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. 2011 Sep;9(3):437–448. doi: 10.2174/157015911796557920

Table 2.

Clinical, Laboratory, Imaging and Histopathologic Characteristics Useful to Distinguish RVCS from PACNS

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.