Neuropathies compressives du nerf ulnaire au poignet (et dans la main) et du nerf fibulaire commun. Données récentes

Rare Case of C4 Perineurinoma with Lambdatic Parieto-occipital Neuroma in Von Recklinghausen Disease in a 5 Years Old Child: A Case Report

“…The male/female ratio is 47/50. The nerves affected were, in order of frequency, the trunk of the sciatic nerve in 28 cases, then the common fibular in 8 cases [6]. In the series by M.L.…”

“…He is a carrier of congenital neurofibromatosis; he presents the cardinal signs of neurofibromatosis typ1. Although biological tests have not been carried out, he has had VON Recklinghausen disease [2, 5,6].…”

“…Intra neural perineuroma is a rather rare tumour, which was considered by some authors as a neurofibroma before being considered as a perineurinoma by H. Mitsumoto et al [10], who had the honour of being the first to have attributed to a perineu rioma, previously defined by S.S. Lazarus and L.D. Trombetta [11], the origin of certain cases of localised hypertrophic neuropathy, the perineurinoma representing about 1% of peripheral nerve tumours but with very particular clinical, electrophysiological and radiological characteristics [6,[10][11][12], the evolution is very slow, with a clear predominance of motor involvement, and the absence of extension to adjacent nerve trunks during followup [12]. Of 97 cases reported in the literature by P. Bouche [6].…”

“…Trombetta [11], the origin of certain cases of localised hypertrophic neuropathy, the perineurinoma representing about 1% of peripheral nerve tumours but with very particular clinical, electrophysiological and radiological characteristics [6,[10][11][12], the evolution is very slow, with a clear predominance of motor involvement, and the absence of extension to adjacent nerve trunks during followup [12]. Of 97 cases reported in the literature by P. Bouche [6].…”

“…Their size is greater than 0.5 cm in childhood and at least 1.5 cm after puberty have diagnostic value provided they are more than 6 in number.  Lentigines have the appearance of a small café au lait spot, and correspond to macules of 1 to 3 cm in diameter located preferentially in the axillary, inguinal and sub-mammary can be due to a localized form of generalized hypertrophic neuropathy or to an intranervous tumour, in this case a perineurioma, a benign intranervous tumour developed from the perineural cells of the nerve [3,6].…”