To report a case of CNS Whipple’s disease in an elderly diabetic male who presented with diarrhea followed by rapidly progressive extrapyramidal symptoms.

Whipple’s disease is a gastrointestinal infection caused by Tropheryma whipplei. It usually presents with diarrhea, abdominal pain, arthralgia and weight loss. CNS involvement in Whipple’s disease is rare, as low as one in a million, and usually presents with extrapyramidal symptoms. Hence accurate diagnosis becomes important as it is a treatable condition, with its common differentials being neurodegenerative disorders with varying prognosis.

A 78-year old male from Western India presented with a 3 month history of rapidly progressive tremors, bradykinesia and recurrent falls associated with REM sleep behavior disorder (in the form of dream enactment), following fever and diarrhea. Examination showed frontal predominant cognitive impairment, extrapyramidal signs with myoclonus and supranuclear gaze palsy. Neuroimaging showed mineralization in basal ganglia, and CSF studies were found to be within normal limits. Probable differentials of infective and autoimmune etiologies were considered, and workup was done on these lines. However, autoimmune encephalitis panel was found to be negative. Further, upper GI endoscopy was done, which was normal, with no PAS positive staining in the duodenal biopsy. However, in view of high index of suspicion, Tropheryma whipplei PCR was also done, which was found to be positive. As the patient befitted the diagnosis of CNS Whipple’s disease, he was started on appropriate antibiotics, with which he had improvement in extrapyramidal symptoms.

Although CNS involvement in Whipple’s disease is rare, it should be considered in patients with combined gastrointestinal and neurological symptoms. Clinical suspicion with appropriate testing helps in diagnosis, timely initiation of appropriate management and complete reversal of symptoms.