Case history

A 36 year old man presented in May 2006 with sudden onset of left facial droop, together with weakness and numbness affecting his left side. There were no prodromal symptoms and no history of trauma. His Glasgow coma score was 15/15, the left nasolabial fold was partially obliterated, and there was a left sided hemiparesis (Medical Research Council (MRC) grade 4/5) with pronator drift and an extensor plantar reflex. His pulse was 72 beats/min and regular, his blood pressure was 116/71 mm Hg; a non-radiating aortic ejection systolic murmur was heard, but no carotid bruits. The rest of the examination was unremarkable.

His haemoglobin was 228 g/l (reference range 133-170 g/l); his packed cell volume was 0.68% (0.39-0.52%), and his erythrocyte count 6.96×10¹²/l (4.3-5.6×10¹²/l); his red cell indices were normal; his white cell count was normal at 5.3×10⁹/l (3.5-11×10⁹/l) but his platelet count was low at 92×10⁹/l (140-400×10⁹/l). His urea and electrolytes were normal; his serum γ glutamyl transpeptidase (GGT) was 81 U/l (9-54 U/l).

After daily venesection for the next five days his haemoglobin was 147 g/l and his platelet count was 152×10⁹/l. His neurological signs resolved by the third day.

Questions

• 1What is the neurological diagnosis?

• 2 What is the underlying cause?

• 3 What further investigations are needed?