Makindo Medical Notes

🧠 Cerebral Angiitis (Primary Angiitis of the CNS, PACNS) is a rare, inflammatory vasculitis affecting only the brain and spinal cord vessels. It may mimic stroke, infection, or neurodegenerative disease, making diagnosis challenging. Early recognition is essential as treatment can be life-saving.

📖 About

• A rare granulomatous vasculitis affecting CNS vessels (arteries, arterioles, capillaries, or veins).
• Distinct from systemic vasculitis with secondary CNS involvement.
• Presents in a segmental pattern, causing patchy vessel inflammation.
• Often misdiagnosed due to overlapping features with stroke, infection, and autoimmune conditions.

🧬 Aetiology

• Mostly idiopathic, but may occur secondary to:
  • 🦠 Infection: Varicella-zoster virus, HIV, bacterial or fungal infections.
  • 🧩 Systemic inflammation: SLE, rheumatoid arthritis, sarcoidosis.
  • 💊 Drug-induced: Cocaine, amphetamines, or medications.
  • 🎗️ Paraneoplastic syndromes: Associated with malignancies.
  • 🧪 Genetic predisposition or immune dysregulation.
• Can follow trauma, radiotherapy, or post-infectious states.

⚠️ Clinical Features

• Presentation is variable and may mimic many other conditions.
• 🧩 Stroke-like episodes: Ischaemic or haemorrhagic, often recurrent.
• 🤕 Headache: Persistent, severe, and a common early symptom.
• 🧠 Cognitive decline: Confusion, memory loss, or dementia-like picture.
• ⚡ Seizures: Focal or generalized.
• 🌡️ Systemic signs: Fever, malaise, weight loss.
• 💭 Psychiatric changes: Depression, psychosis, or personality change.
• Rare: movement disorders or spinal cord involvement.

🔍 Investigations

• CSF analysis: Protein ↑, pleocytosis (WCC ↑), glucose usually normal unless infective.
• MRI: More sensitive than CT – shows bilateral infarcts, small-vessel disease, or haemorrhage. Contrast may reveal vessel inflammation.
• Cerebral angiography: "Beading" (alternating narrowing/dilatation). May be normal in early disease.
• Biopsy: 🏅 Gold standard – vessel wall inflammation, granulomas, or fibrinoid necrosis.
• Vasculitis screen: ANCA, ANA, dsDNA, rheumatoid factor – mainly to rule out systemic disease.
• Blood cultures & viral/fungal serology to exclude infection.

💊 Management

• 🚫 Rule out infection first – critical before immunosuppression.
• Corticosteroids: High-dose steroids (e.g., IV methylprednisolone) are first-line.
• Immunosuppressants: Cyclophosphamide, azathioprine, or mycophenolate for refractory cases.
• Antithrombotics: Consider if ischaemic events, provided no haemorrhage.
• Supportive care: Antiepileptics for seizures, pain relief for headache, rehab for deficits.
• Infectious angiitis: Treat underlying infection with antivirals, antibiotics, or antifungals.

📊 Prognosis

• Highly variable – depends on extent of vessel involvement and timeliness of treatment.
• ⚠️ Without treatment → progressive neurological decline, stroke, and death.
• ✅ With early therapy → many improve, but relapses are possible.