Altered axonal excitability properties in juvenile muscular atrophy of distal upper extremity (Hirayama disease)

Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology
Setsu SawaiSatoshi Kuwabara

Abstract

Juvenile muscular atrophy of distal upper extremity (Hirayama disease) is characterized by juvenile-onset of asymmetric amyotrophy, but the pathophysiology has not been fully clarified. "Cold paresis", aggravation of muscle weakness with exposure to cold, is a characteristic feature of this disease. The aim of this study was to investigate changes in axonal excitability properties in Hirayama disease. Threshold tracking was used to measure strength-duration time constant (SDTC), threshold electrotonus, refractoriness, and supernormality in median motor axons at the wrist of 14 patients and 10 age-matched normal controls. The measurements were performed at room temperature and after cooling. Patients showed prolonged SDTC, fanning-out of threshold electrotonus curves, and increased refractoriness and supernormality. After cooling, there were similar changes in patients and normal subjects, compatible with axonal depolarization possibly due to paralysis of sodium-potassium pump. Motor axonal excitability in Hirayama disease were characterized by increased persistent sodium currents, and dysfunction of transient sodium and potassium channels possibly associated with prominent collateral sprouting in young individuals. Further stud...Continue Reading

References

Aug 1, 1987·Pflügers Archiv : European journal of physiology·J R Schwarz, G Eikhof
Mar 1, 1987·Journal of Neurology, Neurosurgery, and Psychiatry·K HirayamaK Arai
Feb 18, 1998·Muscle & Nerve·H BostockD Burke
Oct 19, 2000·Neuropathology : Official Journal of the Japanese Society of Neuropathology·K Hirayama
Dec 2, 2000·Brain : a Journal of Neurology·M C Kiernan, H Bostock
Apr 5, 2001·Brain : a Journal of Neurology·M C KiernanH Bostock
Aug 22, 2001·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·D BurkeH Bostock
Mar 5, 2003·Brain : a Journal of Neurology·Kazuaki KanaiTakamichi Hattori
May 1, 1963·Neurology·K HIRAYAMAS OKINAKA
Feb 26, 2005·Annals of Neurology·Matthew C KiernanHugh Bostock
Sep 26, 2006·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Noriko TamuraTakamichi Hattori
Mar 18, 2008·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Setsu SawaiSatoshi Kuwabara

❮ Previous
Next ❯

Citations

Mar 15, 2015·Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia·Emma FosterAron Hill
Jun 19, 2017·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·José Manuel MatamalaMatthew C Kiernan
May 27, 2021·Clinical Neurophysiology : Official Journal of the International Federation of Clinical Neurophysiology·Chaojun ZhengJianyuan Jiang

❮ Previous
Next ❯

Related Concepts

Related Feeds

Amyloid Lateral Sclerosis

Amyotrophic Lateral Sclerosis (ALS) is a progressive nervous system disease associated with the death of neurons that control voluntary muscles. Discover the latest research on ALS here.

ALS: Transposon de-silencing

Transposon silencing is a form of transcriptional gene silencing. These gene silencing mechanisms are impaired in Amyotrophic lateral sclerosis (ALS). Here are the latest discoveries pertaining to transposon silencing and this disease.

Related Papers

Pediatric Neurology
Takatoshi HosokawaYasushi Oosaki
Journal of Clinical Neuroscience : Official Journal of the Neurosurgical Society of Australasia
Hyun-Jung JungMyeong-Kyu Kim
© 2021 Meta ULC. All rights reserved