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J Korean Neurol Assoc. 1987;5(2):255-261.
- Two Cases of von Hippel-Lindau Disease
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Hong-Ki Song, M.D., Ki-Bum Sung, M.D., Dae-Il Chang, M.D., Ju-Han Kim, M.D., Kyung-Cheon Chung, M.D., Myung-Ho Kim, M.D.
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Department of Neurology, College of Medicine, Han Yang University
- Von Hippel-Lindau Disease 2례
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송홍기, 성기범, 장대일 , 김주한, 정경천, 김명호
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한양대학교 신경과.
- Abstract
- Von Hippel-Lindau disease is a rare disorder which involves multi-organs including cerebellum and retina. Recently we have experienced 2 cases of von Hippel-Lindau disease.
The case 1 was a 19-year-old female with chief complaint of severe headache
accompanied by vomiting on admission. Computed tomogram of brain showed space
occupying lesion in the posterior fossa. She was rapidly deteriorated and diagnosed as
brain death on the basis of physical and neurological examination and
electroencephalogram. Kidney specimens removed for the transplantation and cerebellum
obtained by partial autopsy reveled bilateral renal cell carcinomas and hemangioblastoma,
respectively, on the microscopic examination..
The case 2 was a 23-year-old male who visited to our hospital due to headache,
gait disturbance, and visual loss in the right eye. The findings of brain CT, vertebral
angiography, ophthalmoscope, retinal angiogram, and operation were compatable to
cerebello-retinal angiomatosis.
With reviewing of some literatures, we present 2 cases of von Hippel-Lindau
disease.
Keywords :
- 초록
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