Choroba moyamoya – Rokowania, prognozy i postęp choroby

Choroba moyamoya
Rokowania, prognozy i postęp choroby

Choroba moyamoya (MMD) to postępująca arteriopatia prowadząca do zwężenia tętnic szyjnych wewnętrznych i ich odgałęzień, co zwiększa ryzyko udaru mózgu. Śmiertelność wynosi około 10% u dorosłych i 4,3% u dzieci, a progresja choroby obserwowana jest u 18,8% pacjentów. Kluczowe czynniki predykcyjne progresji to młodszy wiek (SMD 0,29; 95% CI 0,03–0,55; P=0,03), obciążenie rodzinne (OR 3,97; 95% CI 1,96–8,03; P<0,001) oraz nieprawidłowości kontralateralne (OR 3,95; 95% CI 1,10–14,20; P=0,04). Biochemiczne markery progresji obejmują podwyższone trójglicerydy ≥200 mg/dl (HR 2,292; 95% CI 1,00–4,979; P=0,03), podwyższone CRP, zwiększoną liczbę leukocytów oraz obniżoną względną rezerwę naczyniową mózgu (rCVR) w SPECT z Diamoksem (HR 3,431; 95% CI 1,254–9,389; P=0,02). Wartości te korelują z ryzykiem udaru krwotocznego i niedokrwiennego, co ma znaczenie prognostyczne.

Prognozy w chorobie moyamoya

Wskaźniki śmiertelności i czynniki ryzyka progresji
Markery biochemiczne i perfuzyjne jako predyktory progresji
Ocena funkcji poznawczych i rezerwy naczyniowej

Skale prognostyczne dla choroby moyamoya

Pediatryczna skala MRI Moyamoya (PMMS)

Czynniki wpływające na rokowanie

Wpływ leczenia na rokowanie
Typy procedur rewaskularyzacyjnych i ich wpływ na rokowanie

Wyzwania w przewidywaniu prognozy

Kolejne rozdziały

Prognozy w chorobie moyamoya

Choroba moyamoya (MMD) to postępująca arteriopatia charakteryzująca się zwężeniem wewnątrzczaszkowych tętnic szyjnych wewnętrznych i ich proksymalnych odgałęzień, co predysponuje pacjentów do udaru mózgu. Przewidywanie progresji choroby stanowi wyzwanie kliniczne, lecz jest kluczowe dla wczesnej interwencji i redukcji śmiertelności oraz niepełnosprawności.¹²

Wskaźniki śmiertelności i czynniki ryzyka progresji

Śmiertelność w chorobie moyamoya wynosi około 10% u dorosłych i 4,3% u dzieci. Około 50-60% pacjentów doświadcza stopniowego pogorszenia funkcji poznawczych, prawdopodobnie wskutek nawracających udarów. Zgon najczęściej następuje z powodu krwotoku, a rokowanie zależy od jego ciężkości, charakteru oraz nawrotowości incydentów.³

Badania wskazują, że wskaźnik progresji choroby moyamoya wynosi około 18,8%. Metaanalizy zidentyfikowały trzy główne czynniki predykcyjne progresji MMD:⁴

Młodszy wiek – pacjenci w młodszym wieku mają większe ryzyko progresji (różnica średnich standardowych [SMD]: 0,29, 95% przedział ufności [CI]: 0,55 do 0,03; P = 0,03)⁵
Obciążenie rodzinne – zwiększa ryzyko progresji prawie czterokrotnie (iloraz szans [OR] 3,97, 95% CI: 1,96 do 8,03; P<0,001)⁶⁷
Nieprawidłowości kontralateralne – zwiększają ryzyko progresji (OR 3,95, 95% CI: 1,10 do 14,20; P = 0,04)⁸⁹

Powyższe trzy czynniki są również związane z progresją choroby moyamoya z postaci jednostronnej do obustronnej.¹⁰

Markery biochemiczne i perfuzyjne jako predyktory progresji

Badania wykazały, że czynniki powodujące zmiany zapalne w naczyniach krwionośnych mogą wpływać na progresję choroby moyamoya. Do markerów biochemicznych i perfuzyjnych związanych z progresją choroby należą:¹¹

Podwyższone trójglicerydy (≥200) – znacząco zwiększają prawdopodobieństwo wystąpienia zdarzeń końcowych (HR: 2,292, CI 1,00-4,979, P=0,03)¹²
Podwyższone poziomy białka C-reaktywnego (CRP) – niezależnie związane z progresją choroby moyamoya¹³¹⁴
Zwiększona liczba białych krwinek (WBC) – istotnie związana z progresją choroby¹⁵
Znacznie zmniejszona względna rezerwa naczyniowa mózgu (rCVR) w badaniu SPECT z Diamoksem – istotnie związana z zdarzeniami końcowymi (HR: 3,431, CI 1,254-9,389, P=0,02)¹⁶¹⁷

Wykazano również korelację między podwyższonymi poziomami trójglicerydów a udarem krwotocznym oraz między znacznie obniżonym rCVR a udarem niedokrwiennym, co może pomóc w przewidywaniu rodzaju powikłań u pacjentów z chorobą moyamoya.¹⁸

Ocena funkcji poznawczych i rezerwy naczyniowej

Wyniki poznawcze u dzieci z chorobą moyamoya są zmienne i trudne do przewidzenia na podstawie początkowych objawów neurologicznych i badań. Analiza retrospektywna wykazała, że wyniki poznawcze są najlepiej prognozowane przez pomiar rezerwy naczyniowej mózgowej (CRC) po pierwszym jednostronnym zespoleniu. Jest to optymalny wczesny moment do przewidywania indywidualnego rokowania.¹⁹

Skale prognostyczne dla choroby moyamoya

Pediatryczna skala MRI Moyamoya (PMMS)

Opracowano systematyczną metodę punktacji opartą na zmianach miąższowych w przedoperacyjnym MRI mózgu, aby przewidzieć długoterminowe wyniki leczenia chirurgicznego dzieci z chorobą moyamoya. PMMS (Pediatric Moyamoya MRI Score) to proste narzędzie, które można szybko i łatwo obliczyć, a które koreluje z niepełnosprawnością po operacji.²⁰²¹

Kluczową zaletą tej skali jest potencjał stratyfikacji wyników klinicznych po operacji w porównaniu z obecnie stosowanymi skalami oceny. Korelacja między punktacją PMMS a skalą mRS (zmodyfikowana skala Rankina) w okresie obserwacji sugeruje, że system punktacji PMMS może być wykorzystywany do lepszego informowania pacjentów o ich rokowaniu po leczeniu chirurgicznym choroby moyamoya.²²

Czynniki wpływające na rokowanie

Rokowanie w chorobie moyamoya zależy od kilku kluczowych czynników:²³

Wczesność diagnozy – im wcześniej choroba została zdiagnozowana, tym lepsze rokowanie
Zakres uszkodzeń w momencie poszukiwania leczenia
Podjęcie leczenia – pacjenci, którzy poddają się leczeniu, mają lepsze rokowanie
Wiek pacjenta

Pacjenci z chorobą moyamoya, którzy zgłaszają się do leczenia podczas rozwijających się objawów, mają lepsze rokowanie niż ci, którzy zgłaszają się z objawami statycznymi (prawdopodobnie wskazującymi na dokonany udar).²⁴

Wpływ leczenia na rokowanie

Wczesne leczenie chirurgiczne może pomóc spowolnić progresję choroby moyamoya. Chociaż leczenie nie leczy choroby, może zapobiec udarom i poprawić przepływ krwi do mózgu. Celem leczenia jest zmniejszenie objawów i obniżenie ryzyka powikłań, takich jak udar niedokrwienny, krwawienie mózgowe i zgon.²⁵²⁶

Neurolog może zalecić operację rewaskularyzacji, jeśli u pacjenta występują objawy lub doszło do udarów. Operacja może być również zalecana, jeśli badania wykazują niski przepływ krwi do mózgu. Mimo że istnieją potencjalne ryzyko związane z operacją rewaskularyzacji, takie jak zmiany ciśnienia w naczyniach krwionośnych mózgu, korzyści z zabiegu przeważają nad ryzykiem.²⁷

Bez operacji choroba moyamoya może powodować pogorszenie funkcji poznawczych z powodu zwężających się naczyń krwionośnych. Oprócz leczenia chirurgicznego, terapia poznawczo-behawioralna może pomóc w rozwiązaniu problemów emocjonalnych związanych z chorobą moyamoya, takich jak radzenie sobie z obawami i niepewnością dotyczącą przyszłych udarów.²⁸²⁹

Typy procedur rewaskularyzacyjnych i ich wpływ na rokowanie

W chirurgii rewaskularyzacyjnej chirurdzy omijają zablokowane tętnice, łącząc naczynia krwionośne na zewnątrz czaszki z wewnętrzną częścią czaszki, aby przywrócić przepływ krwi do mózgu. Może to obejmować bezpośrednie lub pośrednie procedury rewaskularyzacyjne lub kombinację obu.³⁰³¹

Procedury rewaskularyzacyjne obejmują:³²³³

Bezpośrednia rewaskularyzacja (direct bypass) – chirurdzy przyszywają tętnicę skórną bezpośrednio do tętnicy mózgowej (tzw. bypass tętnicy skroniowej powierzchownej do tętnicy środkowej mózgu). Ta procedura natychmiast zwiększa przepływ krwi do mózgu. Jest preferowanym rozwiązaniem u dorosłych, ale może być trudna do wykonania u dzieci ze względu na rozmiar naczyń krwionośnych.
Pośrednie procedury rewaskularyzacyjne, takie jak:
- Encefaloduroarteriosynangioza (EDAS) – chirurg odsłania tętnicę skórną, wykonuje otwór w czaszce bezpośrednio pod tętnicą i układa nietkniętą tętnicę skórną na powierzchni mózgu, co pozwala naczyniom krwionośnym z tętnicy rozrastać się do mózgu z czasem.
- Encefalomiosynangioza (EMS) – chirurg oddziela mięsień w okolicy skroniowej czoła i umieszcza go na powierzchni mózgu po przyłączeniu tętnicy skórnej do powierzchni mózgu. Mięsień pomaga utrzymać tętnicę na miejscu, gdy naczynia krwionośne rozrastają się do mózgu.

Interwencja chirurgiczna może być bezpiecznie i skutecznie przeprowadzona przez doświadczony zespół chirurgiczny, który codziennie leczy osoby z chorobą moyamoya.³⁴

Wyzwania w przewidywaniu prognozy

Mimo postępów w diagnostyce i leczeniu choroby moyamoya, nadal istnieje wiele nierozwiązanych kwestii, w tym brak jasnych kryteriów diagnostycznych, konkretnych biomarkerów, precyzyjnego zrozumienia podstawowej patofizjologii oraz silniejszych dowodów dla wytycznych leczenia. Te luki w wiedzy utrudniają dokładne przewidywanie rokowania dla poszczególnych pacjentów.³⁵³⁶

Konieczne są dalsze badania prospektywne w celu oceny uogólnialności systemów punktacji, takich jak PMMS, oraz walidacji zidentyfikowanych czynników predykcyjnych progresji choroby.³⁷³⁸³⁹

Kolejne rozdziały

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Materiały źródłowe

#1 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
Predicting the progression of the disease can achieve timely warning, early intervention, and reduce the disability and mortality of the disease, but the factors that predict the progression of MMD were still controversial. […] In this systematic review and meta-analysis of clinical studies reporting predictors of disease progression in MMD patients, a total of 842 patients were included, and we found three important findings: (1) the progression rate for MMD patients was around 18.8%; (2) lower age, family history and contralateral abnormality were associated with progression in MMD patients, while sex, initial lesion side, Suzuki grade, hypertension, diabetes and smoking were not predictors for progression; (3) lower age, family history and contralateral abnormality were associated with progression in unilateral MMD patients.
#2 Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya in: Neurosurgical Focus Volume 51 Issue 3 (2021) Journals
https://thejns.org/focus/view/journals/neurosurg-focus/51/3/article-pE8.xml
Moyamoya is a progressive arteriopathy that predisposes patients to stroke due to stenosis of the intracranial internal carotid arteries and their proximal branches. Despite the morbidity caused by this condition, the ability to accurately predict prognosis for individual patients remains challenging. […] The goal of this study was to develop a systematic scoring method based on parenchymal findings on preoperative brain MRI to predict long-term outcomes for surgically treated pediatric patients with moyamoya. […] The PMMS was found to be a simple tool based on preoperative MRI data that could be quickly and easily calculated and correlated with disability. This scoring method may aid future development of predictive models of outcomes for children with moyamoya disease and moyamoya syndrome.
#3 Moyamoya Disease: Practice Essentials, Background, Etiology
https://emedicine.medscape.com/article/1180952-overview
Death from moyamoya disease is usually from hemorrhage. The outcome of the disease depends on the severity and nature of the hemorrhage; the prognosis depends on recurrent attacks. […] Mortality rates from moyamoya disease are approximately 10% in adults and 4.3% in children. About 5060% of affected individuals experience a gradual deterioration of cognitive function, presumably from recurrent strokes. […] Patients with moyamoya disease who present for treatment while symptoms are evolving have a better prognosis than do those who present with static symptoms (which probably indicate a completed stroke). […] There is still lots of work to be done, as there are still many unresolved issues about moyamoya disease and syndrome, including lack of clear diagnostic criteria, particular biomarkers, precise understanding of the underlying pathophysiology, and stronger evidence for treatment guidelines.
#4 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
Predicting the progression of the disease can achieve timely warning, early intervention, and reduce the disability and mortality of the disease, but the factors that predict the progression of MMD were still controversial. […] In this systematic review and meta-analysis of clinical studies reporting predictors of disease progression in MMD patients, a total of 842 patients were included, and we found three important findings: (1) the progression rate for MMD patients was around 18.8%; (2) lower age, family history and contralateral abnormality were associated with progression in MMD patients, while sex, initial lesion side, Suzuki grade, hypertension, diabetes and smoking were not predictors for progression; (3) lower age, family history and contralateral abnormality were associated with progression in unilateral MMD patients.
#5 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
The progress of Moyamoya disease (MMD) is often accompanied by the occurrence of new ischemia or hemorrhagic events, which was difficult to predict. This systematic review and meta-analysis aimed to identify predictors for progression in MMD patients. […] The estimated pooled means indicated lower age (standard mean difference [SMD]: 0.29, 95% confidence interval [CI]: 0.55 to 0.03; P = 0.03), family history (odds ratio [OR] 3.97, 95% CI: 1.96 to 8.03; P 0.001) and contralateral abnormality (OR 3.95, 95% CI: 1.10 to 14.20; P = 0.04) were associated with progression in MMD patients. […] This meta-analysis revealed that lower age, family history and contralateral abnormality were associated with progression in MMD patients. The same three factors are associated with the progression of unilateral to bilateral MMD. Further studies are needed to validate our results.
#6 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
The progress of Moyamoya disease (MMD) is often accompanied by the occurrence of new ischemia or hemorrhagic events, which was difficult to predict. This systematic review and meta-analysis aimed to identify predictors for progression in MMD patients. […] The estimated pooled means indicated lower age (standard mean difference [SMD]: 0.29, 95% confidence interval [CI]: 0.55 to 0.03; P = 0.03), family history (odds ratio [OR] 3.97, 95% CI: 1.96 to 8.03; P 0.001) and contralateral abnormality (OR 3.95, 95% CI: 1.10 to 14.20; P = 0.04) were associated with progression in MMD patients. […] This meta-analysis revealed that lower age, family history and contralateral abnormality were associated with progression in MMD patients. The same three factors are associated with the progression of unilateral to bilateral MMD. Further studies are needed to validate our results.
#7 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
Our results suggested that MMD patients with family history were more likely to progress. Therefore, patients with moyamoya disease with a family history should take precautions to reduce the probability of adverse events. […] We found that contralateral abnormality was a robust predictor for progression in MMD patients.
#8 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
The progress of Moyamoya disease (MMD) is often accompanied by the occurrence of new ischemia or hemorrhagic events, which was difficult to predict. This systematic review and meta-analysis aimed to identify predictors for progression in MMD patients. […] The estimated pooled means indicated lower age (standard mean difference [SMD]: 0.29, 95% confidence interval [CI]: 0.55 to 0.03; P = 0.03), family history (odds ratio [OR] 3.97, 95% CI: 1.96 to 8.03; P 0.001) and contralateral abnormality (OR 3.95, 95% CI: 1.10 to 14.20; P = 0.04) were associated with progression in MMD patients. […] This meta-analysis revealed that lower age, family history and contralateral abnormality were associated with progression in MMD patients. The same three factors are associated with the progression of unilateral to bilateral MMD. Further studies are needed to validate our results.
#9 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
Our results suggested that MMD patients with family history were more likely to progress. Therefore, patients with moyamoya disease with a family history should take precautions to reduce the probability of adverse events. […] We found that contralateral abnormality was a robust predictor for progression in MMD patients.
#10 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
Predicting the progression of the disease can achieve timely warning, early intervention, and reduce the disability and mortality of the disease, but the factors that predict the progression of MMD were still controversial. […] In this systematic review and meta-analysis of clinical studies reporting predictors of disease progression in MMD patients, a total of 842 patients were included, and we found three important findings: (1) the progression rate for MMD patients was around 18.8%; (2) lower age, family history and contralateral abnormality were associated with progression in MMD patients, while sex, initial lesion side, Suzuki grade, hypertension, diabetes and smoking were not predictors for progression; (3) lower age, family history and contralateral abnormality were associated with progression in unilateral MMD patients.
#11 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Elevated triglyceride levels (200) were significantly associated with higher likelihood of end-point events (HR: 2.292, CI 1.004.979, P=0.03). […] Severe decreased rCVR findings on Diamox SPECT were also significantly associated with end-point events (HR: 3.431, CI 1.2549.389, P=0.02). […] Increased CRP levels and white blood cell (WBC) count were significantly associated with moyamoya disease progression. […] Elevated triglyceride, CRP, and inflammation markers, as well as decreased rCVR, are potential predictors of moyamoya disease progression and complication types. […] These findings confirmed that factors causing inflammatory changes in blood vessels, such as triglycerides, C-reactive protein (CRP), and white blood cell (WBC) count, can influence the progression of Moyamoya disease.
#12 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Elevated triglyceride levels (200) were significantly associated with higher likelihood of end-point events (HR: 2.292, CI 1.004.979, P=0.03). […] Severe decreased rCVR findings on Diamox SPECT were also significantly associated with end-point events (HR: 3.431, CI 1.2549.389, P=0.02). […] Increased CRP levels and white blood cell (WBC) count were significantly associated with moyamoya disease progression. […] Elevated triglyceride, CRP, and inflammation markers, as well as decreased rCVR, are potential predictors of moyamoya disease progression and complication types. […] These findings confirmed that factors causing inflammatory changes in blood vessels, such as triglycerides, C-reactive protein (CRP), and white blood cell (WBC) count, can influence the progression of Moyamoya disease.
#13 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Elevated triglyceride levels (200) were significantly associated with higher likelihood of end-point events (HR: 2.292, CI 1.004.979, P=0.03). […] Severe decreased rCVR findings on Diamox SPECT were also significantly associated with end-point events (HR: 3.431, CI 1.2549.389, P=0.02). […] Increased CRP levels and white blood cell (WBC) count were significantly associated with moyamoya disease progression. […] Elevated triglyceride, CRP, and inflammation markers, as well as decreased rCVR, are potential predictors of moyamoya disease progression and complication types. […] These findings confirmed that factors causing inflammatory changes in blood vessels, such as triglycerides, C-reactive protein (CRP), and white blood cell (WBC) count, can influence the progression of Moyamoya disease.
#14 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Moreover, in line with previous studies, it was determined that decreased relative cerebral vascular reserve (rCVR) can affect the prognosis of patients with this condition. […] Overall, these findings provide insights into the potential role of chemical markers, such as triglyceride and CRP, in predicting the progression of moyamoya disease. […] The observed association between inflammatory markers and moyamoya disease warrants further investigation and may inform future studies aimed at understanding the pathophysiology of the disease and developing effective treatment strategies. […] Overall, these findings suggest that chemical and perfusion markers may be useful in predicting the type of complication in moyamoya disease. […] The observed association between triglyceride levels and hemorrhagic stroke, and severe decreased rCVR findings on Diamox SPECT and ischemic stroke, warrants further investigation and may inform future studies aimed at understanding the pathophysiology of moyamoya disease and developing effective treatment strategies. […] In conclusion, our study has demonstrated that elevated CRP levels and high triglycerides are independently associated with the progression of Moyamoya disease.
#15 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Elevated triglyceride levels (200) were significantly associated with higher likelihood of end-point events (HR: 2.292, CI 1.004.979, P=0.03). […] Severe decreased rCVR findings on Diamox SPECT were also significantly associated with end-point events (HR: 3.431, CI 1.2549.389, P=0.02). […] Increased CRP levels and white blood cell (WBC) count were significantly associated with moyamoya disease progression. […] Elevated triglyceride, CRP, and inflammation markers, as well as decreased rCVR, are potential predictors of moyamoya disease progression and complication types. […] These findings confirmed that factors causing inflammatory changes in blood vessels, such as triglycerides, C-reactive protein (CRP), and white blood cell (WBC) count, can influence the progression of Moyamoya disease.
#16 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Elevated triglyceride levels (200) were significantly associated with higher likelihood of end-point events (HR: 2.292, CI 1.004.979, P=0.03). […] Severe decreased rCVR findings on Diamox SPECT were also significantly associated with end-point events (HR: 3.431, CI 1.2549.389, P=0.02). […] Increased CRP levels and white blood cell (WBC) count were significantly associated with moyamoya disease progression. […] Elevated triglyceride, CRP, and inflammation markers, as well as decreased rCVR, are potential predictors of moyamoya disease progression and complication types. […] These findings confirmed that factors causing inflammatory changes in blood vessels, such as triglycerides, C-reactive protein (CRP), and white blood cell (WBC) count, can influence the progression of Moyamoya disease.
#17 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Moreover, in line with previous studies, it was determined that decreased relative cerebral vascular reserve (rCVR) can affect the prognosis of patients with this condition. […] Overall, these findings provide insights into the potential role of chemical markers, such as triglyceride and CRP, in predicting the progression of moyamoya disease. […] The observed association between inflammatory markers and moyamoya disease warrants further investigation and may inform future studies aimed at understanding the pathophysiology of the disease and developing effective treatment strategies. […] Overall, these findings suggest that chemical and perfusion markers may be useful in predicting the type of complication in moyamoya disease. […] The observed association between triglyceride levels and hemorrhagic stroke, and severe decreased rCVR findings on Diamox SPECT and ischemic stroke, warrants further investigation and may inform future studies aimed at understanding the pathophysiology of moyamoya disease and developing effective treatment strategies. […] In conclusion, our study has demonstrated that elevated CRP levels and high triglycerides are independently associated with the progression of Moyamoya disease.
#18 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Moreover, in line with previous studies, it was determined that decreased relative cerebral vascular reserve (rCVR) can affect the prognosis of patients with this condition. […] Overall, these findings provide insights into the potential role of chemical markers, such as triglyceride and CRP, in predicting the progression of moyamoya disease. […] The observed association between inflammatory markers and moyamoya disease warrants further investigation and may inform future studies aimed at understanding the pathophysiology of the disease and developing effective treatment strategies. […] Overall, these findings suggest that chemical and perfusion markers may be useful in predicting the type of complication in moyamoya disease. […] The observed association between triglyceride levels and hemorrhagic stroke, and severe decreased rCVR findings on Diamox SPECT and ischemic stroke, warrants further investigation and may inform future studies aimed at understanding the pathophysiology of moyamoya disease and developing effective treatment strategies. […] In conclusion, our study has demonstrated that elevated CRP levels and high triglycerides are independently associated with the progression of Moyamoya disease.
#19
https://link.springer.com/article/10.1007/s00381-023-05884-6
Cognitive outcomes of pediatric moyamoya disease are variable and difficult to predict on the basis of initial neurological signs and examinations. […] To determine the best early time point for outcome prediction, we retrospectively analyzed the correlation between cognitive outcomes and the cerebrovascular reserve capacity (CRC) measured before, between, and after staged bilateral anastomoses. […] Cognitive outcomes were first clearly discriminated by the CRC after the first-side unilateral anastomosis, which is the optimal early timing for the prediction of individual prognosis.
#20 Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya in: Neurosurgical Focus Volume 51 Issue 3 (2021) Journals
https://thejns.org/focus/view/journals/neurosurg-focus/51/3/article-pE8.xml
Moyamoya is a progressive arteriopathy that predisposes patients to stroke due to stenosis of the intracranial internal carotid arteries and their proximal branches. Despite the morbidity caused by this condition, the ability to accurately predict prognosis for individual patients remains challenging. […] The goal of this study was to develop a systematic scoring method based on parenchymal findings on preoperative brain MRI to predict long-term outcomes for surgically treated pediatric patients with moyamoya. […] The PMMS was found to be a simple tool based on preoperative MRI data that could be quickly and easily calculated and correlated with disability. This scoring method may aid future development of predictive models of outcomes for children with moyamoya disease and moyamoya syndrome.
#21 Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya in: Neurosurgical Focus Volume 51 Issue 3 (2021) Journals
https://thejns.org/focus/view/journals/neurosurg-focus/51/3/article-pE8.xml
A current major deficiency in the understanding of moyamoya is the ability to accurately predict prognosis for individual patients. […] The correlation between PMMSs and mRS scores on follow-up suggests that the PMMS scoring system may be used to better inform patients of their prognosis following surgical treatment for moyamoya. […] The PMMS is a straightforward grading system that correlates with postoperative disability in children with moyamoya vasculopathy after surgical treatment, regardless of treatment modality. […] The key benefit of this score is its potential for stratifying clinical outcome after surgery compared with currently employed grading scales. […] The PMMS is a simple tool based on preoperative MRI data that can be quickly and easily calculated and can potentially aid in the prediction of outcomes and clinical decision-making for patients with moyamoya disease and moyamoya syndrome undergoing surgical treatment. Future prospective studies are needed to assess the generalizability of this scoring system.
#22 Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya in: Neurosurgical Focus Volume 51 Issue 3 (2021) Journals
https://thejns.org/focus/view/journals/neurosurg-focus/51/3/article-pE8.xml
A current major deficiency in the understanding of moyamoya is the ability to accurately predict prognosis for individual patients. […] The correlation between PMMSs and mRS scores on follow-up suggests that the PMMS scoring system may be used to better inform patients of their prognosis following surgical treatment for moyamoya. […] The PMMS is a straightforward grading system that correlates with postoperative disability in children with moyamoya vasculopathy after surgical treatment, regardless of treatment modality. […] The key benefit of this score is its potential for stratifying clinical outcome after surgery compared with currently employed grading scales. […] The PMMS is a simple tool based on preoperative MRI data that can be quickly and easily calculated and can potentially aid in the prediction of outcomes and clinical decision-making for patients with moyamoya disease and moyamoya syndrome undergoing surgical treatment. Future prospective studies are needed to assess the generalizability of this scoring system.
#23 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
Moyamoya treatment consultation at Mayo Clinic […] The outlook for moyamoya disease, also called the prognosis, depends on several factors, including: […] How early the disease was diagnosed. […] How much damage has happened when you seek treatment. […] Whether or not you undergo treatment. […] Age. […] Early surgical treatment can help slow progression of moyamoya disease. […] Your neurologist may recommend revascularization surgery if you develop symptoms or strokes. […] Surgery also may be recommended if tests show evidence of low blood flow to your brain. […] Possible surgery risks of revascularization procedures for moyamoya disease include changes in pressure in the blood vessels in the brain. […] However, the benefits of surgery largely outweigh the risks. […] Without surgery, moyamoya disease can cause cognitive decline due to narrowing blood vessels.
#24 Moyamoya Disease: Practice Essentials, Background, Etiology
https://emedicine.medscape.com/article/1180952-overview
Death from moyamoya disease is usually from hemorrhage. The outcome of the disease depends on the severity and nature of the hemorrhage; the prognosis depends on recurrent attacks. […] Mortality rates from moyamoya disease are approximately 10% in adults and 4.3% in children. About 5060% of affected individuals experience a gradual deterioration of cognitive function, presumably from recurrent strokes. […] Patients with moyamoya disease who present for treatment while symptoms are evolving have a better prognosis than do those who present with static symptoms (which probably indicate a completed stroke). […] There is still lots of work to be done, as there are still many unresolved issues about moyamoya disease and syndrome, including lack of clear diagnostic criteria, particular biomarkers, precise understanding of the underlying pathophysiology, and stronger evidence for treatment guidelines.
#25 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
Moyamoya treatment consultation at Mayo Clinic […] The outlook for moyamoya disease, also called the prognosis, depends on several factors, including: […] How early the disease was diagnosed. […] How much damage has happened when you seek treatment. […] Whether or not you undergo treatment. […] Age. […] Early surgical treatment can help slow progression of moyamoya disease. […] Your neurologist may recommend revascularization surgery if you develop symptoms or strokes. […] Surgery also may be recommended if tests show evidence of low blood flow to your brain. […] Possible surgery risks of revascularization procedures for moyamoya disease include changes in pressure in the blood vessels in the brain. […] However, the benefits of surgery largely outweigh the risks. […] Without surgery, moyamoya disease can cause cognitive decline due to narrowing blood vessels.
#26 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
Cognitive behavioral therapy can help address emotional issues related to having moyamoya disease, such as how to cope with fears and uncertainties about future strokes. […] […] […] Mayo Clinic healthcare professionals provide comprehensive treatment for people with moyamoya. […] Healthcare professionals evaluate your condition and determine the treatment that is right for you. […] Treatment doesn’t cure moyamoya disease. […] But treatment can help prevent strokes. […] The goal of treatment is to reduce your symptoms and improve blood flow to the brain. […] Treatment also aims to lower your risk of complications. […] Complications include ischemic stroke caused by a lack of blood flow, bleeding in your brain and death. […] […] […] In revascularization surgery, surgeons bypass blocked arteries.
#27 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
Moyamoya treatment consultation at Mayo Clinic […] The outlook for moyamoya disease, also called the prognosis, depends on several factors, including: […] How early the disease was diagnosed. […] How much damage has happened when you seek treatment. […] Whether or not you undergo treatment. […] Age. […] Early surgical treatment can help slow progression of moyamoya disease. […] Your neurologist may recommend revascularization surgery if you develop symptoms or strokes. […] Surgery also may be recommended if tests show evidence of low blood flow to your brain. […] Possible surgery risks of revascularization procedures for moyamoya disease include changes in pressure in the blood vessels in the brain. […] However, the benefits of surgery largely outweigh the risks. […] Without surgery, moyamoya disease can cause cognitive decline due to narrowing blood vessels.
#28 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
Moyamoya treatment consultation at Mayo Clinic […] The outlook for moyamoya disease, also called the prognosis, depends on several factors, including: […] How early the disease was diagnosed. […] How much damage has happened when you seek treatment. […] Whether or not you undergo treatment. […] Age. […] Early surgical treatment can help slow progression of moyamoya disease. […] Your neurologist may recommend revascularization surgery if you develop symptoms or strokes. […] Surgery also may be recommended if tests show evidence of low blood flow to your brain. […] Possible surgery risks of revascularization procedures for moyamoya disease include changes in pressure in the blood vessels in the brain. […] However, the benefits of surgery largely outweigh the risks. […] Without surgery, moyamoya disease can cause cognitive decline due to narrowing blood vessels.
#29 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
Cognitive behavioral therapy can help address emotional issues related to having moyamoya disease, such as how to cope with fears and uncertainties about future strokes. […] […] […] Mayo Clinic healthcare professionals provide comprehensive treatment for people with moyamoya. […] Healthcare professionals evaluate your condition and determine the treatment that is right for you. […] Treatment doesn’t cure moyamoya disease. […] But treatment can help prevent strokes. […] The goal of treatment is to reduce your symptoms and improve blood flow to the brain. […] Treatment also aims to lower your risk of complications. […] Complications include ischemic stroke caused by a lack of blood flow, bleeding in your brain and death. […] […] […] In revascularization surgery, surgeons bypass blocked arteries.
#30 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
Cognitive behavioral therapy can help address emotional issues related to having moyamoya disease, such as how to cope with fears and uncertainties about future strokes. […] […] […] Mayo Clinic healthcare professionals provide comprehensive treatment for people with moyamoya. […] Healthcare professionals evaluate your condition and determine the treatment that is right for you. […] Treatment doesn’t cure moyamoya disease. […] But treatment can help prevent strokes. […] The goal of treatment is to reduce your symptoms and improve blood flow to the brain. […] Treatment also aims to lower your risk of complications. […] Complications include ischemic stroke caused by a lack of blood flow, bleeding in your brain and death. […] […] […] In revascularization surgery, surgeons bypass blocked arteries.
#31 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
They do this by connecting blood vessels on the outside of the skull to the inside of the skull to help restore blood flow to your brain. […] This may include direct or indirect revascularization procedures. […] Or it may include a combination of both. […] […] […] In direct revascularization surgery, surgeons stitch the scalp artery directly to a brain artery. […] This also is known as superficial temporal artery to middle cerebral artery bypass surgery. […] This procedure increases blood flow to your brain immediately. […] Direct bypass surgery may be difficult to perform in children due to the size of the blood vessels. […] But it’s the preferred option in adults. […] This intervention can be performed safely and effectively by an experienced surgical team that treats people with moyamoya disease daily.
#32 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
They do this by connecting blood vessels on the outside of the skull to the inside of the skull to help restore blood flow to your brain. […] This may include direct or indirect revascularization procedures. […] Or it may include a combination of both. […] […] […] In direct revascularization surgery, surgeons stitch the scalp artery directly to a brain artery. […] This also is known as superficial temporal artery to middle cerebral artery bypass surgery. […] This procedure increases blood flow to your brain immediately. […] Direct bypass surgery may be difficult to perform in children due to the size of the blood vessels. […] But it’s the preferred option in adults. […] This intervention can be performed safely and effectively by an experienced surgical team that treats people with moyamoya disease daily.
#33 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
[…] […] Indirect revascularization procedures include encephaloduroarteriosynangiosis. […] This also is called EDAS. […] They include encephalomyosynangiosis, also called EMS. […] Or they include a combination of both. […] In EDAS, a surgeon makes a small temporary opening on the scalp to expose the artery. […] Then the surgeon makes an opening in your skull directly beneath the artery. […] The surgeon lays the intact scalp artery onto the surface of your brain, which allows blood vessels from the artery to grow into your brain over time. […] The surgeon then replaces the bone and closes the opening in your skull. […] […] […] Your surgeon may perform EMS with EDAS. […] In this procedure, your surgeon separates a muscle in the temple region of your forehead. […] The surgeon places it onto the surface of your brain after attaching the scalp artery to the surface of your brain. […] The muscle helps to hold the artery in place as blood vessels grow into your brain over time.
#34 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
They do this by connecting blood vessels on the outside of the skull to the inside of the skull to help restore blood flow to your brain. […] This may include direct or indirect revascularization procedures. […] Or it may include a combination of both. […] […] […] In direct revascularization surgery, surgeons stitch the scalp artery directly to a brain artery. […] This also is known as superficial temporal artery to middle cerebral artery bypass surgery. […] This procedure increases blood flow to your brain immediately. […] Direct bypass surgery may be difficult to perform in children due to the size of the blood vessels. […] But it’s the preferred option in adults. […] This intervention can be performed safely and effectively by an experienced surgical team that treats people with moyamoya disease daily.
#35 Moyamoya Disease: Practice Essentials, Background, Etiology
https://emedicine.medscape.com/article/1180952-overview
Death from moyamoya disease is usually from hemorrhage. The outcome of the disease depends on the severity and nature of the hemorrhage; the prognosis depends on recurrent attacks. […] Mortality rates from moyamoya disease are approximately 10% in adults and 4.3% in children. About 5060% of affected individuals experience a gradual deterioration of cognitive function, presumably from recurrent strokes. […] Patients with moyamoya disease who present for treatment while symptoms are evolving have a better prognosis than do those who present with static symptoms (which probably indicate a completed stroke). […] There is still lots of work to be done, as there are still many unresolved issues about moyamoya disease and syndrome, including lack of clear diagnostic criteria, particular biomarkers, precise understanding of the underlying pathophysiology, and stronger evidence for treatment guidelines.
#36 Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya in: Neurosurgical Focus Volume 51 Issue 3 (2021) Journals
https://thejns.org/focus/view/journals/neurosurg-focus/51/3/article-pE8.xml
A current major deficiency in the understanding of moyamoya is the ability to accurately predict prognosis for individual patients. […] The correlation between PMMSs and mRS scores on follow-up suggests that the PMMS scoring system may be used to better inform patients of their prognosis following surgical treatment for moyamoya. […] The PMMS is a straightforward grading system that correlates with postoperative disability in children with moyamoya vasculopathy after surgical treatment, regardless of treatment modality. […] The key benefit of this score is its potential for stratifying clinical outcome after surgery compared with currently employed grading scales. […] The PMMS is a simple tool based on preoperative MRI data that can be quickly and easily calculated and can potentially aid in the prediction of outcomes and clinical decision-making for patients with moyamoya disease and moyamoya syndrome undergoing surgical treatment. Future prospective studies are needed to assess the generalizability of this scoring system.
#37 Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
https://pmc.ncbi.nlm.nih.gov/articles/PMC10018164/
The progress of Moyamoya disease (MMD) is often accompanied by the occurrence of new ischemia or hemorrhagic events, which was difficult to predict. This systematic review and meta-analysis aimed to identify predictors for progression in MMD patients. […] The estimated pooled means indicated lower age (standard mean difference [SMD]: 0.29, 95% confidence interval [CI]: 0.55 to 0.03; P = 0.03), family history (odds ratio [OR] 3.97, 95% CI: 1.96 to 8.03; P 0.001) and contralateral abnormality (OR 3.95, 95% CI: 1.10 to 14.20; P = 0.04) were associated with progression in MMD patients. […] This meta-analysis revealed that lower age, family history and contralateral abnormality were associated with progression in MMD patients. The same three factors are associated with the progression of unilateral to bilateral MMD. Further studies are needed to validate our results.
#38 Pediatric moyamoya MRI score: an imaging-based scale to predict outcomes in surgically treated pediatric patients with moyamoya in: Neurosurgical Focus Volume 51 Issue 3 (2021) Journals
https://thejns.org/focus/view/journals/neurosurg-focus/51/3/article-pE8.xml
A current major deficiency in the understanding of moyamoya is the ability to accurately predict prognosis for individual patients. […] The correlation between PMMSs and mRS scores on follow-up suggests that the PMMS scoring system may be used to better inform patients of their prognosis following surgical treatment for moyamoya. […] The PMMS is a straightforward grading system that correlates with postoperative disability in children with moyamoya vasculopathy after surgical treatment, regardless of treatment modality. […] The key benefit of this score is its potential for stratifying clinical outcome after surgery compared with currently employed grading scales. […] The PMMS is a simple tool based on preoperative MRI data that can be quickly and easily calculated and can potentially aid in the prediction of outcomes and clinical decision-making for patients with moyamoya disease and moyamoya syndrome undergoing surgical treatment. Future prospective studies are needed to assess the generalizability of this scoring system.
#39 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
https://www.nature.com/articles/s41598-023-47984-y
Moreover, in line with previous studies, it was determined that decreased relative cerebral vascular reserve (rCVR) can affect the prognosis of patients with this condition. […] Overall, these findings provide insights into the potential role of chemical markers, such as triglyceride and CRP, in predicting the progression of moyamoya disease. […] The observed association between inflammatory markers and moyamoya disease warrants further investigation and may inform future studies aimed at understanding the pathophysiology of the disease and developing effective treatment strategies. […] Overall, these findings suggest that chemical and perfusion markers may be useful in predicting the type of complication in moyamoya disease. […] The observed association between triglyceride levels and hemorrhagic stroke, and severe decreased rCVR findings on Diamox SPECT and ischemic stroke, warrants further investigation and may inform future studies aimed at understanding the pathophysiology of moyamoya disease and developing effective treatment strategies. […] In conclusion, our study has demonstrated that elevated CRP levels and high triglycerides are independently associated with the progression of Moyamoya disease.