Evolution of nerve conduction abnormalities in children with dominant hypertrophic neuropathy of the charcot‐marie‐tooth type

Gutmann, Ludwig; Fakadej, Alexander V.; Riggs, Jack E.

doi:10.1002/mus.880060708

Cited by 56 publications

(26 citation statements)

References 6 publications

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“…The initial dysmyelination and the subsequent cycles of demyelination and remyelination lead to a kind of balance in myelination in the first decade of life. This is supported by human longitudinal electrophysiological studies showing that motor nerve conduction velocity, an electrophysiological marker of myelination, is already abnormal at 2 years of age [11,13], and remains stable from the age of 5 years onwards [20,33].…”

Section: Discussionmentioning

confidence: 74%

Clinical disease severity and axonal dysfunction in hereditary motor and sensory neuropathy Ia

et al. 2004

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Section: Discussionmentioning

confidence: 74%

Clinical disease severity and axonal dysfunction in hereditary motor and sensory neuropathy Ia

et al. 2004

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“…8 Few have reported longitudinal conduction studies in adolescents and adults with HMSNTwo of these reports noted no significant median nerve conduction velocity change in 4 HMSN-I patients for up to 36…”

Section: Discussionmentioning

confidence: 98%

Longitudinal conduction studies in hereditary motor and sensory neuropathy type 1

1989

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Motor conduction studies were performed serially in 10 patients, ages 10-62 years, with clinical and electrophysiological criteria of hereditary motor and sensory neuropathy type 1 (HMSN-1) over periods of 11-19 years. Median nerve conduction velocity (MNCV) and distal motor latency showed no significant change on serial studies. Mean median compound muscle action potential (CMAP) amplitude values, however, decreased 66% in 8 patients. Observed clinical progression in HMSN-1, over prolonged periods of time, was not associated with MNCV slowing. However, CMAP amplitude reduction, reflecting progressive axonal loss, correlated with clinical deterioration.

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“…The first neurophysiologic abnormality seen in CMT1A is prolongation of the DML ( Garcia et al, 1998; Ryan and Jones, 2004 ) . The DML increases progressively until the age of 10 and thereafter remains generally stable ( Gutmann et al, 1983 ) . In our study, there was a trend for DML to increase with age, but this did not reach statistical significance after DML values were corrected to a standard distance of 5 cm ( Slomic et al, 1968 ) .…”

Section: Discussionmentioning

confidence: 99%

Neurophysiologic abnormalities in children with Charcot‐Marie‐Tooth disease type 1A

Yiu

Burns

Ryan

et al. 2008

J Peripheral Nervous Sys

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Although Charcot-Marie-Tooth disease type 1A (CMT1A) initially manifests in the first decade, there are no large studies describing its neurophysiologic features in childhood. We report neurophysiologic findings in 80 children aged 2-16 years with CMT1A who underwent median motor and sensory nerve conduction studies. Neurophysiologic abnormalities were present in all children. Median motor nerve conduction velocity was invariably less than 33 m/s (mean 18.7 m/s, range 9.0-32.9 m/s), with conduction velocities significantly slower in children aged 7-16 years compared with children aged 6 years and below. All children had prolonged distal motor latencies (mean 7.3 ms, range 4.0-12.3 ms). The compound muscle action potential (CMAP) amplitude was reduced from an early age (mean 7.1 mV, range 2.1-13.5 mV), and its normal increase with age was attenuated. Median sensory responses were present in only seven children, all aged less than 9 years and with slowed sensory conduction. Neurophysiologic abnormalities are present in all children with CMT1A from the age of 2 years. Motor conduction slowing progresses through the first 6 years of life and thereafter remains stable. CMAP amplitude is reduced from an early age, and the normal physiologic increase with age is attenuated. Median sensory responses may be recorded in younger children, and their presence does not exclude the diagnosis of CMT1A.

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Evolution of nerve conduction abnormalities in children with dominant hypertrophic neuropathy of the charcot‐marie‐tooth type

Cited by 56 publications

References 6 publications

Clinical disease severity and axonal dysfunction in hereditary motor and sensory neuropathy Ia

Clinical disease severity and axonal dysfunction in hereditary motor and sensory neuropathy Ia

Longitudinal conduction studies in hereditary motor and sensory neuropathy type 1

Neurophysiologic abnormalities in children with Charcot‐Marie‐Tooth disease type 1A

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