[Hypothalamic form of Whipple's disease. Favorable effect of rifampicin]

An unusual case of Whipple's disease is reported. The diagnosis was difficult as the characteristic digestive sign and symptoms (malabsorption, diarrhea, mucosal infiltration by PAS-positive macrophages) were absent. After a ten-year history of seronegative arthritis, myocardiopathy, with aortic insufficiency, basilar pulmonary infiltrates, enlarged lymph nodes, the patient, a 56 years old man, was referred to us for a severe vegetative and neurological dysfunction: stupor, dysarthria, akinesia, hypertonia, hypothermia and abnormal thirst. A CT-scan showed a low-density area of the right hypothalamus, and PAS-positive macrophages were found in a lymph node, in the CSF and in a cerebral biopsy. The patient then received a classical antibiotic treatment, yet the neurologic dysfunction remained severe. Finally, a trial with rifampicin brought a striking improvement of the patient's condition, which has now lasted for three years.