Interspike interval analysis in a patient with peripheral nerve hyperexcitability and potassium channel antibodies

2008 ◽  
Vol 37 (2) ◽  
pp. 269-274 ◽  
Author(s):  
Bert U. Kleine ◽  
Dick F. Stegeman ◽  
Gea Drost ◽  
Machiel J. Zwarts
Keyword(s):  
Peripheral Nerve ◽  
Potassium Channel ◽  
Interval Analysis ◽  
Interspike Interval ◽  
Peripheral Nerve Hyperexcitability

Differential behaviour of Kv7.2 (KCNQ2) potassium channel mutants in neonatal epilepsy and peripheral nerve hyperexcitability

2007 ◽  
Vol 34 (S 2) ◽  
Author(s):  
TV Wuttke ◽  
F Lehmann-Horn ◽  
W Paulus ◽  
J Penzien ◽  
K Jurkat-Rott ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Potassium Channel ◽  
Peripheral Nerve Hyperexcitability ◽  
Differential Behaviour

CLINICAL SPECTRUM OF MORVAN SYNDROME: A SINGLE CENTRE STUDY

2021 ◽  
pp. 22-23
Author(s):  
Jagiasi Kamlesh A ◽  
Bagul Abhay S
Keyword(s):  
Central Nervous System ◽  
Potassium Channel ◽  
Strong Association ◽  
Autoimmune Disorder ◽  
Response To Therapy ◽  
Review Of Literature ◽  
Single Centre ◽  
Voltage Gated ◽  
Single Centre Study ◽  
Peripheral Nerve Hyperexcitability

INTRODUCTION: Morvan syndrome (MoS) is a rare autoimmune disorder characterised by peripheral nerve hyperexcitability, autonomic dysfunction and central nervous system symptoms. It has strong association with autoantibodies to voltage gated potassium channel complex. This was a prospective observationa METHODS: l study. We reported 6 cases of Morvan syndrome. Clinical features, investigations, treatment and outcome were described and review of literature was done. All RESULT: subjects were male. Myokymia and insomnia were present in all patients and no patient had seizure. Voltage gated potassium channel (VGKC) antibody was positive in 66% (n=4) of subjects, all four were positive for CASPER2 and 2 were also weakly positive for LGI1. All patients were treated with immunomodulation and we found good response to therapy. Pain was treated with carbamazepine and phenytoin. CONCLUSION: Morvan syndrome, a VGKC antibody spectrum disorder, is a clinical diagnosis. We found myokymia and insomnia in all patients and no patient had seizure. In our study, response to immunotherapy was good. Awareness about MoS is necessary, as it can be treated successfully.

scholarly journals A Patient with Double-Negative VGKC, Peripheral Nerve Hyperexcitability, and Central Nervous System Symptoms: A Postinfectious Autoimmune Disease

2020 ◽  
Vol 2020 ◽  
pp. 1-7
Author(s):  
Birte Eikeland
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Autoimmune Disease ◽  
Peripheral Nerve ◽  
Skin Lesions ◽  
Double Negative ◽  
Pathogenic Potential ◽  
Peripheral Nerve Hyperexcitability ◽  
Systemic Symptoms ◽  
Kinetics Of

Research in the last few years has indicated that most voltage-gated potassium channel- (VGKC-) complex antibodies without leucine-rich glioma-inactivated protein 1 or contactin-associated protein-like 2 antibody specificity lack pathogenic potential and are not clear markers for autoimmune inflammation. Here we report on a patient with double-negative VGKC who developed severe peripheral nerve hyperexcitability, central nervous system symptoms with agitation and insomnia, dysautonomia, and systemic symptoms with weight loss, itch, and skin lesions. The disease started acutely one month after an episode of enteroviral pericarditis and responded well to immunotherapy. The patient is presumed to have developed a postinfectious immunotherapy-responsive autoimmune disease. In the setting of anti-VGKC positivity, it seems likely that anti-VGKC contributed to the pathogenesis of the patient’s symptoms of nerve hyperexcitability and that the disease was caused by an acquired autoimmune effect on the neuronal kinetics of VGKC. It is still unknown whether or not there are unidentified extracellular molecular targets within the VGKC-complex, i.e., a novel surface antigen and a pathogenic antibody that can cause affected individuals to develop a peripheral nerve hyperexcitability syndrome. This case highlights the fact that less well-characterized autoimmune central and peripheral nervous system syndromes may have infectious triggers.

THUR 153 Antibodies against the voltage-gated potassium channel complex

2018 ◽  
Vol 89 (10) ◽  
pp. A18.2-A18
Author(s):  
Joyutpal Das ◽  
Vanisha Chauhan ◽  
Ryan Keh ◽  
Daniel Mills ◽  
Johal Nicholas ◽  
...  
Keyword(s):  
Potassium Channel ◽  
Intravenous Immunoglobulins ◽  
Voltage Gated ◽  
Morvan’S Syndrome ◽  
Classic Phenotype ◽  
Antibody Titres ◽  
Peripheral Nerve Hyperexcitability ◽  
Clinical Phenotyping ◽  
Positive Results ◽  
Autoimmune Encephalopathy

Voltage-gated potassium channel (VGKC) complex antibodies have been associated with a spectrum of presentations including peripheral nerve hyperexcitability (PNH), Morvan’s syndrome, autoimmune encephalopathy, epilepsy and recently psychosis.We retrospectively reviewed the medical records of 70 patients from the Greater Manchester Neuroscience Centre, who had tested positive for VGKC-complex antibodies between 2012 and 2015 to identify the clinical relevance of positive results.The majority were diagnosed with autoimmune encephalopathy(19) followed by epilepsy(14), psychosis(10) and PNH(6). The remaining fifteen had other neurological presentations and six had no primary neurological disorder. 39/70 patients who had antibody titres>400 pM, were diagnosed with autoimmune encephalopathy(19), epilepsy(9), psychosis(4), PNH(3) and other disorders(4). 24/39 patients, who received treatment with one or a combination of corticosteroids, intravenous immunoglobulins, cyclophosphamide, plasma exchange, azathioprine or rituximab, had a diagnosis of autoimmune encephalopathy(18), epilepsy(2), psychosis(2) and malignancy(2). 16/24 were treatment responsive. 3/31 patients with lower titres were also treated, but only one with the classic phenotype (PNH) responded to treatment.The classic phenotype often had a titre >400 pM. PNH may have a titre ≤400 pM. The patients without classic presentations typically had titres≤400 pM. Consistent with previous studies, clinical phenotyping and antibody titre helped to determine the relevance of VGKC-complex antibodies.

scholarly journals Insomnia and Dysautonomia with Contactin-Associated Protein 2 and Leucine-Rich Glioma Inactivated Protein 1 Antibodies: A “Forme Fruste” of Morvan Syndrome?

2019 ◽  
Vol 11 (1) ◽  
pp. 80-86
Author(s):  
Ezgi Bakırcıoğlu-Duman ◽  
Zeynep Acar ◽  
Gülçin Benbir ◽  
Hande Yüceer ◽  
Hürtan Acar ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Neuropsychological Assessment ◽  
Executive Dysfunction ◽  
Cognitive Symptoms ◽  
Neurologic Examination ◽  
Cognitive Domains ◽  
Needle Electromyography ◽  
Muscle Twitching ◽  
Peripheral Nerve Hyperexcitability ◽  
History Of

Morvan syndrome (MoS) is typically characterized by neuromyotonia, sleep dysfunction, dysautonomia, and cognitive dysfunction. However, MoS patients with mild peripheral nerve hyperexcitability (PNH) or encephalopathy features have been described. A 46-year-old woman presented with a 2-month history of constipation, hyperhidrosis, and insomnia. Neurologic examination revealed muscle twitching and needle electromyography showed myokymic discharges in all limbs. No clinical or electrophysiological features of neuromyotonia were present. Although the patient denied any cognitive symptoms, neuropsychological assessment revealed executive dysfunction, while other cognitive domains were preserved. Cranial and spinal MRIs were unrevealing and tumor investigation proved negative. Polysomnography examination revealed total insomnia, which was partially reversed upon immune-modulatory therapy. Investigation of a broad panel of antibodies revealed serum leucine-rich glioma inactivated protein 1 and contactin-associated protein 2 antibodies. The features of this case indicate that the presentation of PNH syndromes may show significant variability and that MoS patients may not necessarily exhibit full-scale PNH and encephalopathy symptoms.

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