Multimodality evoked potentials in progression of metachromatic leukodystrophy |
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Authors: | H Takakura C Nakano S Kasagi S Takashima K Takeshita |
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Institution: | 1. Shimane Seishigakuen Hospital, Goutsu-shi;2. Division of Child Neurology, Institute of Neurological Sciences, Tottori University, Yonago;3. Department of Pediatrics, Matsue National Hospital, Matsue |
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Abstract: | A 2-yr-3-mo-old girl with metachromatic leukodystrophy (MLD) was examined using serial multiple electrophysiological procedures. Sensory nerve conduction velocity was delayed earlier and more severely than motor nerve conduction velocity. Visual evoked potentials (VEPs) showed prolonged latency of wave IV. Auditory brainstem responses (ABRs) showed prolonged latency of waves I and V, and the I-V interval. As to the interpeak latency of somatosensory evoked potentials (SEPs), the P9-P14 and the P14-N20 intervals were prolonged on admission. Two months later, both intervals were more prolonged, but the prolongation of the P9-P14 interval was the most prominent. The demyelination in our case may have started in the cerebral white matter, progressed to the peripheral nerves, and at last via the spinal root reached the brainstem. An electrophysiological follow-up study may be valuable in the understanding of the progressive pathological changes and in the evaluation of therapeutic measures. |
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Keywords: | Metachromatic leukodystrophy visual evoked potential (VEP) auditory brainstem response (ABR) somatosensory evoked potential (SEP) short latency somatosensory evoked potential (S·SEP) |
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