Introduction: Whipple’s disease (WD) is a rare multisystem infectious disease caused by a slow growing Grampositive bacillus Tropheryma whippleii. Humans are the only known host for the infection. This chronic infection is characterized by predominant intestinal involvement. Weight loss, diarrhea, low-grade fever and arthralgia have all been recorded to be major symptoms of WD. The central nervous system (CNS) may be involved in 10-43% of patients with multisystem WD. The neurological manifestations can mimic almost any neurological condition. These manifestations occur in three circumstances: CNS relapse of previously treated classic WD, neurological involvement in untreated classic WD, and isolated neurological symptoms due to T. whippleii.

We present the first case of isolated cerebral Whipple’s disease presenting as a binocular cyst.

Case report: A 68-year-old right handed man was referred to our institution after suffering two simple focal epileptic seizures localized on the left side of his body. He had experienced increasing irritability for a month without any headache. There was no history of fever, weight loss or recurring episodes of arthralgia. Neurological exam was normal. MRI revealed a right frontal binocular cyst in the subcortical white matter. The lesion was hypo-intense on T1-weighted images and hyper-intense on T2-weighted images. Transient contrast enhancement was noticed after injection of Gadolinium. There was a marked edema surrounding the lesion. The lesion was thought to be a glioblastoma or a hydatid cyst. The lesion was removed by a right frontal craniotomy. The neuropathological examination concluded to a Whipple disease. Upper intestinal endoscopy with duodenal and jejunal biopsies was performed. Histological examination of the specimens showed mostly normal tissue. PCR showed a positive result in the cerebrospinal fluid (CSF). Antibiotic therapy with ceftriaxone was given for 2 weeks followed by TMP-SMZ for 12 months. At regular follow-up, the neurological examination remained stable. Follow-up MRI investigation performed 6 months after surgery showed no recurrence, but a persisting pseudocyst defect.

Conclusion: Isolated cerebral Whipple’s disease often poses a great diagnosis challenge since its symptoms and neuroimaging signs are not specific. This diagnosis should be suspected when no tumor tissue is found at surgery.