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Assessing Cortical Cerebral Microinfarcts on High Resolution MR Images
Published on: November 20, 2015
Multiple cerebral microinfarcts: an uncommon presentation of Cerebral Amyloid Angiopathy-related inflammation
Aikaterini Theodorou1, Athanasios Tsibonakis1, Ioannis S Pateras2
1Second Department of Neurology, School of Medicine, "Attikon" University Hospital, National and Kapodistrian University of Athens, Athens, Greece.
Background:
Cerebral Amyloid Angiopathy-related inflammation (CAA-ri) is a distinct but rare subset of CAA. The greater availability of high resolution Magnetic Resonance Imaging (MRI) has currently allowed the increasing recognition and diagnosis of this entity, without the risk of a brain biopsy. However, in rare cases with typical clinical characteristics but uncommon neuroimaging findings at presentation, the brain-biopsy is required for an early and reliable diagnosis.
Case Description:
A 71-year-old man with arterial hypertension presented due to 1-week history of headache, vomiting, disorientation and impaired consciousness. Brain MRI revealed multiple acute cortical/subcortical microinfarcts, scarce microbleeds, extensive right parietooccipital and left frontotemporal leptomeningeal enhancement. After an extensive diagnostic work-up, excluding infectious, neoplastic and autoimmune etiologies, the patient underwent brain-biopsy. Histology disclosed amyloid deposition in an arteriolar wall and the patient fulfilled diagnostic criteria for probable CAA-ri with supporting pathology. He received intravenous methylprednisolone, followed by oral tapering with steroids showing clinical and radiological improvement with complete resolution of gadolinium enhancement. Follow-up MRI revealed an increase of cerebral microbleeds and the patient fulfilled CAA-ri neuroimaging criteria.
Conclusions:
This case highlights the importance of continuous vigilance from clinical neurologists to detect CAA-ri diagnosis and the diagnostic value of brain-biopsy in CAA-ri patients with atypical neuroimaging presentation, such as acute microinfarcts. The early diagnosis and the prompt treatment initiation can improve the prognosis and the evolution of this rare disorder.
Insights
Cerebral Amyloid Angiopathy-related inflammation (CAA-ri) is a rare condition. Early diagnosis, even with atypical MRI findings, and prompt steroid treatment are crucial for improving outcomes in CAA-ri patients.
Area of Science:
- Neurology
- Neuroimaging
- Pathology
Background:
- Cerebral Amyloid Angiopathy-related inflammation (CAA-ri) is a rare subset of Cerebral Amyloid Angiopathy (CAA).
- High-resolution MRI aids CAA-ri diagnosis, but brain biopsy remains essential for atypical cases.
- CAA-ri diagnosis is increasingly recognized due to advanced neuroimaging techniques.
Discussion:
- A 71-year-old male with hypertension presented with neurological symptoms.
- Brain MRI showed microinfarcts and leptomeningeal enhancement, leading to a brain biopsy.
- Histology confirmed amyloid deposition, meeting criteria for probable CAA-ri.
Key Insights:
- Brain biopsy is crucial for diagnosing CAA-ri with atypical neuroimaging findings like acute microinfarcts.
- Prompt treatment with steroids (methylprednisolone) led to clinical and radiological improvement.
- Follow-up MRI showed increased microbleeds, confirming CAA-ri neuroimaging criteria.
Outlook:
- Vigilance in clinical neurology is key for timely CAA-ri detection.
- Early diagnosis and treatment significantly improve prognosis in CAA-ri.
- This case underscores the diagnostic utility of brain biopsy in complex CAA-ri presentations.
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