Cerbral amyloid angiopathy-Related inflammation(CAA-ri) presenting with a seizure and stroke-like episode: case report

Mohammadreza Malek Zadeh ℗, Pourandokht Mousavian, Mohammad Dehghani Firozabadi ©

Cerbral amyloid angiopathy-Related inflammation(CAA-ri) presenting with a seizure and stroke-like episode: case report

کد: G-1016

نویسندگان: Mohammadreza Malek Zadeh ℗, Pourandokht Mousavian, Mohammad Dehghani Firozabadi ©

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خلاصه مقاله

Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare neurological condition characterized by perivascular inflammation triggered by the deposition of β-amyloid (Aβ) in cerebral blood vessels. This inflammation leads to progressive dementia, focal neurological deficits, seizures, and intracerebral hemorrhages. Magnetic resonance imaging (MRI) typically shows patchy or confluent hyperintensities on T2/fluid attenuation inversion recovery (FLAIR) sequences in the cortex and subcortical white matter, often corresponding to areas with pre-existing microhemorrhages. While a definitive diagnosis usually requires a brain biopsy, it is rarely performed due to its invasive nature. We present the case of a 69-year-old woman who presented with a range of symptoms, including headache, generalized tonic-clonic seizures, aggressive behaviors, decreased level of consciousness with restlessness, and mild right hemiparesis over the past week. Extensive diagnostic evaluations were conducted to rule out other potential causes, including viral encephalitis, autoimmune encephalitis, acute stroke, neoplastic syndromes, neurosarcoidosis, primary brain lymphoma, metastasis, posterior reversible encephalopathy syndrome, and other important differentials. Ultimately, the patient met the criteria for a diagnosis of CAA-ri. The patient was treated with high-dose corticosteroid therapy and cyclophosphamide, resulting in an improvement in her overall condition. She was discharged with medication instructions and scheduled for follow-up treatment. This case represents one of the few reported instances of CAA-ri and highlights the importance of considering this condition in the differential diagnosis of patients with specific neurological symptoms. Keywords: Cerebral Amyloid Angiopathy, Seizure, Inflammation, Brain MRI Lesions, Cerebral Small Vessel Disease

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