Zerebrale Amyloidangiopathie und ihre Folgen

Die zerebrale Amyloidangiopathie (CAA) ist charakterisiert durch Amyloidablagerungen in kleinen kortikalen und leptomeningealen Gefäßen, wobei die Boston-Kriterien die Diagnosestellung einer wahrscheinlichen CAA ermöglichen. Bei der CAA-assoziierten Inflammation treten amyloidbedingte bildmorphologische Auffälligkeiten auf, bei denen ein ödematöser und ein hämorrhagischer Subtyp in der MRT unterschieden werden können.

Abstract

Cerebral amyloid angiopathy (CAA) is characterized by the deposition of amyloid in the vessel walls of small cortical and leptomeningeal arteries. Whereas the definite diagnosis requires a brain biopsy, knowledge of clinical and neuroimaging findings in CAA is essential. The Boston criteria version 2.0 including characteristic clinical and neuroradiological features facilitates the diagnosis of probable CAA. Spontaneous and amyloid-beta immunotherapy-associated CAA-related inflammation cause amyloid related imaging abnormalities (ARIA). There are two subtypes: ARIA-E (edema, leptomeningeal effusion) and ARIA-H (haemorrhagic).

Kernaussagen

• Die CAA ist charakterisiert durch Amyloidablagerungen in kleinen kortikalen und leptomeningealen Gefäßen.

• Die Prävalenz der CAA steigt mit dem Alter und beträgt bei kognitiv Beeinträchtigten über 80 Jahre 50–60%.

• Häufige Manifestation ist die spontane lobare intrazerebrale Blutung (ICB) mit hohem Rezidivrisiko.

• Neurologisch typisch sind transiente fokale neurologische Episoden (TFNE) und zunehmende kognitive Ausfälle bis hin zur Demenz vom Alzheimer-Typ.

• Charakteristische neuroradiologische Befunde sind:

  • lobare ICB

  • kortikale/subkortikale Mikroblutungen (MB)

  • kortikale Subarachnoidalblutung (SAB)

  • kortikale superfizielle Siderose (SS)

  • erweiterte perivaskuläre Räume und T2-hyperintense Läsionen im Centrum semiovale

• Hämosensitive MRT-Sequenzen (T2*, besser SWI) sind essenziell.

• Die Boston-Kriterien Version 2.0 erleichtern die klinische Diagnosestellung.

• Die CAA-assoziierte Inflammation (CAA-ai) entspricht dem Subtyp mit 2 Varianten von ARIA: vasogenes Ödem und leptomeningeale Effusionen (ARIA-E) und hämorrhagische Läsionen (ARIA-H).

• ARIA treten bei ca. 20% von Patienten mit monoklonaler Antikörpertherapie gegen Amyloid-β (Aβ) auf.

Schlüsselwörter

zerebrale Amyloidangiopathie (CAA) - Boston-Kriterien - amyloidbedingte bildmorphologische Auffälligkeiten (ARIA)

Keywords

cerebral amyloid angiopathy (CAA) - Boston criteria - amyloid related imaging abnormalities (ARIA)

Publikationsverlauf

Artikel online veröffentlicht:
06. Dezember 2024

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