Longitudinal study of auditory brainstem response in leigh syndrome.

Full 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.

Author information

Author/s: Yoshinaga, H (H); Ogino, T (T); Endo, F (F); Yasuhara, S (S); Murakami, N (N); Ohtsuka, Y (Y); Oka, E (E);

Affiliation: Department of Child Neurology, Okayama University Medical School. Okayama, Japan. magenta(-atsign-)md.okayama-u.ac.jp

Journal and publication information

Publication Type: Case Reports; Comparative Study; Evaluation Studies; Journal Article

Journal: Neuropediatrics (Neuropediatrics), published in Germany. (Language: eng)

Reference: 2003-Apr; vol 34 (issue 2) : pp 81-6

Dates: Created 2003/05/30; Completed 2003/07/09; Revised 2008/01/16;

PMID: 12776229, status: MEDLINE (last retrieval date: 2/18/2009, IMS Date: )

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