Table 2.
RVCS | PACNS | |
---|---|---|
Clinical data | ||
age | 20-40 years | 40-60 years |
gender | primarily women | more frequent in men |
trigger (drugs, postpartum etc) | frequently identified | absent |
headache | acute and severe | insidious |
cognitive impairment | unusual | frequent |
CSF | Normal or minimal protein increase | Abnormal (increased protein concentration and mild pleocytosis) |
MRI | Normal (>70%) * | Abnormal in 90%. Small infarcts in grey and white matter in multiple vascular territories, diffuse white matter lesions, mass lesions |
Angiography | Abnormal:diffuse areas of multiple stenoses and dilatations ** | May be normalSingle or multiple abnormalities(cut-offs, lumen irregularities, avascular mass lesion) |
CNS / leptomeningeal biopsy | Normal | Vasculitis |
Except when complicated by stroke, intraparenchymal or cortical subarachnoid hemorrhage or posterior reversible leukoencephalopathy.
Angiographic abnormalities are required for diagnosis but must be reversible in 6-12 weeks.