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KMID : 0391520100180020326
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Journal of the Korean Child Neurology Society
2010 Volume.18 No. 2 p.326 ~ p.331
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A Case of Primary Intraspinal Peripheral Primitive Neuroectodermal Tumor (pPNET) Mimicking Guillain-Barre Syndrome
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Lee Jeong-Yong
Lee Eun-Hye
Jeong Min-Hee
Yum Mi-Sun
Im Ho-Joon
Seo Jong-Jin
Ra Young-Shin
Ko Tae-Sung
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Abstract
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Primary intraspinal peripheral primitive neuroectodermal tumors (pPNET) are extremely rare. We report a 10-month-old girl presented with rapidly progressive paraparesis and raised cerebrospinal fluid protein. A magnetic resonance image demonstrated an intramedullary mass from C3 to T3 level and intradural extramedullary nodules in the lumbosacral area. Surgery was performed with partial tumor removal and histologic examination revealed a small round cell tumor and immunohistochemical characteristics of pPNET. She died due to tumor progression at four months after initial diagnosis. This case has been reported to raise awareness among clinicians to include the possibility of intraspinal tumors in the differential diagnosis of progressive neurological deficits mimicking Guillain-Barre syndrome.
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KEYWORD
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Guillain-Barre Syndrome, Neuroectodermal Tumors, Primitive, Peripheral
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