Middle latency auditory-evoked potentials in myotonic dystrophy: Relation to the size of the CTG trinucleotide repeat and intelligent quotient

Kenji Arakawa, Hideaki Tomi, Shozo Tobimatsu, Jun Ichi Kira

Research output: Contribution to journalArticle

11 Citations (Scopus)

Abstract

Objective: Major components of MLAEPs are thought to originate in the temporal lobe. Absence of the Pb potential has been demonstrated in MLAEPs in Alzheimer's disease and demented Parkinson's disease patients. To validate usefulness of middle latency auditory-evoked potentials (MLAEPs) in evaluating the central nervous system (CNS) involvement of myotonic dystrophy (MyD). Methods: MLAEPs were recorded in eight patients with MyD and nine normal control subjects. In the patient group, the size of the CTG triplet repeat expansion within the dystrophia myotonica protein kinase (DMPK) gene and the revised Wechsler Adult Intelligence Scale (WAIS-R) were also assessed. Results: The latency of the Nb potential showed a significant correlation with the size of the CTG repeat expansion (r=0.734, P=0.036). The Pb latency also tended to prolong according to CTG amplification (r=0.644, P=0.087). The amplitudes of Na and Pa significantly increased compared with those of normal control subjects (P=0.024 and 0.016, respectively). However, they did not correlate with IQ or CTG amplification. Conclusions: Abnormal MLAEPs may indicate CNS involvement in MyD. Although the precise generating mechanisms of Nb are unclear, the correlation of Nb latency with CTG amplification suggests that MLAEPs can reflect the extent of genetic abnormality.

Original languageEnglish
Pages (from-to)31-36
Number of pages6
JournalJournal of the Neurological Sciences
Volume207
Issue number1-2
DOIs
Publication statusPublished - Mar 15 2003

Fingerprint

Trinucleotide Repeats
Auditory Evoked Potentials
Myotonic Dystrophy
Central Nervous System
Temporal Lobe
Intelligence
Protein Kinases
Parkinson Disease
Alzheimer Disease
Genes

All Science Journal Classification (ASJC) codes

  • Neurology
  • Clinical Neurology

Cite this

@article{f80a04e48436410289443553858f8b11,
title = "Middle latency auditory-evoked potentials in myotonic dystrophy: Relation to the size of the CTG trinucleotide repeat and intelligent quotient",
abstract = "Objective: Major components of MLAEPs are thought to originate in the temporal lobe. Absence of the Pb potential has been demonstrated in MLAEPs in Alzheimer's disease and demented Parkinson's disease patients. To validate usefulness of middle latency auditory-evoked potentials (MLAEPs) in evaluating the central nervous system (CNS) involvement of myotonic dystrophy (MyD). Methods: MLAEPs were recorded in eight patients with MyD and nine normal control subjects. In the patient group, the size of the CTG triplet repeat expansion within the dystrophia myotonica protein kinase (DMPK) gene and the revised Wechsler Adult Intelligence Scale (WAIS-R) were also assessed. Results: The latency of the Nb potential showed a significant correlation with the size of the CTG repeat expansion (r=0.734, P=0.036). The Pb latency also tended to prolong according to CTG amplification (r=0.644, P=0.087). The amplitudes of Na and Pa significantly increased compared with those of normal control subjects (P=0.024 and 0.016, respectively). However, they did not correlate with IQ or CTG amplification. Conclusions: Abnormal MLAEPs may indicate CNS involvement in MyD. Although the precise generating mechanisms of Nb are unclear, the correlation of Nb latency with CTG amplification suggests that MLAEPs can reflect the extent of genetic abnormality.",
author = "Kenji Arakawa and Hideaki Tomi and Shozo Tobimatsu and Kira, {Jun Ichi}",
year = "2003",
month = "3",
day = "15",
doi = "10.1016/S0022-510X(02)00354-4",
language = "English",
volume = "207",
pages = "31--36",
journal = "Journal of the Neurological Sciences",
issn = "0022-510X",
publisher = "Elsevier",
number = "1-2",

}

TY - JOUR

T1 - Middle latency auditory-evoked potentials in myotonic dystrophy

T2 - Relation to the size of the CTG trinucleotide repeat and intelligent quotient

AU - Arakawa, Kenji

AU - Tomi, Hideaki

AU - Tobimatsu, Shozo

AU - Kira, Jun Ichi

PY - 2003/3/15

Y1 - 2003/3/15

N2 - Objective: Major components of MLAEPs are thought to originate in the temporal lobe. Absence of the Pb potential has been demonstrated in MLAEPs in Alzheimer's disease and demented Parkinson's disease patients. To validate usefulness of middle latency auditory-evoked potentials (MLAEPs) in evaluating the central nervous system (CNS) involvement of myotonic dystrophy (MyD). Methods: MLAEPs were recorded in eight patients with MyD and nine normal control subjects. In the patient group, the size of the CTG triplet repeat expansion within the dystrophia myotonica protein kinase (DMPK) gene and the revised Wechsler Adult Intelligence Scale (WAIS-R) were also assessed. Results: The latency of the Nb potential showed a significant correlation with the size of the CTG repeat expansion (r=0.734, P=0.036). The Pb latency also tended to prolong according to CTG amplification (r=0.644, P=0.087). The amplitudes of Na and Pa significantly increased compared with those of normal control subjects (P=0.024 and 0.016, respectively). However, they did not correlate with IQ or CTG amplification. Conclusions: Abnormal MLAEPs may indicate CNS involvement in MyD. Although the precise generating mechanisms of Nb are unclear, the correlation of Nb latency with CTG amplification suggests that MLAEPs can reflect the extent of genetic abnormality.

AB - Objective: Major components of MLAEPs are thought to originate in the temporal lobe. Absence of the Pb potential has been demonstrated in MLAEPs in Alzheimer's disease and demented Parkinson's disease patients. To validate usefulness of middle latency auditory-evoked potentials (MLAEPs) in evaluating the central nervous system (CNS) involvement of myotonic dystrophy (MyD). Methods: MLAEPs were recorded in eight patients with MyD and nine normal control subjects. In the patient group, the size of the CTG triplet repeat expansion within the dystrophia myotonica protein kinase (DMPK) gene and the revised Wechsler Adult Intelligence Scale (WAIS-R) were also assessed. Results: The latency of the Nb potential showed a significant correlation with the size of the CTG repeat expansion (r=0.734, P=0.036). The Pb latency also tended to prolong according to CTG amplification (r=0.644, P=0.087). The amplitudes of Na and Pa significantly increased compared with those of normal control subjects (P=0.024 and 0.016, respectively). However, they did not correlate with IQ or CTG amplification. Conclusions: Abnormal MLAEPs may indicate CNS involvement in MyD. Although the precise generating mechanisms of Nb are unclear, the correlation of Nb latency with CTG amplification suggests that MLAEPs can reflect the extent of genetic abnormality.

UR - http://www.scopus.com/inward/record.url?scp=12244311199&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=12244311199&partnerID=8YFLogxK

U2 - 10.1016/S0022-510X(02)00354-4

DO - 10.1016/S0022-510X(02)00354-4

M3 - Article

C2 - 12614928

AN - SCOPUS:12244311199

VL - 207

SP - 31

EP - 36

JO - Journal of the Neurological Sciences

JF - Journal of the Neurological Sciences

SN - 0022-510X

IS - 1-2

ER -