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Resident's Flash Card: Neurofibromatosis

Neurofibromatosis - I (von Recklinghausen’s disease)
- autosomal dominant (abnormality is on chromosome 17)
- diagnostic criteria include six or more cafe-au-lait spots, axillary or inguinal freckling, two (or more) neurofibromas, optic glioma (most common CNS neoplasm - occurs in 30-90% and is bilateral in 10-20%), iris hamartoma, distinctive osseous lesions, or a first-degree relative with NF-1
- hypertension may be found with renal artery fibromuscular dysplasia
- neurofibromas can occur in the urinary and gastrointestinal tracts
- increased incidence of all types of neoplasms 
- malignant transformation of the neurofibroma into neurofibrosarcoma occurs in about 5%
- lateral thoracic meningoceles are a common manifestation of spinal neurofibromatosis; most authors believe that they are secondary to menigeal dysplasia (dural ectasia). 80% of patients with lateral thoracic meningoceles have neurofibromatosis.
- a plexiform type of neurofibroma is virtually pathognomonic of neurofibromatosis

Neurofinbromatosis - II
- autosomal dominant (abnormality is on chromosome 22)
- bilateral 8th cranial nerve tumors or
- unilateral 8th cranial nerve tumor or neurofibroma, menigioma, glioma, Schwannoma, or capsular cataract and a first degree relative with NF-2
Resident's Flash Card: Neurofibromatosis Reviewed by Sumer Sethi on Monday, August 19, 2013 Rating: 5

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