Articles: vasculitis-pathology.
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Case Reports
Biopsy-proven case of Epstein-Barr virus (EBV)-associated vasculitis of the central nervous system.
A 75-year-old woman was admitted to our hospital with rapidly deteriorating consciousness disturbance. She had a 7-year history of rheumatoid arthritis (RA), which had been treated with methotrexate (MTX) and prednisolone. Brain T2-weighted MRI showed diffuse high-intensity lesions in the cerebral subcortical and deep white matter, bilateral basal ganglia and thalamus. ⋯ Immunosuppressive drugs such as steroids and MTX are widely used to treat autoimmune disorders, but may exacerbate the reactivation of EBV. This is the first case of biopsy-proven EBV-positive/HIV-negative vasculitis during the treatment of RA with MTX and steroids. This case indicates that EBV-associated vasculitis needs to be considered as a differential diagnosis of CNS vasculitis.
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The aneurysm wall has been reported to play a critical role in the formation, development, and even rupture of an aneurysm. We used high-resolution magnetic resonance imaging (HRMRI) to investigate the aneurysm wall in an effort to identify evidence of inflammation invasion and define its relationship with aneurysm behavior. ⋯ Aneurysm wall enhancement on HRMRI might be a sign of inflammatory change. Symptomatic aneurysms exhibited wall enhancement on HRMRI. Wall enhancement had a high consistent correlation of symptomatic aneurysms. Therefore, wall enhancement on HRMRI might predict an unsteady state of an intracranial saccular aneurysm.
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J. Neurol. Neurosurg. Psychiatr. · May 2016
Case ReportsHereditary leukoencephalopathy with axonal spheroids: a spectrum of phenotypes from CNS vasculitis to parkinsonism in an adult onset leukodystrophy series.
Hereditary diffuse leukoencephalopathy with neuroaxonal spheroids (HDLS) is a hereditary, adult onset leukodystrophy which is characterised by the presence of axonal loss, axonal spheroids and variably present pigmented macrophages on pathological examination. It most frequently presents in adulthood with dementia and personality change. HDLS has recently been found to be caused by mutations in the colony stimulating factor-1 receptor (CSF1R) gene. ⋯ We estimate that CSF1R mutations account for 10% of idiopathic adult onset leukodystrophies and that genetic testing for CSF1R mutations is essential in adult patients presenting with undefined CNS vasculitis or a leukodystrophy with prominent neuropsychiatric signs or dementia.