Clinical, laboratory and electrophysiological features of Morvan’s Fibrillary Chorea

Lee, Will; Day, Timothy; Williams, David R.

doi:10.1016/j.jocn.2012.10.029

Cited by 19 publications

(10 citation statements)

References 24 publications

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“…4 This patient classically demonstrated a strongly positive serum anti-VGKC titer; however, this is not a requirement for the diagnosis of Morvan syndrome. 2,[5][6][7][8][9] The increasing spectrum of diseases associated with anti-VGKC complex antibodies has recently been summarized. 10,11 These conditions initially included disorders of the peripheral nervous system such as acquired neuromyotonia or Isaacs syndrome, and cramp-fasciculation syndrome.…”

Section: Discussionmentioning

confidence: 99%

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Morvan Syndrome

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Chhetri

Dayanandan

et al. 2015

The Neurohospitalist

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A 74-year-old gentleman was admitted to the regional neurosciences center with encephalopathy, myokymia, and dysautonomia. Chest imaging had previously identified an incidental mass in the anterior mediastinum, consistent with a primary thymic tumor. Antivoltage-gated potassium channel (anti-VGKC) antibodies were positive (titer 1273 pmol/L) and he was hypokalemic. Electromyogram and nerve conduction studies were in keeping with peripheral nerve hyperexcitability syndrome, and an electroencephalogram was consistent with encephalopathy. A diagnosis of Morvan syndrome was made, for which he was initially treated with high-dose steroids, followed by a 5-day course of intravenous immunoglobulin (IVIG) therapy. He also underwent thymectomy, followed by a postexcision flare of his symptoms requiring intensive care management. Further steroids, plasmapheresis, and IVIG achieved stabilization of his clinical condition, enabling transfer for inpatient neurorehabilitation. He was commenced on azathioprine and a prolonged oral steroid taper. A subsequent presumed incipient relapse responded well to further IVIG treatment. This case report documents a thymoma-associated presentation of anti-VGKC-positive Morvan syndrome supplemented by patient and carer narrative and video, both of which provide valuable further insights into this rare disorder. There are a limited number of publications surrounding this rare condition available in the English literature. This, combined with the heterogenous presentation, association with underlying malignancy, response to treatment, and prognosis, provides a diagnostic challenge. However, the association with anti-VGKC antibody-associated complexes and 2 recent case series have provided some scope for both accurate diagnosis and management.

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Section: Discussionmentioning

confidence: 99%

“…4 This patient classically demonstrated a strongly positive serum anti-VGKC titer; however, this is not a requirement for the diagnosis of Morvan syndrome. 2,5 -9…”

Section: Discussionmentioning

confidence: 99%

Morvan Syndrome

Maskery

Chhetri

Dayanandan

et al. 2015

The Neurohospitalist

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“…The diagnosis of Morvan's syndrome prompted an examination of the underlying antigenic source. Thymoma was ruled-out, and a thorough investigation excluded the possibility of an active neoplastic process [4].…”

Section: Section-2mentioning

confidence: 99%

Clinical Reasoning: Myokymia, Dysautonomia, and Uveitis Researching a Common Denominator

Mikhail

Maurice

2020

Asp Biomed Clin Case Rep

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A 56-year old man, originally from Pakistan, presented with bilateral avascular necrosis of the hips on a background of emphysema, pulmonary fibrosis, coronary artery disease, diabetes type 2 and psoriasis. The cause of the avascular necrosis was unclear, with no recent trauma or steroid use. During his preoperative consultation, he presented dysautonomia requiring an inpatient investigation; the surgery was canceled. He reported a thirty-pound weight loss and a three-week history of night sweats and shortness of breath. While hospitalized, this gentleman presented subacute confusion and fluctuation of his sensorium, compatible with limbic encephalitis. Furthermore, he developed diffuse myokymia involving the axial and appendicular musculature, confirmed by EMG.

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“…Das Morvan Syndrom beschreibt die Kombination der geschilderten Symptome der Neuromyotonie mit zusätzlicher Dysautonomie und Veränderungen der Kognition und des Verhaltens [ 7 -9 ] . Die häufi gsten Symptome sind Insomnie, visuelle Halluzinationen, Verwirrtheit, Änderungen der Persönlichkeit sowie Hyperhidrose, Tachycardie, Hypertension, Constipation und Inkontinenz [ 8 ] .…”

Section: Klinische Beschwerdenunclassified

“…Neoplasien fi nden sich in ca. 50 % der Patienten mit Morvan Syndrom [ 8 ] und 10 % mit NMT [ 28 ] , das KFS ist kein paraneoplastisches Syndrom.…”

Section: Nachentladungen Bei Stimulation Peripherer Nervenunclassified

Periphere Nervenübererregbarkeit – Krampf-Faszikulationssyndrom, Neuromyotonie und Morvan Syndrom

et al. 2014

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Zusammenfassung Periphere Nerven?bererregbarkeitssyndrome sind seltene Erkrankungen die sich je nach Schweregrad und Auspr?gung durch verschiedene klinische und elektrophysiologische Befunde auszeichnen. Bei dem Krampf-Faszikulationssyndrom (KFS) finden sich entsprechend Muskelkr?mpfe und Faszikulationen, die sich auch elektromyografisch nachweisen lassen. Die Neuromyotonie (NMT) ist klinisch durch Muskelkr?mpfe, Muskelzuckungen, Myokymien, verz?gerte Muskelrelaxation, Par?sthesien und vermehrtes Schwitzen gekennzeichnet und elektromyografisch finden sich neben Doublets, Triplets und Multiplets die charakteristischen hochfrequenten neuromyotonen Entladungen. Das Morvan Syndrom erweitert die Neuromyotonie um Dysautonomie, Ver?nderungen der Kognition und des Verhaltens. Autoantik?rper, die nicht gegen die VGKC selbst gerichtet sind, sondern gegen Proteine, die mit den VGKC einen Komplex bilden (Contactin-2, CASPR-2 und LGI-1) finden sich in bis zu 25% beim KFS, in bis zu 50% bei der NMT und bis zu 90% beim Morvan Syndrom. NMT und Morvan Syndrom kommen als paraneoplastische Syndrome vor, vor allem assoziiert mit Thymom oder kleinzelligem Bronchuskarzinom. Bei paraneoplastischen Formen kann die Behandlung des Tumors zu Besserungen der neuromyotonen Symptome f?hren. Zur symptomatischen Behandlung werden Na-Kanal Blocker wie z.?B. Carbamazepin eingesetzt, zur Behandlung autoimmuner Formen intraven?se Immunglobuline oder Plasmapharese.

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Clinical, laboratory and electrophysiological features of Morvan’s Fibrillary Chorea

Cited by 19 publications

References 24 publications

Morvan Syndrome

Morvan Syndrome

Clinical Reasoning: Myokymia, Dysautonomia, and Uveitis Researching a Common Denominator

Periphere Nervenübererregbarkeit – Krampf-Faszikulationssyndrom, Neuromyotonie und Morvan Syndrom

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