Amyloidassoziierte Krankheiten des Zentralnervensystems: multimodale Befundung

Die Ablagerung von β-Amyloid im Zentralnervensystem ist die Ursache ganz verschiedener Krankheitsbilder. Trotz identischer Pathophysiologie unterscheiden sie sich markant hinsichtlich Klinik und bildgebenden Befunden. Dieser Artikel schildert die wichtigsten klinischen und bildgebenden Merkmale amyloidassoziierter Krankheiten, um dem Radiologen die für die korrekte Diagnose erforderlichen Kenntnisse an die Hand zu geben.

Abstract

Amyloid-b (Ab) is ubiquitous in the central nervous system (CNS), but pathologic accumulation of Ab results in four distinct neurologic disorders that affect middle-aged and elderly adults, with diverse clinical presentations ranging from chronic debilitating dementia to acute life-threatening intracranial hemorrhage. The characteristic imaging patterns of Ab-related CNS diseases reflect the pathophysiology of Ab deposition in the CNS. Ab is recognized as a key component in the neuronal damage that characterizes the pathophysiology of Alzheimer disease, the most common form of dementia. Targeted molecular imaging shows pathologic accumulation of Ab and tau protein, and fluorine 18 fluorodeoxyglucose positron emission tomography and anatomic imaging allow differentiation of typical patterns of neuronal dysfunction and loss in patients with Alzheimer disease from those seen in patients with other types of dementia. Cerebral amyloid angiopathy (CAA) is an important cause of cognitive impairment and spontaneous intracerebral hemorrhage in the elderly. Hemorrhage and white matter injury seen at imaging reflect vascular damage caused by the accumulation of Ab in vessel walls. The rare forms of inflammatory angiopathy attributed to Ab, Ab-related angiitis and CAA-related inflammation, cause debilitating neurologic symptoms that improve with corticosteroid therapy. Imaging shows marked subcortical and cortical inflammation due to perivascular inflammation, which is incited by vascular Ab accumulation. In the rarest of the four disorders, cerebral amyloidoma, the macroscopic accumulation of Ab mimics the imaging appearance of tumors. Knowledge of the imaging patterns and pathophysiology is essential for accurate diagnosis of Ab-related diseases of the CNS.

Kernaussagen

• Die Akkumulation von Aβ im Gehirn gilt als entscheidender, die Alzheimer-Krankheit auslösender Faktor. Er stößt die Kaskade von Prozessen an, die zur Bildung von neurofibrillären Bündeln und zu zunehmendem Neuronenuntergang führen.

• In der Vergangenheit dienten CT oder MRT bei Patienten mit Demenz zum Ausschluss anderer Demenzursachen als Alzheimer-Krankheit. Heute können die strukturelle MRT und die molekulare Bildgebung mittels PET zur Darstellung Alzheimer-typischer abnormer Befunde eingesetzt werden. Dies kann besonders bei Patienten hilfreich sein, bei denen die Ursache der Demenz ungewiss ist.

• Lobäre und zerebelläre Blutungen, Leukenzephalopathie, kleine Infarkte in der Hirnrinde und Mikroblutungen sind die charakteristischen Merkmale einer CAA. Ihre Ursache besteht in einer erhöhten Brüchigkeit der Gefäße aufgrund der Ablagerung von Aβ-Protein in der Media kortikaler und leptomeningealer Gefäße. Das Auftreten multipler Hirnblutungen und deren charakteristische Verteilung bei einem älteren Patienten mit normalem Blutdruck sprechen für die Diagnose einer sporadischen CAA.

• Zwar ist die Ablagerung von Aβ in den Gefäßwänden ein gemeinsames Merkmal beider CAA-Formen. Jedoch erleichtert die assoziierte ausgeprägte entzündliche Reaktion die Abgrenzung der inflammatorischen von der typischen CAA sowohl bei der pathologischen Untersuchung als auch bei der bildgebenden Darstellung.

• Charakteristisches Zeichen eines zerebralen Amyloidoms ist eine Läsion mit solider Kontrastmittelanreicherung, deren Zentrum in der weißen Substanz liegt und die von einem Ödem umgeben ist. Erkrankungen mit ähnlichem Bildgebungsbefund wie beim zerebralen Amyloidom sind ZNS-Lymphom, primäres ZNS-Neoplasma und ZNS-Vaskulitis.

Schlüsselwörter

Pathologische β-Amyloidablagerung - bildgebende Merkmale - Alzheimer-Krankheit - zerebrale Amyloidangiopathie

Keywords

pathologic accumulation of Amyloid-b - imaging patterns - Alzheimer disease - cerebral amyloid angiopathy

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