Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0085584 (encephalopathy)
 18,178 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Review of literature data concerning genetically determined blood vessel diseases is presented. These disorders are a cause of a process similar to Binswanger's disease but occurring familial. Recurrent TIA, ischemic strokes and other kind of blood supply disturbances lead to numerous and various intensity vasogenic brain tissue damage, particularly in the white matter. Progressive neurological symptoms and dementia form the picture of subcortical leukoencephalopathy in several members of family. Moyamoya disease, fibromuscular dysplasia, hereditary hemorrhagic telangiectasia, hereditary cerebral hemorrhage with amyloidosis, pseudoxanthoma elasticum, two types of subcortical encephalopathy in Japan, HERNS and CADASIL are described.
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PMID:Genetically determined vascular diseases. 1070 39

The clinicopathological findings reported by Binswanger are insufficient to qualify as distinct entity the condition named "Binswanger's disease", and subsequently by Olszewski (1962) "subcortical arteriosclerotic encephalopathy (SAE) (Binswanger's type)". A short summary of the characteristic pathological, clinical and neuroimaging features of SAE is reported. The white matter changes detected by neuroimaging must be considered aspecific, since identical changes may be found in normal elderly as well as in patients with different diseases: different biochemical mechanisms can undoubtedly underlie identical neuroimaging patterns. Two other relevant points are noteworthy: the occurrence of pathological features of SAE in other diseases (CADASIL, pseudoxanthoma elasticum, antiphospholipid antibody syndrome) and the observation of some patients with pathological changes of SAE but an incomplete clinical picture. The clinicopathological features described as Binswanger's disease do not qualify as a separate entity since they are common to a variety of illnesses. The pathological picture identified by Olszewski can rightly be named, according to Caplan, "chronic microvascular leukoencephalopathy" (CML). The clinicopathological features of the so-called Binswanger's disease constitute a syndrome, the CML syndrome (CMLS), which can be found in some hereditary diseases and in acquired conditions. This syndrome shows peculiar cerebrovascular changes and, when clinically associated with dementia, identifies one of the subtypes of vascular dementia.
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PMID:Binswanger's disease is not a single entity. 1144 70

A review is presented of literature data concerning vascular disease occurring in families. They manifested clinically as recurrent TIA, ischaemic and haemorrhagic strokes and other blood supply disturbances and lead to numerous vasogenic brain tissue damage of various intensity. Particularly evident lesions are observed in hemispheric white matter. Progressive neurological symptoms and dementia form the picture of subcortical leucoencephalopathy in several members of a family. Moyamoya disease, fibromuscular dysplasia, hereditary haemorrhagic telangiectasia, hereditary cerebral haemorrhage with amyloidosis, pseudoxanthoma elasticum, two types of subcortical encephalopathy in Japan, HERNS and CADASIL are described.
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PMID:[Encephalopathy and other neurologic syndromes with familial occurrence]. 1146 8