Moyamoya disease outcome
Moyamoya disease (MMD) is an idiopathic disease with a progressive nature leading to recurrent stroke due to occlusion of the terminal internal carotid arteries.
The effectiveness of surgery for long-term protection of cognitive function is unclear even if the initial surgical treatment may result in good neurological outcomes 1).
Brain functional and structural connectivity between the supplementary motor area and inferior frontal gyrus in the left hemisphere are damaged in moyamoya disease (MMD). These findings could be useful in the evaluation of disease progression and prognosis of MMD 2).
The disorder can lead to negative mood and stress, which, left unresolved, may increase adverse health outcomes. Yang et al. conducted a cross-sectional survey to examine the stress and mood of adults with moyamoya disease. Participants were recruited at a university hospital in Seoul, Korea. Data were collected through questionnaires and review of participants' electronic medical records. A total of 109 adult patients participated. Significant correlations were found between perceived stress, anxiety, and depression. Adults with moyamoya disease experience anxiety, depression, and stress-related to the risk of cerebral hemorrhage or ischemia, similar to patients with other cerebrovascular diseases. If uncontrolled, negative mood and stress can cause adverse health outcomes. Health professionals caring for patients with moyamoya disease should carefully observe patients' stress and mood and develop interventions tailored to stages of the disease to help patients manage stress and mood. The study results provide baseline information for understanding the level of and the factors associated with stress and mood 3).
Pediatric Moyamoya disease patients have greater patency and a greater ability to establish good leptomeningeal collateral circulation (LMC) status than adult patients, and poor LMC status has a strong correlation with severe clinical symptoms and poor postoperative outcomes. LMC status may be an important factor in the differences in clinical characteristics and prognosis between pediatric and adult MMD patients 4).
Recurrent stroke after surgical revascularization is still a big issue for moyamoya disease (MMD).
Female, left-sided surgery, and edematous lesion were independent risk factors for postoperative TNEs; the left-sided surgery and edematous lesion were also independently associated with the severity of TNE. Although patients with postoperative TNEs had worse neurological status during the perioperative period, postoperative TNEs had no associations with worse mRS score at the time of discharge 5).
The outcome following surgery is very difficult to judge, and there is no standardised measurement to assess it. It is therefore important to know which approach for such patient is adequate.
Comparing to patients with acute idiopathic primary intraventricular hemorrhage (PIVH), patients with acute MMD-related PIVH have younger age, lower blood pressure, and better renal function. Moreover, patients with acute MMD-related PIVH have lower short-term mortality 6).
Sundaram et al., compared the long-term outcome of moyamoya patients treated conservatively to those who underwent RS.
A study population included all patients with moyamoya disease/syndrome from 2002 to 2012. The demographic, clinical characteristic and imaging details were reviewed. The outcome was obtained prospectively.
Of the 36 patients, 26 (72.2%) had MMD and 10 (27.8%) had moyamoya syndrome. The median age at onset of symptoms was 17.5 years (range, 10 months-55 years). Fifteen patients belonged to pediatric group and 21 were adults. All the pediatric patients had ischemic events at onset and 10 (47.6%) of the adults presented with hemorrhage. Twenty (55.6%) patients received conservative treatment and 16 (44.4%) underwent revascularization procedures. The median duration of follow-up was 28 months (range, 3-90 months). Three (18%) of the surgically treated patients had recurrent ischemic events on follow-up, but none of the conservatively treated patients had events. An excellent outcome (Modified Rankin Scale of ≤2) was seen in 12 (75%) surgically treated and 16 (94%) conservatively treated patients (p=0.17).
Compared to East Asians, our patients had a lower stroke recurrence rate and good functional outcome even with conservative treatment. Future studies should focus on clinical and imaging predictors of progression to select moyamoya patients for RS 7).