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Young male with headache, blindness, and hypogonadism
  1. A S Kashyap
  1. Department of Medicine, Armed Forces Medical College, Pune 411040, India
  1. Dr Kashyap (email:skashyap{at}pn2.vsnl.net.in)

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Answers on p 519.

A 20 year old man presented with a history of diffuse, moderately severe headache of six years' duration. He had noticed gradual onset, slowly progressive diminution of vision in both eyes, and poorly developed secondary sexual characters of the same duration. On clinical examination his height was 152 cm, upper segment to lower segment ratio of 0.82 (mean 0.92 in white adults, 0.85 in black adults), arm span 162 cm (normal <5 cm more than height), there were no pubic, axillary, or secondary sexual facial hair. His testicular volume was 3 ml (normal adult 12–25 ml), stretched penile length 4 cm [mean in Asians 10.6 cm). His bone age by the Tanner-Whitehouse 2 method was 18 years. His visual acuity was reduced to perception of light in both eyes; fundoscopy revealed bilateral optic atrophy. Clinically he was euthyroid and eucortisolic. Other general and systemic examinations were normal. The skull radiograph lateral view taken earlier was reviewed (fig 1). Luteinising hormone concentration was 1 IU/l (normal range 1.3–13 IU/l), testosterone 3 nmol/l (10–35 nmol/l), prolactin 100 μg/l (2–15 μg/l), thyroxine 140 nmol/l (64–154 nmol/l), cortisol at 8am 600 nmol/l (140–690 nmol/l) and at 4pm 300 nmol/l (80–330 nmol/l), simultaneous plasma and urine osmolality were 287 mmol/kg serum water (285–295 mmol/kg serum water) and 800 mmol/kg respectively. The urine volume was 2000 ml/day and specific gravity 1.020. Serum sodium, potassium, and blood glucose (fasting and postprandial) concentrations were normal. He could not afford tests for growth hormone.

Figure 1

Radiograph of the skull.

Questions

(1)
What are the abnormalities shown in the skull radiograph?
(2)
What is the diagnosis?
(3)
What are the various clinical presentations of this condition?

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