Clinical management of infectious cerebral vasculitides

ABSTRACT

A wide range of infections (virus, bacteria, parasite and fungi) may cause cerebral vasculitides. Headache, seizures, encephalopathy and stroke are common forms of presentation. Infection and inflammation of intracranial vessels may cause pathological vascular remodelling, vascular occlusion and ischemia. Vasculitis in chronic meningitis may cause ischemic infarctions, and is associated with poor outcome. Appropriate neuroimaging (CT-angiography, MR-angiography, conventional 4-vessel angiography) and laboratory testing (specific antibodies in blood and CSF, CSF culture and microscopy) and even brain biopsy are needed to quickly establish the aetiology. Enhancement of contrast, wall thickening and lumen narrowing are radiological signs pointing to an infectious vasculitis origin. Although corticosteroids and prophylactic antiplatelet therapy have been used in infectious cerebral vasculitis, there are no randomized clinical trials that have evaluated their efficacy and safety. Stable mycotic aneurysms can be treated with specific antimicrobial therapy. Endovascular therapy and intracranial surgery are reserved for ruptured aneurysms or enlarging unruptured aneurysms.

KEYWORDS:

• Cerebral vasculitis
• infectious disease
• infectious vasculitides
• Lyme disease
• mycotic aneurysm
• stroke
• syphilis
• VZV vasculopathy

Financial & competing interests disclosure

The author has no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Key issues

• Although different pathogenic mechanisms may be involved in infection-associated stroke, vasculitis and embolism are the most common.

• VZV causes inflammation and remodeling of cerebral arteries, resulting in ischemic and hemorrhagic strokes. GCA seems to be a VZV vasculopathy primarily affecting the temporal arteries.

• A multifocal cerebral vasculitis involving small and medium-sized vessels has been described in neuroborreliosis. Early antimicrobial treatment with ceftriaxone or doxycycline is recommended.

• Meningovascular syphilis should be included in the differential diagnosis of stroke, particularly in young and male patients with cryptogenic stroke.

• Antituberculous chemotherapy and corticosteroids are the mainstays of treatment of TBM. However, they seem to have a low efficacy to avoid the progression of cerebrovascular complications in TBM.

• Invasive fungal infections can cause meningitis and cerebral vessel invasion. Guidelines about the optimal length of drug treatment and the method for monitoring the response for treatment are still needed.

• Subarachnoid NCC causes an inflammatory exudate, resulting in the thickening of leptomeninges. Inflammatory cells invade the vessel wall, leading to endarteritis and endothelial hyperplasia causing cysticercotic angeitis. Cysticide drugs (albendazole) and steroids are the treatment of choice.

• Corticosteroids and prophylactic antiplatelet therapy may be indicated when there is evidence of cerebral vasculitis. Aspirin has been shown to reduce stroke risk and decrease mortality in TBM.

• Intracranial aneurysmal vasculopathy and stable para-infectious mycotic aneurysms should be treated with specific antimicrobial therapy. Endovascular therapy and intracranial surgery should be considered for ruptured aneurysms or enlarging unruptured mycotic aneurysms.