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Visualization of Amyloid β Deposits in the Human Brain with Matrix-assisted Laser Desorption/Ionization Imaging Mass Spectrometry
Published on: March 7, 2019
Amyloid-β-related angiitis presenting as leptomeningitis
Pablo Gómez-Porro1, Beatriz De La Puente1, Lorena Fernández1
1Neurology, Puerta de Hierro University Hospital of Majadahonda, Majadahonda, Madrid, Spain.
Inflammatory cerebral amyloid angiopathy (ICAA) can be challenging to diagnose. This case highlights subtle radiological findings in amyloid beta-related angiitis (ABRA) and the utility of the kappa index.
Area of Science:
- Neurology
- Immunology
- Pathology
Background:
- Inflammatory cerebral amyloid angiopathy (ICAA) is an autoimmune condition targeting cerebral beta-amyloid.
- ICAA encompasses cerebral amyloid angiopathy-related inflammation (CAA-RI) and amyloid beta-related angiitis (ABRA).
Purpose of the Study:
- To report a case of ABRA with subtle initial radiological findings.
- To emphasize the diagnostic challenges and potential utility of biomarkers in ABRA.
Main Methods:
- Histological confirmation of ABRA.
- Cerebrospinal fluid analysis, including kappa free light chain index.
- Brain biopsy for definitive diagnosis.
Main Results:
- Initial radiological findings were subtle, posing a diagnostic challenge.
- Elevated kappa free light chain index supported an inflammatory etiology with leptomeningitis.
- Brain biopsy confirmed ABRA.
Conclusions:
- ABRA can present with subtle radiological signs, necessitating its inclusion in the differential diagnosis of leptomeningitis.
- The kappa index may serve as a valuable biomarker in diagnosing ICAA spectrum disorders.
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