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ograms will have the typical ndings on biopsy As pointed out by Alrawi and colleagues, many patients prove to have an alternative condition, mainly an infectious encephalitis and brain or intravascular lymphoma, abscess, or Creutzfeldt-Jakob disease Tissue excised during an operation (or brain biopsy) for a suspected brain tumor, lymphoma, or white matter disease has revealed the characteristic vasculitis in some cases; in others the diagnosis has been made only at autopsy, the ndings coming as a distinct surprise The affected vessels are in the 100- to 500-mm range and are surrounded and in ltrated by lymphocytes, plasma cells, and other mononuclear cells; giant cells are distributed in small numbers in the media, adventitia, or perivascular connective tissue Infarction of brain tissue can be traced to thrombosis The meninges are variably in ltrated with in ammatory cells Sometimes only a part of the brain has been affected in one of our cases the cerebellum, in another, one frontal lobe and the opposite parietal lobe Among the most important considerations is the cerebral arteritis caused by varicella zoster virus, which can simulate in radiographic appearance granulomatous arteritis and giant cell arteritis (page 643) The viral form of arteritis follows or evolves in association with herpes zoster ophthalmicus On occasion, intravascular lymphoma may present a similar picture The clinical and radiologic appearance of brain arteritis also raises the question of sarcoidosis, which is sometimes limited to the nervous system, of brain lymphoma or intravascular lymphoma, of CADASIL, multiple sclerosis, 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 eosinophilia, increase in sedimentation rate or antineutrophil cytoplasmic antibodies (ANCA), or anemia As mentioned, brain lymphoma of the intravascular type and multiple sclerosis are diagnostic considerations from both a clinical and a radiologic perspective Some patients with isolated central nervous system angiitis (those presenting as an aseptic meningitis with multiple infarcts) have responded to corticosteroid and cyclophosphamide therapy (Moore) Takayasu Disease (Aortic Branch Disease, Occlusive Thromboaortopathy) This is a nonspeci c arteritis involving mainly the aorta and the large arteries arising from its arch It is similar in many ways to giant-cell arteritis except for its propensity to involve the proximal rather than the distal branches of the aorta Most of the patients have been young Asian women, but there are scattered reports of similar cases from the United States, Latin America, and eastern Europe The exact 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 disease The erythrocyte sedimentation rate is elevated in the early and active stages Later there is evidence of occlusion of the innominate, subclavian, carotid, vertebral, and other arteries The affected arteries no longer pulsate, hence the descriptive term pulseless disease However, it should be noted that in non-Asian individuals, the disease is usually due to atherosclerosis When renal arteries are involved, hypertension may result, and there may be coronary occlusion, which is often 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, dizziness, and hemiparetic and hemisensory syndromes are the usual neurologic manifestations (Lupi-Herrera et al) Several au-
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thors have emphasized the frequency of posturally induced neurologic symptoms as well as the relative infrequency of major strokes, despite multiple TIA-like spells The in amed vessels in the thorax are well demonstrated in radionuclide scans using gallium Pathologic studies disclose a periarteritis, often with giant cells and reparative brosis Many of the patients die in 3 to 5 years According to Ishikawa and colleagues, the administration of corticosteroids in the acute in ammatory stage of the disease improves the prognosis Reconstructive vascular surgery has helped some of the patients in the later stages of the disease Polyarteritis (Periarteritis) Nodosa In this disorder there is an in ammatory necrosis of arteries and arterioles throughout the body The lungs are usually spared, however, which is the basis of distinguishing this form of vasculitis from the allergic granulomatous angiitis of Churg and Strauss, mentioned above The vasa nervorum are frequently involved by the lesions of polyarteritis, giving rise to a mononeuropathy multiplex or to a symmetrical axonal type of polyneuropathy (see page 1137) Involvement of the CNS is unusual (occurring in fewer than 5 percent of cases) and takes the form of widespread microinfarcts; macroscopic infarction is a rarity The clinical manifestations vary: included are headache, confusion and uctuating cognitive disorders, convulsions, hemiplegia, and brainstem signs We have also observed acute spinal cord lesions The CNS lesions and brain hemorrhage are rare and usually occur in a setting of renal hypertension Wegener Granulomatosis This is a rare disease of unknown cause, affecting adults as a rule and favoring males slightly A subacutely evolving vasculitis with necrotizing granulomas of the upper and lower respiratory tracts followed by necrotizing glomerulonephritis are its main features Neurologic complications come later in one-third to one-half of cases and take two forms: (1) a peripheral neuropathy either in a pattern of polyneuropathy or, far more frequently, in a pattern of mononeuropathy multiplex (page 1138) and (2) multiple cranial neuropathies as a result of direct extension of the nasal and sinus granulomas into adjacent upper cranial nerves and from adjacent to pharyngeal lesions to the lower cranial nerves (see also page 1188) The basilar parts of the skull may be eroded, with spread of granuloma to the cranial cavity and more remote parts Cerebrovascular events, seizures, and cerebritis are less common neurologic complications Spastic paraparesis, temporal arteritis, Horner syndrome, and papilledema have been observed but are rare (see Nishino et al) The orbits are involved in 20 percent of patients, and lesions here simulate the clinical and radiologic appearance of orbital pseudotumor, cellulitis, or lymphoma Pulmonary granulomas, usually asymptomatic but evident on a chest lm, are also common The vasculitis implicates both small arteries and veins There is a brinoid necrosis of their walls and an in ltration by neutrophils and histiocytes The sedimentation rate is elevated, as are the rheumatoid and antiglobulin factors The presence in the blood of antineutrophil cytoplasmic antibodies (c ANCA) has been found to be relatively speci c and sensitive for Wegener disease but it may also be present in intravascular lymphoma A degree of therapeutic success in this formerly fatal disease has been achieved by the use of cyclophosphamide, chlorambucil, or azathioprine Cyclophosphamide in oral doses of 1 to 2 mg/kg per day has cured 90 to 95 percent of the cases In acute cases, rapidly acting steroids prednisone, 50 to 75 mg/day should be given in conjunction with the immunosuppressant drug(s)
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