[Non-specific angiitis and the central nervous system]

Central nervous system manifestations of systemic lupus erythematosus are reported in 25 to 60 p. cent of cases and include mental disturbances, epilepsy, focal deficits, and headache. Cerebrospinal fluid (CSF) changes are inconstantly observed. Cerebral scintigraphy may be useful. CT Scan imaging shows infarcts, hemorrhages, or cortical atrophy. Cerebral angiography is usually normal. Pathological examination shows frequent arteriolar lesions in the CNS but their appearance is rarely that of an angiitis. The mechanism of the CNS lesion involves both angiitis and antineuronal antibodies. Diagnosis is difficult when presenting signs are those of CNS involvement, particularly as the ESR can be normal. Serum complement and anti-DNA antibody levels are frequently normal when there is an isolated CNS involvement. Overall prognosis is poor in cases with CNS lesions. The use of corticoids is discussed. Cerebral angiitis is an exceptional finding during the course of rheumatoid arthritis and scleroderma. Central neurological manifestations of Sharp's and Gougerot-Sjögren's syndromes have recently been reported. CNS lesions are reported in 10 to 20 p. cent of patients with panarteritis nodosa and include mental disorders and disturbance of vigilance, epilepsy, focal deficits, meningeal signs and headache. The CSF is often normal. The CT scan provides images of infarcts, hemorrhages or cortical atrophy. Cerebral angiography may show segmental stenoses and distal occlusions. Pathological examination of the CNS shows mainly lesions in the intracerebral and leptomeningeal arterioles and small caliber arteries. Treatment is by corticoids and immunosuppressors. Angiitis of the CNS is rare and of late onset in Wegener's granulomatosis. Hypersensitivity angiitis rarely affects the CNS, those cases where it is involved being of poor prognosis and probably related to panarteritis nodosa. CNS manifestations in giant cell arteritis and Takayasu's arteritis result from neck artery lesions. The CNS is affected in 20 p. cent of cases in Behcet's disease with resulting isolated aseptic meningitis or a meningo-encephalomyelitis, and intracranial hypertension. The CSF is nearly always abnormal. Cerebral angiography should be directed towards the search for cerebral thrombophlebitis. Pathologically lesions predominate in the brain stem. Meningeal lesions are almost constantly present. Neurological involvement is of poor prognosis, and early corticotherapy must be instituted. The nosological autonomy of Buerger's thromboangiitis obliterans is questioned. Lymphomatoid granulomatosis is a particular type of angiitis in which malignant cell infiltration occurs. The CNS is affected in 20 p. cent of cases, and whatever the treatment the prognosis is poor. Granulomatous angiitis of the CNS has the distinctive feature of lesions affecting the CNS vessels either exclusively or predominantly. The antemortem diagnosis is based on biopsy of the leptomeninges, and treatment with corticoids and immunosuppressors may prove effective.