Cerebral amyloid angiopathy‐related inflammation presented with posterior reversible encephalopathy syndrome
Background Cerebral amyloid angiopathy (CAA) is characterized by the deposition of amyloid beta protein deposits within the media and adventitia of small and medium‐sized leptomeningeal and cortical blood vessels of the brain. CAA‐related inflammation (CAA‐ri) is a relatively rare and aggressive sub...
Gespeichert in:
Veröffentlicht in: | Alzheimer's & dementia 2023-12, Vol.19 (S16), p.n/a |
---|---|
Hauptverfasser: | , , , , , |
Format: | Artikel |
Sprache: | eng |
Online-Zugang: | Volltext |
Tags: |
Tag hinzufügen
Keine Tags, Fügen Sie den ersten Tag hinzu!
|
Zusammenfassung: | Background
Cerebral amyloid angiopathy (CAA) is characterized by the deposition of amyloid beta protein deposits within the media and adventitia of small and medium‐sized leptomeningeal and cortical blood vessels of the brain. CAA‐related inflammation (CAA‐ri) is a relatively rare and aggressive subtype of CAA with diverse neurologic deficits and characteristic radiological findings. Posterior reversible encephalopathy syndrome (PRES) is a neurological disorder of acute or subacute onset characterized by various neurological symptoms and reversible entity both radiologically and clinically. We report a case of CAA‐ri presented with PRES.
Method
A 80‐year‐old man with a history of hypertension presented to the Emergency department with sudden onset of drowsy mentation and disorientation. At admission arterial blood pressure was 210/100mmHg. Brain computed tomography revealed hypodense lesion in both parieto‐occipito‐temporal lobes with the predominance on the left side. Brain MRI susceptibility weighted imaging (SWI) presented multiple microbleeds with diffuse cerebral edema in both parieto‐occipital and left temporal cortex and subcortex as can be seen in cerebral amyloid angiopathy (CAA). Obtained images suggested CAA coexisting with posterior reversible encephalopathy syndrome (PRES). Electroencephalopgraphy (EEG) revealed regionalized sharp wave in the right parieto‐occipital area. There was no evidence of venous thrombosis. CSF analysis showed no pleocytosis with protein elevation of 105 mg/dl. PRES was considered due to neurologic deficit of acute onset, elevated arterial blood pressure and vasogenic edema on MRI. 18F‐Florbetaben PET CT showed amyloid positivity.
Result
A diagnosis of CAA‐ri was considered, and the patient was started on intravenous steroid for 5 days and valproic acid with clinical improvement. Arterial blood pressure showed gradual normalization within 5 days of hospitalization. After 2 weeks of hospitalization, the patient was able to walk without assistance. MMSE score was 17 with normal orientation of place, acalculia and memory impairment. The modified Rankin Scale was 1 and Barthel index score was 20 at the time of discharge.
Conclusion
CAA‐ri may present with PRES‐like features. Early detection and appropriate management of trigger factors of PRES which may caused by CAA‐ri would facilitate acute phase recovery and long term prognosis. |
---|---|
ISSN: | 1552-5260 1552-5279 |
DOI: | 10.1002/alz.076256 |