Moore listed criteria for making the diagnosis of isolated angiitis of the central nervous system (IAC). The author is from Wayne State University in Detroit, Michigan.
Features of IAC:
(1) It is an idiopathic, recurrent vasculitis.
(2) It is confined to the central nervous system (CNS).
(3) It affects small blood vessels primarily but it may also affect medium-sized vessels.
(4) It is often fatal unless treated aggressively with cyclophosphamide and corticosteroids.
(5) Most patients have multiple focal lesions in cerebrum, cerebellum and/or brainstem.
(6) Neurologic signs and symptoms include headaches, confusion, intellectual deterioration, memory loss, malaise, stroke, seizures, cranial nerve palsies and/or myelopathies.
Criteria for the diagnosis:
(1) headaches and multifocal neurologic deficits with one or more of the following:
(1a) present for >= 6 months
(1b) severe at onset
(1c) rapidly progressive
(2) cerebral angiography shows segmental arterial narrowing. Other findings include vascular occlusion, delayed emptying, and anastomotic channels.
(3) exclusion of systemic inflammation and/or infection. The presence of an elevated ESR, ANA, RF, immune complexes or autoantibodies indicate an alternative diagnosis.
(4) leptomeningeal or brain biopsy shows vascular inflammation and excludes alternative diagnoses (infection, cancer, atherosclerosis).
where:
• The preferred biopsy site is the tip of the nondominant temporal lobe tip. The biopsy should attempt to include a longitudinally oriented surface vessel.
