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Cerebral amyloid angiopathy-related inflammation (CAA-ri): A case report
Yao Meng1,2, Yining Xiao1,3, Ruohan Sun1,4
1Department of Neurology, Hebei General Hospital, Shijiazhuang, China.
Rationale:
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a treatable condition characterized by an acute or subacute onset, with its primary pathological hallmark being the deposition of amyloid, predominantly β-amyloid (Aβ), within intracranial microvessels. Despite its potential for treatment, CAA-ri is a rare disorder that is frequently underrecognized by clinicians in practice. This article provides a comprehensive overview of the clinical manifestations and therapeutic approaches associated with CAA-ri, aiming to enhance awareness among healthcare professionals.
Patient Concerns:
A 67-year-old male patient who suffered from a sudden decline in cognitive functioning, intermittent headache, and dysphoria underwent brain magnetic resonance imaging, susceptibility weighted imaging, and cerebrospinal fluid analysis and was considered probable CAA-ri.
Diagnosis Interventions:
During the course of disease development, the patient suffered from a sudden decline in cognitive functioning, mainly in the form of unresponsiveness, decreased comprehension, and increased repetitive language, accompanied by intermittent headaches and dysphoria. Brain magnetic resonance imaging showed numerous white matter in both hemispheres. Susceptibility weighted imaging showed multiple spots of hypointensity in the bilateral cerebral and cerebellar hemispheres, a hypointensity signal in the left occipital lobe, and extensive zones of hypointensity in bilateral sulci. Cerebrospinal fluid analysis was abnormal with elevated levels of protein and low levels of P-tau, Aβ42, and Aβ1-42/Aβ1-40. The use of glucocorticoids greatly reduced his symptoms. This lends credence to the probable CAA-ri diagnosis. The symptoms can be successfully alleviated by administering methylprednisolone sodium succinate.
Outcomes:
During the patient's hospitalization, immunosuppressive therapy, primarily consisting of methylprednisolone sodium succinate and methylprednisolone, was administered, resulting in a significant improvement in symptoms. Post-discharge, the patient was monitored regularly, revealing a gradual enhancement in cognitive function without recurrence. Consequently, immunosuppressive therapy was discontinued 1 year following the patient's discharge.
Lessons:
CAA-ri is a rare clinical condition, and timely diagnosis and early treatment are very critical for patient prognosis.
Insights
Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare, treatable condition. Early diagnosis and glucocorticoid treatment, such as methylprednisolone, significantly improve cognitive function and alleviate symptoms in patients with CAA-ri.
Area of Science:
- Neurology
- Immunology
Background:
- Cerebral amyloid angiopathy-related inflammation (CAA-ri) is a rare but treatable condition.
- It is characterized by amyloid deposition in intracranial microvessels and often underrecognized.
- This overview aims to increase awareness of CAA-ri's clinical features and treatments.
Purpose of the Study:
- To provide a comprehensive overview of Cerebral amyloid angiopathy-related inflammation (CAA-ri).
- To enhance clinician awareness of CAA-ri's manifestations and therapeutic options.
Main Methods:
- A case study of a 67-year-old male with cognitive decline, headache, and dysphoria.
- Diagnostic tools included MRI, SWI, and cerebrospinal fluid analysis.
- Treatment involved glucocorticoids, specifically methylprednisolone sodium succinate.
Main Results:
- The patient presented with cognitive decline, headache, and dysphoria, diagnosed as probable CAA-ri.
- MRI and CSF analysis supported the diagnosis.
- Methylprednisolone treatment led to significant symptom improvement and cognitive recovery.
Conclusions:
- Cerebral amyloid angiopathy-related inflammation (CAA-ri) is rare.
- Timely diagnosis and early treatment are crucial for favorable patient outcomes.
- Glucocorticoid therapy is effective in managing CAA-ri symptoms.
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