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Query: UMLS:C0042373 (vascular disease)
 17,070 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 53-year-old man presented with a lumbosacral polyradiculoneuropathy and developed fluctuating encephalopathy suggestive of multifocal small vessel disease. Postmortem examination demonstrated multifocal vascular occlusion by undifferentiated cells confined to the intravascular space. Extravascular spread was found only in spleen and liver. The presence of lysozyme and absence of factor VIII in the cytoplasm of the malignant cells confirmed their histiocytic nature. This patient had an unusual intravascular form of malignant histiocytosis that must be included in the differential diagnosis of multifocal vascular disease.
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PMID:Intravascular malignant histiocytosis mimicking central nervous system vasculitis: an immunopathological diagnostic approach. 675 80

For the assessment of the anatomical distribution of cerebral amyloid angiopathy (CAA) as well as that of amyloid plaques (AP), 955 post-mortem brain specimens from 653 patients (aged 14 to 95 years) were made available from a general hospital. Using histological and immunohistochemical techniques, we demonstrated amyloid angiopathy chiefly in the occipital, parietal and temporal regions. Senile plaques were mostly found in parietal, temporal and occipital areas. In our cases, neurofibrillary tangles were rarely prominent in the regions examined. There was often a significant difference in the frequency of amyloid angiopathy and of plaques in distinct areas between men and women. Congophilic angiopathy was associated with senile plaques in 82.3%, and neurofibrillary tangles with plaques in 76.9%. In comparison, the correlation of amyloid plaques with CAA (32.5%) and of plaques with neurofibrillary tangles (11.5%) was less pronounced. There was a strong association of cerebral vascular amyloid with age in both sexes, it was more pronounced in women than in men. Unexpectedly, CAA showed a decrease in frequency in men in the 7th and 8th decade of life. In addition, there was a positive correlation between the amount of CAA and spontaneous hemorrhages, but no correlation with ischemic encephalopathy. Immunohistochemistry showed that cerebral amyloid was neither amyloid of the AA- nor of the ATTR-(Antitransthyretin-, formerly anti-AF)-type. An antibody to the beta-protein (anti-A beta) showed cerebral vascular deposits to be congruent with the Congo red method, whereas senile plaques stained weakly and neurofibrillary tangles could not be stained at all with these antibodies.
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PMID:Cerebral amyloid angiopathy. Frequency, significance and immunohistochemistry. 769 58

Binswanger's type encephalopathy is characterized by progressive dementia and diffuse subcortical ischemic lesions associated with arteriosclerosis. Hypertension is believed to be a major pathogenic factor in causing this encephalopathy but there are some cases of the encephalopathy not suffering from hypertension. In 1985, Yamamura et al. and Fukutake et al. reported familial cases of normotensive juvenile Binswanger's type encephalopathy with alopecia and lumbago, and considered it to be possibly a new clinical syndrome. We reported three cases of relatively young-onset (under the age of 40) Binswanger's type encephalopathy with persistent hypotension. All three patients suffered from neither alopecia nor lumbago. Patient (male aged 40) had repeated episodes of ischemic stroke and had progressive dementia. Patients 2 (male aged 41) and 3 (male aged 34) were not in a state of dementia, but had a history of transient ischemic attacks, and at present are completely symptom-free. Though there were no risk factors for cerebrovascular disease in these cases, the repeated episodes of ischemic stroke and the existence of small multiple lacunes in the basal ganglia on CT and MRI suggest that the white matter damage was principally due to a vascular disorder. In these cases, persistent hypotension was characteristic and might be a factor for the induction and exacerbation of this encephalopathy. These three cases are different from the classic form of Binswanger's type encephalopathy based on hypertension. Normotensive cases have been described before, but our cases do not seem to fall into this category because the blood pressure constantly remained hypotensive.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Binswanger's type encephalopathy without alopecia and lumbago in young hypotensive patients]. 772 96

Although cerebral amyloid angiopathy is a well-known cause of cerebral lobar hemorrhage, subacute dementia, seizures, and acute encephalopathy without lobar hemorrhage are infrequently recognized as manifestations of this disease. In this report, we describe a case of cerebral amyloid angiopathy in a 74-year-old woman who had subacute progressive dementia and a superimposed rapid acute neurologic deterioration associated with seizures and the presence of cerebral edema on computed tomographic scans and leukoencephalopathy and cortical petechial hemorrhages on magnetic resonance imaging. A diagnosis of cerebral amyloid angiopathy in conjunction with small cortical infarcts and petechial hemorrhages was confirmed by antemortem biopsy. This clinical and radiologic picture is being increasingly recognized as characteristic of cerebral amyloid angiopathy.
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PMID:Cortical petechial hemorrhage, leukoencephalopathy, and subacute dementia associated with seizures due to cerebral amyloid angiopathy. 773 Dec 59

In four patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes (MELAS) in which mutated mitochondrial deoxyribonucleic acid was seen, hypertrophic cardiomyopathy and angiopathy was demonstrated by echocardiography, dipyridamole stress scintigraphy, and cardiac catheterization. On stress scintigraphy with dipyridamole, three patients showed hypoperfusion in the early image and a "filling-in" pattern in the late image. However, coronary angiography did not demonstrate narrowing of the large vessels in these patients. Light and electron microscopy of endomyocardial biopsy specimens indicated abnormal mitochondria, with marked increase in the number and size of mitochondria in endothelium. Modified Gomori's trichrome staining in biopsied endomyocardial specimens revealed a red-purple deposit similar in appearance of the ragged-red fibers in skeletal muscle, a characteristic finding of mitochondrial disease. Deterioration of complex I in the mitochondrial electron transfer system, which is widely observed in various mitochondrial diseases, appeared in biopsied skeletal muscle of our patients, indicating deficiency of some subunits of complex I. These results indicate that mitochondrial diseases such as MELAS show not only cardiomyopathy but also angiopathy. We speculate that proliferation of mitochondria leads to narrowing of the lumen of arterioles, which might be responsible for the ischemic findings observed scintigraphically.
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PMID:Cardiomyopathy and angiopathy in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and strokelike episodes. 794 44

This article summarizes various concepts (Binswangers encephalopathy, multiinfarct-dementia, lacunar state, mixed dementia, dementia due to amyloid angiopathy or vasculitis) and classifications (DSM, ICD, ADDTC) on vascular dementia. It reviews historical, clinical, and diagnostic aspects (i.e. neuroradiology, SPECT, PET) as well as therapeutic approaches. The confusing nomenclature on vascular dementia is discussed, considering especially the non-convincing concept of multiinfarct-dementia that often has been misused as a synonym for vascular dementia. Multiinfarct-dementia is now restricted to a syndrome of vascular dementia due to several large vessels strokes. A current definition and classification of vascular dementia as suggested by NINDS-AIREN international workshop is described. It defines criteria consistent with the diagnosis of "possible", "probable" and "definite" vascular dementia based on clinical, radiologic and neuropathologic features. The criteria of "probable" vascular dementia include all the following: 1. the presence of dementia and cerebrovascular disease defined by focal signs on neurologic investigation and evidence of relevant cerebrovascular disease by brain imaging (multiple lacunae, extensive white matter lesions, multiple large-vessels infarcts or a strategically placed infarct) 2. A relationship between dementia and cerebrovascular disease (onset of dementia within 3 months following a recognized stroke; abrupt deterioration, stepwise progression). This classification of vascular dementia emphasises pathogenetic aspects and includes dementia resulting from small- and large-vessels disease as well as hypoperfusion, haemorrhagic dementia and dementia due to still unknown factors. Operational criteria for the frequent Binswangers encephalopathy--a prototype of vascular dementia--are presented. Thereby a basis for further research and discussion in this exciting area should have been formed.
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PMID:[Progress in vascular dementia--an overview of vascular dementia from past to new concepts]. 805 Jul 74

We present the clinical, morphological and neuropathological findings in a 44-year-old male suffering from the acquired immunodeficiency syndrome (AIDS) (CDC stage IV C2) who presented with rapidly progressive right-side hemiparesis and developed hemianopia and aphasia. Scans showed multiple, not contrast-enhancing, not space-occupying echo-intensive lesions in T2-weighted MR-imaging. No hint for an opportunistic infection, necrotizing vasculitis or vascular disease was found. All therapeutic regimens failed and 8 weeks after onset of neurological symptoms the patient died because of cardiorespiratory arrest. Post-mortem examination excluded opportunistic infection, progressive multifocal leukoencephalopathy, lymphoma, vasculitis and ischemia of the brain. In the presence of an unusually high amount of HIV-infected macrophages at immunohistochemical examination, the overall pathological findings were atypical both for HIV encephalitis and HIV leukoencephalopathy. We describe a pathologically distinct new form of HIV associated encephalopathy.
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PMID:A pathologically distinct new form of HIV associated encephalopathy. 815 18

Sixty-five patients with a diagnosis of acute encephalitis or encephalopathy were discharged from a regional neurological unit over a 17-year period. Investigation during the acute illness, or subsequent clinical and laboratory observations, yielded a definite or probable diagnosis in 34 of these patients, including herpes simplex encephalitis (8 cases), encephalitis due to other identified viruses (7 cases), vascular disease (7 cases) and multiple sclerosis (4 cases). In these 34 patients, mortality relating to the presenting illness was 50% and a further 29% had significant long-term neurological morbidity. In the other 31 patients, no cause for the encephalopathy was identified, despite extensive investigation. These patients had an alteration in conscious state, often with recurrent seizures (45%), focal neurological signs (52%), pyrexia (65%), abnormal electroencephalogram (85%) and cerebrospinal fluid lymphocytosis (80%). During follow-up (6 months to 15 years) none had recurrent encephalopathy, and 65% eventually made a complete recovery, although delayed by seizures in 6% and psychiatric illness in 13%. The mortality in this group relating to the acute illness was 6%. Overall, nearly half the patients with a discharge diagnosis of acute encephalitis or encephalopathy had a good prognosis for recovery, following a monophasic illness of undetermined cause.
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PMID:Acute encephalopathy: diagnosis and outcome in patients at a regional neurological unit. 820 5

An autopsy case of portal systemic encephalopathy and senile dementia of the Alzheimer type coexisting in a 77-year-old man is described. The patient had suffered recurrent episodes of delirium after a subtotal gastrectomy for gastric carcinoma. He died of DIC 45 months after the gastrectomy. A pathological examination revealed a vascular plexus around the liver which might have served as collateral circulation. Neuropathologically, spongy necrosis and Alzheimer type II changes of astrocytes were found in the basal ganglia and fronto-occipital cortices. In the same anatomical regions, only immunohistological staining using antibody against amyloid beta-protein and the periodic-acid methenamine silver method revealed abundant neuriticplaques, cerebral amyloid angiopathy and diffuse plaques. We discussed the clinicopathological findings in this case.
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PMID:An autopsy case of coexisting portal systemic encephalopathy and senile dementia of the Alzheimer type. 830 83

Vascular dementia is the most common cause of dementia in the elderly after Alzheimer's disease. Many forms of vascular dementia have been described: multi-infarct dementia, lacunar dementia, Binswanger's subcortical encephalopathy, cerebral amyloid angiopathy, white matter lesions associated with dementias, single infarct dementia, dementia linked to hypoperfusion and haemorrhagic dementia. The difficulty of diagnosing vascular dementia must not be underestimated and an international consensus is needed for epidemiological studies. The NINCDS-AIREN group has recently published diagnostic criteria. The State of California Alzheimer's Disease Diagnostic and Treatment Centers also proposed some which differ from the NINCDS-AIREN criteria in considering only ischaemic vascular dementia and not other mechanisms such as haemorrhagic or hypoxic lesions. Most studies stress hypertension as the most powerful risk factor for all forms of vascular dementia. The incidence rate ranges from 7 per 1000 person-years in normal volunteers to 16 per 1000 person-years in hypertensive patients. No therapeutic attempt has influenced the course of the disease once the dementing condition is established. The only effective approach is preventive treatment. The objective of the SYST-EUR Vascular Dementia project is to confirm that the treatment of isolated systolic hypertension is able to reduce its incidence.
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PMID:Assessing vascular dementia. 853 23


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