Background: Morvan's syndrome is characterized by peripheral nervous system hyperexcitibility (myokymia and neuromyotonia), hyperhydrosis, sleep disorder, limb paresthesias, and encephalopathy. Voltage gated potassium channel antibodies (VGKC abs) are frequently present. Reduplicative paramnesia (RP) has not been reported with this disorder.
Objective: To describe a patient with Morvan's syndrome presenting with RP.
Design: Single case study.
Patient: A 64-year-old man with several years of myokymia and myoclonus with escalating parasomnia and confusion developed the delusion that a replica of his house and its contents existed 40 mi away.
Results: Serum VGKC ab titer was elevated. Neuropsychological testing disclosed executive function and memory deficits. Electromyography demonstrated diffuse myokymia. Treatment with intravenous immunoglobulin and prednisone produced improvement of RP and myoclonus, but not myokymia.
Conclusion: RP may occur in patients with VGKC ab-associated Morvan's syndrome. Both RP and nervous system hyperexcitability may respond to immunotherapy including intravenous immunoglobulin and corticosteroids.
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http://dx.doi.org/10.1016/j.jns.2007.09.030 | DOI Listing |
Ann Indian Acad Neurol
September 2024
Department of Neurology, National Institute of Mental Health and Neurosciences, Bengaluru, Karnataka, India.
Medicine (Baltimore)
October 2024
Clinical Department of Traditional Chinese Medicine, Hubei University of Chinese Medicine, Wuhan, China.
Cureus
September 2024
Internal Medicine, California Hospital Medical Center, Los Angeles, USA.
Med Clin (Barc)
December 2024
Department of Neurology, Guangdong Neuroscience Institute, Guangdong Provincial People's Hospital (Guangdong Academy of Medical Sciences), Southern Medical University, Guangzhou, China.
Rev Neurol (Paris)
November 2024
Service de neurologie clinique et fonctionnelle, groupe hospitalier Sud, hospices civils de Lyon, Lyon, France; Centre de référence pour les encéphalites auto-immunes et les syndromes neurologiques paranéoplasiques, hospices civils de Lyon, Lyon, France. Electronic address:
CASPR2-associated neurological disorders encompass a wide clinical spectrum broadly divided into overlapping three autoimmune syndromes: CASPR2 limbic encephalitis, Morvan syndrome, and Isaacs syndrome. CASPR2 is a neuronal protein expressed at different sites in the central and peripheral nervous system and has a variety of roles and functions regarding neuronal excitability, synaptic plasticity, and homeostasis of inhibitory networks, most of which are only partially understood. CASPR2 antibodies have various pathogenic effects including internalization of CASPR2, disruption of protein-protein interactions, and, possibly, complement activation.
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