Moyamoya disease (MMD) is a specific chronic cerebrovascular occlusive disease first reported by Japanese surgeons in 1957 (1). It is named for the smoke-like cerebrovascular morphology observed during cerebral angiography in the skull base (2). There are regional differences in the incidence of moyamoya disease worldwide. Studies have shown that the MMD is more prevalent in Japan and East Asia, while it is less common in European and North American populations. For example, the annual incidence in East Asian countries ranges from 0.5 to 1.5 per 100,000 people, compared to as low as 0.1 per 100,000 people in North America (3). In East Asia, the incidence rates also varies among countries. Japan and South Korea specifically have reported incidence rates of 0.94/100,000 and 2.3/100,000, respectively (4). In China, several epidemiological studies have also been conducted. A study conducted in Taiwan reported that the average incidence rate of MMD was 0.15/100000 (5). In the epidemiological study of Nanjing, the incidence rate from 2000 to 2007 was 3.92/100000 (6), lower than that of South Korea and Japan in general. However, national epidemiological studies of MMD in mainland China seem to have not yet been carried out.

MMD may present a series of clinical symptoms, including transient ischemic attack, ischemic stroke, hemorrhagic stroke, epilepsy, headache and cognitive impairment (7). The severity and progression of the disease may vary depending on the age of onset of symptoms and the type of first attack. MMD in children is mostly manifested as progressive cerebral ischemia, while adults with MMD is mostly manifested as cerebral ischemia and hemorrhage (8). It has also been found that even asymptomatic MMD patients may have cognitive impairments, particularly in areas such as intelligence, spatial ability, verbal working memory, and numerical operations (9). MMD and its complications bring not only physical damage to patients, but also great psychological challenges (10). A study showed that 46.7% of MMD patients were diagnosed with depression, 50% with anxiety, and 47.5% with post-traumatic stress disorder (11). The psychological problems caused by these diseases undoubtedly greatly affect the quality of life of patients (12). Therefore, for patients with MMD, it is not enough to only receive medical treatment for physiological diseases. It is necessary to conduct relevant researches on the mental health problems of MMD patients.

Illness uncertainty refers to the ambiguity and anxiety individuals experience due to unpredictable aspects of their health condition, commonly observed in cancer and chronic disease cohorts. It is also associated with depression and reduced quality of life in individuals with chronic illnesses (13). This uncertainty, stemming from the unpredictable trajectory and treatment outcomes of chronic and life-threatening diseases, also leads to reduced compliance with treatment regimens. Consequently, this not only undermines the effectiveness of therapeutic interventions but also adversely affects the overall prognosis of such conditions (14). Exploring the associated factors of illness uncertainty is therefore critical in improving patient outcomes and ensuring a more holistic approach to the management of chronic diseases like MMD.

Resilience is an individual’s ability to adapt and cope with adversity while maintaining well-being (15). It involves the development of skills and support systems for effective management and recovery, as well as the capacity to recover from traumatic experiences. Resilience serves as a buffer against stress and enhances one’s ability to manage stress, confront challenges, develop coping strategies, and adapt to changing circumstances (16, 17). Initially introduced by Block, the concept of resilience includes traits such as intelligence, strength, and adaptability that individuals display in response to ever-changing needs (18). These traits are considered protective factors. Patients with MMD require focused attention due to their unique psychological needs. However, research on resilience and its correlation with uncertainty in illness in patients with MMD seems to be rare. Fortunately, some related studies (19) have been conducted on stroke, which belongs to the same cerebrovascular disease as MMD, and their findings have certain reference value for us.

Perceived social support is a complex, multidimensional concept characterized by reciprocity, encompassing social, psychological, and material assistance an individual receipted from social networks (20). In other words, it refers to the help provided by family, friends, and significant others, playing a crucial role in directly and indirectly reducing illness uncertainty. The social support needs of patients are diverse and largely depend on the tasks they are facing at the time (21). Social support is closely related to the prognosis of patients with MMD. A study have found that illness uncertainty among patients with MMD decreases their quality of life, while a higher level of social support can improve their quality of life (22). In addition, it has been suggested that higher social support level is associated with lower illness uncertainty (23). This finding has been replicated in other populations of patients with chronic diseases (24, 25).

Therefore, although the study of illness uncertainty is very important for MMD patients, and relevant studies have also confirmed that resilience and perceived social support are related to illness uncertainty. However, there seems to be a lack of relevant research on the status quo of illness uncertainty in MMD patients. The relationship between resilience, perceived social support and illness uncertainty in MMD patients is not clear. Further research is needed to fill this gap. So, the present study aims to investigate the levels of illness uncertainty in MMD patients and to determine the association of socio-demographic characteristics, perceived social support and resilience with illness uncertainty in MMD patients. This would be of great benefit in understanding the mental state of MMD patients and provide valuable insights into future intervention strategies for MMD patients.

The present study aims to investigate the levels of illness uncertainty in MMD patients and to determine the association of socio-demographic characteristics, perceived social support and resilience with illness uncertainty in MMD patients. Therefore, we will follow the discussion in this order.

At present, there seems to be little relevant research in MMD patients. Most previous studies tend to focus on mental characters of cerebrovascular diseases patients in general, such as brain bleeds, stroke (45). Considering that MMD is also a kind of cerebrovascular disease, these research results focusing on cerebrovascular disease may have a certain reference for the current research.

The average score of illness uncertainty in MMD patients was (100.03 1008.59), which was at a moderate level. This result was similar to acute stroke patients (46). The reason for this may be that the high disability and mortality rates may increase the uncertainty of patients (47). McIntosh (48) has pointed out the ambiguity of patients regarding the occurrence, development, and prognosis of the disease greatly affects their uncertainty. What’s worse, as time goes by, patients’ concerns increase, thus increasing their sense of uncertainty, which may affect their psychological adjustment, treatment compliance and outlook on life.

The current study found that illness uncertainty of MMD patients was associated with gender. Specifically, female patients generally had higher disease uncertainty scores than male patients, which was consistent with the results of previous studies observed in other patients with chronic diseases (49, 50). This may be attributed to various factors, such as men’s psychological quality, coping strategies and social roles in dealing with diseases. In current Chinese society, many workers from the countryside. Also, the level of illness uncertainty was also related to the occupation of patients. In general, the illness uncertainty score of the workers was higher than that of the farmers, which was similar to another study results (51). The reason for this situation may be related to the social situation in China. These people no longer work as farmers and choose to work in the city itself may be due to the huge life pressure and economic burden they face, which leads to farming is no longer enough to support their daily expenses. Illness means the loss of main labor force of the family and the aggravation of economic burden. These factors make patients have more anxiety, and then produce a higher level of illness uncertainty (52).

What’s more, patients’ illness uncertainty scores were also observed to differ according to their education level, which was similar to previous studies (53, 54). According to the results of ANOVA analysis and the multiple hierarchical regression, scores of illness uncertainty of patients with different education levels showed that except for patients with secondary technical school or high school education levels, the patient ‘s education level was negatively correlated with the score of disease uncertainty, indicating that the higher the patient ‘s education level, the lower the score of disease uncertainty. The reason for this phenomenon may be that the higher the patient ‘s overall education level, the greater the ability and access to information may be (55).

The study found that MMD patients exhibited a moderate level of resilience, with an average score of (78.75 78.8.92), which was higher than a previous study conduct in elderly ischemic stroke patients. The reason for this difference may be due to the different types of diseases and the age of the study population. In addition, the results of this study exhibited a negative correlation between resilience and illness uncertainty, which indicating that the higher the resilience level of MMD patients, the lower the level of disease uncertainty. The results has been confirmed in other previous studies (56, 57). In addition, one study showed that resilience and disease uncertainty jointly affect sleep quality in female systemic lupus erythematosus patients (56). Another study revealed the elastic in emergency surgery patients disease play a mediating role between uncertainty and anxiety (58). Thus, resilience may play a protective role for patients in avoiding the negative effects arising from illness uncertainty. Future research may focus on cultivating tenacity to reduce the uncertainty in illness in patients with a negative influence on the patients.

We also found that the present study identified a negative correlation between illness uncertainty and perceived social support, which was a discovery that aligns with previous studies (59, 60). This suggests that higher levels of social support from family, friends, and healthcare professionals are associated with reduced illness uncertainty. Medical staff should work closely with patients’ families to provide support and information. Group activities and communication can also help address challenges and improve well-being for MMD patients. Healthcare professionals can make a significant impact on patients’ psychological well-being and overall quality of life.

Although every effort has been made to better the design of this study, some limitations are unavoidable. First, the use of a convenience sampling method, while practical, may limit the representativeness of the sample. Second, the cross-sectional study design can only represent the psychological status of patients at the time of the study, but it cannot reflect the full and dynamic psychological changes of patients with the diagnosis of MMD. Future research could employ longitudinal designs for causal exploration. Third, reliance on self-reported data may introduce subjective and recall biases, potentially impacting assessment accuracy. The study’s sample was drawn exclusively from two hospitals in China, possibly limiting the generalizability of findings to other regions within the country. Further investigation in diverse organizational and cultural settings is warranted.

In this survey involving 263 MMD patients, illness uncertainty levels were found to be at a moderate level. Additionally, illness uncertainty exhibited negative correlations with resilience and perceived social support. Multiple linear regression analysis revealed that gender, working status, education level, smoking history, current residential status, diagnosis duration of MMD, total score of perceived social support, and total score of resilience were independent factors associated with illness uncertainty, collectively explaining a significant portion of its variance. These findings highlight the importance of greater attention and intervention for patients with underlying poor psychological status, while we observe that fostering resilience and giving patients adequate social support may have a positive effect on reducing the uncertainty of their illness and improving their overall well-being.

The original contributions presented in the study are included in the article/supplementary material. Further inquiries can be directed to the corresponding authors.

WZ: Conceptualization, Writing – original draft. ZP: Data curation, Writing – original draft. YZ: Data curation, Writing – original draft. DL: Conceptualization, Investigation, Validation, Writing – original draft. HZ: Investigation, Writing – original draft, Methodology, Validation. SL: Methodology, Writing – original draft. CL: Methodology, Writing – original draft. XX: Methodology, Writing – original draft. QL: Methodology, Writing – original draft. GY: Methodology, Writing – original draft. SHY: Methodology, Writing – original draft. RS: Methodology, Writing – original draft. SY: Writing – original draft, Supervision. DW: Supervision, Writing – original draft. ML: Investigation, Methodology, Writing – review & editing. HL: Data curation, Methodology, Supervision, Conceptualization, Formal analysis, Project administration, Validation, Investigation, Resources, Writing – original draft, Writing – review & editing.