Background: Whipple disease (WD) is an infectious disease, which may affect the central nervous system. Central nervous system symptoms are eventually present in as many as 43% of the cases. To our knowledge, cerebellar ataxia in WD has never been formally studied in any large series.
Objective: To determine the prevalence of cerebellar ataxia in central nervous system WD.
Results: Between January 1974 and December 2003, we identified 11 patients who met criteria for definite central nervous system WD, the second largest series to date. Surprisingly, while oculomasticatory myorrhythmia was recorded in only 1 patient (9%), cerebellar ataxia had been documented in 5 cases (45%).
Conclusion: Our data suggest that cerebellar ataxia should be considered a more common feature of central nervous system WD.