MRI features of pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL).
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MRI features of pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL). / Ding, Xiaoqi; Hagel, Christian; Ringelstein, Bernd; Buchheit, Stefan; Zeumer, Hermann; Kuhlenbäumer, Gregor; Appenzeller, Silke; Fiehler, Jens.
in: J NEUROIMAGING, Jahrgang 20, Nr. 2, 2, 2010, S. 134-140.Publikationen: SCORING: Beitrag in Fachzeitschrift/Zeitung › SCORING: Zeitschriftenaufsatz › Forschung › Begutachtung
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TY - JOUR
T1 - MRI features of pontine autosomal dominant microangiopathy and leukoencephalopathy (PADMAL).
AU - Ding, Xiaoqi
AU - Hagel, Christian
AU - Ringelstein, Bernd
AU - Buchheit, Stefan
AU - Zeumer, Hermann
AU - Kuhlenbäumer, Gregor
AU - Appenzeller, Silke
AU - Fiehler, Jens
PY - 2010
Y1 - 2010
N2 - ABSTRACT 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. J Neuroimaging 2008;XX:1-7.
AB - ABSTRACT 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. J Neuroimaging 2008;XX:1-7.
M3 - SCORING: Zeitschriftenaufsatz
VL - 20
SP - 134
EP - 140
JO - J NEUROIMAGING
JF - J NEUROIMAGING
SN - 1051-2284
IS - 2
M1 - 2
ER -
