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Central form of Von Reklinghausen nervous disease

The authors present a systematization of the lesions seen in Recklinghausen's disease. Four cases of the nervous central type of the disease were studied. In all the cases there were bilateral pontine-cerebellar tumors, one of the most important findings for diagnostic purposes, which was the only manifestation of the disease in one of them (pure central form). In the others, with mixed types, besides various central lesions (neurofibromas, meningeomas) there were mild peripheral lesions. Intravertebral tumors orinating from radicular sheaths were the cause of the initial symptoms in three patients. In the two cases where the cerebral cortex was histologically studied, groups of atypical glia cells characteristic of the central type of neurofibromatosis were found. Two relatively rare lesions were found in the second case : cortical angioma and hypertrophic intersticial neuritis of the brachial plexus. In patients with peripheral or intravertebral neurofibromatosis, as well as in their relatives, central lesions must be sought for, especially tumors of the acoustic nerve. The neurological diagnostic procedure of choice in such tumors is the contrasted ventriculography with Lipiodol.


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