Primary angiitis of the central nervous system

Other Names: PACNS; Primary central nervous system vasculitis; Primary CNS vasculitis; Granulomatous angiitis of the central nervous system

Primary angiitis of the central nervous system is a rare form of vasculitis (inflammation of blood vessels) affecting the blood vessels that nourish the brain, spinal cord and peripheral nerves. This condition can lead to narrowing and blockage of the blood vessels of the central nervous system which can eventually cause aneurysms, ischemia and/or hemmorrhage.

Cause

The cause of this condition is unknown.

Signs and symptoms

Signs and symptoms of this condition may begin suddenly or develop over time. Some of the symptoms may incude headaches that do not go away, fever, rapid weight loss, confusion or forgetfulness, and general malaise.

For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed.

80%-99% of people have these symptoms

• Cerebral vasculitis

30%-79% of people have these symptoms

• Abnormal CSF protein level
• Aphasia(Difficulty finding words)
• CSF pleocytosis
• Headache(Headaches)
• Hemiparesis(Weakness of one side of body)
• Multifocal cerebral white matter abnormalities
• Nausea and vomiting
• Paralysis(Inability to move)
• Recurrent subcortical infarcts
• Transient ischemic attack(Mini stroke)

5%-29% of people have these symptoms

• Abnormal visual field test
• Ataxia
• Blurred vision
• Diplopia(Double vision)
• Dysarthria(Difficulty articulating speech)
• Fever
• Intracranial hemorrhage(Bleeding within the skull)
• Paraparesis(Partial paralysis of legs)
• Reduced visual acuity(Decreased clarity of vision)
• Seizure
• Tetraparesis
• Transient global amnesia

1%-4% of people have these symptoms

• Amaurosis fugax
• Constitutional symptom
• Parkinsonism
• Pseudopapilledema
• Vertigo(Dizzy spell)

Diagnosis

The diagnosis of PACNS is based on the combination of clinical presentation, imaging findings (magnetic resonance imaging and angiography), brain biopsy, and laboratory and cerebral spinal fluid (CSF) values. PACNS can either be confirmed by magnetic resonance angiography (MRA)/conventional angiography or tissue biopsy showing the presence of typical histopathological patterns. Identification of PACNS mimics is often challenging in clinical practice, but crucial to avoid far-reaching treatment decisions.

Treatment

The current treatment recommendation is to start with oral prednisone at a dose of 1 mg/kg per day and cyclophosphamide at a dose of 2 mg/kg per day. Most centers use prednisone and cyclophosphamide for 4-6 months to induce clinical remission, and then taper prednisone off. Patients generally stay on cyclophosphamide therapy between three and six months, depending on when remission occurs and if there are any potential side effects from cyclophosphamide.

Once cyclophosphamide is discontinued, it should be replaced with a less toxic medication for an additional six to twelve months of maintenance therapy. Some doctors switch from cyclophosphamide to azathioprine (2 mg/kg) or mycophenolate mofetil. Methotrexate can also be used, but may be limited by its difficulty to cross the blood brain barrier. There is limited data on how long the maintenance therapy lasts so the decision on the duration of the therapy should be individualized, based upon how the patient responds to therapy.

NIH genetic and rare disease info

• NIH info on Primary angiitis of the central nervous system

Primary angiitis of the central nervous system is a rare disease.

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