Autopsy findings of atheromatous embolism to the spinal cord]

To study vascular lesions of the spinal cord in the elderly, a pathological study of atheromatous emboli in the spinal cord was done. Among 604 patients examined at autopsy, atheromatous emboli of the spinal cord were found in 7 (1.2%). The average age of these patients was 76 years. The most common underlying disorders in these patients were hypertension, severe aortic atherosclerosis, and diabetes mellitus. Atheromatous emboli were also often found in the arteries of the kidneys, spleen, pancreas, and colon. The small arteries of the spinal arachnoid at the lumbosacral level were most frequently affected by the atheromatous emboli. Two patients had spinal-cord infarctions associated with atheromatous emboli: one had a cystic infarction of the lateral column at the T9 segment, and the other had cystic infarctions of the lateral column at the C7 and T3 segments. The low incidence of spinal cord infarction was attributed to good collateral circulation in the spinal cord. Atheromatous embolism should be considered as a possible cause of vascular lesions of the spinal cord in elderly persons with aortic atherosclerosis.     

Characterization of a new HLA-B39 allele, B*3913, in a Brazilian Caucasian

We report a case of paraplegia in the immediate postoperative period following right bilobectomy for carcinoma of the lung. An epidural catheter had been inserted following induction of anaesthesia and an infusion of bupivacaine 0.15% was used for postoperative pain relief. Magnetic resonance imaging failed to reveal any spinal or epidural haematoma or spinal cord ischaemia. The patient developed respiratory failure on the third postoperative day and required assisted ventilation. He was weaned from the ventilator on day 15. Two days later he sustained a cardiac arrest and died. Post-mortem examination demonstrated spinal cord infarction and severely stenosed spinal arteries. The thoracotomy position and/or intra-operative hypotension might have compromised the blood flow to the spinal cord and although suspected as a possible cause, the use of epidural analgesia was not implicated.     

Diagnosis of canine babesiosis by Percoll gradient separation of parasitized erythrocytes

PURPOSE: To report the development of a bilateral ocular ischemic syndrome despite corticosteroid treatment in a patient with giant cell arteritis. METHOD: Case report. RESULTS: Despite receiving high-dose intravenous methylprednisolone and oral prednisone for biopsy-proven giant cell arteritis that presented as a severe anterior ischemic optic neuropathy in the right eye, a patient developed progressive ocular ischemia in that eye as well as an ocular ischemic syndrome in the fellow eye. CONCLUSIONS: Some patients with giant cell arteritis, possibly patients with other underlying systemic vasculopathies, are refractory to what should be adequate treatment with systemic corticosteroids and may develop a bilateral ocular ischemic syndrome.     

Giant cell arteritis involving the lower limbs

Giant cell arteritis is an inflammatory disease that can affect the arteries anywhere in the body. Two cases are reported in which the arteries of the lower limbs were involved. Intermittent claudication with a walking distance of only 30 m was the inaugural manifestation in both cases. A biopsy of the superficial femoral artery provided the diagnosis in the first case. Ergotamine toxicity was considered initially in the second case. Acute ischemia and gangrene requiring amputation can complicate giant cell arteritis of the lower limbs and consequently corticosteroid therapy in an effective dose should be given as soon as the diagnosis is made. The inflammatory arterial lesions improve under therapy, but irreversible fibrosis with stenosis can develop if treatment is initiated late.     

Giant cell arteritis

Giant cell arteritis is a form of vasculitis affecting medium- and large-size arteries, with a predilection for vessels arising from the aortic arch. The origins of giant cell arteritis are unknown. However, ethnic affinities are prominent, with the majority of cases involving white, elderly patients. In addition, there is evidence of a familial pattern and an association with HLA-DR4, suggesting a genetic predisposition in select cases. With an increase in the elderly population, clinicians must become cognizant of this age-related malady and its treatment to ensure quality care for elderly patients.     

The enigma of giant cell arteritis: multidisciplinary management of two cases

BACKGROUND: Giant cell arteritis is an enigmatic disease that is characterized by chronic granulomatous inflammation of the walls of large and medium-sized arteries. The process has a predilection for the extradural cranial arteries, which include the ophthalmic and the posterior ciliary arteries. A multisymptom disease of older individuals, giant cell arteritis often manifests challenging issues and diagnostic dilemmas. CASE REPORTS: We illustrate two cases with initial symptoms of intermittent headache, malaise, and decreased visual acuity that were incorrectly diagnosed or ultimately misdiagnosed. The first case represents a patient who was diagnosed as having migraine headache and an erythrocyte sedimentation rate (ESR) that was interpreted by the attending physician as too low to warrant temporal artery biopsy. The second case is that of a patient who had a history of headaches, jaw claudication, and numerous medical evaluations. CONCLUSION: Giant cell arteries is an enigmatic disease with multiple manifestations. The differential diagnoses can range from temporal mandibular joint dysfunction to tension headache. Imminent vision loss as a sequelae of this condition warrants careful review of ocular and constitutional history and prompt treatment.     

Factors influencing completion of treatment among tuberculosis patients in Mbarara District, Uganda

We report one case of acute cervical spinal cord infarction characterized by motor impairment of the upper limbs with respect of the lower limbs. It occurs infrequently. The purpose of this paper is to discuss the clinical, neurophysiological and magnetic resonance imaging findings, and to review the presumed mechanisms of spinal cord infarction.     

Cotton-wool spots and the early diagnosis of giant cell arteritis

BACKGROUND: Giant cell arteritis is a common cause of severe visual loss in older individuals. Patients often present to the ophthalmologist having already lost vision in one eye. Detection of early ophthalmoscopic signs that precede irreversible visual loss in giant cell arteritis would allow preventative treatment in an otherwise frequently blinding disease. METHODS: Case presentations. RESULTS: Seven patients with mild visual symptoms and results of an ophthalmologic examination significant for cotton-wool spots were found to have giant cell arteritis. On specific questioning, six of seven patients described constitutional symptoms consistent with giant cell arteritis. Six patients had an abnormally elevated Westergren erythrocyte sedimentation rate. Temporal artery biopsy confirmed giant cell arteritis in six patients. The seventh patient received a diagnosis of polymyalgia rheumatica. Prompt treatment with corticosteroids led to preservation of vision and uneventful resolution of the cotton-wool spots in all seven patients. CONCLUSION: Cotton-wool spots are an early ophthalmoscopic finding in giant cell arteritis and can precede severe visual loss. Recognition of the significance of cotton-wool spots, use of laboratory studies, and prompt treatment may preserve vision in an otherwise frequently blinding disease.     

Large vessel vasculitis (giant cell arteritis, Takayasu arteritis)

Giant cell arteritis and Takayasu arteritis are separate but similar idiopathic diseases clinically characterized by constitutional symptoms, shared surrogate markers of systemic inflammation and indistinguishable granulomatous pan-arteritis of large vessels. This review emphasizes and analyses changing perceptions about the diseases. Recent series suggest that aortic involvement in giant cell arteritis may be more common than was previously appreciated. The case for and against inflammatory arthritis in giant cell arteritis is discussed. Ethnic new geographical variation in Takayasu arteritis-disease expression is reviewed. New philosophies of treatment are presented for both diseases. Prognosis in giant cell arteritis and its relationship to treatment is analysed. The utility of the laboratory for diagnosis and monitoring disease activity is appraised for each.     

Meditation in palliative care--a practical tool for self-management

A thirty four-year-old-white man in good health developed an acute anterior wall myocardial infarction (AMI), Killip II with normal coronary arteries. No thrombolytic therapy was given. Selective angiography revealed multiple aneurysms in mesenteric and renal arteries. The diagnosis of polyarteritis nodosa (PAN) was performed. AMI in PAN is secondary to arteritis with thrombosis, or to atherosclerosis due to steroid therapy. This case, having multiorgan vascular aneurysms involvement without previous cardiac symptomatology nor steroid therapy, presented as his first cardiac complication an AMI with normal coronary arteries probably due to selective arteritis.     

Temporal artery involvement revealing AL amyloidosis and IgD monoclonal gammopathy

We report a patient who presented with clinical symptoms suggesting giant cell arteritis. A diagnosis of AL amyloidosis was made on temporal artery biopsy. Temporal artery involvement is unusual in AL amyloidosis, but its frequency may be underestimated. Another uncommon finding was the presence of an IgD monoclonal gammopathy without diagnostic features of multiple myeloma.     

Spinal epidural hematoma progressing to Brown-Sequard syndrome: report of a case

Horton giant cell arteritis of the legs is a very rare and unusual occurrence. A very interesting case of acute ischemia of the right leg in a 51-year-old woman treated with emergency thromboendarterectomy is described. Histological findings led to the diagnosis of Horton giant cell arteritis and the patient was submitted to steroid and vascular therapy. Good results were obtained and follow-up after five years confirms the good general condition of the patient.     

Basic studies of adreno-sympathetic function test-glucagon test and histamine test (author''s transl)

Sera from 74 patients with polymyalgia rheumatica or giant cell arteritis or both were tested for immune complexes by using the Raji cell radioimmunoassay. Levels in patients with active disease were higher than in patients whose disease had become inactive. There was no difference in levels of immune complex-like materials between patients with polymyalgia rheumatica alone and those with giant cell arteritis. Density gradient analysis of one serum showed immune complex-like materials mainly in the 19S region. Immune complexes may be important in the pathogenesis of these conditions.     

Subarachnoid neurocysticercosis with occlusion of the major intracranial arteries: case report

Cysticercosis is the most common parasitic disease affecting the central nervous system. Stroke is a recognized complication of neurocysticercosis, occurring in 2 to 12% of cases, mostly in the form of small lacunar infarcts. We report a case of hemiparesis and aphasia in a 51-year-old Hispanic woman, which was secondary to complete occlusion of the left internal carotid and bilateral anterior cerebral arteries. Magnetic resonance imaging demonstrated the presence of enhancing subarachnoid material surrounding these occluded cerebral arteries, providing antemortem, noninvasive documentation of the inflammatory meningeal cysticercotic reaction that was presumably responsible for the occlusive arteritis causing the cerebral infarction. This represents the third reported case of internal carotid artery occlusion and the first reported case of anterior cerebral artery occlusion secondary to neurocysticercosis.     

Ischemic spinal cord disorders

Infarcts in the territory of the anterior spinal artery usually are located in the Adamkiewicz artery. Onset is either sudden or progressive with clinical signs of deficit and spinal pain of short duration. The extent and distribution of the deficit depends on the extension and the level of the spinal lesions. A perfect knowledge of the spinal cord vascularization is required to discern the complex and variable clinical expression. Unlike cerebral vascular events, emboli or cardiac origin are rarely involved in spinal cord events. Aortic pathology, including surgery of the aorta, is however of major importance in ischaemic spinal cord syndromes. Several reports have shown that atheroma causing spinal cord ischaemia are not located in the perispinal arterial network but lie on the aorta and its spinal branches. Dissection of the aorta is found responsible in 2 to 8% of the cases. Isthma stenosis can also give spinal cord signs and syphylitic arteritis is exceptionally encountered. Other rare causes are gas emboli and fibrocalcium emboli.     

Immunosuppressive agents in Horton's disease. Which drug for which indication?

Corticosteroids remain the cornerstone treatment of giant cell arteritis. Steroid-related toxicity and needs for alternative therapeutic regimens in patients experiencing recurrent active or steroid-resistant diseases, are the main reasons for searching a steroid-sparing agent. Regarding disease pathogenesis, immunosuppressive agents might be useful drugs. Methotrexate is the most widely studied agent in this indication. However, available data are incomplete and studies have included only polymyalgia rheumatica or both polymyalgia rheumatica and giant cell arteritis affected patients. Results of these studies evoke the possibility of using methotrexate in steroid-dependent or resistant diseases, as well as a steroid-sparing agent in order to taper off and stop steroids quickly; with an acceptable toxicity. Azathioprine may be used to control steroid-dependant or resistant diseases, but toxicity appears to be higher. An open prospective study compared azathioprine and methotrexate and concluded to the superiority of methotrexate. Available data concerning cyclophosphamide and cyclosporine A are too incomplete. A satisfactory steroid-sparing agent in giant cell arteritis is still unknown. Currently, methotrexate, and probably azathioprine, appear to be the most useful agents in this indication. However, there is no demonstration that using an immunosuppressive agent in giant cell arteritis could reduce steroid-related morbidity.     

Magnetic resonance imaging of a case of central nervous system tuberculosis with tuberculous arachnoiditis and multiple tuberculomas

A 62-year-old woman developed headache, vomiting and fever. On admission to hospital, she showed an imparied level of consciousness, diplopia on left lateral gaze, bilateral hearing loss and left hemiparesis. Cranial contrast computed tomography (CT) revealed basal meningeal enhancement. Lumbar cerebrospinal fluid (CSF) showed an increase in cell count (80/mm3) and total protein (3000 mg/dl), and a decrease in glucose (65 mg/dl) in comparison with blood sugar (173 mg/dl). Polymerase chain reaction was positive for Mycobacterium tuberculosis in the CSF. She was diagnosed as having tuberculous meningitis and was treated with anti-tuberculous chemotherapy. Her level of consciousness recovered and other clinical signs improved gradually the first month after admission. However, in spite of the combination of anti-tuberculous chemotherapy and steroid therapy, her combination of anti-tuberculous chemotherapy and steroid therapy, her consciousness level worsened again in association with paraplegia at the sixth week after admission and magnetic resonance imaging (MRI) revealed multiple tuberculomas, spinal arachnoiditis and spinal cord infarction. On T2-weighted imaging some of the tuberculomas showed a central hyperintense area (a central bright core) with an isointense periphery, which was surrounded by a hyperintense area. The lesion appeared hypointense with an isointense rim on T1-weighted imaging, showing a ring enhancement on post-contrast T1W imaging. The spinal cord infarction was situated at the third thoracic cord, which corresponded to the borderline of spinal artery perfusion. This is a rare case of progression of spinal arachnoiditis and spinal cord infarction during anti-tuberculous chemotherapy, and who had tuberculoma with a central bright core on MRI.     

Vertebral body infarction as a confirmatory sign of spinal cord ischemic stroke: report of three cases and review of the literature

BACKGROUND: Acute spontaneous spinal cord syndromes often remain etiologically ambiguous despite extensive diagnostic efforts. In the previous literature five cases are described with acute spinal cord syndromes interpreted as spinal cord ischemic strokes because of association with vertebral body infarctions on MRI. CASE DESCRIPTIONS: Three cases are presented, and the literature is reviewed. In addition to an extensive diagnostic battery including an initial MRI without pathological signs, follow-up MRI at different time intervals from the onset of symptoms showed T2 hyperintense signals in vertebral bodies. Patient 1, who had plaques in the abdominal aorta, had suffered a thoracolumbar spinal infarction; this and a concomitant infarction of the left portion of T-12 could be demonstrated on follow-up MRI on day 12. Patient 2, who had incomplete transverse spinal artery syndrome below T-3, had an abnormal signal at the T-2 level of the spinal cord on follow-up MRI on day 5; this was one segment above infarction of the dorsal area of T-3, corresponding to the ascending course of the medullary artery. The spinal cord of patient 3, who had a posterior spinal artery syndrome below T-11, was unremarkable on follow-up MRI on day 14, but a T2 hyperintense signal was noted in the dorsal area of T-10. CONCLUSIONS: Vertebral body infarction represents the only confirmatory sign for the otherwise exclusionary diagnostic procedure for spinal cord ischemic stroke and must be searched for on follow-up MRI as a key to correct diagnosis.     

A long-term follow-up study of serial magnetic resonance images in patients with delayed encephalopathy after acute carbon monoxide poisoning

We report a case of vasculitis in a 67-year-old woman who successively developed over a four-month period clinical manifestations suggestive of rheumatoid arthritis, lupus, sicca, syndrome and finally giant cell arteritis. All her symptoms resolved promptly upon discontinuation of enalapril and none recurred over the five-year follow-up period. The only residual manifestation is Jaccoud's arthropathy of the hands.     

The incidence of giant cell arteritis in Olmsted County, Minnesota: apparent fluctuations in a cyclic pattern

OBJECTIVE: To investigate trends in the incidence of giant cell arteritis over a 42-year period in Olmsted County, Minnesota. DESIGN: Population-based incidence study. SETTING: Olmsted County, Minnesota. METHODS: All incidence cases of giant cell arteritis first diagnosed between 1950 and 1991 were identified using the unified record system at Mayo Clinic. Age- and sex-specific incidence rates were calculated using the number of incidence cases as the numerator and population estimates as the denominator. Overall rates were age- and sex-adjusted to the 1980 United States white population. The annual incidence rates were graphically illustrated using a 3-year centered moving average. RESULTS: Between 1950 and 1991, 125 Olmsted County residents (103 women and 22 men) were diagnosed with giant cell arteritis. The age- and sex-adjusted incidence per 100,000 persons 50 years of age or older was 17.8 (95% CI, 14.7 to 21.0); incidence was significantly higher in women (24.2 [CI, 19.5 to 28.9]) than in men (8.2 [CI, 4.8 to 11.6]). Age-specific incidence rates increased with age (P < 0.0001). The annual incidence rates increased significantly over the study period (P = 0.002) and appear to have clustered in five peak periods, which occurred about every 7 years. A significant calendar-time effect was identified; it predicted an increase in incidence of 2.6% (CI, 0.9% to 4.3%) every 5 years. CONCLUSIONS: Our observation of a regular cyclic pattern in incidence rates over time supports the hypothesis of an infectious cause for giant cell arteritis. Similar studies in other populations are needed to confirm our findings.