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Nationwide survey on cerebral amyloid angiopathy in Japan
K Sakai1, M Ueda2, W Fukushima3
1Department of Neurology and Neurobiology of Aging, Kanazawa University Graduate School of Medical Sciences, Kanazawa, Japan.
Insights
This study estimates the prevalence of cerebral amyloid angiopathy-related intracerebral hemorrhage (CAA-related ICH) and inflammation (CAA-ri) in Japan. It found approximately 5900 cases of CAA-related ICH and 170 cases of CAA-ri, detailing their clinical features.
Area of Science:
- Neurology
- Epidemiology
- Clinical Research
Background:
- Cerebral amyloid angiopathy (CAA) is a growing concern, leading to intracerebral hemorrhage (ICH) and inflammation/vasculitis (CAA-ri).
- Understanding the epidemiology of these conditions in Japan is crucial for public health planning.
Purpose of the Study:
- To estimate the total number of patients diagnosed with CAA-related ICH and CAA-ri in Japan.
- To analyze the clinical features and demographic characteristics of these patient populations.
Main Methods:
- A nationwide survey involving questionnaires sent to randomly selected hospitals across Japan.
- Data collection spanned from January 2012 to December 2014, with response rates analyzed.
Main Results:
- An estimated 5900 cases of CAA-related ICH and 170 cases of CAA-ri were identified in Japan.
- CAA-related ICH predominantly affected women, with onset around 78.4 years, often in the frontal lobe.
- CAA-ri presented with cognitive impairment and headache, median onset at 75 years, and frequent neurological signs.
Conclusions:
- The study provides crucial epidemiological data on CAA-related ICH and CAA-ri in Japan.
- Estimated prevalence rates were 4.64 per 100,000 for CAA-related ICH and 0.13 per 100,000 for CAA-ri.
Background And Purpose:
A nationwide survey was conducted to understand the epidemiology of cerebral amyloid angiopathy-related intracerebral hemorrhage (CAA-related ICH) and cerebral amyloid angiopathy-related inflammation/vasculitis (CAA-ri) in Japan.
Methods:
To estimate the total number and clinical features of patients with CAA-related ICH and CAA-ri between January 2012 and December 2014 and to analyze their clinical features, questionnaires were sent to randomly selected hospitals in Japan.
Results:
In the first survey, 2348 of 4657 departments responded to the questionnaire (response rate 50.4%). The total numbers of reported patients with CAA-related ICH and CAA-ri were 1338 and 61, respectively, and their total numbers in Japan were estimated to be 5900 [95% confidence interval (CI) 4800-7100] and 170 (95% CI 110-220), respectively. The crude prevalence rates were 4.64 and 0.13 per 100 000 population, respectively. The clinical information of 474 patients with CAA-related ICH obtained in the second survey was as follows: (i) the average age of onset was 78.4 years; (ii) the prevalence increased with age; (iii) the disease was common in women; and (iv) hematoma most frequently occurred in the frontal lobe. Sixteen patients with CAA-ri for whom data were collected in the second survey had the following characteristics: (i) median age of onset was 75 years; (ii) cognitive impairment and headache were the most frequent initial manifestations; and (iii) focal neurological signs, such as motor paresis and visual disturbance, were frequently observed during the clinical course.
Conclusions:
The numbers of patients with CAA-related ICH and CAA-ri in Japan were estimated.
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