children s health issues
Neurofibromatosis
Symptoms of Neurofibromatosis
NF1 usually causes no symptoms, but most people with NF1develop the characteristic café-au-lait spots or lumps under the skin. People may not notice these spots or lumps. However, if neurofibromas put pressure on the nerve they surround, people may notice tingling or weakness in areas near the lumps. Café-au-lait spotsdevelop in about 90% of affected children. They are the medium-brown color of coffee with milk (café-au-lait) and develop on the skin of the chest, back, pelvis, and creases of the elbows and knees. These flat spots typically exist at birth or appear during infancy. Children who do not have neurofibromatosis may have 2 or 3 café-au-lait spots, but children who have NF1 have 6 or more of these spots. Neurofibromas that develop in the skinare common. Between ages 10 and 15, neurofibromas of varying sizes and shapes may begin to appear. There may be fewer than 10 of these growths or up to thousands of them. They may be the color of skin or have a pink or tan discoloration and usually do not cause other symptoms. can cause structural abnormalities, such as an abnormally curved spine (kyphoscoliosis), rib deformities, enlarged long bones in the arms and legs, and bone defects of the leg bones or skull. If the bone surrounding an eyeball is affected, the eye bulges. Neurofibromas that develop under the skin can also affect the nerves that lead from the brain to various parts of the head, neck, and trunk ( Overview of the Cranial Nerves ). Neurofibromas that develop in the nervesmay affect any nerve in the body. They frequently grow on spinal nerve roots (the parts of the spinal nerve that emerge from the spinal cord through the spine). There, they often cause few or no problems. However, if they put pressure on (compress) the spinal cord, they can cause paralysis or disturbances in sensation in different parts of the body, depending on which part of the spinal cord is compressed. If neurofibromas compress peripheral nerves, the nerves may not function normally, and pain, tingling, numbness, or weakness may result. Tumors that affect nerves in the head can cause blindness, dizziness, incoordination, or weakness. Other problems may develop. They include A tumor on the optic nerve (called an optic Gliomas ) Lisch nodules (benign growths on the iris, the colored part of the eye) Bulges in the walls of arteries ( Overview of Aortic Aneurysms and Aortic Dissection ) or blockages in arteries Slightly enlarged head Learning Disorders None Overview of Leukemia None Cancerous tumors, sometimes in the brain or along the peripheral nerves Optic gliomas may not cause any symptoms, or they may enlarge enough to put pressure on the optic nerve and impair vision or even cause blindness. These tumors can usually be identified by the time children are 5 years old and rarely develop after age 10. Neurofibromatosis usually progresses slowly. As the number of neurofibromas increases, more neurologic problems may develop. In NF2, Vestibular Schwannoma develop on 1 or both auditory nerves. The tumors compress the nerves and may cause People may also have other types of tumors, including Gliomas and In schwannomatosis, benign tumors (called schwannomas) can develop on almost any nerve in the body, except the auditory nerves, so people with non-NF2 schwannomatosis do not have vestibular schwannomas. Some people have many schwannomas, but others have only a few. The first symptom of schwannomatosis is usually pain, which can occur anywhere in the body and which may become chronic and severe. Some people have numbness, tingling, or weakness in the fingers and toes. Other symptoms may develop, depending on the location of the schwannomas. Symptoms develop when schwannomas compress nerves in the head, spine, or trunk. 