Vasculitis of the CNS

Definitions

• Vasculitis of the CNS can be primary or secondary
• Primary = Primary angiitis of the CNS (PACNS) also called:
• Isolated or primary CNS vasculitis
• Granulomatous angiitis of the CNS
• Discussion relates to primary condition

Epidemiology

• Mean age 42 (range 3-78)

Pathogenesis

• Affects small and medium sized blood vessels within CNS
• By definition does not affect blood vessels outside CNS
• Granulomatous inflammation
• Similar appearance to GCA and Takayasu arteritis
• Predominantly affects cerebral cortex and meninges rather than subcortical regions.

Clinical manifestations

• Non-specific, diffuse disease
• Decreased cognition
• Headache
• Seizure
• Stroke
• Haemorrhage
• Spinal cord involvement
• Can occur in isolation of combination
• Myelopathy

Diagnosis

• Main process is to rule out other causes.
• LP
• Elevated opening pressure
• Elevated total protein
• Angiography (gold standard)
• Diffuse of localized changes in arteries
• Focal narrowing
• Irregularity
• Bleeding
• Aneurysms
• MRI/MRA
• Can be indicative but not enough to make diagnosis on its own.
• Brain biopsy (gold standard)
• Still miss a significant amount on first biopsy

Treatment

• Steroids
• Cyclophosphamide

Prognosis

• Average survival 45 days
• Maximal survival <10 months.