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Neuropediatrics 1987; 18(3): 182-183
DOI: 10.1055/s-2008-1052476
© Georg Thieme Verlag KG Stuttgart · New York

Late Infantile Krabbe Leukodystrophy: MRI and Evoked Potentials in a Japanese Girl

T.  Kurokawa1 , Y. -J. Chen1 , M.  Nagata1 , K.  Hasuo2 , T.  Kobayashi3 , T.  Kitaguchi3
  • 1Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka 812, Japan
  • 2Department of Radiology, Faculty of Medicine, Kyushu University, Fukuoka 812, Japan
  • 3Department of Neurology, Faculty of Medicine, Kyushu University, Fukuoka 812, Japan
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Publication History

Publication Date:
19 March 2008 (online)

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Abstract

A Japanese girl showed deterioration in development from the age of 13 months. At the age of 16 months, there were mild spastic diplegia, increase in cerebrospinal fluid protein to 61.5 mg/dl and deficient galactosylceramidase I. Magnetic resonance imaging (MRI) demonstrated a high signal intensity with increased T2 in the centrum semiovale. Short latency somatosensory evoked potentials (SSEPs) showed a prolonged N13-N20 interpeak latency followed by abolition of N20. Brainstem auditory evoked potentials (BAEPs) were normally followed by prolonged interpeak latencies of wave I-V. This may be the first report of what we consider to be the late infantile form of Krabbe disease with MRI and evoked potential examinations.

Key words

Globoid cell leukodystrophy - Magnetic resonance imaging - Evoked potential

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