Disease Information for Cerebral vasculitis/arteritis: Definition

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  • Cerebral angiitis causes rapid dementia; Primary angiitis of the CNS is a rare vasculitis and is restricted to the small and medium-sized arteries of the CNS; Small leptomeningeal vessels are preferentially involved, although there may be occasional involvement of the internal carotid or vertebral arteries; Healing lesions appear in the same biopsy specimen as active lesions; The average age at onset is 45 years; male predominance; Clinical onset is subacute, with headache, weakness, and delirium or dementia; Focal signs then develop; fewer than 25 percent of patients have fever or other systemic signs or symptoms; clinical subgroups: “True” primary angiitis of the CNS lasts longer than 3 months and is characterized by focal and diffuse neurological involvement and typical pathological findings; “Benign angiopathy” of the CNS has an acute onset with normal CSF, angiographic changes, and a benign course ; occasional reports of an associated systemic vasculitis, but this is unusual; diagnostic criteria include (1) unexplained neurological deficit, (2) documentation of an arteritic process within the CNS by angiography or biopsy, and (3) absence of systemic vasculitis; sedimentation rate is elevated; LP/ CSF shows lymphocytic pleocytosis or elevated protein concentration and Diffuse slowing of the EEG is seen in 81 percent; Computed tomography (CT) and magnetic resonance imaging (MRI) may show infarcts in multiple vascular territories; Biopsy-proven primary angiitis of the CNS is almost never seen when both the lumbar puncture and neuroimaging studies are normal, so these studies may be useful in deciding in whom to perform invasive studies; Brain Biopsy is the diagnostic test of choice; disorder is rapidly progressive, even fulminant: Confusion, loss of memory, and delirium are common; untreated patients, 90 percent eventually develop focal CNS signs, which are multiple in 80 percent; Focal weakness of at least one limb is present in more than 50 percent of patients; Focal or generalized seizures; Aphasia and hemiparesis ; progression to stupor or coma follows often; Extrapyramidal and cerebellar or brainstem signs can develop; Myelopathy occurs with differential diagnosis of multiple sclerosis; Prognosis: primary angiitis of the CNS is dismal, with inexorable progression to death in an average of 45 days; corticosteroid therapy with immunosuppressive agents has produced remissions of significant duration; death in 95 percent of untreated patients, 46 percent of patients receiving corticosteroids, and 8 percent of patients treated with corticosteroids and immunosuppression; current recommendations include high-dose corticosteroid treatment with oral cyclophosphamide; Aggressive treatment should be continued for 12 months after signs of the illness have disappeared

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