Longitudinal study of auditory brainstem response in Leigh syndrome

Harumi Yoshinaga, T. Ogino, Fumika Endoh, S. Yasuhara, N. Murakami, Y. Ohtsuka, E. Oka

Research output: Contribution to journalArticle

6 Citations (Scopus)

Abstract

To assess the utility of auditory brainstem response (ABR) in diagnosing brainstem changes in patients with Leigh syndrome (LS), we performed a longitudinal study of five patients with LS using both ABR and neuroimaging techniques (CT and MRI). The brainstem components of the initial ABRs we performed on the patients were abnormal in all five patients. In four of the patients, these abnormal findings preceded any clinical signs of brainstem impairment. Improvements in clinical findings were reflected in improvements in ABR findings in three patients. In one of these three patients, improvements in clinical findings were also reflected in improvements in MRI findings. In the other two patients, MRI findings showed no improvements, despite the improvements in clinical findings. In two of our patients, ABR clearly revealed functional improvements in the brainstem which were not revealed by MRI. Therefore, we conclude that ABR is an essential diagnostic technique for patients with LS.

Original languageEnglish
Pages (from-to)81-86
Number of pages6
JournalNeuropediatrics
Volume34
Issue number2
DOIs
Publication statusPublished - Apr 2003

Fingerprint

Leigh Disease
Brain Stem Auditory Evoked Potentials
Longitudinal Studies
Brain Stem
Neuroimaging

Keywords

  • ABR
  • DCA
  • Leigh syndrome
  • MRI
  • PLEDs

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

Cite this

Yoshinaga, H., Ogino, T., Endoh, F., Yasuhara, S., Murakami, N., Ohtsuka, Y., & Oka, E. (2003). Longitudinal study of auditory brainstem response in Leigh syndrome. Neuropediatrics, 34(2), 81-86. https://doi.org/10.1055/s-2003-39603

Longitudinal study of auditory brainstem response in Leigh syndrome. / Yoshinaga, Harumi; Ogino, T.; Endoh, Fumika; Yasuhara, S.; Murakami, N.; Ohtsuka, Y.; Oka, E.

In: Neuropediatrics, Vol. 34, No. 2, 04.2003, p. 81-86.

Research output: Contribution to journalArticle

Yoshinaga, H, Ogino, T, Endoh, F, Yasuhara, S, Murakami, N, Ohtsuka, Y & Oka, E 2003, 'Longitudinal study of auditory brainstem response in Leigh syndrome', Neuropediatrics, vol. 34, no. 2, pp. 81-86. https://doi.org/10.1055/s-2003-39603
Yoshinaga H, Ogino T, Endoh F, Yasuhara S, Murakami N, Ohtsuka Y et al. Longitudinal study of auditory brainstem response in Leigh syndrome. Neuropediatrics. 2003 Apr;34(2):81-86. https://doi.org/10.1055/s-2003-39603
Yoshinaga, Harumi ; Ogino, T. ; Endoh, Fumika ; Yasuhara, S. ; Murakami, N. ; Ohtsuka, Y. ; Oka, E. / Longitudinal study of auditory brainstem response in Leigh syndrome. In: Neuropediatrics. 2003 ; Vol. 34, No. 2. pp. 81-86.
@article{e893dd05549148dcacbeed10fe33f45d,
title = "Longitudinal study of auditory brainstem response in Leigh syndrome",
abstract = "To assess the utility of auditory brainstem response (ABR) in diagnosing brainstem changes in patients with Leigh syndrome (LS), we performed a longitudinal study of five patients with LS using both ABR and neuroimaging techniques (CT and MRI). The brainstem components of the initial ABRs we performed on the patients were abnormal in all five patients. In four of the patients, these abnormal findings preceded any clinical signs of brainstem impairment. Improvements in clinical findings were reflected in improvements in ABR findings in three patients. In one of these three patients, improvements in clinical findings were also reflected in improvements in MRI findings. In the other two patients, MRI findings showed no improvements, despite the improvements in clinical findings. In two of our patients, ABR clearly revealed functional improvements in the brainstem which were not revealed by MRI. Therefore, we conclude that ABR is an essential diagnostic technique for patients with LS.",
keywords = "ABR, DCA, Leigh syndrome, MRI, PLEDs",
author = "Harumi Yoshinaga and T. Ogino and Fumika Endoh and S. Yasuhara and N. Murakami and Y. Ohtsuka and E. Oka",
year = "2003",
month = "4",
doi = "10.1055/s-2003-39603",
language = "English",
volume = "34",
pages = "81--86",
journal = "Neuropediatrics",
issn = "0174-304X",
publisher = "Hippokrates Verlag GmbH",
number = "2",

}

TY - JOUR

T1 - Longitudinal study of auditory brainstem response in Leigh syndrome

AU - Yoshinaga, Harumi

AU - Ogino, T.

AU - Endoh, Fumika

AU - Yasuhara, S.

AU - Murakami, N.

AU - Ohtsuka, Y.

AU - Oka, E.

PY - 2003/4

Y1 - 2003/4

N2 - To assess the utility of auditory brainstem response (ABR) in diagnosing brainstem changes in patients with Leigh syndrome (LS), we performed a longitudinal study of five patients with LS using both ABR and neuroimaging techniques (CT and MRI). The brainstem components of the initial ABRs we performed on the patients were abnormal in all five patients. In four of the patients, these abnormal findings preceded any clinical signs of brainstem impairment. Improvements in clinical findings were reflected in improvements in ABR findings in three patients. In one of these three patients, improvements in clinical findings were also reflected in improvements in MRI findings. In the other two patients, MRI findings showed no improvements, despite the improvements in clinical findings. In two of our patients, ABR clearly revealed functional improvements in the brainstem which were not revealed by MRI. Therefore, we conclude that ABR is an essential diagnostic technique for patients with LS.

AB - To assess the utility of auditory brainstem response (ABR) in diagnosing brainstem changes in patients with Leigh syndrome (LS), we performed a longitudinal study of five patients with LS using both ABR and neuroimaging techniques (CT and MRI). The brainstem components of the initial ABRs we performed on the patients were abnormal in all five patients. In four of the patients, these abnormal findings preceded any clinical signs of brainstem impairment. Improvements in clinical findings were reflected in improvements in ABR findings in three patients. In one of these three patients, improvements in clinical findings were also reflected in improvements in MRI findings. In the other two patients, MRI findings showed no improvements, despite the improvements in clinical findings. In two of our patients, ABR clearly revealed functional improvements in the brainstem which were not revealed by MRI. Therefore, we conclude that ABR is an essential diagnostic technique for patients with LS.

KW - ABR

KW - DCA

KW - Leigh syndrome

KW - MRI

KW - PLEDs

UR - http://www.scopus.com/inward/record.url?scp=0037911579&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=0037911579&partnerID=8YFLogxK

U2 - 10.1055/s-2003-39603

DO - 10.1055/s-2003-39603

M3 - Article

C2 - 12776229

AN - SCOPUS:0037911579

VL - 34

SP - 81

EP - 86

JO - Neuropediatrics

JF - Neuropediatrics

SN - 0174-304X

IS - 2

ER -