Central Nervous System Vasculitis

Imaging of Central Nervous System Autoimmune, Paraneoplastic, and Neuro-rheumatologic Disorders

“…Primary angiitis of the CNS refers to inflammation affecting the vessels of the brain or spinal cord without overt systemic vasculitis or an underlying potential cause. Secondary CNS vasculitis occurs in the setting of a systemic vasculitis or CNS infection (such as syphilis, tuberculosis, and varicella-zoster virus) 61 . Depending on the cause, patients may present with subacute onset of progressive headache, cognitive impairment, and neuropsychiatric disturbances or multifocal cerebral infarcts across multiple vascular territories.…”

“…Secondary CNS vasculitis occurs in the setting of a systemic vasculitis or CNS infection (such as syphilis, tuberculosis, and varicella-zoster virus). 61 Depending on the cause, patients may present with subacute onset of progressive headache, cognitive impairment, and neuropsychiatric disturbances or multifocal cerebral infarcts across multiple vascular territories. Imaging may help differentiate CNS vasculitis from mimics such as intracranial atherosclerosis, vasospasm such as in reversible cerebral vasoconstriction syndrome (RCVS), and noninflammatory vasculopathies such as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL).…”

“…Imaging may help differentiate CNS vasculitis from mimics such as intracranial atherosclerosis, vasospasm such as in reversible cerebral vasoconstriction syndrome (RCVS), and noninflammatory vasculopathies such as cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL). 61 MRI is abnormal in 95% to 100% of patients with CNS vasculitis, so a negative MRI can essentially exclude the diagnosis. Typical findings include infarcts of varying ages in multiple vascular territories with foci of restricted diffusion acutely and T2-hyperintense foci from subacute to chronic infarcts.…”

“…62 Prominent meningeal gadolinium enhancement is usually associated with negative angiography (because of involvement of small meningeal arteries) but may have a more favorable response to immunosuppressive therapy. 61,63 Tumorlike infiltrative lesions have been described in 4% to 11% of patients with primary angiitis of the CNS with neoplasm as the most common differential diagnosis. A biopsy is typically required for diagnosis in these cases (FIGURE [10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25].…”

“…Ischemic lesions are sometimes accompanied by subarachnoid or intraparenchymal hemorrhagic lesions 62 . Prominent meningeal gadolinium enhancement is usually associated with negative angiography (because of involvement of small meningeal arteries) but may have a more favorable response to immunosuppressive therapy 61,63 . Tumorlike infiltrative lesions have been described in 4% to 11% of patients with primary angiitis of the CNS with neoplasm as the most common differential diagnosis.…”