exchange, a standard treatment for VGKC antibody-associated conditions.
- However, the patient showed a positive response to corticosteroid therapy.
Implications:
- This case challenges the notion that VGKC antibodies are essential for Morvan's syndrome diagnosis.
- It suggests alternative autoimmune mechanisms may underlie Morvan's syndrome.
- The findings support an autoimmune etiology for Morvan's syndrome, even in the absence of VGKC antibodies, and highlight corticosteroids as a potential treatment.
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