[Continuous clinical management of patients with neurofibromatosis type 1].
Neurofibromatosis type 1(NF1) is an autosomal dominant disorder with variable expression. The complications are age specific. Serious complication during early childhood is rare but optic glioma, brain tumors or leukemia may appear. Learning difficulties and attention deficit hyperactive disorders occur in as many as 60% of patients during school age. Overall, intelligence in neurofibromatosis is normal and mental retardation occurs in 6-7%. Managements for learning difficulties and attention deficit hyperactive disorders are especially important for quality of life in these patients. Skin neurofibromas or subcutaneous plexiform neurofibromas appear during childhood and may cause pain or spinal cord involvement. Malignant peripheral nerve sheath tumors that arise from plexiform neurifibromas are a particularly devastating complication during middle age.
Ohno
Kousaku
K
Department of Child Neurology, Tottori University Hospital.
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D002648:Child; D002675:Child, Preschool; D003266:Continuity of Patient Care; D017353:Gene Deletion; D016514:Genes, Neurofibromatosis 1; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008875:Middle Aged; D009456:Neurofibromatosis 1 / Q000175:diagnosis / Q000235:genetics / Q000503:physiopathology / Q000628:therapy; D055815:Young Adult