Utility of stimulus induced after discharges in the evaluation of peripheral nerve hyperexcitability: Old wine in a new bottle?

2020 ◽  
Author(s):  
Aditya Vijayakrishnan Nair ◽  
Arun Mani ◽  
Asish Vijayaraghavan ◽  
Pullumpallil Alexander ◽  
Atif Shaikh ◽  
...  
Keyword(s):  
Peripheral Nerve ◽  
Peripheral Nerve Hyperexcitability

Variants and Differential Diagnosis of Peripheral Nerve Hyperexcitability

2006 ◽  
Vol 37 (01) ◽  
Author(s):  
HJ Gdynia ◽  
AC Ludolph ◽  
AD Sperfeld
Keyword(s):  
Differential Diagnosis ◽  
Peripheral Nerve ◽  
Peripheral Nerve Hyperexcitability

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.

Therapeutic Implications of Peripheral Nerve Hyperexcitability in Muscle Cramping

2016 ◽  
Vol 33 (6) ◽  
pp. 560-563 ◽  
Author(s):  
Rebecca L. Hurst ◽  
Lisa D. Hobson-Webb
Keyword(s):  
Peripheral Nerve ◽  
Therapeutic Implications ◽  
Peripheral Nerve Hyperexcitability

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.

SY7.4 Autoimmune Peripheral Nerve Hyperexcitability Syndromes

2009 ◽  
Vol 120 ◽  
pp. S11
Author(s):  
Kerry R. Mills
Keyword(s):  
Peripheral Nerve ◽  
Peripheral Nerve Hyperexcitability

scholarly journals A Mouse Model of Schwartz-Jampel Syndrome Reveals Myelinating Schwann Cell Dysfunction with Persistent Axonal Depolarization in Vitro and Distal Peripheral Nerve Hyperexcitability When Perlecan Is Lacking

2012 ◽  
Vol 180 (5) ◽  
pp. 2040-2055 ◽  
Author(s):  
Marie Bangratz ◽  
Nadège Sarrazin ◽  
Jérôme Devaux ◽  
Désirée Zambroni ◽  
Andoni Echaniz-Laguna ◽  
...  
Keyword(s):  
Schwann Cell ◽  
Mouse Model ◽  
Peripheral Nerve ◽  
Cell Dysfunction ◽  
Peripheral Nerve Hyperexcitability ◽  
Myelinating Schwann Cell

Clinical electrophysiological characterization of the acquired neuromyotonia phenotype of autoimmune peripheral nerve hyperexcitability

2006 ◽  
Vol 33 (6) ◽  
pp. 801-808 ◽  
Author(s):  
Paul Maddison ◽  
Kerry R. Mills ◽  
John Newsom-Davis
Keyword(s):  
Peripheral Nerve ◽  
Peripheral Nerve Hyperexcitability ◽  
Electrophysiological Characterization

PERIPHERAL NERVE HYPEREXCITABILITY WITH ANTI–VGKC ANTIBODIES PRESENTING AS MUSICIAN'S DYSTONIA

2013 ◽  
Vol 84 (11) ◽  
pp. e2.53-e2
Author(s):  
Christopher Kobylecki ◽  
Andrew Marshall ◽  
Jon Sussman ◽  
Hisham Hamdalla
Keyword(s):  
Peripheral Nerve ◽  
Peripheral Nerve Hyperexcitability
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