Review: Diagnosis and treatment of cerebral vasculitis

Abstract

Vasculitides are characterized by inflammation and necrosis of the blood vessel wall. Large vessels including the aorta are affected in giant-cell arteritis, medium-size arteries in classic polyarteritis nodosa. The small-vessel vasculitides are separated in those with antineutrophil cytoplasm antibodies (ANCA) and those without. The primary angiitis of the central nervous system (PACNS) is a rare disorder affecting both medium- and small-sized vessels. Major symptoms of cerebral vasculitis are stroke, headache and encephalopathy. Diagnosis is based on laboratory and imaging findings. When cerebral affection occurs in systemic vasculitis an acute inflammatory response with raised erythrocyte sedimentation rate and increased values of C-reactive protein is present. In many cerebral vasculitides including PACNS, CSF studies reveal inflammatory findings. Magnetic resonance imaging, including ADC maps, diffusion and gradient echo sequences, is the investigation of choice to detect and monitor cerebral involvement. Certain MRI techniques and 18-fluorodeoxyglucose positron emission tomography allow the visualization of vessel wall inflammation when the lumen is still unaffected on angiography. The treatment recommendations for cerebral angitis are derived from protocols for systemic vasculitides. In general, a combination of steroids and pulse cyclophosphamide (CYC) is recommended for induction treatment. An alternative option is the use of the anti- CD20 antibody rituximab. Methotrexate, azathioprine and mycophenolate mofetil are recommended as alternatives to CYC once remission is achieved.

Headache in the Emergency Room

“…34 In vasculitis, some of the symptoms include headache, encephalopathy, and even cerebral infarcts, although diagnosis is often challenging to the clinician. 35 In older women, who present with more subacute headaches, giant cell arteritis is a type of vasculitis that should be ruled out. Giant cell arteritis (GCA), or temporal arteritis, is a chronic vasculitis of large-and medium-sized vessels, that affects, almost exclusively, the elderly population, with mean age of presentation of 65 years or more.…”

“…36 Immediate initiation of treatment with prednisone or prednisolone is recommended if GCA is suspected. 35 Other cerebral vasculitides include Takayasu's arteritis, polyarteritis nodosa, Churg-Strauss syndrome, Behçet's disease, and primary angiitis of the CNS (PACNS).…”

“…36 Elevated inflammatory markers, erythrocyte sedimentation rate (ESR) or C-reactive protein (CRP), can be valuable tools in diagnosis, but negative values do not rule out GCA. 35 Diagnosis may be aided by neuroimaging, either ultrasonography or MRI, and a temporal artery biopsy may also be done for a definitive diagnosis. 36 Immediate initiation of treatment with prednisone or prednisolone is recommended if GCA is suspected.…”

Vasculitis of the Central Nervous System – A Rare Cause of Stroke

“…Imaging radiological techniques which demonstrate arterial vessels play significant role in securing the diagnosis of vasculitis, and also demonstrate the cerebral involvement [12,14,16,17,18]. Computed tomography (CT) and magnetic resonance imaging (MRI) detect multiple CNS vascular lesions when only one is clinically apparent.…”

“…Then appropriate laboratory tests and other ancillary procedures must be performed to asses organ damage [12,14]. Laboratory findings suggestive of a systemic vasculitis include an acute inflammatory response like: raised erythrocyte sedimentation rate (ESR) and increased values of C-reactive protein (CRP).…”