MRI findings in isolated cerebral manifestation of Whipple's disease—Case report and review of the literature

Abstract

Cases of Whipple's disease with isolated involvement of the brain without any systemic affection appear to be rare. We report a case of Whipple's disease with exclusively cerebral manifestation ascertained through repeated cerebro-spinal fluid analyses. The diagnosis of Whipple's disease involving solely the nervous system remains difficult. Mostly it is based on a combination of various neurological symptoms and the evidence of PAS-positive macrophages detected in cerebro-spinal fluid. Unfortunately the abnormalities on MRI are not specific in every case. In our patient the cranial MRI revealed areas of high signal intensity on FLAIR images in both temporomesial regions, around the third ventricle, along the quadrigeminal plate, and in the right thalamus. On T2-weighted spin-echo images similar changes were seen. No signal changes were detected on T1-weighted images and there was no contrast enhancement. Even on diffusion weighted images no altering were seen. Follow-up MRI after 6 months of broad-spectrum antibiotic treatment demonstrated the almost complete resolution of the signal abnormalities. Examination of the cerebro-spinal fluid was negative for Tropheryma whippelii after the treatment. Brain lesions detected by MRI appear to correlate well with neuropathologic lesions reported in cerebral manifestations of Whipple's disease, namely the basal parts of the telencephalon, the hypothalamus, the thalamus, the periaqueductal grey matter, and the quadrigeminal plate. The MRI findings in cases of isolated cerebral Whipple's disease do not seem to differ substantially from those with systemic manifestation and additional cerebral involvement. The literature concerning cerebral manifestation of Whipple's disease and associated MRI findings is reviewed.