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Brain Dev. 2010 Apr;32(4):338-41. doi: 10.1016/j.braindev.2009.11.005. Epub 2009 Dec 9.

A case of intraneural perineurioma presenting with monomelic atrophy in a child.

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Department of Child Neurology, National Center of Neurology and Psychiatry (NCNP), Masayuki Sasaki, 4-1-1 Ogawahigashi-cho, Kodaira, Tokyo 187-8551, Japan.


We report the case of an 11-year-old girl who developed slowly progressive atrophy of the left lower extremity. She suffered from mild dilated cardiomyopathy of unknown cause since 4years of age. When she was 7years old, her family noticed that her left extremity was thinner compared to the right one. Computed tomography showed atrophy and areas of low density in the left gluteus maximus, thigh, and calf muscles. The left sciatic nerve showed gadolinium enhancement on magnetic resonance imaging. A biopsy of the left sural nerve revealed pseudo-onion bulbs. Immunohistochemical staining was positive for epithelial membrane antigen and negative for S100 protein. Electron microscopy demonstrated myelinated or unmyelinated nerve fibers surrounded by concentric layers of perineurial cells. These results indicated intraneural perineurioma. The tumor was estimated at least from the nerve root to the ankle joint. The length of nerve involvement in this patient was the highest recorded in the literatures. Intraneural perineurioma is a very rare disorder, but is tend to be found in youth. This disorder should be considered when we see children with monomelic weakness and/or atrophy.

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