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Primary angiitis of the central nervous system: MRI features and clinical presentation
Surendra Singh1, Susan John, Thykkoothathil Pappy Joseph
1Department of Radiodiagnosis, Christian Medical College and Hospital, Vellore, Tamilnadu, India. singhsurendra26@hotmail.com
Abstract:
We report the MRI features in a series of four patients with primary angiitis of the central nervous system (PACNS). Based on MRI features, clinical presentation, paraclinical investigations and laboratory tests, including cerebrospinal fluid (CSF) analysis, various differentials were considered. In two patients with MRI findings of cortical, subcortical and deep white matter lesions, lack of mass effect, focal areas of bleed and heterogeneous parenchymal, leptomeningeal or pial enhancement and a normal magnetic resonance angiography, a differential diagnosis of primary angiitis of the CNS was also considered. In all patients, an open brain biopsy was advised to establish a definitive diagnosis of PACNS. Here, we briefly discuss the MRI features, correlation with clinical presentation and paraclinical parameters for the diagnosis of this entity. We also briefly review the literature.
Insights
MRI can reveal key features of primary angiitis of the central nervous system (PACNS). These findings, alongside clinical data and CSF analysis, aid in diagnosing this rare condition, often requiring brain biopsy for confirmation.
Area of Science:
- Neurology
- Neuroradiology
- Immunology
Background:
- Primary angiitis of the central nervous system (PACNS) is a rare condition affecting blood vessels in the brain and spinal cord.
- Accurate diagnosis is challenging due to nonspecific clinical and imaging findings.
- Distinguishing PACNS from other neurological disorders is critical for appropriate treatment.
Purpose of the Study:
- To describe the magnetic resonance imaging (MRI) features observed in a cohort of patients with PACNS.
- To correlate MRI findings with clinical presentation and laboratory results for diagnostic insights.
- To review the literature on MRI in PACNS diagnosis.
Main Methods:
- Retrospective analysis of MRI scans from four patients diagnosed with PACNS.
- Review of clinical data, cerebrospinal fluid (CSF) analysis, and other paraclinical investigations.
- Comparison of imaging findings with differential diagnoses.
Main Results:
- MRI revealed white matter lesions (cortical, subcortical, deep) in two patients.
- Absence of mass effect, focal bleeding, and heterogeneous enhancement were noted.
- Normal magnetic resonance angiography (MRA) was observed in some cases.
- Open brain biopsy was deemed necessary for definitive PACNS diagnosis in all patients.
Conclusions:
- Specific MRI patterns, in conjunction with clinical and CSF data, can suggest PACNS.
- Heterogeneous enhancement and white matter lesions are important imaging clues.
- Brain biopsy remains the gold standard for confirming PACNS.