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.     

Giant cell arteritis as a cause of first-ever stroke

The aims of this study were to assess how frequently giant cell arteritis (GCA) was a cause of first-ever stroke in 4,086 patients in the Lausanne Stroke Registry and to determine the risk factors, patterns, latency and current therapy at onset in patients with GCA plus stroke. GCA was recognized using the criteria of the American College of Rheumatology. We report on 6 patients (0.15%) with a histologically proven diagnosis of temporal arteritis and clinical and neuroradiological evidence of cerebral ischemia. The CT and MRI scans showed lacunar infarction in 3 patients, territorial infarction in 2 and were normal in 1. Stroke latency ranged from 0 to 2 months. All patients suffered from headache. We conclude that stroke is a rare, but dangerous, complication of GCA and that a combination of antiplatelet drugs and corticosteroids may be advisable for preventing stroke occurrence.     

Takayasu arteritis: a treatable cause of stroke in infancy

Takayasu disease is an arteritis of unknown etiology involving the aorta and its major branches. Untreated, 75% of patients die within two years. A 6-month-old black female presented with a right focal seizure, a flaccid right hemiparesis, decreased pulses in the right arm, a large left frontoparietal hypodense area on computed tomography, and an elevated erythrocyte sedimentation rate. Cerebral angiography demonstrated irregular dilatation of both carotid arteries and narrowing of the left middle cerebral artery. Aneurysm of the right sinus of Valsalva, dilatation of the aortic root, narrowing of the origins of both carotid arteries, and beading of the descending aorta were demonstrated by cardiac angiography. The patient was treated with prednisone (2 mg/kg/day) and azathioprine (1 mg/kg/day). There was gradual return of the erythrocyte sedimentation rate to normal over the ensuing 3-10 months, resolution of the hemiparesis, and acquisition of normal developmental milestones. Digital subtraction angiography revealed improvement in the appearance of the descending aorta and of the common carotid arteries with the disappearance of the arterial wall irregularities. Early diagnosis and vigorous immunosuppression may improve outcome in this rare and often fatal vascular disease in childhood.     

Giant cell arteritis (cranial arteritis,polymyalgia rheumatica)

Summary Giant cell arteritis, which is probably due to disturbed immune mechanisms, has a spectrum of clinical symptoms in elderly people. In nearly all cases such general signs as loss of appetite, loss of weight and fever are present. The sedimentation rate is almost without exception about 100 mm in the first hour. The two most frequent and typical clinical syndromes are polymyalgia rheumatica and cranial arteritis. The polymyalgia rheumatica is characterized by periarticular pain which is mostly symmetrical and accentuated in the shoulder girdle. Increasingly severe temporal headache and ocular disturbances are found with cranial arteritis in more than 50% of cases. A combination of both diseases is frequent. Other arterial branches are rarely involved. The course of the disease is over a period of 1 1/2 to 2 years. Treatment with corticosteroids is indicated mainly because of the severe ocular complications with blindness. It should begin immediately, be intensive and last over a long period. Regular followup is necessary over several years in order to avoid relapses.

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Zusammenfassung Die Riesenzellarteriitiden können sich — wohl als Ausdruck gestörter Immunmechanismen — durch ein Spektrum klinischer Symptome bei älteren Leuten manifestieren. Fast immer sind ein gestörter Allgemeinzustand, Anorexie, Abmagerung und Fieber vorhanden. Die Blutsenkungsreaktion ist so gut wie ausnahmslos sehr stark, meist auf Werte um 100 mm in der 1. Stunde, beschleunigt. Die zwei häufigsten und markantesten Symptomengruppen sind die Polymyalgia rheumatica und die Arteriitis cranialis. Die Polymyalgia rheumatica ist durch meist symmetrische, stammnahe, paraartikuläre Schmerzen, vorwiegend im Schultergürtelbereich, gekennzeichnet. Die Arteriitis cranialis ist durch oft temporale, zunehmend intensive Dauerkopfschmerzen und vor allem durch Sehstörungen in mehr als der Hälfte der Fälle charakterisiert. Ein kombiniertes Vorkommen von Polymyalgia rheumatica und Arteriitis cranialis ist häufig. Der Befall anderer Gefäßbezirke ist seltener, kann aber dann auch zu Durchblutungsstörungen des zentralen Nervensystems und der Extremitäten führen. Der spontane Krankheitsverlauf dauert 1 1/2–2 Jahre. Eine Therapie mit Corticosteroiden ist besonders wegen der schwerwiegenden oculären Komplikationen mit Erblindung indiziert. Die Behandlung muß sofort, intensiv und langdauernd sein, die Kontrollen im Hinblick auf Rezidive müssen sich über mehrere Jahre erstrecken.

     

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。     

Giant cell arteritis

Giant cell arteritis (GCA) is an immune-mediated vasculitis affecting individuals over 50 years of age. It is characterised by granulomatous inflammation that affects medium-sized and large arteries. The wide spectrum of clinical manifestations can be divided into those related to tissue ischemia from vascular lesions and those related to a systemic inflammatory response. The pathogenesis of these groups also appears distinct, with vascular lesion formation thought to be an adaptive immune response, and the systemic inflammatory reaction an innate immune response. Clinical suspicion of GCA must remain especially high in those with neurological or visual symptoms and if warranted, prompt treatment with high-dose corticosteroids is invaluable in halting disease progression.     

Transient epileptic amnesia in dementia: a treatable unrecognized cause of episodic amnestic wandering

The authors present two patients with dementia who displayed recurrent transient episodes of amnestic wandering and disorientation characterized by getting lost in familiar environments. At other times these patients did not wander or become disoriented. The inability to recall any information during these episodes, and the marked difference of the episodic amnesia exacerbations from the progressive amnesia characteristic of Alzheimer disease seen in these patients led to their evaluation. These clinical episodes and the bilateral interictal epileptiform electroencephalographic changes found in both patients led to the diagnosis of transient epileptic amnesia, a syndrome that can be diagnostically elusive. These transient amnestic wandering events subsided after treatment with antiepileptic drugs in both patients. The authors suggest that transient wandering of this type may be caused by ictal events or postictal confusional states. This report emphasizes the importance of recognizing transient epileptic amnesia as an easily treatable cause of episodic behavioral abnormalities responsive to antiepileptic therapy, especially in those patients who have a markedly inconsistent pattern of wandering, disorientation in familiar settings, and amnesia exacerbation manifested by no recall of the emotional stress of getting lost or of any information during these episodes. Recognition of this type of behavioral disruption and its proper treatment can lead to improved quality of life for these patients, maintain these patients in their homes and out of chronic care institutions longer, and facilitate the community's and caretaker's interactive roles with the patient.     

Neurologic disease in biopsy-proven giant cell (temporal) arteritis

Neurologic findings were studied in 166 consecutive patients with biopsy-proven giant cell (temporal) arteritis. Neurologic problems occurred in 51 patients (31%): neuropathies (23), TIA/strokes (12), neuro-otologic syndromes (11), tremor (6), neuropsychiatric syndromes (5), tongue numbness (3), and myelopathy (1). Neuro-ophthalmologic problems occurred in 35 patients (21%): amaurosis fugax (AF) (17), permanent vision loss (PVL) (14), scintillating scotoma (8), and diplopia (3). Abnormalities in large arteries in 52 patients (31%) included bruits and diminished pulses. The carotid artery was involved in 31 patients (bilateral in 58%). Overall, 35% of patients with carotid disease had TIA/stroke, AF, or PVL.     

Peripheral neuropathic syndromes in giant cell (temporal) arteritis

Of 166 consecutive patients with histologically confirmed giant cell (temporal) arteritis (GCA) seen during a 3-year period, 23 (14%) had clinically diagnosed peripheral neuropathic syndromes temporally coincident with clinically active GCA. Electromyography and nerve conduction studies were performed in 16, confirming abnormalities in all. Of the 23 patients, 11 had a generalized peripheral neuropathy, nine had multiple mononeuropathies, and three had a mononeuropathy. The nerves affected as mononeuropathies were the median, ulnar, peroneal, tibial, and sural nerves, and the C-5 and L-5 nerve roots. Angiography, performed in two patients, demonstrated widespread arteritis involving the lower limbs and, after 3 months of oral corticosteroid treatment in one of these patients, an amputation specimen showed chronic arteritis.     

Survival and cause of death in Alzheimer''s disease and multi-infarct dementia

Survival and causes of death of 218 patients with Alzheimer's disease (AD) and of 115 patients with multi-infarct dementia (MID) were examined. The patients were originally found in a community-based epidemiological survey of dementia, and all patients with AD or MID alive on the prevalence day were included. The 6-years survival rate for AD was 21.1% vs. the expected rate 48.5%, that for MID 11.9% vs. 45.2% expected. A comparison of relative survival rates suggested that MID carries a less favorable survival prognosis than AD. The mean durations were: AD 5.7 years and MID 5.2 years; median duration being 5 years in both diseases. The excess mortality in both AD and MID was independent of age. In AD, the survival rate decreased with increasing severity of dementia, while in MID the mortality was the same regardless of the severity of the dementia. The dementia disorder was the underlying cause of death in 68% of AD patients, and in 38% of MID patients, bronchopneumonia being the most frequent immediate cause of death in both groups. As a cause of death, acute cerebrovascular accidents occurred more often in MID patients than in the general population of comparable age. Malignant diseases were less frequent as a cause of death in both dementia groups than in the general population.     

Relation between multiple infarcts and vascular (multi-infarct) dementia

77 patients with multiple infarcts in computerized tomography have been investigated. In 10 patients no definitive stroke could be found in the history, 2 had TIAs only. In 20 cases a PRIND and in 45 a completed stroke could be observed. More than one stroke was only present in 28 patients. In 68 of the patients without remarkable aphasia or decreased level of consciousness the psychiatric findings showed 37 patients with and 31 without a dementia. The CT findings showed in the patients with dementia significantly more often infarcts in the dominant hemisphere, whereas atrophy was found in a similar distribution in both groups.     

Epidemiology of multi-infarct dementia

Multi-infarct dementia (MID), thought to result from the cumulative effect of repeated episodes of cerebral infarction, represents a sizable proportion of the dementia cases in the population. The frequency of MID as the cause of dementia is commonly quoted as 12-20%, but a more conservative figure on the order of 10% is suggested from prospective epidemiologic studies. Its prevalence increases steadily with advancing age, and a male predominance has been noted. Geographical variations in prevalence of MID may reflect true geographic/ethnic differences or lack of uniformity in the use of diagnostic criteria. Risk factors possibly associated with dementia after stroke include advancing age, previous cerebral or myocardial infarction, an atherothrombotic stroke mechanism, and cerebral atrophy as detected by imaging studies. In order to clarify the role of cerebrovascular disease as a cause of dementia, future epidemiologic studies should define the entity in precise terms, and place emphasis on clearly establishing the temporal relationship between stroke and dementia; new onset of dementia should be correlated with the clinical and anatomic features of the stroke.     

Types of multi-infarct dementia

A series of 79 patients with multi-infarct dementia (MID) were divided into 2 groups designated cortical MID and subcortical MID, according to whether the computed tomography (CT) scan showed the presence or absence of cortical infarcts, and an absent to mild or moderate to severe degree of white matter low attenuation (WMLA). Cortical MID was characterized by repeated atherothrombotic and cardiogenic strokes, moto-sensory hemiparesis, a severer degree of aphasia, and abrupt onset of cognitive failure. Subcortical MID typically showed the following features: lacunar strokes, bulbar signs including dysarthria, pure motor hemiparesis, depression and emotional lability. WMLA was found in all patients with subcortical MID but also in over 60% of those with cortical MID. In the 2 groups CT scans showed equal frequencies of deep infarcts. When divided according to severity of WMLA, 92% of patients in the cortical MID group and 44% of those in the subcortical MID group were found to have at least one cortical infarct on the CT scan. Although cortical and subcortical MID differed in several clinical features, they did not show major differences in the risk factors for stroke, and clearly overlapped each other as regards ischaemic scores and the findings in neurological examinations and CT. Thus, it is still an open question whether cortical MID and subcortical MID, including the lacunar state and Binswanger's disease, are 2 distinct entities or merely represent the expression of biological variation while having the same etiopathogenesis.     

The nucleus basalis of Meynert in multi-infarct (vascular) dementia

Summary The number and nucleolar volume of nerve cells within the nucleus basalis of Meynert were estimated in 10 patients with Alzheimer's disease, 12 with multi-infarct dementia, 9 with a mixed Alzheimer/multi-infarct dementia and in 10 age-matched controls. As reported previously in Alzheimer's disease, both the number and nucleolar volume of surviving cells was reduced, whereas in multi-infarct dementia no significant change in either measure was noted. In patients with Alzheimer's disease/multi-infarct dementia the loss of nerve cells and reduction in nucleolar volume varied greatly in severity from patient to patient according to the relative balance of Alzheimer and vascular type pathological changes present within each patient.Supported in part by a grant from the North Western Regional Health Authority to one of us (BM)     

Giant cell arteritis and its variants

In addition to cranial arteritis, the three other variants of giant cell arteritis may also lead to neurological symptoms: polymyalgia rheumatica, occult giant cell arteritis, and inflammatory aortic arch syndrome of elderly persons. This survey discusses the nosological, clinical, diagnostic, and therapeutic aspects of giant cell arteritis and its subgroups on the basis of experience with over 100 cases.