Giant-Cell Arteritis Involving Small Meningeal and Intracerebral Vessels

McCormick, Harry M.; Neubuerger, Karl T.

doi:10.1097/00005072-195807000-00007

Cited by 66 publications

(12 citation statements)

References 7 publications

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“…One problem is that the diagnosis of giant cell arteritis was not confirmed bioptically in all published cases. Therefore in some papers an isolated granuloma tous angiitis of the CNS could be assumed [4,5,22,23]. Further, etiologic relationships between arteritis tempo ralis and brain infarction seem to be unclear in some reported patients.…”

Section: Discussionmentioning

confidence: 99%

Temporal Arteritis with Cerebral Complications: Report of Four Cases

Büttner

Heye²,

Przuntek³

1994

Eur Neurol

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We report on four patients with a histologically proven diagnosis of arteritis temporalis and clinical and/or neuroradiological evidence of severe focal cerebral ischemia due to intracranial vasculitis. While one patient suffered from a transient ischemic attack, CCT and MRI scans of the other patients showed multiple lacunar infarctions, combined with territorial infarctions in two cases. Necropsy in one patient demonstrated generalized giant cell vasculitis in large and small cerebral vessels. We suppose that the cerebral involvement was provoked by insufficient steroid therapy of arteritis temporalis in two patients. In one case, remission could be achieved by a combination of high-dose steroids and cyclophosphamide; one further patient remitted under lower steroid dosage. Steroid therapy was ineffective in two patients, one of whom died due to secondary complications. We conclude that central nervous system affection is a rare but dangerous complication of arteritis temporalis and may present as cerebral micro- and macroangiopathy.

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Section: Discussionmentioning

confidence: 99%

Temporal Arteritis with Cerebral Complications: Report of Four Cases

Büttner

Heye²,

Przuntek³

1994

Eur Neurol

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“…The first microscopic description was by Horton, Magath, and Brown (1932). Definitive surveys of the patho- logical findings at biopsy and necropsy have been published by several workers (Harrison, 1948;Heptinstall et al, 1954;McCormick and Neuberger, 1958). The gross features of the disease are not characteristic.…”

Section: Discussionmentioning

confidence: 99%

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confidence: 99%

Electron microscopy of giant-cell (temporal) arteritis.

Smith¹

1969

Journal of Neurology, Neurosurgery & Psychiatry

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Definitive histological studies of giant-cell (temporal) arteritis have been published by several workers (Harrison, 1948;Heptinstall, Porter, and Barkley, 1954;McCormick and Neuberger, 1958). The lesions are fairly uniform and have involved all layers of many different arteries and veins-large and small, intracranial as well as extracranial. The distinctive histological finding in this disease has been a giantcell reaction with disruption of the internal elastic membrane. Aetiological factors are unknown. Biopsy specimens from a patient with a classical clinical and pathological case of giant-cell (temporal) arteritis have been examined electron microscopically. This report describes the ultrastructural details of this diseased artery. CASE REPORTA 64-year-old man was referred to the Saint Louis University Hospital in July 1967 by Dr. James Mulvill, Alton, Illinois, with a main complaint of right frontotemporal headaches for three months. He also had noted malaise, weight loss, and mild intermittent fever. His past history was remarkable for a partial loss of vision in the right eye for eight years with an attack of gout two years ago. He took digitalis and probenecid regularly and quinidine intermittently. He denied arthralgia and myalgia, and had no symptoms of cerebral dysfunction. He never noted redness or tenderness of the temporal regions.Examination revealed normal vital signs. The temporal arteries were firm and cord-like with weak pulsations, but were not tender, and there were no signs of inflammation surrounding them. There were no bruits and pulses were good in the extremities. Funduscopic examination showed mild arteriosclerotic changes bilaterally with old perimacular exudates on the right. There was a central scotoma of the right visual field. There was no evidence of recent ocular disease. The cause of his old visual loss was not ascertained. The remainder of the general and neurological examination was normal.Laboratory studies were normal except for erythrocytic sedimentation rate of 116 mm/hr, a diabetic glucose tolerance test, and serum uric acid 7-0 mg%. There was no eosinophilia, serum protein electrophoresis was Hartford Foundation. normal, and cerebrospinal fluid was normal. Mercury-203 chlormerodrin brain scan was normal. An electroencephalogram showed mild, diffuse, slow dysrhythmia. Radiographs of chest, skull, and paranasal sinuses were normal except for bilateral maxillary sinusitis.The left superficial temporal artery was biopsied. The lumen was obliterated in some regions. Histological sections (Fig. 1) demonstrated a marked inflammatory reaction associated with proliferative changes of intima, media, and adventitia. Numerous eosinophils, lymphocytes, and plasma cells were present. The intima had a nodular proliferation. At the junction of the intima and media many giant cells were seen. Some were of foreignbody type, but most were of the Langhan's type. No tuberculoid granulomata were noted.The patient was treated with prednisone 30 mg per day for one month and then the drug w...

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“…[1][2][3][4][5][6][7][8][9] In 1988, Calabrese and Mallek 5 suggested diagnostic criteria for PCNSV that included development of a neurological deficit unexplained by other processes, an angiogram with characteristic features of vasculitis, or a central nervous system (CNS) biopsy specimen showing vasculitis. Because of the more invasive nature of CNS biopsy, angiography has become preferred for evaluating patients with suggestive symptoms.…”

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confidence: 99%

Primary central nervous system vasculitis: analysis of 101 patients

Salvarani

Brown

Calamia

et al. 2007

Annals of Neurology

562

578

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Objective: To analyze the clinical findings, response to therapy, outcome, and incidence of primary central nervous system vasculitis (PCNSV) in a large cohort from a single center. Methods: We retrospectively studied 101 patients with PCNSV, selected by predetermined diagnostic criteria, who were seen during a 21-year period. This was a collaborative study by five departments at a large multispecialty clinic. Clinical findings and outcomes were compared among patients categorized by method of diagnosis, response to therapy, survival, and degree of disability. An annual incidence rate was calculated. Results: Seventy patients were diagnosed by angiography and 31 by central nervous system biopsy. Three histological patterns were observed during biopsy. Although most patients responded to therapy, an increased mortality rate was observed. Relapses occurred in one fourth of patients. Mortality rate and disability at last follow-up were greater in those who presented with a focal neurological deficit, cognitive impairment, cerebral infarctions, and angiographic large-vessel involvement but were lower in those with prominent gadolinium-enhanced lesions when evaluated by magnetic resonance imaging. The annual incidence rate of PCNSV was 2.4 cases per 1,000,000 person-years.

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Giant-Cell Arteritis Involving Small Meningeal and Intracerebral Vessels

Cited by 66 publications

References 7 publications

Temporal Arteritis with Cerebral Complications: Report of Four Cases

Temporal Arteritis with Cerebral Complications: Report of Four Cases

Electron microscopy of giant-cell (temporal) arteritis.

Primary central nervous system vasculitis: analysis of 101 patients

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