Central nervous system lesions in von hippel-lindau syndrome

Traen, Sophie; Seynaeve, Patrick; Cardoen, Liesbeth; Carels, Kenneth; Lecluyse, Carmen

doi:10.5334/jbr-btr.551

Journal of the Belgian Society of Radiology

2011

DOI: 10.5334/jbr-btr.551

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Central nervous system lesions in von hippel-lindau syndrome

Sophie Traen¹,

Patrick Seynaeve²,

Liesbeth Cardoen³

et al.

Abstract: Background:A 48-year-old man presented with persisting tinnitus and progressive worsening occipital headaches, neckpain, ataxia and paresthesia of the scalp. There was no recent trauma in his recent history.

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“…Hemangioblastomas originate from the blood vessels composed of both endothelial and stromal cells (11). CNS hemangioblastomas commonly involve cerebellum, spine, and medulla.…”

mentioning

confidence: 99%

An Unusual Cause of Extremity Weakness: Von Hippel-Lindau Disease and Advantages of Radiologic Imaging on Diagnosis

Görmeli¹,

Aydın²,

Görmeli³

et al. 2015

J Turgut Ozal Med Cent

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Von Hippel-Lindau disease is a rare autosomal dominant disease with the disorder matched with the short arm of chromosome 3. The prevalence of Von Hippel-Lindau disease has been estimated to be between 1:35000-1:40000. Retinal hemangioma and central nervous system (CNS) hemangioma are the main manifestations with later accompanying adrenal pheochromacytoma, paraganglioma, renal cell carcinoma, islet cell tumors, pancreatic cysts and neuroendocrine tumors, epididymal cysts, or cystadenoma and endolmphatic sac tumor of the inner ear. The disease is a multisystem disorder with phenotypic variability. A 50-year-old man presented with a 2-month history of weakness of the right upper and lower limbs. Magnetic resonance imaging revealed cerebellar and spinal hemangioblastomas. In this case report, we present the radiologic images and clinical features of apatient with von Hippel-Lindau disease along with symptomatic central nervous system hemangioblastomas and multiple renal and pancreatic cysts.

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“…Hemangioblastomas originate from the blood vessels composed of both endothelial and stromal cells (11). CNS hemangioblastomas commonly involve cerebellum, spine, and medulla.…”

mentioning

confidence: 99%

An Unusual Cause of Extremity Weakness: Von Hippel-Lindau Disease and Advantages of Radiologic Imaging on Diagnosis

Görmeli¹,

Aydın²,

Görmeli³

et al. 2015

J Turgut Ozal Med Cent

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show abstract

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Central nervous system lesions in von hippel-lindau syndrome

Abstract: Background:A 48-year-old man presented with persisting tinnitus and progressive worsening occipital headaches, neckpain, ataxia and paresthesia of the scalp. There was no recent trauma in his recent history.

Cited by 1 publication

References 2 publications

An Unusual Cause of Extremity Weakness: Von Hippel-Lindau Disease and Advantages of Radiologic Imaging on Diagnosis

An Unusual Cause of Extremity Weakness: Von Hippel-Lindau Disease and Advantages of Radiologic Imaging on Diagnosis

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