Literature DB >> 31307606

Peripheral nerve hyperexcitability.

Bashar Katirji1.   

Abstract

Neuromyotonic and myokymic discharges are abnormal electrical muscular discharges caused by ectopic discharges from motor axons and represent the hallmarks of peripheral nerve hyperexcitability. Neuromyotonic discharges are specific for peripheral nerve hyperexcitability syndromes, whereas myokymic discharges may occur either focally or in a more generalized fashion in many other peripheral nerve disorders. Isaacs syndrome and Morvan syndrome are rare acquired peripheral nerve hyperexcitability disorders that share common clinical features and are often associated with elevated voltage-gated potassium channel-complex antibodies. Central nervous system symptomatology is more common in Morvan syndrome, which also overlaps with limbic encephalitis. Cramp-fasciculation syndrome, a more common syndrome, may represent a milder form of peripheral nerve hyperexcitability. Peripheral nerve hyperexcitability syndromes should be distinguished from stiff person syndrome, myotonic disorders, and rippling muscle disease. When severe, Isaacs syndrome and Morvan syndrome may be disabling but often respond to membrane-stabilizing drugs and immunomodulatory treatments. The electrophysiologic features of these disorders are described.
Copyright © 2019 Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Cramp-fasciculation syndrome; Episodic ataxia; Isaacs syndrome; Morvan syndrome; Myokymia; Neuromyotonia; Peripheral nerve hyperexcitability

Year:  2019        PMID: 31307606     DOI: 10.1016/B978-0-444-64142-7.00054-0

Source DB:  PubMed          Journal:  Handb Clin Neurol        ISSN: 0072-9752


  2 in total

Review 1.  Guidelines on clinical presentation and management of nondystrophic myotonias.

Authors:  Bas C Stunnenberg; Samantha LoRusso; W David Arnold; Richard J Barohn; Stephen C Cannon; Bertrand Fontaine; Robert C Griggs; Michael G Hanna; Emma Matthews; Giovanni Meola; Valeria A Sansone; Jaya R Trivedi; Baziel G M van Engelen; Savine Vicart; Jeffrey M Statland
Journal:  Muscle Nerve       Date:  2020-05-27       Impact factor: 3.217

2.  Isaac Syndrome with Intractable Neuropathic Pain Features: A Case Report.

Authors:  Mustafa Al-Chalabi; Nicholas R DelCimmuto; Pratyush Pavan Devarasetty; Jayasai Jeyarajan; Blair N Baumle; Noor Pirzada
Journal:  Case Rep Neurol       Date:  2022-03-29
  2 in total

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