A 60 year old right handed Indian man presented with a 2 month history of worsening confusion and weakness. He had been aware of increasing unsteadiness of gait with several falls. For several months he had experienced weight loss, poor appetite, abdominal pain and constipation. He was admitted via A&E as he had become acutely confused and agitated with word finding difficulties and right sided facial droop. There was a past medical history of ‘arthritis’, hypertension, NIDDM and hypercholesterolaemia. Medications included aspirin, pravastatin, bendroflumethiazide, amlodipine and gaviscon. He was a non-smoker who drank 35 units alcohol/week.

On examination he was clinically anaemic. Systemic examination was unremarkable.

He was confused and became increasingly drowsy, developing a lobar pneumonia and klebsiella septicaemia for which he was intubated and ventilated. Following recovery 1 week later, he was noted to have marked facial and bulbar weakness. In the limbs, there was extensive wasting but no fasciculations. Tone was flaccid and there was severe distal weakness (grade 1–2/5) in the arms and legs with moderate limb girdle weakness (grade 4/5). He was areflexic. Co-ordination could not be assessed and sensation was normal.