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Neurologist and Director, Neurodegenerative Disorders Research, 185 York Street, Subiaco, WA 6008, Australia; [email protected]
Whipple’s disease is a rare condition caused by a soil-born gram positive bacillus Tropheryma whipplei (T whipplei).1 Whipple’s disease of the brain is even rarer. It may occur in the context of a systemic illness with gastrointestinal or rheumatological features, or as a primary central nervous system infection.2 On the basis of my experience with two recent patients contrasting primary and secondary Whipple’s disease of the brain, I will suggest an approach to its diagnosis and management.
PATIENT 1. PRIMARY WHIPPLE’S DISEASE OF THE BRAIN
A 46-year-old woman presented with generalised tonic-clonic seizures. She subsequently developed diplopia and ataxia. Four months later she had a tremor, a fluctuating left VI nerve palsy, eye movement abnormality with impaired saccadic pursuit, and hyponatraemia secondary to inappropriate ADH secretion. Later blepharospasm, complete paralysis of upgaze, worsening ataxia and a positive Romberg’s sign appeared. She had a severe amnesic syndrome including impaired verbal and visual spatial memory. Five months after onset she rapidly deteriorated with fluctuating consciousness—her Glasgow Coma Score ranged from 3 to 14. She also experienced hallucinations and confusion.
At presentation her brain MRI had revealed atrophy and gliosis of the right hippocampus. However, concomitant with her deterioration she developed multiple nodular enhancing lesions on MRI involving the right temporal lobe, caudate nucleus, anterior commissure, right globus pallidus, left insula cortex, left hippocampus, lenticular striate territory, mesencephalon and perivascular spaces (fig 1). At this time a lumbar puncture showed an opening pressure of 17 cm of water with 464 white cells/μl (90% polymorphs, 10% lymphocytes). There were 30 red cells/μl. Cytology showed polymorphs and lymphocytes without malignant cells. The protein was 0.35 g/l (0.15–0.45); glucose was 1.6 mmol/l (concurrent blood glucose 3.1). Gram stain, India ink preparation and cultures for Mycobacteria were all negative.
She was treated...