THE INITIAL STORY

A young man in his late teens presented with a four week history of malaise, vomiting, anorexia, and 7 kg weight loss. He was dehydrated and barium follow through revealed a band like obstruction of the 3rd and 4th parts of the duodenum. Following rehydration and normalisation of his biochemistry he had three generalised tonic-clonic seizures and was started on sodium valproate. This failed to control his seizures and phenytoin was added. He also experienced visual hallucinations, for instance seeing toilet seats being swallowed up. Laparotomy confirmed duodenal obstruction secondary to Wilkie’s syndrome (obstruction between the angle of the aorta and superior mesenteric artery, seen in association with weight loss). A duodeno-jejunal anastamosis was performed.

Postoperatively his neurological condition deteriorated with increasing confusion, headache, and left sided myoclonus and weakness so he was transferred to the neurology service. The past medical history was revisited. He had had a normal birth and childhood milestones but he had always been thin and weak with reduced exercise tolerance compared to his two siblings. He had complained of intermittent abdominal pain and vomiting since the age of 5. He had no family history of neurological or gastrointestinal disease. It also turned out that he had been bitten by a tick three weeks before admission.

On examination he was underweight at 40 kg with generalised muscle wasting and bilateral pes cavus. The most striking finding was epilepsia partialis continua (EPC) affecting the left arm and leg. He had a left homonymous hemianopia, dysarthria, and horizontal nystagmus on right gaze. In addition he had a left hemiparesis (MRC grade 2) and left sided sensory impairment (all modalities). Upper limb reflexes were normal but lower limb reflexes were absent.

INVESTIGATIONS

He had a very large number of investigations during the course of this admission and these are summarised …