ArticleJournal of clinical neurology (Seoul, Korea)2012
Comparison between clinical disabilities and electrophysiological values in Charcot-Marie-Tooth 1A patients with PMP22 duplication.
Article in Journal of clinical neurology (Seoul, Korea), 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 10 papers.
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10 citing papers in PubMed, 25 citations in OpenAlex.
- A study concept of expeditious clinical enrollment for genetic modifier studies in Charcot-Marie-Tooth neuropathy 1A.Journal of the peripheral nervous system : JPNS · 2024Article
- New evidence for secondary axonal degeneration in demyelinating neuropathies.Neuroscience letters · 2021Review
- One PMP22/MPZ and Three MFN2/GDAP1 Concomitant Variants Occurred in a Cohort of 189 Chinese Charcot-Marie-Tooth Families.Frontiers in neurology · 2021Article
- Paternal gender specificity and mild phenotypes in Charcot-Marie-Tooth type 1A patients with de novo 17p12 rearrangements.Molecular genetics & genomic medicine · 2020Article
- [A genotyping study of 13 cases of early-onset Charcot-Marie-Tooth disease].Zhongguo dang dai er ke za zhi = Chinese journal of contemporary pediatrics · 2019Article
- Nine-hole Peg Test and Ten-meter Walk Test for Evaluating Functional Loss in Chinese Charcot-Marie-Tooth Disease.Chinese medical journal · 2017Article
- Clinical and Pathological Variation of Charcot-Marie-Tooth 1A in a Large Chinese Cohort.BioMed research international · 2017Article
- Studying cerebellar dysfunction in neuropathy-related tremor.Clinical neurophysiology : official journal of the International Federation of Clinical Neurophysiology · 2015Article
- Severe phenotypes in a Charcot-Marie-Tooth 1A patient with PMP22 triplication.Journal of human genetics · 2015Article
- Segregation analysis in families with Charcot-Marie-Tooth disease allows reclassification of putative disease causing mutations.BMC medical genetics · 2014Article
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31 authors at 20 institutions in 1 country.
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Abstract
background and purposeCharcot-Marie-Tooth disease (CMT) type 1A (CMT1A) is the demyelinating form of CMT that is significantly associated with PMP22 duplication. Some studies have found that the disease-related disabilities of these patients are correlated with their compound muscle action potentials (CMAPs), while others have suggested that they are related to the nerve conduction velocities. In the present study, we investigated the correlations between the disease-related disabilities and the electrophysiological values in a large cohort of Korean CMT1A patients.
methodsWe analyzed 167 CMT1A patients of Korean origin with PMP22 duplication using clinical and electrophysiological assessments, including the CMT neuropathy score and the functional disability scale.
resultsClinical motor disabilities were significantly correlated with the CMAPs but not the motor nerve conduction velocities (MNCVs). Moreover, the observed sensory impairments matched the corresponding reductions in the sensory nerve action potentials (SNAPs) but not with slowing of the sensory nerve conduction velocities (SNCVs). In addition, CMAPs were strongly correlated with the disease duration but not with the age at onset. The terminal latency index did not differ between CMT1A patients and healthy controls.
conclusionsIn CMT1A patients, disease-related disabilities such as muscle wasting and sensory impairment were strongly correlated with CMAPs and SNAPs but not with the MNCVs or SNCVs. Therefore, we suggest that the clinical disabilities of CMT patients are determined by the extent of axonal dysfunction.
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