A 57 year old woman presented with a slowly progressive neurological d
isorder consisting of myoclonus, hypersomnia and episodic alterations
of consciousness. She had a history of biopsy-proven gastrointestinal
Whipple's disease for which she had been treated with tetracycline 6 y
ears earlier. A lumbar puncture showed a moderate lymphocytic pleocyto
sis and additional laboratory tests revealed intestinal malabsorption.
These findings suggested a relapse of Whipple's disease with predomin
ant CNS involvement. However, the duodenal biopsy did not show the his
tological changes typical of Whipple's disease. The patient was treate
d with penicillin and streptomycin followed by trimethoprime and sulfa
methoxazole and showed a complete remission of her neurological sympto
ms as well as normalised laboratory tests. The diversity of neurologic
al symptoms as well as the current state of diagnosis and therapy of W
hipple's disease are reviewed.