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Postgrad Med J 2001;77:471 ( July )

Self assessment questions

Answers on p 481.

A complicated case of von Hippel-Lindau disease

G Thomas, R Hillson

Department of Diabetes and Endocrinology, Hillingdon Hospital, Uxbridge, Middlesex, UK

Correspondence to: Dr G Thomas, Flat 1, 134 Shirland Road, Maida Vale, London W9 2BT, UK gthomas_uk@hotmail.com

Submitted 2 September 1999; Accepted 4 April 2000

The first 150 words of the full text of this article appear below.

A 17 year old female shop assistant presented with a three week history of generalised headache, associated with nausea, vomiting, and vertigo. She had no past medical history, and was taking no regular medication. Her mother was currently receiving radiotherapy for anaplastic carcinoma of the thyroid gland. On examination she had an ataxic gait, bilateral papilloedema, and horizontal nystagmus to right lateral gaze. Left sided dysdiadokinesis and hyper-reflexia were demonstrated. Power and sensation were preserved. Computed tomography of the brain revealed a cystic lesion within the left cerebellum. Subsequent magnetic resonance imaging (MRI) revealed a second, non-cystic lesion within the region of the right vermis (fig 1). Both images were consistent with cerebellar haemangioblastomata. A posterior fossa craniotomy was performed, with successful excision of both tumours. She made an uneventful recovery, with complete resolution of all symptoms, and was subsequently discharged.

Figure Removed (Available Only in the Full Text)

During a follow up outpatient appointment six months later, . . . [Full text of this article]







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