Among the most important considerations in this disease is the cerebral arteritis caused by varicella zoster virus of the ophthalmic division of the trigeminal nerve; it simulates in imaging appearance granulomatous arteritis and giant cell arteritis. On occasion, intravascular lymphoma may present a similar picture and sympathomimetic agents, as mentioned earlier, cause a vasculopathy with segmental narrowing of cerebral vessels that has many similarities. The clinical and radiologic appearance of brain arteritis also raises the question of sarcoidosis, which is sometimes limited to the nervous system, of CADASIL, antiphospholipid antibody syndrome, or of the polyarteritis (allergic granulomatous angiitis) described by Churg and Strauss. Unlike some of these diseases, however, the lungs and other organs are spared; there is no systemic eosinophilia, increase in sedimentation rate or antineutrophil cytoplasmic antibodies (ANCA), or anemia.
Some patients with isolated angiitis of nervous system presenting as an aseptic meningitis and multiple cerebral infarcts have responded to corticosteroid and cyclophosphamide therapy (Moore, 1994), and we have used this combined approach from the time the diagnosis is established. The severity and configurations of the process are so variable that judging the effects of treatment is difficult, but our untreated patients have uniformly deteriorated or died without treatment.
This is a nonspecific inflammatory chronic arteritis involving the aorta and the large arteries arising from its arch. It is similar in some ways to giant cell arteritis except for its propensity to involve the proximal rather than the distal branches of the aorta. The name of the disorder comes from Takayasu’s 1908 article. Most of the patients have been young Asian women, but there are numerous reports from the United States, Latin America, and Europe. The etiology has never been ascertained but an autoimmune mechanism is suspected.
Constitutional symptoms such as malaise, fever, anorexia, weight loss, and night sweats usually introduce the illness. The erythrocyte sedimentation rate is elevated in the early and active stages. Later there is evidence of occlusion of the brachiocephalic, subclavian, carotid, vertebral, and other arteries that may be asymptomatic or cause neurologic ischemic symptoms. The affected arteries no longer pulsate, hence the descriptive term pulseless disease. When renal arteries are involved, hypertension results, and there may be coronary occlusion, which may be fatal. Involvement of the pulmonary artery may lead to pulmonary hypertension. Coolness of the hands and weak radial pulses are common indicators of the disease and headaches are frequent. Blurring of vision, especially upon physical activity or fever, dizziness, and hemiparetic and hemisensory syndromes are the usual neurologic manifestations (Lupi-Herrera et al). The frequency of posturally induced neurologic symptoms has been emphasized, as well as the relative infrequency of major strokes despite multiple TIA-like spells. The inflamed vessels in the thorax are revealed by radionuclide scans using gallium. Pathologic studies disclose a periarteritis of the large vessels, often with giant cells and reparative fibrosis.
Many of the patients die in 3 to 5 years. According to Ishikawa and colleagues, the administration of corticosteroids in the acute inflammatory stage of the disease improves the prognosis. Reconstructive vascular surgery has helped some of the patients in the later stages of the disease.