The significance of color duplex ultrasonography for the diagnosis of temporal arteritis]

We examined the usefulness of color duplex ultrasonography in patients suspected of having temporal arteritis. Five patients, who were all aged 70 or older, developed a new onset of localized headache with temporal artery abnormalities, and had an elevated erythrocyte sedimentation rate of > 100 mm/hour. The final diagnoses were temporal arteritis in three patients, polymyalgia rheumatica in one, and probable healed temporal arteritis in one. Color duplex ultrasonography showed stenoses, which were confirmed histologically as well, in the superficial temporal artery of all patients. The characteristic findings of active temporal arteritis were, however, demonstrated in only three biopsy specimens, and in the remaining two the stenoses were thought to be related to previous arteritis. The hypoechoic halo, which has been reported to be a characteristic finding of color duplex ultrasonography in active temporal arteritis, was detected in only one patient with active temporal arteritis and another one with probable healed temporal arteritis. No stenoses were demonstrated in the superficial temporal arteries of 30 control subjects (20 with at least one risk factor of atherosclerosis and 10 without it). Color duplex ultrasonography can therefore be considered a powerful method for detecting stenoses in the superficial temporal artery. Its ability to identify their etiology is, however, unsatisfactory, so that temporal artery biopsy remains undoubtedly the most reliable test for etiological evaluation. We thus recommend color duplex ultrasonography as a supplementary method for the diagnosis of temporal arteritis, because it can provide useful information concerning the appropriate site of temporal artery biopsy.     

Concordance of bilateral temporal artery biopsy in giant cell arteritis.

OBJECTIVE: To determine whether the diagnostic sensitivity of bilateral temporal artery biopsy is superior to that of unilateral biopsy in cases of suspected temporal arteritis. MATERIALS AND METHODs: A retrospective analysis of the results of 60 bilateral temporal artery biopsies examined in an ophthalmic pathology laboratory. RESULTS: The histopathologic diagnosis in 13% of the biopsy pairs was discordant. There was a 5% chance of obtaining a positive biopsy result on the side opposite an initially negative biopsy result. CONCLUSIONS: Bilateral temporal artery biopsy is 5% more likely than unilateral biopsy to detect the characteristic histopathologic findings in patients with temporal arteritis.     

Giant cell arteritis (temporal arteritis): A report of four cases from north east India

Giant cell arteritis (GCA) is a common disease of the geriatric age group in the western world, with a prevalence of 0.2% in the fifty plus age group. It is an important cause of morbidity, with irreversible visual loss being the most ominous complication. This diagnosis is an important consideration in all cases of new onset headache in elderly subjects. Reports of giant cell arteritis are few and far between in the Indian subcontinent. In this report, we describe the clinical details of four cases of giant cell arteritis, detected at Guwahati, Assam. The four patients were in the 70-82 age group. Sex distribution was equal. All of them had polymyalgia rheumatica (PMR), with one case displaying an initial presentation as only PMR. Cardinal manifestation was a severe headache, frequently accompanied by scalp allodynia and abnormalities of the superficial temporal artery (STA) on examination. STA biopsy yielded histopathological confirmation in three patients. Permanent visual loss was noted in one patient. These cases highlight the importance of assessing the possibility of giant cell arteritis through appropriate clinical history, estimation of acute phase reactants and the judicious use of superficial temporal artery biopsy, to clinch the diagnosis.     

Biopsy negative giant cell arteritis–Revised diagnostic criteria: Giant cell arteritis diagnostic criteria

BackgroundDiagnosis of giant cell arteritis has traditionally relied on demonstration of pathologic changes on temporal artery biopsy.MethodTo highlight recent advances in large vessel imaging resulting in revised diagnostic criteria for giant cell arteritis.ConclusionWe call attention to the revised diagnostic criteria imaging evidence of extracranial large vessel thickening as an alternative to temporal artery biopsy in diagnosis of giant cell arteritis in a patient with heralding anterior fornix infarct.     

An Unusual Presentation of Rheumatoid Meningitis

BACKGROUND: Central nervous system involvement in rheumatoid arthritis can rarely occur in the absence of systemic disease. Rheumatoid meningitis has not been reported to present as spells of neurologic dysfunction. PATIENT AND METHODS: The authors describe a woman with a history of well-controlled rheumatoid arthritis who presented with headaches and spells of focal neurological dysfunction. Brain magnetic resonance imaging, brain biopsy, and temporal artery biopsy were required to make the diagnosis of rheumatoid meningitis with arteritis. RESULTS: Neuroimaging revealed abnormal leptomeningeal enhancement. Necrotizing granulomatous inflammation was seen on meningeal and brain biopsy. A temporal artery biopsy showed evidence of arteritis without giant cells. CONCLUSIONS: The possibility of central nervous system involvement by rheumatoid arthritis should be considered in patients with a history of rheumatoid arthritis even in the absence of systemic symptoms. Making the diagnosis may require meningeal and brain biopsy. The condition may be steroid responsive.     

Recurrent brachial plexus neuropathy and giant cell arteritis

A 73-year-old women presented with a recurrent form of sporadic brachial plexus neuropathy, the so-called Parsonage and Turner syndrome. This diagnosis is based on clinical and electromyographic findings. Interestingly a biopsy of the temporal artery demonstrated a giant cell arteritis. The clinical picture started 2 weeks after an upper respiratory tract illness. The possible viral etiology of giant cell arteritis is considered. We think an immunological rather than ischemic disturbance may have caused the recurrent brachial plexus neuropathy. This case report suggests that giant cell arteritis be considered in the investigation of the Parsonage and Turner syndrome.     

巨细胞动脉炎:颞动脉活检光镜研究

目的 探讨中国人巨细胞动脉炎(GCA)颞动脉活检的病理学特征和意义。方法 诊断为GCA的患者20例，非GCA患者7例为对照；以临床症状、颞动脉活检、类固醇激素治疗及随访结果作为诊断标准。结果 20例患者被诊为GCA，其中16例符合美国风湿病学会(ACR)的GCA诊断标准，18例显示活跃期血管炎，14例显示跳跃性损害，灵敏度、特异度和阳性预告值分别为90．0％、83．3％～94．8％。结论 提示颞动脉活检对GCA的诊断和治疗都具有重要意义。     

Late ipsilateral recurrence of ischemic optic neuropathy in giant cell arteritis.

A patient with arteriosclerosis, diabetes mellitus, and giant cell arteritis (GCA) treated continuously with low-dose prednisone developed anterior ischemic optic neuropathy (AION) at 5 and 13 months after clinical diagnosis of GCA. At the time of late recurrent AION, there were no systemic symptoms or elevations in acute phase reactants to signal active arteritis, yet temporal artery biopsy disclosed dramatic inflammation, forcing the presumption that the infarct was arteritic. Recurrent systemic symptoms and elevation of acute phase reactants are not reliable warning signs of reactivated GCA. In patients at high risk for corticosteroid complications, late biopsy may be a reasonable guide to corticosteroid weaning.     

Giant cell (temporal) arteritis: a treatable cause of multi-infarct dementia

Dementia occurs infrequently in patients with giant cell (temporal) arteritis (GCA). Three elderly women with biopsy-proven GCA showed abrupt cognitive decline during periods of clinically active GCA, 1 to 6 months after diagnostic temporal artery biopsy, during periods of corticosteroid taper. One patient had additional clinical signs of cerebral infarction and other ischemic phenomena. Reinstitution of higher oral doses of corticosteroids successfully prevented further cognitive losses and permitted gradual but incomplete improvement of cognitive function in 1 patient. Neuropsychologic data from 2 patients 7 to 10 months after temporal artery biopsy suggested multifocal cognitive impairment, and the 3rd patient appeared clinically to be globally, severely demented. Neuroimaging studies revealed multiple areas of infarction, predominantly in the posterior circulation territory. One patient had bilateral vertebral artery occlusions (digital subtraction angiography) and bilaterally reduced carotid system perfusion pressures (oculoplethysmography). There were no associated cardiovascular risk factors or family history of dementia in these patients.     

Recent Advances in Diagnostic Strategies for Giant Cell Arteritis

Giant cell arteritis (GCA) is a systemic vasculitis that affects the aorta and its major branches. Involvement of the ciliary artery can result in ischemic optic neuropathy and subsequent blindness, which is typically irreversible. If GCA is suspected, treatment with glucocorticoids should be initiated promptly to prevent further vision loss. However, given the need for prolonged therapy with glucocorticoids and the morbidity associated with their use, diagnosis should be confirmed. Clinical features and laboratory findings are neither sensitive nor specific for GCA. The mainstay of diagnosis remains histopathologic examination of a section of the superficial temporal artery. Several imaging studies have been used to evaluate the temporal artery but, at present, their utility as alternatives to a temporal artery biopsy is limited. Recent advances in imaging modalities have allowed detailed noninvasive imaging of the large arteries and are a useful adjunct for the diagnosis of GCA, particularly in patients with primarily large-vessel involvement in whom temporal artery biopsy is often negative.     

Internal carotid stenosis and giant cell arteritis

We report on the case of a 69-year-old man admitted with a transient ischemic attack preceded by a two months history of severe headache. Giant cell arteritis was diagnosed by means of temporal artery biopsy. Angiography showed an intra- and extracranial stenosis of the left internal carotid artery. The possible relationship between this stenosis and vasculitis is discussed and stroke as a clinical manifestation of the giant cell arteritis is reviewed.     

Immunohistology of temporal arteritis: phenotyping of infiltrating cells and deposits of complement components

Summary Deposition of complement factors, immunoglobulins and infiltrating cells was evaluated by immunohistochemical staining in 30 temporal artery biopsy specimens from patients suffering from temporal arteritis and/or polymyalgia rheumatica and in controls. In the temporal arteritis group infiltrating cells, classic complement, alternative complement and lytic complex activation were detected. In specimens from patients suffering from only polymyalgia rheumatica there was unexpected evidence of classic complement and lytic complex activation. We conclude that immuno-histochemistry provides support for the concept of temporal arteritis and polymyalgia being based on the same pathological process.     

Bilateral third nerve palsy and temporal arteritis

BACKGROUND: Bilateral oculomotor palsy is a rare manifestation of temporal arteritis, and to our knowledge only 1 case has been described in the literature. OBJECTIVE: To investigate a possible case of temporal arteritis in a patient with bilateral third nerve palsy. DESIGN: Case report and review. SETTING: University hospital. PATIENT: A 65-year-old man had subacute pupil-sparing bilateral third nerve palsy. RESULTS: Temporal artery biopsy findings and response to corticosteroids are consistent with temporal arteritis. CONCLUSION: Temporal arteritis is a rare cause of ophthalmoplegia in elderly persons and may be unrecognized.     

巨细胞动脉炎跳跃区域的超微结构特征及其意义

目的　研究巨细胞动脉炎 (GCA)颞动脉活检跳跃区域的超微结构特征及其意义。方法　对 2 0例GCA和 7例非GCA患者进行一侧颞动脉活检 ,根据光镜观察的结果 ,将GCA患者分为跳跃区域组 (14例 )和活跃期血管炎组 (6例 ) ;电镜观察 3组患者的血管超微结构 ,病理改变按 0～ 3分 4级评分。结果　超微结构的病理变化得分为 :GCA跳跃区域组 0分 1例 ,2～ 6分 9例 ,8～ 9分 4例 ;GCA活跃期血管炎组 7～ 15分 6例 ;非GCA组 0～ 1分 6例 ,5分 1例。经H检验 ,3组间差异有显著性 (P <0 0 1) ;两两比较发现 ,GCA跳跃区域组得分明显高于非GCA组 ,但低于GCA活跃期血管炎组得分。GCA跳跃区域组 ,其内膜超微结构变化突出 ,与GCA活跃期血管炎组比 ,差异无显著性 (P >0 0 5 )。结论　GCA颞动脉跳跃区域内有明显的病理改变 ,尤其是内膜。提示GCA跳跃区域超微结构的研究有助于GCA的诊断和治疗。     

Giant cell arteritis of the occipital arteries

CONTEXT: Occipital headache and nuchal pain may indicate the involvement of the occipital arteries (OCCA) in temporal arteritis (TA). Recently high resolution color coded sonography (CCDS) has greatly improved the imaging of small lumen arteries. OBJECTIVE: The aim of the present study was the demonstration of TA, of the OCCA in comparison with the superficial temporal artery (STA) by means of CCDS in patients with nuchal and occipital pain suspected of suffering from TA. DESIGN: Prospective study of 78 patients comparing CCDS findings of the OCCA and of the STA with the clinical diagnosis and the biopsy results. RESULTS: 27 patients received the diagnosis TA; there were 51 other diagnoses. CCDS of the OCCA reached a sensitivity in diagnosis of 63 % and a specificity of 100 % and in histology of 65 % and 100% respectively. CCDS of the STA reached a diagnostic sensitivity of 78 % and a specificity of 94% and of a histological sensitivity of 77 % and specificity of 82 %. Reversibility of CCDS abnormalities was monitored in 5 patients over a period of 13 to 42 days. CONCLUSIONS: Involvement of the OCCA in TA patients is a frequent finding and may be the only pathological phenomenon in some patients with nuchal pain, occipital headache and occipital scalp tenderness. CCDS of the STA and OCCA contributed to the diagnosis of TA with a high rate of perivascular hypoechogenic abnormalities (stenoses and occlusions) and a low rate of these abnormalities in the control patients. However, CCDS cannot differentiate between inflammatory and degenerative artery disease and has limitations concerning spatial resolution. Before CCDS may replace biopsy in clinical practice the accuracy of the criteria recommended above should be tested in larger groups of patients.     

Internal carotid artery occlusion caused by giant cell arteritis.

A case of hemiplegia in a 46 year old woman is described. Total occlusion of the right internal carotid artery was discovered at angiography. Because of persistent elevation of the ESR, and characteristic plasma protein abnormalities, biopsy of the temporal artery was carried out and demonstrated the typical features of giant cell arteritis.     

Accuracy of frameless and frame-based image-guided stereotactic brain biopsy in the diagnosis of glioma: comparison of biopsy and open resection specimen

OBJECTIVES: Tissue heterogeneity and rapid tumor progression may decrease the accuracy a prognostic value of stereotactic brain biopsy in the diagnosis of gliomas. Correct tumor grading is therefore dependent on the accuracy of biopsy needle placement. There has been a dramatic increase in the utilization of frameless image-guided stereotactic brain biopsy; however, its accuracy in the diagnosis of glioma remains unstudied. METHODS: The diagnoses of 21 astrocytic brain tumors were derived using image-guided stereotactic biopsy (12 frame-based, nine frameless) and followed by open resection of the lesion 1.5 (0.5-4) months later. The histologic diagnoses yielded by the biopsy were compared with subsequent histologic diagnosis from open tumor resection. RESULTS: Histology of 21 stereotactic biopsies accurately represented the greater lesion at open resection a median of 45 days later in 16 (76%) cases and correctly guided therapy in 19 (91%) cases. Biopsy accuracy of frameless versus frame-based stereotaxis was similar (89 versus 66%, p=0.21). In three (14%) cases, biopsy specimens were adequate to diagnose glioma; however, histology was insufficient for definitive tumor grading. Anaplastic oligodendroglioma (ODG) was under-graded as low-grade ODG in one (5%) case. Biopsy of new onset glioblastoma multiforme (GBM) yielded necrosis/gliosis and was termed non-diagnostic in one patient. Tumors <50 cm(3) were 8-fold less likely to accurately represent the grade of the entire lesion at resection compared with lesions <50 cm(3) (OR, 8.8; 95% CI, 0.9-100, p=0.05). DISCUSSION: Both frameless and frame-based MRI-guided stereotactic brain biopsy are safe and accurately represent the larger glioma mass sufficiently to guide subsequent therapy. Large tumor volume had a higher incidence of non-concordance. Increasing the number of specimens taken through the long dimension of large tumors may improve diagnostic accuracy.     

Highly located transverse lesion of the cord with quadriplegia caused by giant cell arteritis (author's transl)]

This is a report of a 64 years old patient who experienced a transverse lesion of the spinal cord with quadriplegia caused by giant cell arteritis. It is stressed that giant cell arteritis not only affects the temporal arteries but on principle any other artery of the body. The giant cell arteritis generally is accompanied with similar humoral findigns as a malignant neoplasm. Therefor in a few cases with high spinal cord lesion suspected to be caused by a malignant tumour the giant cell arteritis may be the real cause, if a spinal mass is excluded. In order to prevent irreversible paralytic defects a cortisone therapy has to be started with as early as possible. It must be carried on for a long period to prevent a restarting of the inflammatory process.     

Thrombocytosis in temporal arteritis rising platelet counts: a red flag for giant cell arteritis.

OBJECTIVES: To determine whether early recognition and detection of thrombocytosis in patients with giant cell arteritis can help secure an earlier diagnosis, and whether it can help differentiate cases of arteritic optic neuropathy from other forms of optic neuropathy. METHODS: Medical and ophthalmologic records from 1993 to 1998 of the authors' patients with biopsy-proven temporal arteritis versus the authors' patients with nonarteritic anterior ischemic optic neuropathy and idiopathic demyelinating optic neuritis were prospectively collected. Past and present blood analyses were collected, and platelet counts were compared between patients with giant cell arteritis and control populations. This was done to determine whether thrombocytosis could help with the diagnosis and differentiation of these different disease states. RESULTS: There was a significant difference in the frequency of thrombocytosis in patients with giant cell arteritis (13 out of 19 patients), with or without arteritic ischemic optic neuropathy, as compared with those with nonarteritic anterior ischemic optic neuropathy (zero out of 30 patients), idiopathic optic neuritis (zero out of 26 patients), and healthy age-matched controls (one out of 22 control subjects). This difference was especially helpful in patients whose sedimentation rates were within the normal range (adjusting for age). Also noted was the finding that the rise in the platelet counts was not acute, but rather it was a slow gradual increase for approximately 12 months before the onset of significant systemic or visual symptoms. CONCLUSION: Thrombocytosis should be considered an important marker in patients being referred for evaluation of ischemic optic neuropathy, diplopia, amaurosis fugax, headache, or even generalized malaise. Westegren sedimentation rates <50 mm/hr are often erroneously viewed as nondiagnostic or equivocal in the elderly and just followed. An over-the-phone review of patients' sedimentation rates, complete blood counts, and platelet counts can lead to expedited evaluation and treatment of patients who may be at high risk of visual loss from temporal arteritis. Thrombocytosis should lower a physician's threshold to acutely treat patients for possible arteritic ischemic optic neuropathy until the disease is definitely ruled out.     

拟诊为颞动脉炎的纤维肌性发育不良3例分析并文献回顾

目的 探讨纤维肌性发育不良的特点,提高神经内科医生对纤维肌性发育不良的认识。方法 收集3例被拟诊为颞动脉炎而行颞动脉活检并经病理诊断为纤维肌性发育不良的病例,复习并分析国内外相关病例报道。结果 3例均表现为头痛,其中2例仅累及颞动脉,另外1例合并双侧股浅动脉病变; 病理检查均表现为内膜纤维组织病变。结论 纤维肌性发育不良是一种血管病,几乎可累及全身血管床,可以“头痛、高血压病、腹痛、癫痫、神经功能缺损等”为首发症状; 神经内科医师应警惕此病,正确诊断及治疗。