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Final clinical long case 18

Created: 27/6/2006
Updated: 27/6/2006
A 36-year-old male with neurofibromatosis presents with convulsions. He also has had right-sided homonymous hemianopia for the past few weeks. He is a smoker and an alcoholic.

The patient is conscious, with no other focal neurology than the hemianopia.
Pulse rate: 56 beats/min
Blood pressure 140/90 mmHg
ECG: Right-axis deviation, up slurring of ST segments in V4 and V5.
Chest X-ray: Right upper lobe collapse, consolidation and a well circumscribed mass in the mid-zone.
Full blood count: Unremarkable except that the mean cell volume (MCV) is 89 fL
Biochemistry: Unremarkable except for a high alkaline phosphatase
Computed tomography: Posterior cranial fossa tumour, which looks malignant. The neurosurgeons want to perform a craniotomy.

1. Summarise the case.
2. Should this be an elective or emergency case?
3. What is the differential diagnosis?
4. What percentage of neurofibromatosis cases become malignant?
5. What is right homonymous hemianopia?
6. What is the recommendation for alcohol intake?
7. How much is a unit of alcohol?
8. Describe the ECG findings.
9. Describe the chest X-ray findings.
10. Why might the MCV be high? What are the causes of high MCV?
11. Why might alkaline phosphatase be high in this patient, when the bilirubin is normal?
12. What other investigations would you need?
13. Will you proceed with the case?
14. What position will the patient be placed in?

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