| MRI features of pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL). | |
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MedLine Citation:
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PMID: 19187480 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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BACKGROUND AND PURPOSE: Cerebral small vessel disease is a topic of growing interest for both the scientific community and the aging society. We report the magnetic resonance imaging (MRI) characteristics of a recently found autosomal dominantly inherited microangiopathy. METHODS: Eighteen members (35 to 77 years) of a large German family underwent MR scanning with a standardized MRI protocol for cerebrovascular diseases. Images were evaluated independently by two neuroradiologists. RESULTS: Six family members revealed an unequivocally pathological phenotype on MRI with lacunar infarcts of the pons (6/6) and lesions of the subcortical and periventricular white matter (5/6). Lesions in the temporal lobes (1/6) and cerebral microbleeds (1/6) were uncommon. None of the patients revealed atherosclerotic changes in MR angiography. Retrospective analysis of 5 brain autopsies from previously reported patients of the same family confirmed the regular involvement of the pons. CONCLUSION: This cerebral autosomal dominant arteriopathy with pontine infarcts and leukoencephalopathy is characterized by a special lesion pattern strikingly different from CADASIL. The distinct MRI characteristics with pontine lesions and rare occurrence of temporal lesions argue for a new nosological entity and may be helpful for the differential diagnosis. |
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Authors:
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Xiao-Qi Ding; Christian Hagel; E Bernd Ringelstein; Stefan Buchheit; Hermann Zeumer; Gregor Kuhlenbäumer; Silke Appenzeller; Jens Fiehler |
Publication Detail:
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Type: Journal Article Date: 2009-01-29 |
Journal Detail:
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Title: Journal of neuroimaging : official journal of the American Society of Neuroimaging Volume: 20 ISSN: 1552-6569 ISO Abbreviation: J Neuroimaging Publication Date: 2010 Apr |
Date Detail:
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Created Date: 2010-05-21 Completed Date: 2010-09-29 Revised Date: - |
Medline Journal Info:
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Nlm Unique ID: 9102705 Medline TA: J Neuroimaging Country: United States |
Other Details:
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Languages: eng Pagination: 134-40 Citation Subset: IM |
Affiliation:
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Department of Neuroradiology, University Medical Center Hamburg-Eppendorf, University of Hamburg, Germany. Ding.Xiaoqi@mh-hannover.de |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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Adult Aged Cerebrovascular Disorders / genetics*, pathology* Chromosome Disorders / genetics*, pathology* Dementia, Vascular / genetics*, pathology* Female Humans Magnetic Resonance Imaging / methods* Male Middle Aged Syndrome |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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