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Fortschr Neurol Psychiatr 2016; 84(09): 534-541
DOI: 10.1055/s-0042-109537
Übersicht
© Georg Thieme Verlag KG Stuttgart · New York

Die sporadische zerebrale Amyloidangiopathie – eine aktuelle Übersicht mit klinischen Fallbeispielen

Sporadic Cerebral Amyloid Angiopathy: An Overview with Clinical Cases
F. Schöberl
1   Neurologische Klinik und Poliklinik & Deutsches Schwindel- und Gleichgewichtszentrum DSGZ, Klinikum der Universität München
,
O. E. Eren
1   Neurologische Klinik und Poliklinik & Deutsches Schwindel- und Gleichgewichtszentrum DSGZ, Klinikum der Universität München
,
F. A. Wollenweber
2   Institut für Schlaganfall- und Demenzforschung, Klinikum der Universität München
,
T. Kraus
3   Zentrum für Neuropathologie und Prionforschung, Klinikum der Universität München
,
L. Kellert
1   Neurologische Klinik und Poliklinik & Deutsches Schwindel- und Gleichgewichtszentrum DSGZ, Klinikum der Universität München
4   Neurologische Klinik, Universitätsklinikum Heidelberg
› Author Affiliations
Further Information

Publication History

Publication Date:
08 September 2016 (online)

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Zusammenfassung

Die sporadische zerebrale Amyloidangiopathie (CAA) ist eine im fortgeschrittenen Lebensalter auftretende Mikroangiopathie. Neuropathologisch gekennzeichnet ist die CAA durch die Ablagerung von ß-Amyloid (Aß) in dünn- bis mittelkalibrigen Arteriolen, Kapillaren und Venolen des Hirnkortex sowie der Leptomeningen. Vor allem in den letzten Jahren wurde evident, dass die CAA eine wichtige Ursache spontaner intrazerebraler Blutungen sowie auch kognitiver Beeinträchtigungen im höheren Lebensalter darstellt. Die klinischen und bildgebenden Manifestationen können sehr unterschiedlich sein und reichen von akut auftretenden fokalen neurologischen Defiziten infolge einer intrazerebralen Lobärblutung (ICB) bis hin zu einem Symptomkomplex aus subakut auftretenden kognitiven und psychischen Veränderungen, Kopfschmerzen sowie epileptischen Anfällen aufgrund multifokaler, teilweise konfluierender Marklagerödeme durch einen Aß-vermittelten Entzündungsprozess. In dieser Übersichtsarbeit sollen die aktuellen Erkenntnisse über die CAA strukturiert und ausführlich anhand eigener Fallbeispiele dargestellt werden.

Abstract

Sporadic cerebral amyloid angiopathy (CAA) is a cerebral small vessel disease in the elderly. Neuropathologically, it is characterized by deposition of amyloid-ß (Aß) in the wall of small to medium-sized arteries, capillaries and venules of the cerebral cortex and leptomeninges. Over the last years it was recognized as an important cause of spontaneous intracerebral hemorrhage and cognitive deficits in the elderly. The clinical and radiological manifestations are diverse ranging from acute onset focal neurological deficits due to intracerebral lobar hemorrhage to subacute progressive cognitive impairment due to Aß-mediated inflammation confluent subcortical edema. The wide clinico-radiological spectrum of CAA is a major challenge for the neurologist and stroke physician. This review provides a structured and detailed look at recent developments in CAA, and is illustrated with case studies.

Schlüsselwörter

sporadische zerebrale Amyloidangiopathie - inflammatorische Amyloidangiopathie - „amyloid spells“ - intrazerebrale Blutung - kognitive Defizite

Key words

sporadic cerebral amyloid angiopathy - inflammatory amyloid angiopathy - amyloid spells - intracerebral bleeding - cognitive impairment
 

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