title: [Neurologic syndromes in children with phakomatosis].
abstract: The author studied 189 patients with the main forms of phakomatosis (Louis-Barre syndrome, Sturge-Weber, Hippel-Lindau disease and Recklinghausens neurofibromatosis.) The use of pathogenetical treatment in the Louis-Barre syndrome (a transplantation of a neonatal thymus with the bone marrow) promoted an improvement in the clinico-immunological indices. A study of familial forms of the Louis-Barre syndrome and the Sturge-Weber disease demonstrated a similarity according to the main clinical indices among the sibs in 80% which may speak of the role of the genetical factors in the formation of the disease.
mesh_terms: D000798:Angiomatosis / Q000175:diagnosis*; D001260:Ataxia Telangiectasia / Q000175:diagnosis* / Q000628:therapy; D001854:Bone Marrow Cells; D016026:Bone Marrow Transplantation; D002648:Child; D002675:Child, Preschool; D006801:Humans; D007223:Infant; D009456:Neurofibromatosis 1 / Q000175:diagnosis; D009461:Neurologic Manifestations; D013341:Sturge-Weber Syndrome / Q000175:diagnosis*; D013950:Thymus Gland / Q000637:transplantation; D014184:Transplantation, Homologous; D006623:von Hippel-Lindau Disease / Q000175:diagnosis*
vernacular_title: Nevrologicheskie sindromy pri fakomatozakh u deteĭ