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MR imaging of central nervous system Whipple disease: a 15-year review
D F Black1, A J Aksamit, J M Morris
1Department of Radiology, Mayo Clinic Radiology, Rochester, MN 55905, USA. black.david@mayo.edu
Abstract:
CNS WD is fatal if antibiotics are not begun early, but knowledge regarding the variety of presentations on MR imaging is limited. In order to more effectively recognize this entity on MR imaging, the Mayo Clinic medical records were reviewed for subjects diagnosed with CNS WD from 1992-2006 who had also undergone MR imaging of the neuraxis. Seven subjects were identified and their imaging findings were reviewed by the authors. Four of 7 had head MR imaging findings indicative of WD. Two subjects demonstrated high T2 signal within the corticospinal tracts. CNS WD may demonstrate high T2 signal with minimal enhancement and no restricted diffusion, primarily in the midline of the midbrain, hypothalamus, and mesial temporal lobes and occasionally the corticospinal tracts. MR imaging may also be normal. Radiologists should be aware of these presentations and be prepared to mention CNS WD as a diagnostic possibility since early antibiotic therapy may significantly impact morbidity and mortality.
Insights
Central nervous system Wilson disease (CNS WD) requires early antibiotics for survival. Recognizing diverse MR imaging findings, including high T2 signal in specific brain regions and corticospinal tracts, is crucial for timely diagnosis and improved patient outcomes.
Area of Science:
- Neurology
- Radiology
- Infectious Disease
Background:
- Central nervous system Wilson disease (CNS WD) is a fatal condition if not treated promptly with antibiotics.
- Limited knowledge exists regarding the spectrum of MR imaging presentations for CNS WD.
Observation:
- A review of 7 patients diagnosed with CNS WD between 1992-2006 at Mayo Clinic was conducted.
- MR imaging findings of the neuraxis were analyzed for characteristic patterns of CNS WD.
Findings:
- Four out of seven patients exhibited MR imaging findings suggestive of WD.
- Specific findings included high T2 signal within the corticospinal tracts in two subjects.
- CNS WD can present with high T2 signal, minimal enhancement, and no restricted diffusion, particularly in the midline brain structures (midbrain, hypothalamus, mesial temporal lobes) and occasionally corticospinal tracts. MR imaging may also appear normal.
Implications:
- Radiologists must be aware of the varied MR imaging presentations of CNS WD.
- Considering CNS WD in differential diagnoses is vital, as early antibiotic intervention significantly impacts patient morbidity and mortality.
