Fasciculations, Autonomic Symptoms and Limbic Encephalitis: A Thymoma-Associated Morvan’s-Like Syndrome

Deymeer, Feza; Akca, Sukriye; Kocaman, Gulsen; Parman, Yeşim; Serdaroğlu, Pıraye; Öktem-Tanör, Öget; Çoban, Oğuzhan; Vincent, Angela

doi:10.1159/000090719

Cited by 13 publications

(7 citation statements)

References 12 publications

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“…VGKC-Abs positive LE was first reported in 2001 [1] and a number of cases have been reported since then. LE associated with VGKC-Abs is generally non-paraneoplastic [2,3] and there are few cases of VGKC-Abs associated LE with thymoma [2][3][4]14,21]. In contrast to previously reported cases of LE associated with VGKC-Abs, this case showed prominent autonomic symptoms such as intestinal pseudo-obstruction, hyperhidrosis, and severe hypertension.…”

Section: Discussioncontrasting

confidence: 60%

Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction

Sekiguchi¹,

Takahashi²,

Mori³

et al. 2008

Journal of the Neurological Sciences

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Section: Discussioncontrasting

confidence: 60%

Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction

Sekiguchi¹,

Takahashi²,

Mori³

et al. 2008

Journal of the Neurological Sciences

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“…Additional features that distinguished these patients from classical LE were the presence of a neuropathic lower limb pain, weight loss, male gender, and thymoma (±MG), although thymomas can also be found in rare cases diagnosed with LE 24. Six patients who each lacked 1 of the core features of NMT, autonomic disturbance, and insomnia (Supplementary Table 4), and others previously reported,1, 25–27 suggest the existence of conditions with only 2 of these 3 core components.…”

Section: Discussionmentioning

confidence: 69%

Morvan syndrome: Clinical and serological observations in 29 cases

Irani

Pettingill

Kleopa

et al. 2012

Annals of Neurology

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461

386

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MoS is clinically distinct from other VGKC-complex antibody-associated conditions, and usually is associated with high-titer CASPR2 antibodies, often accompanied by lower-titer LGI1 antibodies. CASPR2 and LGI1 antibodies bind to multiple brain regions, which helps to explain the multifocal clinical features of this disease, but other antibodies are likely to play a role in some patients and need to be characterized in future studies.

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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%

Morvan Syndrome

Maskery

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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Fasciculations, Autonomic Symptoms and Limbic Encephalitis: A Thymoma-Associated Morvan’s-Like Syndrome

Cited by 13 publications

References 12 publications

Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction

Potassium channel antibody-associated encephalitis with hypothalamic lesions and intestinal pseudo-obstruction

Morvan syndrome: Clinical and serological observations in 29 cases

Morvan Syndrome

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