Grand Rounds—Hammersmith Hospital: Cerebral Whipple's disease

Relapse presenting with spinal myoclonus

Whipple's disease is a rare disease caused by infection with the newly identified organism Tropheryma whippelii. Relapses of the disease most commonly occur in the central nervous system and are often resistant to antibiotics. We present the case of a man with recurring ophthalmoplegia, myoclonus, and hypothalamic symptoms while on treatment with cotrimoxazole for a previous relapse of Whipple's disease in the central nervous system. The patient had the clinical and electromyographic features of spinal myoclonus, a movement disorder not previously described in isolation in Whipple's disease. We then discuss the clinical features and pharmacological treatment of cerebral Whipple's disease.

Case history

A 28 year old right handed man was admitted to the neurology ward of his local hospital for further investigations. The patient was Italian and worked as a bank clerk in Pisa.

He gave an 18 month history of gradually worsening involuntary movements of both arms and the upper trunk. He also had impaired eye movements and a six month history of ejaculatory and erectile difficulties, weight gain, somnolence, and depression. He also complained of poor memory and difficulty with concentration.

His medical history was that in 1986 he had presented with nausea, anorexia, abdominal pain, and weight loss. Endoscopy was performed, and duodenal mucosal biopsies showed macrophages that yielded a positive result when stained with the periodic acid Schiff reagent. Intestinal Whipple's disease was diagnosed, and he was treated for six months with oral amoxycillin.

In 1992 he had presented with increased skin pigmentation, lethargy, weight gain, and decreased libido. He had paralysed vertical ocular movements. Staining with periodic acid Schiff reagent did not yield a positive result in duodenal biopsies but did in 15 mononuclear cells from the cerebrospinal fluid.

Magnetic resonance imaging of the brain showed multiple focal lesions in the hypothalamus, striatum, and midbrain (fig 1 …