Long-term follow-up in cerebral Whipple's disease

To evaluate the long-term outcome in patients with cerebral Whipple's disease.

We reviewed the literature and contacted authors who had reported on cerebral Whipple's disease within the last 10 years. Fifteen patients were evaluated, including one patient treated at our hospital and the one presented in this paper. The mean observation period was 41 +/- 37 months (minimum 1 month, maximum 120 months).

Four patients had improved and were able to pursue an independent lifestyle, three patients had improved but are still dependent on help with simple activities of daily life, one patient was unchanged, and seven patients had died. Patients with initial penicillin alone had a worse prognosis than patients with initial penicillin plus streptomycin.

Third generation cephalosporins have been shown to be beneficial in cerebral Whipple's disease, therefore initial antibiotic treatment of Whipple's disease should consist of ceftriaxone (instead of penicillin) combined with streptomycin. Since five of 12 patients (40%) treated with co-trimoxazole (trimethoprim plus sulphamethoxazole) did not respond, we conclude that the combination of trimethoprim and sulphamethoxazole does not prevent or cure central nervous system (CNS) involvement in all patients with Whipple's disease. If CNS relapse occurs during treatment with trimethoprim and sulphamethoxazole, oral third generation cephalosporins might be a useful alternative.