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Acute cerebrovascular disease in the young: the Stroke in Young Fabry Patients study
Arndt Rolfs1, Franz Fazekas, Ulrike Grittner
1Albrecht-Kossel-Institute for Neuroregeneration, Medical Faculty, University of Rostock, Gehlsheimer St.20, 18147 Rostock, Germany. arndt.rolfs@med.uni-rostock.de
Insights
Young adults experiencing stroke have a 0.5% chance of having Fabry disease. The study highlights the prevalence of silent infarcts and risk factors, indicating a need for new preventive strategies for young stroke patients.
Area of Science:
- Neurology
- Cardiovascular Research
- Genetics
Background:
- Strokes in young adults have severe consequences, yet their characteristics are poorly understood.
- Traditional risk factors like atherosclerosis are less common in young stroke patients, suggesting different underlying causes.
- The Stroke in Young Fabry Patients (sifap) study was initiated to investigate acute cerebrovascular disease in individuals aged 18-55.
Purpose of the Study:
- To characterize a multinational cohort of young stroke patients.
- To identify the prevalence of Fabry disease in young stroke survivors.
- To analyze the causes and imaging findings of cerebrovascular events in young adults.
Main Methods:
- Prospective, multinational European study (sifap) enrolling 5023 patients aged 18-55.
- Standardized clinical, laboratory, and radiological assessments were performed.
- Data collection occurred between April 2007 and January 2010 across 15 countries and 47 centers.
Main Results:
- Definite Fabry disease was diagnosed in 0.5% of patients, with probable cases in an additional 0.4%.
- Large artery atherosclerosis (18.6%) and dissection (9.9%) were the most frequent causes of ischemic stroke.
- Silent infarcts were observed in 20% of first-ever stroke patients, and white matter lesions were prevalent.
Conclusions:
- Fabry disease is a relevant, though rare, cause of stroke in young adults.
- The high prevalence of silent infarcts and risk factors underscores the necessity for early detection and prevention strategies.
- Further research is needed to develop targeted interventions for preventing strokes in younger populations.
Background And Purpose:
Strokes have especially devastating implications if they occur early in life; however, only limited information exists on the characteristics of acute cerebrovascular disease in young adults. Although risk factors and manifestation of atherosclerosis are commonly associated with stroke in the elderly, recent data suggests different causes for stroke in the young. We initiated the prospective, multinational European study Stroke in Young Fabry Patients (sifap) to characterize a cohort of young stroke patients.
Methods:
Overall, 5023 patients aged 18 to 55 years with the diagnosis of ischemic stroke (3396), hemorrhagic stroke (271), transient ischemic attack (1071) were enrolled in 15 European countries and 47 centers between April 2007 and January 2010 undergoing a detailed, standardized, clinical, laboratory, and radiological protocol.
Results:
Median age in the overall cohort was 46 years. Definite Fabry disease was diagnosed in 0.5% (95% confidence interval, 0.4%-0.8%; n=27) of all patients; and probable Fabry disease in additional 18 patients. Males dominated the study population (2962/59%) whereas females outnumbered men (65.3%) among the youngest patients (18-24 years). About 80.5% of the patients had a first stroke. Silent infarcts on magnetic resonance imaging were seen in 20% of patients with a first-ever stroke, and in 11.4% of patients with transient ischemic attack and no history of a previous cerebrovascular event. The most common causes of ischemic stroke were large artery atherosclerosis (18.6%) and dissection (9.9%).
Conclusions:
Definite Fabry disease occurs in 0.5% and probable Fabry disease in further 0.4% of young stroke patients. Silent infarcts, white matter intensities, and classical risk factors were highly prevalent, emphasizing the need for new early preventive strategies. Clinical Trial Registration Information- URL: http://www.clinicaltrials.gov.Unique identifier: NCT00414583.
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