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PubMed · 8027633

Multi-infarct dementia.

Abstract

Vascular dementia and its most common subtype, multi-infarct dementia, are pathologically proven clinical entities. Their prevalence is not as high as previously thought, but they do represent a significant percentage of the population of demented patients. The diagnosis is more difficult to make than is the diagnosis of Alzheimer's disease; however, there are excellent criteria to guide the physician in making the diagnosis. At present the only treatment available is to control the risk factors responsible for the basic disease process; in the majority of cases this requires controlling hypertension.

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BibTeXRIS

R L Strub. 1994. Multi-infarct dementia.. https://pubmed.ncbi.nlm.nih.gov/8027633/

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Progressive supranuclear palsy phenotype secondary to CADASIL.

BACKGROUND AND PURPOSE: To report a unique case of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy manifesting as a progressive supranuclear palsy phenotype, thereby expanding its recognized presentations. METHODS: Review of the pertinent literature from MEDLINE, cross-referencing cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy, progressive supranuclear palsy, and parkinsonism. Description of a 60-year-old woman who presented with a several year history of step-wise, progressive parkinsonism secondary to cerebral autosomal dominant arteriopathy. RESULTS: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy may present with a progressive supranuclear phenotype. CONCLUSION: Parkinsonism, including a progressive supranuclear palsy phenotype, is one of a growing number of recognized ways that cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy may present.

Dementia, Multi-Infarct↗

[CADASIL: a case with clinical, radiological, histological and genetic diagnoses].

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a rare inherited cerebrovascular disease. The onset of clinical symptoms occurs with migraine with aura, transient ischemic attacks, recurrent subcortical ischemic infarcts, neuropsychiatric changes reaching subcortical dementia. Brain magnetic resonance images show multiple deep cerebral infarcts in white matter and basal ganglia and diffuse leukoencephalopathy. Neuropathologic hallmark consists of deposition of small electron dense granular patches related to the basement membrane of vascular smooth muscle cells with degeneration of smooth muscle cells and media and luminal obliteration. Recently, the genetic characteristics of this disorder have been reported. Missense mutations in notch3 gene localized in chromosome 19 are involved in its pathogenesis. Only three families from Spain have been reported. Here we describe a patient with typical clinical symptoms, neuroimaging and pathology of CADASIL. C406T (Arg110Cys) mutation in notch3 gene was found. We comment on the clinical symptoms of different members of the patient's family.

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