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This Article Full Text Full Text (PDF) CME: Take the course for this article: Volume 63, Number 4, August 24, 2004 Correspondence: Submit a response Alert me when this article is cited Alert me when Correspondence are posted Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Download to citation manager Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Husain, A. M. Articles by Aldosari, M. Search for Related Content PubMed PubMed Citation Articles by Husain, A. M. Articles by Aldosari, M. Related Collections MRI Leukodystrophies All Pediatric EMG

Krabbe disease

Neurophysiologic studies and MRI correlations

From the Departments of Medicine (Neurology) (Dr. Husain) and Pediatrics (Pediatric Neurology) (Dr. Aldosari), Duke University, and Neurodiagnostic Center (Dr. Husain), Veterans Affairs Medical Center, Durham, and School of Pharmacy (M. Altuwaijri), Campbell University, Buies Creek, NC.

Address correspondence and reprint requests to Dr. A.M. Husain, Duke University Medical Center, 202 Bell Bldg., Box 3678, Durham, NC 27710; e-mail: Aatif.Husain{at}duke.edu

Background: Krabbe disease (KD) is a rare hereditary leukodystrophy affecting children mostly in the first 6 months of life; later onset has been reported as well.

Objective: To review abnormalities in neurophysiologic studies in children with KD and determine if there is a correlation between these studies and disease severity as measured by MRI scans.

Methods: KD patients with at least one neurophysiologic study and one MRI scan at the authors’ institution were reviewed. Relationships between KD type, neurophysiologic studies, and severity of disease as measured by MRI were explored.

Results: Data were available for 26 children: 20 with early infantile KD (EIKD) and 6 with late-onset KD (LOKD). Flash visual evoked potentials were abnormal in 53% of EIKD children, whereas none of the LOKD children had an abnormal study. Brainstem auditory evoked potentials were abnormal in 88% of EIKD and 40% of LOKD children. EEGs were abnormal in 65% of EIKD and 33% of LOKD children. Nerve conduction studies were abnormal in all children with EIKD and in 20% of LOKD children. Abnormal neurophysiologic studies correlated with more extensive disease as measured by MRI scans.

Conclusions: Children with early infantile Krabbe disease and late-onset Krabbe disease have different patterns of abnormalities in neurophysiologic studies. These studies offer an objective means of assessing KD and correlate well with disease severity measured by MRI scans.

Received July 29, 2003. Accepted in final form March 22, 2004.

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