On the Pathogenesis of Charcot-Marie-Tooth Disease<i>A Study of the Sensory and Motor Conduction Velocity in the Median Nerve</i>

Nielsen, Viggo Kamp; Pilgaard, S

doi:10.3109/17453677208988658

Cited by 11 publications

(5 citation statements)

References 15 publications

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“…Evidence against the axon progressively dwindling in size derives from the lack of any correlation between conduction velocity and age found in the present series. Nielsen and Pilgaard (1972), in fact, reported a positive correlation between conduction velocity and age. As emphasized by the latter authors, this is of interest as conduction velocity normally falls slightly with age (Wagman and Lesse, 1952).…”

Section: Discussionmentioning

confidence: 91%

“…The explanation in the dominantly inherited disorders, and in the recessively inherited disorders where the clinical features correspond to those of peroneal muscu-lar atrophy, is unknown. In the five families with dominantly inherited Charcot-Marie-Tooth disease studied by Nielsen and Pilgaard (1972), the average values for motor and sensory conduction velocity in the median nerve were 23-6 and 19-8 m/sec respectively. They concluded that as these values were similar to those normally found at birth, the pathological process is likely to involve an impaired post-natal myelination of nerve fibres.…”

Section: Discussionmentioning

confidence: 95%

“…No supporting evidence for a progressive reduction in axon size has so far appeared. Measurements of motor nerve conduction velocity over periods of up to three years (Amick and Lemmi, 1963;Nielsen and Pilgaard, 1972) have shown no significant change, although this may be too short an interval for these very chronic disorders. Evidence against the axon progressively dwindling in size derives from the lack of any correlation between conduction velocity and age found in the present series.…”

Section: Discussionmentioning

confidence: 95%

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Motor nerve conduction velocity in peroneal muscular atrophy: evidence for genetic heterogeneity

Thomas

Calne

1974

Journal of Neurology, Neurosurgery & Psychiatry

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Section: Discussionmentioning

confidence: 91%

Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 95%

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Motor nerve conduction velocity in peroneal muscular atrophy: evidence for genetic heterogeneity

Thomas

Calne

1974

Journal of Neurology, Neurosurgery & Psychiatry

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“…Since the initial description, it has been studied by researchers to understand its etiology and pathophysiology [1][2][3][4][5]. However, the exact cause and mechanisms of the disease remain elusive [6].…”

Section: Introductionmentioning

confidence: 99%

Gait Pattern in Charcot-Marie-Tooth Disease Type 1A According to Disease Severity

Park,

Joo,

Choi

et al. 2023

JPM

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The aim of this study was to evaluate the characteristics of gait patterns in Charcot-Marie-Tooth disease type 1A (CMT1A) patients according to disease severity. Twenty-two CMT1A patients were enrolled and classified into two groups, according to the disease severity. The healthy control group consisted of 22 subjects with no gait impairment. Full barefoot three-dimensional gait analysis with temporospatial, kinematic, and kinetic data was performed among the mild and moderate CMT1A group and the control group. Minimal hip abduction, maximal hip extension generation, peak knee flexion moment at stance, ankle dorsiflexion at initial contact, maximal ankle plantarflexion at push-off and maximal ankle rotation moment at stance in the CMT1A group showed a significant difference compared to the control group (p < 0.05). In the moderate group, there were greater maximal hip flexion angles in swing, and smaller dorsiflexion angles at initial contact compared to the control group and mild group. CMT patients had typical gait characteristics and their gait patterns were different according to severity. The analysis of gait patterns in patients with CMT1A will help to understand gait function and provide important information for the treatment of patients with CMT in the future.

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“…Its causes and pathophysiological mechanism have been multilaterally researched so far1-5 but are still not clear 6. This disease is autosomally, dominantly inherited and a kind of peripheral neuropathy that causes progressive and symmetric motor and sensory disturbance in lower limbs, in which occurrence is focused on the lower limbs.…”

Section: Introductionmentioning

confidence: 99%

Foot Deformity in Charcot Marie Tooth Disease According to Disease Severity

et al. 2011

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ObjectiveTo investigate the characteristics of foot deformities in patients with Charcot-Marie-Tooth (CMT) disease compared with normal persons according to severity of disease.MethodSixty-two patients with CMT disease were recruited for this study. The normal control group was composed of 28 healthy people without any foot deformity. Patients were classified into a mild group and a moderate group according to the CMT neuropathy score. Ten typical radiological angles representing foot deformities such as pes equinus and pes varus were measured. The CMT group angles were compared with those of the normal control group, and those of the mild group were also compared with those of the moderate group.ResultsThe lateral (Lat.) talo-first metatarsal angle, anteroposterior talo-first metatarsal angle, Lat. calcaneal-first metatarsal angle, Lat. naviocuboid overlap, Lat. calcaneal pitch, Lat. tibiocalcaneal angle, and Lat. talocalcaneal angle in the CMT group showed a significant difference compared to the normal control group (p<0.05). These findings revealed CMT patients have pes cavus, forefoot adduction, midfoot supination and pes varus deformity. Compared to the mild group, the moderate group significantly showed an increased Lat. calcaneal pitch and decreased Lat. calcaneal-first metatarsal angle, Lat. tibiocalcaneal angle, Lat. talocalcaneal angle, and Lat. talo-first metatarsal angle (p<0.05). These findings revealed that the pes cavus deformity of CMT patients tend to be worse with disease severity.ConclusionThe characteristic equinovarus foot deformity patterns in CMT patients were revealed and these deformities tended to be worse with disease severity. Radiographic measures may be useful for the investigation of foot deformities in CMT patients.

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On the Pathogenesis of Charcot-Marie-Tooth DiseaseA Study of the Sensory and Motor Conduction Velocity in the Median Nerve

Cited by 11 publications

References 15 publications

Motor nerve conduction velocity in peroneal muscular atrophy: evidence for genetic heterogeneity

Motor nerve conduction velocity in peroneal muscular atrophy: evidence for genetic heterogeneity

Gait Pattern in Charcot-Marie-Tooth Disease Type 1A According to Disease Severity

Foot Deformity in Charcot Marie Tooth Disease According to Disease Severity

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