Clinical Manifestations of CNS Vasculitis
Primary CNS vasculitis typically presents with headache as the most common initial symptom, followed by encephalopathy and behavioral changes, with focal neurological deficits occurring in 20-30% of patients. 1
Core Clinical Presentations
The clinical manifestations vary significantly based on the size of affected vessels:
Medium Vessel Vasculitis
- Focal neurological signs predominate 2
- Stroke-like presentations (46.7% in secondary CNSV) 3
- Ischemic strokes developing in different vascular territories 4
- Unilateral weakness 5
- Transient ischemic attacks 6
Small Vessel Vasculitis
- Cognitive deficits and altered consciousness 2
- Encephalopathy with progressive cognitive changes 5
- Seizures (26.7-44.4% of cases) 1, 2, 7
- Multifocal encephalopathy that may present with normal angiograms 4
- Small-vessel strokes occurring over weeks to months 4
Additional Neurological Manifestations
Headache is nearly universal, presenting as progressive holocephalic headaches 5, 8
Seizures and intracranial hemorrhage may occur 1
Sensorimotor abnormalities (55.6% of cases) 7
Visual disturbances including blurred vision 7
Gait abnormalities and ataxia 5
Important Clinical Context
Diagnostic Clues
The diagnosis requires markers of both vascular disease AND inflammation 2. Key supporting features include:
- CSF abnormalities in 80-90% of patients: lymphocytic pleocytosis (rarely exceeding 250 cells/mm³), elevated protein, increased opening pressure 4, 8
- ESR typically normal or minimally elevated in primary CNS vasculitis 4
- Acute-phase reactants characteristically normal 4
Critical Pitfall
The median age of onset is 50 years for primary CNS vasculitis 8, though younger presentations occur (median 31-33 years in some series) 5, 9. Symptoms may persist for weeks to years before diagnosis—one study showed a median of 36 weeks (range 3 weeks to 4 years) before diagnosis 5.
When to Suspect CNS Vasculitis
Consider cerebral vasculitis in children or adults with:
- Ischemic or hemorrhagic stroke 4
- Recurrent stroke 4
- Stroke associated with encephalopathic changes 4
- Stroke accompanied by fever, multifocal neurological events, unexplained skin lesions, glomerulopathy, or elevated sedimentation rate 4
Imaging Findings
Contrast-enhanced MRI is abnormal in >90% of cases 4, showing:
- Multiple infarcts of variable ages (up to 50% of patients) 1
- White matter hyperintensities 1
- Mass lesions (5% of cases) 1
- Meningeal enhancement (8% of cases) 1
- Hemorrhage (9% of cases) 1
- Vessel wall enhancement on high-resolution vessel wall imaging 7, 6
A normal MRI makes CNS vasculitis diagnosis unlikely 8, with sensitivity of a normal MRI approaching 100% for excluding the diagnosis 1.