SciELO - Scientific Electronic Library Online

vol.53 issue4HTLV-I Associated uveitis, myelopathy, rheumatoid arthritis and Sjögren's syndromeBrown-Vialetto-van Laere syndrome: report of two cases author indexsubject indexarticles search
Home Pagealphabetic serial listing  

Services on Demand




Related links


Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X


BLEGGI-TORRES, Luiz F. et al. Von hippel-lindaus disease: Report of three cases and review of the literature. Arq. Neuro-Psiquiatr. [online]. 1995, vol.53, n.4, pp.782-788. ISSN 0004-282X.

The authors present the autopsy findings of two related patients and the biopsy findings of a thrid member of the family. The oldest member was 34 years old at death and on postmortem examination he had haemangioblastomas in the retina, cerebellum, medulla and spinal cord. Other findings were renal cell carcinoma, phaechromocytoma, cysts of kidney and pancreas, hydromyelia and atypical meningiomas. His brother died when 30 years old. The autopsy revealed haemangioblastomas of cerebellum, renal cell carcinoma and a clear cell cystadenoma of epididymus. The third patient was the daughter of the first and presented with headache and dizziness. CT-scan showed a cerebellar haemangioblastoma. Epidemiological considerations on the commonest visceral and CNS lesions and a review of current diagnostic criteria are discussed.

Keywords : von Hippel-Lindau's disease; angiomatosis; phakomatosis.

        · abstract in Portuguese     · text in English     · English ( pdf )


Creative Commons License All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License