Neurologic Manifestations of von Recklinghausen's Disease of the Nervous System (Part 1 of 2)

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Abstract
The neurologic manifestations occurring in a series of 92 patients with von Recklinghausen''s neurofibromatosis are studied in an effort to learn more about the disease since the "text book" description is usually incomplete and past and current literature consists for the most part of case reports. The frequency of occurrence of neurologic manifestations is higher than generally reported, with males being affected twice as often as females. Symptomatic tumors occurred in 31 patients. Monosymptomatic (solitary) tumors which were amenable to surgical treatment occurred with greater frequency than generally appreciated. All degrees of cutaneous manifestations may be present and frequently minimal cutaneous stigmata are associated with widespread central nervous system involvement. Convulsions, mental retardation and radicular pain syndromes occur with sufficient frequency to be considered separately. They may occur independently of demonstrable CNS [central nervous system] tumors. Many "rare" features of neurofibromatosis which are subjects of case reports occur in a single modest series, i.e., intraspinal ganglioneuromas, Schwannoma of hypoglossal nerve, prococious puberty and sacral meningocele.