1/62. Subcortical arteriosclerotic encephalopathy (Binswanger's disease). A vascular etiology of dementia.A 51-yearold man with moderate intermittent hypertension had a rapidly progressive, profound dementia in the absence of significant localizing neurological signs. Postmortem examination disclosed the vascular alterations and diffuse white matter degeneration which characterize subcortical arteriosclerotic encephalopathy (SAE) or Binswanger's disease. The case underscores the need to consider vascular disease as an etiology of dementia -- even in the absence of focal neurological deficit.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
2/62. Neurofibromatosis, stroke and basilar impression. Case report.Neurofibromatosis type 1 (NF1) can virtually affect any organ, presenting most frequently with "cafe au lait" spots and neurofibromas. Vasculopathy is a known complication of NF1, but cerebrovascular disease is rare. We report the case of a 51-year-old man admitted to the hospital with a history of stroke four months before admission. On physical examination, he presented various "cafe au lait" spots and cutaneous neurofibromas. neurologic examination demonstrated right-sided facial paralysis, right-sided hemiplegia, and aphasia. Computed tomography scan of head showed hypodense areas in the basal ganglia and centrum semiovale. Radiographs of cranium and cervical spine showed basilar impression. Angiography revealed complete occlusion of both vertebral and left internal carotid arteries, and partial stenosis of the right internal carotid artery. A large network of collateral vessels was present (moyamoya syndrome). It is an uncommon case of occlusive cerebrovascular disease associated with NF1, since most cases described in the literature are in young people, and tend to spare the posterior cerebral circulation. Basilar impression associated with this case may be considered a pure coincidence, but rare cases of basilar impression and NF1 have been described.- - - - - - - - - - ranking = 2keywords = vascular disease (Clic here for more details about this article) |
3/62. Painless aortic dissection presenting as hoarseness of voice: cardiovocal syndrome: Ortner's syndrome.Most of the neurological manifestations of the aortic dissection are due to neuronal ischemia secondary to either extension of the dissection process into a branch artery, or compression of an artery by the false lumen of the dissecting aortic hematoma. However, the enlarging false lumen may directly compress on an adjacent nerve, causing neuronal injury resulting in neurological symptoms. This may particularly take place when a distal intimal tear does not decompress the false lumen, resulting in formation of an expanding blind pouch. About 10% of aortic dissections are painless and may present with symptoms secondary to the complications of the dissection. Although cardiovocal syndrome, or Ortner's syndrome (hoarseness of voice due to involvement of recurrent laryngeal nerve in cardiovascular diseases) has been described with aortic dissection, it has not been reported as an initial presenting feature of this disorder. This report describes the first case of painless aortic dissection presenting with hoarseness of voice, the cardiovocal syndrome. The hoarseness remained the only symptom throughout the entire course of the disease. The aortic dissection was not suspected initially. During surgical exploration, the recurrent laryngeal nerve was found compressed by the false lumen at the level of aortic arch. Aortic root replacement was performed successfully, resulting in complete resolution of the hoarseness. The neurological manifestations of aortic dissection, and the cardiovocal syndrome, are discussed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
4/62. popliteal artery entrapment syndrome: specific aspect.A case of popliteal artery entrapment syndrome (PAES) is reported. A non smoker, 63-year-old man, consulted for severe claudication of the lower limb, with a sudden onset. There was no past history of vascular disease. Neither the arteriography nor the arterial doppler led to definite diagnosis. In our case, only the C.T. scan was contributive to the diagnosis. The age, 63, at which this abnormality became symptomatic, the abrupt appearance of ischaemic symptoms and the embryologic type of the arterial stenosis were particular. The surgical management was the only therapeutic option.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
5/62. The C.C.A. syndrome (congenital contractural arachnodactyly): a new differential syndrome for Marfan's syndrome and homocystinuria.The first case in the dental literature of congenital contractural arachnodactyly (C.C.A. syndrome) is presented. This newly delineated syndrome is an autosomal dominant heritable disorder of connective tissue. Its similarities to Marfan's syndrome and homocystinuria, as well as other syndromes, are discussed. The lack of cardiovascular disease, specific ocular anomalies, and mental retardation are presented in the differential diagnosis of the C.C.A syndrome with Marfan's syndrome and homocystinuria.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
6/62. The cognitive syndrome of vascular dementia: implications for clinical trials.dementia is common among patients with cerebrovascular disease, particularly in a setting of one or more clinically evident strokes. Prior cohort and case studies have suggested that the cognitive syndrome of vascular dementia is characterized by predominant executive dysfunction, in contrast to the deficits in memory and language function that are typical of patients with alzheimer disease. The course of cognitive decline may also differ between those dementia subtypes, with many, but not all, patients with vascular dementia exhibiting a stepwise course of decline caused by recurrent stroke and most patients with alzheimer disease exhibiting a gradually progressive course of decline. The findings of prior studies of the cognitive syndrome of vascular dementia must be interpreted with caution, however, because of (1) possible inaccuracies in the determination of the dementia subtype and the loss of precision that might result from pooling heterogeneous subgroups of patients with vascular dementia, (2) difficulties inherent in identifying a pattern of strengths and weaknesses in patients who are required to have memory impairment and other deficits to meet operationalized criteria for dementia, and (3) the use of limited test batteries whose psychometric properties are incompletely understood. Specific questions that should be addressed by future studies are discussed.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
7/62. Diagnostic features of the Favre-Goldmann syndrome.Extensive retinal vascular disease was noted in three patients from two families with Favre-Goldmann syndrome. In addition to classical features they had pronounced leakage from some retinal vessels. Vessels were either opaque ('sclerotic') or non-perfused. Cystoid macular oedema was a contributing cause of decreased vision. Two of the three patients showed a discrepancy on electroretinography between single-flash photopic amplitudes and flicker fusion frequency. This may be characteristic of Favre-Goldmann syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
8/62. Raynaud's phenomenon, anticentromere antibodies, and digital necrosis without sclerodactyly: an entity independent of scleroderma?We describe 4 women of 43, 73, 84, and 86 years with Raynaud's phenomenon, severe digital necrosis, and high serum levels of anticentromere antibodies without skin thickening or internal organ sclerosis. Investigations revealed no diabetes or arterial vascular disease leading to arterial obstruction. Histologic examination did not show any dermal sclerosis or calcinosis. The intravenous infusions of prostaglandin reversed the ischemic lesions in 3 patients. These cases suggest that the triad Raynaud's phenomenon, anticentromere antibodies and necrosis of digits without sclerodactyly and sclerosis of internal organ should be considered as an entity distinct from scleroderma with sclerosis. For this entity we propose the name RACAND syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
9/62. Uveitis masquerade syndromes.BACKGROUND: Incorrect diagnosis of the uveitic masquerade syndromes (UMS) may have severe consequences. In this study, the frequency, clinical manifestations, and informative diagnostic tests for UMS are described. DESIGN: Retrospective observational case series. PARTICIPANTS: Forty patients with UMS were identified in a cohort of 828 consecutive patients with uveitis. The mean follow-up was 4.5 years. methods: A review of clinical, laboratory, photographic, and angiographic records was performed. MAIN OUTCOME MEASURES: Clinical features, associated systemic diseases, diagnostic procedures and their role in the diagnostic process, and systemic and visual outcomes. RESULTS: Nineteen patients had intraocular malignancy (48% of all with UMS; 2.3% of all with uveitis), mainly intraocular lymphoma (n = 13) and leukemia (n = 3). The ophthalmologist was the first to recognize malignant disease in 11 of 19 patients (58%). Of 21 patients with nonmalignant UMS, 10 had an ocular vascular disease and 5 a hereditary ocular disorder. The patients with malignant UMS were older than those with nonmalignant UMS (average age, 50 vs 44 years, P: < 0.05). During follow-up, 9 of 19 patients with malignant UMS died. The most informative diagnostic procedure for malignant UMS was intraocular fluid analysis; for nonmalignant UMS, fluorescein angiography. The cytologic analysis of intraocular fluids yielded the best results for diagnosing intraocular malignancies (7 of 11 patients, 64%); the association of negative cytologic results with the recent administration of systemic corticosteroids was noted. immunophenotyping of the aqueous confirmed the diagnosis of hematologic malignancy for 3 of 5 patients examined. panuveitis was the most frequent manifestation of malignant UMS. Intraocular lymphomas presented with isolated vitreitis (n = 6), chorioretinal lesions (n = 5) and iris infiltration (n = 2). Clinical presentation of nonmalignant UMS was diverse but consisted mainly of abnormalities of the retinal vasculature. CONCLUSIONS: UMS was diagnosed in 5% of the patients with uveitis at a tertiary center. Despite the variety of underlying disorders and different clinical presentations, a high frequency of malignant and vascular diseases was found. awareness of the clinical manifestations of UMS and application of the correct diagnostic procedures should promote timely diagnosis and treatment, which are essential not only for visual acuity but also for the life of the patient.- - - - - - - - - - ranking = 2keywords = vascular disease (Clic here for more details about this article) |
10/62. A newly recognized syndrome--radiation-related bronchiolitis obliterans and organizing pneumonia. A case report and literature review.bronchiolitis obliterans and organizing pneumonia (BOOP) is a syndrome that has been associated with a variety of underlying disorders, including infection, collagen vascular diseases and toxic fume inhalation. Rarely, however, BOOP has been associated with radiation- or chemotherapy-induced pulmonary toxicity. Over the past 3 years, several case series have reported BOOP in the unique setting of radiation in breast cancer patients. This study describes our experience with this newly recognized syndrome and a review of the English-language literature on this syndrome.- - - - - - - - - - ranking = 1keywords = vascular disease (Clic here for more details about this article) |
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