[Vasculitis].
Vasculitis, restricted to the central nervous system, may be an iodiopathic disorder or may be secondary to toxins, infections or neoplasm. Alternatively, it may be part of a systemic autoimmune disease. Temporal arteritis presents with headache, jay claudication, polymyalgia rheumatica and constitutional symptoms. Hemispheric strokes occur in 10%, especially in the territory supplied by the posterior or the middle cerebral arteries. Corticosteroids are the treatment of choice. Isolated angitis of the central nervous system is an idiopathic recurrent inflammatory disease of the small and medium-sized vessels, confirmed to the brain and the spinal cord. Persisting headaches, encephalopathy with personality changes, cognitive disorders and memory disturbances as well as multifocal neurologic deficits resulting from recurrent cerebral ischemia and cranial nerve involvement are the most frequent presently symptoms of the disease. Angiography of the intracranial vessels presents segmental stenoses or dilatations of the medium and small vessels; a leptomeningeal biopsy demonstrating vasculitis is the only reliable diagnostic method. Treatment is instituted with prednisone and cyclophosphamide. At least one year of therapy is necessary. The typical dermatological manifestations of Behçet's disease are aphthous stomatitis, genital ulcerations and ocular inflammation. Neurological involvement has been described in 30% of all patients with Behçhet's disease. Sinus thrombosis presents with the signs of intracranial hypertension. A meningoencephalitis with headaches, disturbances of consciousness and focal neurologic symptoms is frequent; spinal cord involvement occurs. The treatment of choice for Behçet's disease consists of steroids in combination with chlorambucil or azathioprine. While polyarteritis nodosa and the Churg-Strauss syndrome most frequently involve the peripheral nervous system. CNS involvement is encountered in Wegener's granulomatosis. In lupus erythematosus and Sjorgren's syndrome, cerebral symptoms are rarely caused by a true vasculitis. In lupus erythematosus, nervous system involvement has been attributed to autoantibodies which interact directly with neurons [antineuronal antibodies] or indirectly lead to thrombotic vasculopathy by influencing the coagulation system [antiphospholipid antibodies].
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Neurologische Klinik mit klinischer Neurophysiologie, Alfried-Krupp-Krankenhaus, Essen.
D001528:Behcet Syndrome / Q000175:diagnosis; D002539:Cerebral Arterial Diseases / Q000175:diagnosis*; D015267:Churg-Strauss Syndrome / Q000175:diagnosis; D003095:Collagen Diseases / Q000175:diagnosis; D003937:Diagnosis, Differential; D013700:Giant Cell Arteritis / Q000175:diagnosis; D014890:Granulomatosis with Polyangiitis; D006801:Humans; D014657:Vasculitis / Q000175:diagnosis*
Vaskulitis.