Chapter 24: The infertile banker with a headache

A couple attend a private infertility clinic. He is a 38-year-old merchant banker. She complains that he is always stressed and has had headaches for the last year. He says it is from staring at a computer screen for hours at a time and that his vision is deteriorating.

His semen sample is found to contain 5 million spermatozoa/mL (normal range >15). His morning serum total testosterone is 3 nmol/L (normal range 6.7-29) and serum luteinizing hormone LH is 1 IU/L (normal range 1.5-9.3). His serum prolactin is 240 ng/mL (normal range 2.1-17.7).

• 1. What is the likely diagnosis?

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  Correct answer:
  Prolactinoma (prolactin secreting pituitary macroadenoma)

He has a visual field perimetry test.

• 2. What does it show?

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  Correct answer:
  
  Visual field perimetry showing bitemporal hemianopia more notable in the upper fields

He has an MRI scan of his head.

• 3. What does it show?

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  Correct answer:
  
  MRI confirms a large macro-prolactinoma pressing on the optic chiasm.

He was initially treated with the dopamine receptor agonist cabergoline for 6 months and his prolactin levels became normal, his visual fields improved and the tumour shrank. His wife conceived but he was still a banker.

• 4. Name as many phakomatoses as you can.

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  Correct answer:
  

  • Neurofibromatosis (von Recklinghausen’s disease)
  • Tuberous sclerosis (Bourneville’s disease)
  • von Hippel-Lindau disease (cerebroretinal angiomatosis)
  • Sturge-Weber syndrome (encephalotrigeminal angiomatosis)
  • Osler-Rendu-Weber syndrome
  • Fabry’s disease (angiokeratoma corporis diffusum)

  The first three of these are associated with brain tumours.