Kurokawa T, Chen Y J, Nagata M, Hasuo K, Kobayashi T, Kitaguchi T
Department of Pediatrics, Faculty of Medicine, Kyushu University, Fukuoka, Japan.
Neuropediatrics. 1987 Aug;18(3):182-3. doi: 10.1055/s-2008-1052476.
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.
一名日本女孩在13个月大时发育出现恶化。16个月大时，出现轻度痉挛性双瘫，脑脊液蛋白增加至61.5mg/dl，半乳糖神经酰胺酶I缺乏。磁共振成像（MRI）显示半卵圆中心T2加权像信号强度增高。短潜伏期体感诱发电位（SSEPs）显示N13 - N20峰间潜伏期延长，随后N20消失。脑干听觉诱发电位（BAEPs）通常显示I - V波峰间潜伏期延长。这可能是我们认为的伴有MRI和诱发电位检查的晚发性婴儿型克拉伯病的首例报告。
