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Fifty individuals with Von Hippel-Lindau(VHL)were studied with gadolinium- enhanced magnetic resonance imaging(MRI)to determine the frequency and distribution of CNS lesions.The associated clinical features were also reviewed.Thirty-six(72%)of the 50 had 1 or more CNS tumors.The most frequently affected sites in the CNS excluding the retina were the cerebellum(52%),spinal cord(44%),and brainstem(18%).New regional predilections for the craniocervical junction and conus medullaris were demonstrated by this study.Forty-one percent of all VHL patients with CNS tumors were neurologically asymptomatic:cerebellar tumors(50%),spinal cord tumors(50%),and brainstem tumors(44%)were often without clinical signs or symptoms.Multiple lesions were common.The mean age of all VHL patients(34. 5years)was similar to the mean age of all CNS VHL patients(34.4years), suggesting a lack of age association.CNS lesions commonly occurred in the 2nd decade of life.All patients at risk for VHL should be evaluated using gadolinium-enhanced MRI after 10 years of age,although ophthalmic examination should be initiated within the 1st 2 years of life.Enhanced MRI is particularly useful in the detection of CNS tumors in patients with the VHL gene. |
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ataxia
brainstem,neoplasms of
cerebellum,neoplasms of
conus medullaris,lesion of
cranio-cervical junction
gadolinium
genetic neurologic disorders
headache
hemangioblastoma
MRI
MRI,abnormal
MRI,contrast enhanced
MRI,spinal cord
neoplasm,primary of CNS
nystagmus
spinal cord,neoplasm
Von Hippel Lindau
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