Cerebral Angiitis
🧠 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.
