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Titolo:
CEREBRAL AUTOSOMAL-DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL) - A MORPHOLOGICAL-STUDY OF A GERMAN FAMILY
Autore:
BERGMANN M; EBKE M; YUAN Y; BRUCK W; MUGLER M; SCHWENDEMANN G;
Indirizzi:
ZENT KRANKENHAUS BREMEN OST,INST CLIN NEUROPATHOL,ZURICHER STR 40 D-28325 BREMEN GERMANY ZENT KRANKENHAUS BREMEN OST,DEPT NEUROL D-28325 BREMEN GERMANY UNIV GOTTINGEN,INST NEUROPATHOL D-3400 GOTTINGEN GERMANY UNIV AACHEN,INST NEUROPATHOL D-5100 AACHEN GERMANY
Titolo Testata:
Acta Neuropathologica
fascicolo: 4, volume: 92, anno: 1996,
pagine: 341 - 350
SICI:
0001-6322(1996)92:4<341:CAAWSI>2.0.ZU;2-W
Fonte:
ISI
Lingua:
ENG
Soggetto:
ARTERIOSCLEROTIC LEUKOENCEPHALOPATHY; MITOCHONDRIAL ENCEPHALOMYOPATHIES; ALZHEIMERS-DISEASE; YOUNG-ADULT; DEMENTIA; ENCEPHALOPATHY; STROKE; MIGRAINE; EPISODES; BIOPSY;
Keywords:
CEREBRAL AUTOSOMAL DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL); HEREDITARY MULTIINFARCT DEMENTIA; SKIN BIOPSY;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Science Citation Index Expanded
Science Citation Index Expanded
Citazioni:
49
Recensione:
Indirizzi per estratti:
Citazione:
M. Bergmann et al., "CEREBRAL AUTOSOMAL-DOMINANT ARTERIOPATHY WITH SUBCORTICAL INFARCTS AND LEUKOENCEPHALOPATHY (CADASIL) - A MORPHOLOGICAL-STUDY OF A GERMAN FAMILY", Acta Neuropathologica, 92(4), 1996, pp. 341-350

Abstract

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is characterized clinically by recurrent cerebral infarcts, subcortical dementia and pseudobulbar palsy, andmorphologically by a granular degeneration of cerebral and, to a lesser degree, extracerebral blood vessels. We present morphological findings in a further German family affected by CADASIL. The index case showed the typical periodic acid-Schiff-positive granular degeneration ofvascular smooth muscle cells (VSMC) in cerebral vessels, which did not react with antibodies against various immunoglobulins or complement factors. Ultrastructurally, granular osmiophilic material (GOM) covered the VSMC in different cerebral regions as well as in extracerebral organs (muscle, nerve, skin, small and large intestine, liver, kidney and heart). Skin biopsy samples from other family members of the last two generations also revealed GOM irrespective of the clinical symptomatology (CADASIL, migraine only or asymptomatic). Patients in the thirdgeneration had higher amounts of GOM in skin vessels than did asymptomatic or migraine patients in the fourth generation. We conclude that skin biopsy is a useful and less-invasive screening method for the differential diagnosis of CADASIL.

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Documento generato il 16/07/20 alle ore 19:21:06