The online version of this article (doi:10.1186/1752-1947-8-26) contains supplementary material, which is available to authorized users.
The authors declare that they have no competing interests.
KG and AMM analyzed and interpreted the patient data regarding the clinical presentation and were major contributors in writing the manuscript. JBW performed and interpreted the MRI data. MCL reviewed all of the data and made major contributions to writing and editing the manuscript. All authors read and approved the final manuscript.
Primary angiitis of the central nervous system is a rare disease of unclear etiology. There is no single test diagnostic of primary angiitis of the central nervous system. We report an unusual pattern on brain magnetic resonance imaging that might be specific for primary angiitis of the central nervous system.
A 47-year-old Caucasian man developed progressive bilateral hand tremor, difficulty walking, cognitive slowing and headache. A physical examination showed bilateral hand tremor with dysmetria, hyperreflexia and abnormal gait. Magnetic resonance imaging of his brain showed bilateral, symmetrical, increased intensity on T2-weighted images concurrent with linear contrast enhancement in a radial distribution throughout his white matter, sparing subcortical regions in his centrum semiovale, corona radiata, basal ganglia and brainstem. Magnetic resonance spectroscopy demonstrated elevated choline and decreased N-acetyl aspartate. Except for elevated protein and lymphocytic pleocytosis, examination of his cerebrospinal fluid showed no abnormalities. Serological tests for rheumatologic, vasculitic, paraneoplastic, infectious and peroxisomal disorders were negative. A brain biopsy revealed primary angiitis of the central nervous system. Our patient was treated with steroids and intravenous cyclophosphamide, with improvement in signs and symptoms as well as changes on magnetic resonance imaging.
Bilateral, symmetrical, increased intensity on T2-weighted images concurrent with linear contrast enhancement in a radial distribution throughout the white matter on magnetic resonance imaging of the brain should be recognized as a feature of primary angiitis of the central nervous system, and might avoid the need for a brain biopsy to diagnose primary angiitis of the central nervous system.
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- Radial contrast enhancement on brain magnetic resonance imaging could be diagnostic of primary angiitis of the central nervous system: a case report and review of the literature
Aisha Mohsin Malik
James B Wood
Michael C Levin
- BioMed Central