Neurology: Case of the Month

Test Yourself — Patient 82

Von Hipple-Lindau Syndrome

1. What is the leading cause of death in patients with VHL?
   • [ A ] Myocardial infacrtion
   • [ B ] Renal cell carcinoma
   • [ C ] Central nervous system hemangioblastoma
2. What is the most common non-CNS manifestation of VHL?
   • [ A ] Pheochromocytoma
   • [ B ] Pancreatic cysts
   • [ C ] Renal carcinoma
3. Which of the following is the most common CNS manifestation of VHL?
   • [ A ] Hydrocephalus
   • [ B ] Spinal hemangioblastoma
   • [ C ] Cerebellar hemangioblastoma
4. All of the following should be included in your work-up of a patient with suspected VHL except?
   • [ A ] MRI of brain with and without contrast
   • [ B ] MRI of the spinal cord
   • [ C ] Fundoscopic exam
   • [ D ] CT abdomen and pelvis
   • [ E ] Lumbar puncture
   • [ F ] Urine for vanillylmandelic acid
5. All are included in the diagnosis of VHL except
   • [ A ] Retinal hemangioblastoma
   • [ B ] Pheochromacytoma
   • [ C ] Genetic testing for VHL mutation
   • [ D ] Cerebellar hemangioblastoma

 

Email comments: