Cerebral amyloid angiopathy related inflammation with leptomeningeal involvement: a case report and review of the literature

Sowanou, Arlos Verlaine; Ungureanu, Aurelian; Paulin, M.

doi:10.1007/s13760-022-01971-8

Cited by 2 publications

(2 citation statements)

References 26 publications

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“…Previous data have shown that a favorable outcome was seen in between 42% and 75% of CAA-rI patients [10,11]. In addition, a more recent systematic review of both pathologic variants of CAA-rI showed that about half of all patients were asymptomatic or with mild neurological sequelae after a two-year follow-up period [4].…”

Section: Discussionmentioning

confidence: 97%

“…Nevertheless, this inflammatory CAA variant is often characterized with the presence of atypical clinical features, which might lead to misdiagnosing and delayed treatment introduction [5,6]. On the other hand, the most suggestive magnetic resonance (MR) imaging findings in CAA-rI are predominantly asymmetric white matter hyperintensities seen on T2 or fluid-attenuated inversion recovery sequences, lobar cerebral microbleeds or cortical superficial siderosis on susceptibility-weighted imaging, and/or signs of lobar intracerebral hemorrhage [1,5,7,9,10]. As previously underlined, an even more significant feature of this infrequent subtype of CAA is a largely satisfying and mostly rapid response to early administered immunosuppressive therapy.…”

Section: Introductionmentioning

confidence: 99%

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Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review

et al. 2023

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Cerebral amyloid angiopathy-related inflammation (CAA-rI) is a largely reversible, subacute encephalopathy, which is considered as a rare variant of cerebral amyloid angiopathy (CAA). Although the diagnosis of this inflammatory vasculopathy is generally clinico-pathologic, a probable or possible diagnosis can often be established based on current clinico-radiological diagnostic criteria. This is important since CAA-rI is considered as a treatable disorder, which most commonly occurs in the elderly population. Behavioral changes and cognitive deterioration are highlighted as the most common clinical signs of CAA-rI, followed by a heterogeneous spectrum of typical and atypical clinical presentations. However, despite the well-established clinical and radiological features incorporated in the current diagnostic criteria for this CAA variant, this rare disorder is still insufficiently recognized and treated. Here, we have shown three patients diagnosed with probable CAA-rI, with significant heterogeneity in the clinical and neuroradiological presentations, followed by different disease courses and outcomes after the introduction of immunosuppressive treatment. Moreover, we have also summarized up-to-date literature data about this rare, yet underdiagnosed, immune-mediated vasculopathy.

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Section: Discussionmentioning

confidence: 97%

Section: Introductionmentioning

confidence: 99%

Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review

et al. 2023

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Cerebral Amyloid Angiopathy—Related Inflammation: A Single-Center Experience and a Literature Review

Theodorou

Palaiodimou

Safouris

et al. 2022

JCM

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Background: Limited data exist regarding the prevalence of clinical, neuroimaging, and genetic markers among patients diagnosed with Cerebral Amyloid Angiopathy–related inflammation (CAA-ri). We sought to determine these characteristics in patients diagnosed in our center and to summarize available literature published either as single-case reports or small case series (<5 patients). Methods: We reported our single-center experience of patients diagnosed with CAA-ri according to international criteria during a seven-year period (2015–2022), and we abstracted data from 90 previously published cases. Results: Seven patients (43% women, mean age 70 ± 13 years) were diagnosed with CAA-ri in our center. The most common symptom at presentation was focal neurological dysfunction (71%), and the most prevalent radiological finding was the presence of T2/FLAIR white matter hyperintensities (100%). All patients were treated with corticosteroids and had a favorable functional outcome. Among 90 previously published CAA-ri cases (51% women, mean age 70 ± 9 years), focal neurological dysfunction was the most common symptom (76%), followed by a cognitive decline (46%) and headache (34%). The most prevalent neuroimaging findings were cerebral microbleeds (85%), asymmetric T2/FLAIR white matter hyperintensities (81%), and gadolinium-enhancing T1-lesions (37%). Genetic testing for the Apolipoprotein-E gene was available in 27 cases; 59% carried the APOE ε4/ε4 genotype. The majority of the published CAA-ri cases (78%) received corticosteroid monotherapy, while 17 patients (19%) were treated with additional immunosuppressive treatment. Favorable functional outcome following treatment was documented in 70% of patients. Conclusion: Improving the vigilance of clinicians regarding the early recognition and accurate diagnosis of CAA-ri is crucial for swift therapy initiation, which may result in improved functional outcomes.

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Cerebral amyloid angiopathy related inflammation with leptomeningeal involvement: a case report and review of the literature

Cited by 2 publications

References 26 publications

Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review

Cerebral Amyloid Angiopathy-Related Inflammation (CAA-rI): Three Heterogeneous Case Reports and a Focused Literature Review

Cerebral Amyloid Angiopathy—Related Inflammation: A Single-Center Experience and a Literature Review

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