Progressive axonal dysfunction and clinical impairment in amyotrophic lateral sclerosis

Highlights ► Initial 12-week changes in axonal excitability in ALS patients suggest increasing K+ channel dysfunction in motor axons. ► Activity-dependent hyperpolarization contributes to longitudinal abnormalities in ALS patients. ► Changes in axonal excitability correlate with clinical assessments...

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Bibliographic Details
Published in:Clinical neurophysiology 2012-12, Vol.123 (12), p.2460-2467
Main Authors: Cheah, Benjamin C, Lin, Cindy S.Y, Park, Susanna B, Vucic, Steve, Krishnan, Arun V, Kiernan, Matthew C
Format: Article
Language:English
Subjects:
Action Potentials - physiology
Amyotrophic lateral sclerosis
Amyotrophic Lateral Sclerosis - physiopathology
Axons - physiology
Biological and medical sciences
Biomarker
Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases
Disease Progression
Electrodiagnosis. Electric activity recording
Female
Follow-Up Studies
Humans
Investigative techniques, diagnostic techniques (general aspects)
Longitudinal Studies
Male
Medical sciences
Middle Aged
Motor Neurons - physiology
Nerve Degeneration - physiopathology
Nerve excitability
Nervous system
Neurology
Neurophysiology
Potassium Channels - physiology
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Summary:Highlights ► Initial 12-week changes in axonal excitability in ALS patients suggest increasing K+ channel dysfunction in motor axons. ► Activity-dependent hyperpolarization contributes to longitudinal abnormalities in ALS patients. ► Changes in axonal excitability correlate with clinical assessments that demonstrate functional decline. ► Axonal excitability studies may be sensitive to identify progressive dysfunction of surviving axons in ALS patients.
ISSN:1388-2457
1872-8952
DOI:10.1016/j.clinph.2012.06.020