Video-Polysomnography in Peripheral Nerve Hyperexcitability: Clues to Morvan Syndrome in Two Patients and Literature Review

Peripheral nerve hyperexcitability syndromes are characterized by spontaneous motor discharges, fasciculations, and cramps. Morvan syndrome differs from Isaacs syndrome by its central nervous system involvement, autonomic dysfunction, and profound sleep disruption, often presenting as agrypnia excitata. We report two seropositive patients with cramps, fasciculations, insomnia, and autonomic dysfunction but without neuropsychiatric features. Case 1, a 49-year-old man, had LGI1 antibodies and presented with insomnia and autonomic symptoms. Video-polysomnography revealed severe insomnia with absent N3/REM sleep, reduced spindles and K-complexes, and oneiric stupor behaviors. Case 2, a 25-year-old woman, had LGI1 and CASPR2 antibodies, neuropathic pain, and fasciculations. Video-polysomnography demonstrated REM sleep without atonia, rapid N1-to-REM transitions, and microstructural abnormalities of N2 sleep. Tumor screening was negative in both; both improved with immunotherapy. A literature review confirms that video-polysomnography consistently reveals central involvement in Morvan syndrome, characterized by loss of sleep spindles, K-complexes, and pathological REM features, while Isaacs syndrome preserves normal sleep structure. Video-polysomnography is therefore an essential tool to detect subclinical central nervous system dysfunction in Morvan syndrome, especially when neuropsychiatric symptoms are absent, and helps distinguish it from Isaacs syndrome.

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Publication Details

Journal
Journal of Clinical Neurophysiology
Published
2025-12-02
DOI
https://doi.org/10.1097/wnp.0000000000001222
Primary Topic
Autoimmune Neurological Disorders and Treatments
Type
article
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article

Video-Polysomnography in Peripheral Nerve Hyperexcitability: Clues to Morvan Syndrome in Two Patients and Literature Review

Ersin Tan, Merve Melodi Çakar, Fatma Gökçem Yıldız, A GUL et al.
Journal of Clinical Neurophysiology
Autoimmune Neurological Disorders and Treatments
article

Video-Polysomnography in Peripheral Nerve Hyperexcitability: Clues to Morvan Syndrome in Two Patients and Literature Review

Ersin Tan, Merve Melodi Çakar, Fatma Gökçem Yıldız, A GUL, Esqin Meherremov
article en

Abstract

Peripheral nerve hyperexcitability syndromes are characterized by spontaneous motor discharges, fasciculations, and cramps. Morvan syndrome differs from Isaacs syndrome by its central nervous system involvement, autonomic dysfunction, and profound sleep disruption, often presenting as agrypnia excitata. We report two seropositive patients with cramps, fasciculations, insomnia, and autonomic dysfunction but without neuropsychiatric features. Case 1, a 49-year-old man, had LGI1 antibodies and presented with insomnia and autonomic symptoms. Video-polysomnography revealed severe insomnia with absent N3/REM sleep, reduced spindles and K-complexes, and oneiric stupor behaviors. Case 2, a 25-year-old woman, had LGI1 and CASPR2 antibodies, neuropathic pain, and fasciculations. Video-polysomnography demonstrated REM sleep without atonia, rapid N1-to-REM transitions, and microstructural abnormalities of N2 sleep. Tumor screening was negative in both; both improved with immunotherapy. A literature review confirms that video-polysomnography consistently reveals central involvement in Morvan syndrome, characterized by loss of sleep spindles, K-complexes, and pathological REM features, while Isaacs syndrome preserves normal sleep structure. Video-polysomnography is therefore an essential tool to detect subclinical central nervous system dysfunction in Morvan syndrome, especially when neuropsychiatric symptoms are absent, and helps distinguish it from Isaacs syndrome.

Journal of Clinical NeurophysiologyVol. 43(2)
Apple (Israel) (IL), Hacettepe University (TR)
Openalex Percentile: Top 47%
Autoimmune Neurological Disorders and Treatments
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