Choroba moyamoya
Objawy

Choroba moyamoya to przewlekłe, postępujące schorzenie naczyniowe charakteryzujące się zwężeniem tętnic szyjnych wewnętrznych i ich odgałęzień w podstawie mózgu, prowadzącym do niedokrwienia mózgu. W odpowiedzi na niedokrwienie powstaje charakterystyczna sieć drobnych naczyń kolateralnych, widoczna w angiografii jako „kłąb dymu”. Choroba manifestuje się dwoma szczytami wiekowymi: u dzieci (5-10 lat) i dorosłych (30-50 lat). U dzieci dominują udary niedokrwienne i przemijające ataki niedokrwienne (TIA), często prowokowane przez hiperwentylację, natomiast u dorosłych częściej występują udary krwotoczne. Objawy obejmują jednostronne niedowłady, zaburzenia mowy, widzenia, napady padaczkowe oraz bóle głowy o charakterze migrenopodobnym, często oporne na leczenie. Progresja choroby prowadzi do narastającego ryzyka udarów i powikłań neurologicznych, a u około 50-66% nieleczonych pacjentów obserwuje się pogorszenie stanu neurologicznego.

Choroba moyamoya – charakterystyka

Choroba moyamoya to rzadkie, przewlekłe i postępujące schorzenie naczyniowe charakteryzujące się zwężeniem tętnic szyjnych wewnętrznych oraz ich głównych odgałęzień w podstawie mózgu, prowadzącym do ograniczenia dopływu krwi do mózgu. W odpowiedzi na niedokrwienie organizm tworzy sieć drobnych, kruchych naczyń krwionośnych (naczyń kolateralnych), które w badaniach angiograficznych przypominają „kłęby dymu” – stąd japońska nazwa „moyamoya”, oznaczająca właśnie „kłąb dymu”.12

Choroba moyamoya może wystąpić w każdym wieku, jednak najczęściej objawia się w dwóch szczytowych okresach: u dzieci w wieku 5-10 lat oraz u dorosłych między 30 a 50 rokiem życia. Jest to schorzenie postępujące, co oznacza, że z czasem stan pacjenta ulega pogorszeniu, jeśli nie zostanie wdrożone odpowiednie leczenie.12

Objawy kliniczne choroby moyamoya

Objawy choroby moyamoya są bezpośrednio związane ze zmniejszonym przepływem krwi do mózgu, co prowadzi do niedotlenienia tkanki mózgowej. Prezentacja kliniczna może się różnić w zależności od wieku pacjenta, stopnia zaawansowania choroby oraz obszaru mózgu dotkniętego niedokrwieniem.12

Objawy u dzieci

U dzieci pierwszym objawem choroby moyamoya jest zwykle udar niedokrwienny mózgu lub nawracające przemijające ataki niedokrwienne (TIA, tzw. mini-udary). Do charakterystycznych objawów należą:12

Charakterystyczną cechą choroby moyamoya u dzieci jest prowokowanie objawów przez czynniki zwiększające hiperwentylację, takie jak płacz, gra na instrumentach dętych, jedzenie gorących potraw, wysiłek fizyczny czy odwodnienie. Hiperwentylacja powoduje obniżenie poziomu dwutlenku węgla, co prowadzi do skurczu naczyń mózgowych i nasilenia niedokrwienia.12

Objawy u dorosłych

U dorosłych obraz kliniczny choroby moyamoya różni się od obserwowanego u dzieci. Dorośli pacjenci mogą doświadczać zarówno udarów niedokrwiennych lub TIA, jak i – co charakterystyczne – krwawień śródmózgowych (udarów krwotocznych). Krwawienia te wynikają głównie z pęknięcia kruchych naczyń kolateralnych, które powstały w odpowiedzi na niedokrwienie.12

Typowe objawy u dorosłych obejmują:12

  • Nagły, silny ból głowy (w przypadku krwawienia)
  • Jednostronne osłabienie lub porażenie
  • Zaburzenia mowy
  • Zaburzenia widzenia (w tym utrata wzroku w jednym oku, niewyraźne widzenie, pogorszone widzenie obuoczne)
  • Niemożność rozpoznawania przedmiotów
  • Omdlenia
  • Napady drgawkowe
  • Zaburzenia funkcji poznawczych12

Objawy wspólne dla wszystkich grup wiekowych

Niezależnie od wieku pacjenta, choroba moyamoya może powodować następujące objawy:12

  • Bóle głowy o charakterze migrenowym, często oporne na standardowe leczenie
  • Napady drgawkowe
  • Osłabienie, drętwienie lub porażenie twarzy, ramienia lub nogi (zwykle jednostronne)
  • Zaburzenia widzenia
  • Zaburzenia mowy lub rozumienia mowy (afazja)
  • Zaburzenia poznawcze lub opóźnienia rozwojowe
  • Ruchy mimowolne
  • Zawroty głowy i zaburzenia równowagi12

Objawy te mogą być wywoływane lub nasilane przez wysiłek fizyczny, płacz, kaszel, napinanie się lub gorączkę.1

Progresja choroby moyamoya

Choroba moyamoya ma charakter postępujący, co oznacza, że zwężenie naczyń krwionośnych pogłębia się z czasem, prowadząc do nasilenia objawów i zwiększonego ryzyka powikłań, jeśli nie zostanie wdrożone odpowiednie leczenie.12

Naturalny przebieg choroby

Przebieg naturalny choroby moyamoya jest zmienny, jednak u większości nieleczonych pacjentów dochodzi do progresji. Szacuje się, że u około 50-66% nieleczonych pacjentów choroba postępuje, prowadząc do pogorszenia stanu neurologicznego.12

Progresja może mieć charakter:1

  • Powolny, przewlekły przebieg
  • Przebieg przerywany z rzadkimi incydentami
  • Gwałtowny przebieg z szybkim pogorszeniem stanu neurologicznego

U pacjentów z jednostronną chorobą moyamoya (zajęcie naczyń tylko po jednej stronie mózgu) ryzyko progresji do formy obustronnej wynosi około 30% podczas długoterminowej obserwacji.1

Czynniki związane z progresją choroby

Badania zidentyfikowały kilka czynników, które mogą być związane z szybszą progresją choroby moyamoya:12

  • Młodszy wiek w momencie rozpoznania
  • Rodzinne występowanie choroby moyamoya
  • Nieprawidłowości po stronie przeciwnej w początkowym obrazowaniu
  • Wrodzone wady serca
  • Wcześniejsza radioterapia czaszki
  • Pochodzenie azjatyckie
  • Podwyższony poziom trójglicerydów (≥200 mg/dl)
  • Podwyższony poziom białka C-reaktywnego (CRP)
  • Zwiększona liczba białych krwinek12

Fazy progresji choroby

Przebieg choroby moyamoya można podzielić na fazy, które charakteryzują się stopniem zmian naczyniowych i związanym z nimi ryzykiem rozwoju objawów lub powikłań:1

Faza wczesna – Pacjenci mogą zacząć doświadczać objawów związanych ze zmniejszonym przepływem krwi do mózgu, takich jak przemijające ataki niedokrwienne (TIA). Objawy mogą obejmować przejściowe osłabienie, drętwienie, trudności w mówieniu lub zaburzenia widzenia.1

Faza pośrednia – Wraz z postępem choroby wzrasta ryzyko poważniejszych udarów. Udary niedokrwienne, wynikające ze zmniejszonego przepływu krwi, mogą prowadzić do bardziej wyraźnych i trwałych deficytów neurologicznych, takich jak osłabienie, porażenie, trudności w mówieniu lub inne zaburzenia neurologiczne.1

Faza zaawansowana – W tym zaawansowanym stadium ryzyko ciężkich i nawracających udarów może się utrzymywać. Pacjenci mogą doświadczać zwiększonej częstości udarów niedokrwiennych lub krwotocznych, prowadzących do znaczniejszego i trwalszego upośledzenia neurologicznego.1

Powikłania choroby moyamoya

Nieleczona choroba moyamoya może prowadzić do szeregu poważnych powikłań, które mogą mieć trwały wpływ na jakość życia pacjenta.12

Udary mózgu i ich konsekwencje

Najpoważniejszym powikłaniem choroby moyamoya są udary mózgu, które mogą być niedokrwienne (spowodowane niedrożnością naczyń) lub krwotoczne (spowodowane pęknięciem kruchych naczyń kolateralnych).12

Konsekwencje udarów obejmują:12

  • Porażenie lub niedowład (najczęściej jednostronny)
  • Zaburzenia mowy (afazja, dyzartria)
  • Zaburzenia widzenia
  • Zaburzenia równowagi i koordynacji
  • Zaburzenia czucia
  • Napady padaczkowe (wtórne do uszkodzenia mózgu)
  • Zaburzenia funkcji poznawczych
  • Zaburzenia zachowania i nastroju12

Zaburzenia poznawcze i rozwojowe

Choroba moyamoya, nawet bez jawnych klinicznie udarów, może prowadzić do zaburzeń poznawczych u około 30% pacjentów, co sugeruje, że przewlekłe niedokrwienie mózgu jest istotnym czynnikiem przyczyniającym się do tych problemów.1

Zaburzenia poznawcze mogą obejmować:12

  • Upośledzenie szybkości przetwarzania informacji
  • Zaburzenia pamięci werbalnej
  • Obniżenie fluencji słownej
  • Zaburzenia funkcji wykonawczych
  • Trudności w uczeniu się
  • Problemy z koncentracją i uwagą
  • U dzieci – opóźnienia rozwojowe12

Przewlekły ból głowy

Ból głowy jest częstym objawem choroby moyamoya, występującym zarówno u dzieci, jak i dorosłych. Ma on zazwyczaj charakter migrenopodobny i często jest oporny na standardowe leczenie przeciwbólowe.12

Mechanizm powstawania bólu głowy wiąże się prawdopodobnie ze stymulacją nocyceptorów opony twardej przez rozszerzone naczynia kolateralne. Warto zauważyć, że bóle głowy mogą utrzymywać się nawet po skutecznym leczeniu chirurgicznym.12

Zaburzenia ruchowe

U części pacjentów z chorobą moyamoya, szczególnie u dzieci, mogą wystąpić zaburzenia ruchowe w postaci mimowolnych ruchów pląsawiczych lub dystonicznych. Zaburzenia te mogą być bardzo upośledzające i trudne do leczenia farmakologicznego, jednak często ustępują po rewaskularyzacji chirurgicznej.1

Rokowanie w chorobie moyamoya

Rokowanie w chorobie moyamoya zależy od wielu czynników, w tym od wieku zachorowania, wczesności rozpoznania, zastosowanego leczenia oraz występowania powikłań.12

Czynniki wpływające na rokowanie

Do czynników, które mogą wskazywać na gorsze rokowanie, należą:12

  • Udar krwotoczny jako pierwsza manifestacja choroby
  • Płeć żeńska
  • Rodzinna postać choroby
  • Młody wiek zachorowania
  • Współistniejące zaburzenia tarczycy
  • Palenie tytoniu
  • Zaawansowana choroba w momencie rozpoznania
  • Przebyte udary w wywiadzie12

Naturalna historia choroby bez leczenia

Bez odpowiedniego leczenia choroba moyamoya prowadzi do postępującego pogorszenia stanu neurologicznego. U większości nieleczonych pacjentów dochodzi do nawracających udarów i postępującego spadku funkcji poznawczych w miarę zwężania się naczyń krwionośnych.12

Śmiertelność w nieleczonej chorobie moyamoya wynosi około 10% u dorosłych i 4,3% u dzieci, głównie w wyniku krwawienia śródmózgowego. U około 50-60% pacjentów dochodzi do stopniowego pogorszenia funkcji poznawczych, prawdopodobnie w wyniku nawracających udarów.12

Wpływ leczenia na rokowanie

Wczesne rozpoznanie i odpowiednie leczenie, szczególnie chirurgiczne, znacząco poprawiają rokowanie w chorobie moyamoya.12

Badania wykazały, że u pacjentów pediatrycznych poddanych leczeniu chirurgicznemu progresja choroby występuje tylko u około 2,6% w porównaniu do 80% w grupie nieleczonej. Leczenie operacyjne zmniejsza również ryzyko udarów, krwawień śródmózgowych i innych powikłań.12

Przy odpowiednim leczeniu większość pacjentów z chorobą moyamoya może prowadzić stosunkowo normalne życie z dobrą długoterminową prognozą. Szczególnie dzieci, które otrzymały kombinację leczenia zachowawczego i chirurgicznego, mają dobre rokowanie z niskim ryzykiem nawrotu udaru.12

Podsumowanie objawów choroby moyamoya

Grupa wiekowa Główne objawy Charakterystyczne cechy
Dzieci (5-10 lat) Udary niedokrwienne, TIA Objawy często prowokowane przez hiperwentylację (płacz, wysiłek)
Napady drgawkowe, opóźnienia rozwojowe, bóle głowy Rzadko krwawienia śródmózgowe
Dorośli (30-50 lat) Udary krwotoczne Częściej krwawienia śródmózgowe niż u dzieci
Udary niedokrwienne, TIA, zaburzenia poznawcze Bardziej zróżnicowany obraz kliniczny
Wszystkie grupy wiekowe Bóle głowy, napady drgawkowe, jednostronne niedowłady Objawy mogą być przemijające lub trwałe
Zaburzenia mowy, widzenia, funkcji poznawczych Progresywny charakter choroby bez leczenia

Znaczenie wczesnej diagnostyki i leczenia

Wczesne rozpoznanie i leczenie choroby moyamoya ma kluczowe znaczenie dla zapobiegania postępowi choroby i rozwojowi poważnych powikłań, takich jak udary czy trwałe deficyty neurologiczne.12

Pacjenci, u których zdiagnozowano jednostronną chorobę moyamoya, powinni być regularnie monitorowani za pomocą badań obrazowych (MRI i MRA), aby wcześnie wykryć progresję do postaci obustronnej.1

Leczenie chirurgiczne (rewaskularyzacja) jest najskuteczniejszą metodą poprawy przepływu krwi do mózgu i zmniejszenia ryzyka udarów. Pacjenci, którzy przechodzą zabieg we wczesnym stadium choroby, gdy objawy są jeszcze w fazie rozwoju, mają lepsze rokowanie niż ci, u których leczenie rozpoczęto po wystąpieniu trwałych deficytów neurologicznych.12

Wczesna interwencja jest szczególnie istotna u dzieci, ponieważ choroba moyamoya może wpływać na rozwój poznawczy i motoryczny. Ponadto, dzieci mają większą neuroplastyczność, co pozwala na lepszą adaptację i regenerację po leczeniu chirurgicznym.12

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  1. 09.04.2026
  2. www.leksykon.com.pl

Materiały źródłowe

  • #1 Moyamoya disease – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/symptoms-causes/syc-20355586
    In moyamoya disease, arteries to the brain become narrow and may even close, leading to reduced delivery of oxygen-rich blood to the brain. This lack of blood flow to the brain can cause a stroke and other symptoms. […] The condition may cause a ministroke, known as a transient ischemic attack (TIA), or a stroke. It also can cause bleeding in the brain. Moyamoya disease can affect how well the brain functions and can cause cognitive and developmental delays or disability. […] Moyamoya disease may happen at any age. But symptoms are most common in children between ages 5 and 10 and in adults between ages 30 and 50. Spotting symptoms early is very important to prevent complications such as a stroke. […] Moyamoya disease causes different symptoms in adults and children. In children, the first symptom is usually a stroke or recurrent transient ischemic attack, also called TIA. Adults may experience these symptoms, as well. But adults also may experience bleeding in the brain, known as a hemorrhagic stroke. The bleeding happens because of the way blood vessels in the brain formed.
  • #1 Moyamoya disease // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/moyamoya-disease
    Moyamoya disease may happen at any age. But symptoms are most common in children between ages 5 and 10 and in adults between ages 30 and 50. Spotting symptoms early is very important to prevent complications such as a stroke. […] Moyamoya disease causes different symptoms in adults and children. In children, the first symptom is usually a stroke or recurrent transient ischemic attack, also called TIA. Adults may experience these symptoms, as well. But adults also may experience bleeding in the brain, known as a hemorrhagic stroke. The bleeding happens because of the way blood vessels in the brain formed. […] Symptoms of moyamoya disease related to reduced blood flow to the brain include: Headache. Seizures. Weakness, numbness or paralysis in the face, arm or leg. This is typically on one side of the body. Vision problems. Trouble speaking or understanding others, known as aphasia. Cognitive or developmental delays. Involuntary movements. […] These symptoms can be triggered by exercise, crying, coughing, straining or a fever. […] Early detection and treatment can help prevent a stroke and serious complications.
  • #1 Moyamoya Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK535455/
    Cerebral ischemic events in the form of TIA or ischemic infarcts are the commonest presentation of all Moyamoya patients. […] Symptoms can be categorized on the basis of etiology: those due to cerebral ischemia (i.e., stroke, transient ischemic attack (TIA), and seizure) and those due to the growth of collateral vessels that compensate for ischemia (i.e. hemorrhage and headache). […] In the pediatric population, Moyamoya typically manifests with TIA or ischemic stroke. Attacks are usually precipitated by hyperventilation during crying, playing a wind instrument, or eating hot noodles. Hyperventilation decreases carbon dioxide, leading to cerebral vasoconstriction and aggravating cerebral hypo-perfusion. Children could also suffer from an intellectual disability. Deterioration of cognition is in a linear relationship with the number of strokes and chronic hypoxemia from a progressive narrowing of the cerebral vasculature.
  • #1 Moyamoya Disease | Cedars-Sinai
    https://www.cedars-sinai.org/health-library/diseases-and-conditions/m/moyamoya-disease.html
    Symptoms of moyamoya disease vary depending on the age of the patient. Adults with the condition experience bleeding or strokes. Other symptoms in adults include: Fainting or blacking out, Loss of sight in one eye, Blurry vision, Poor vision in both eyes, Inability to recognize objects. […] In children, moyamoya disease tends to cause transient ischemic attacks (TIA), also known as microstrokes or „warning strokes.” In children, it also may cause seizures. Other symptoms may include: Uncontrolled movements, Developmental disabilities, Headaches, Speech difficulties, Periods of being unable to move their feet, legs or arms. Because this condition involves bleeding, patients may also experience a shortage of red blood cells (anemia), which has symptoms such as fatigue and pale skin. […] The condition tends to get worse without treatment, and can lead to severe brain damage or death.
  • #1 Moyamoya Disease Causes, Symptoms, Treatment, Surgery
    https://www.medicinenet.com/moyamoya_disease/article.htm
    Moyamoya disease can occur in children and adults. There are two times the disease incidence peaks: most commonly, at about 10 years of age in children and between ages 30 to 50 in adults. Moyamoya disease may present differently in adults and children. […] In adults, the most common symptoms include bleeding in the brain (hemorrhagic strokes). Other symptoms of Moyamoya disease in adults include: Lightheadedness or fainting, Visual disturbances, Blurred vision, Loss of vision in one eye, Poor vision in both eyes, Inability to recognize objects, Seizures, Headaches, Unilateral (on one side) weakness, numbness, or paralysis in your face, arm, or leg, Cognitive decline. […] Moyamoya disease in children often results in transient ischemic attacks (TIAs), also called „ministrokes,” and seizures.
  • #1 Moyamoya disease – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/symptoms-causes/syc-20355586
    Symptoms of moyamoya disease related to reduced blood flow to the brain include: Headache. Seizures. Weakness, numbness or paralysis in the face, arm or leg. This is typically on one side of the body. Vision problems. Trouble speaking or understanding others, known as aphasia. Cognitive or developmental delays. Involuntary movements. […] These symptoms can be triggered by exercise, crying, coughing, straining or a fever. […] Early detection and treatment can help prevent a stroke and serious complications. […] Most complications from moyamoya disease are linked to the effects of strokes. They include seizures, paralysis and vision problems. Other complications include speech problems, movement disorders and developmental delays. Moyamoya disease can cause serious and permanent damage to the brain.
  • #1 Moyamoya Disease | University of Michigan Health
    https://www.uofmhealth.org/conditions-treatments/brain-neurological-conditions/moyamoya-disease
    The first signs of moyamoya disease are often typical stroke symptoms such as: […] Other symptoms may include seizures, headaches, involuntary movements, or progressive difficulty in thinking or memory.
  • #1 Moyamoya disease | Causes, Symptoms & Treatment | UW Medicine
    https://www.uwmedicine.org/conditions-symptoms/brain-nervous-system/moyamoya
    Moyamoya disease can affect children and adults. It’s also progressive, meaning it gets worse over time. […] Many people with moyamoya disease don’t have any symptoms until their condition has reached an advanced stage. In some cases, the first sign of the disease is a stroke, transient ischemic attack (TIA) or brain bleeding. This is true in both children and adults. […] Strokes, TIAs (ministrokes) and brain bleeds can happen quickly in people with moyamoya disease. And these conditions share some of the same sudden symptoms. […] Some children and adults with moyamoya disease have symptoms that come on gradually and then get worse over time. […] It’s important to let your provider know if you have any of these symptoms. The sooner you’re diagnosed with moyamoya disease, the sooner you can begin treatment.
  • #1 Moyamoya Disease
    https://practicalneurology.com/diseases-diagnoses/stroke/moyamoya-disease/31617/
    Clinical symptoms vary according to the location of the hemorrhage, which can be intraventricular, intraparenchymal, or subarachnoid. Hemorrhage has been attributed to rupture of fragile collateral vessels and may also be caused by moyamoya-associated microaneurysms in some cases. […] Headache is a frequent presenting symptom and is typically of a migraine-like quality and refractory to medical treatment. Headache is generally believed to be caused by dilatation of the collateral vessels that may stimulate the dural nociceptors. […] The natural history of moyamoya is variable; however, moyamoya progresses in the majority of cases. Progression may have a slow indolent course, an intermittent pattern with rare events, or be fulminant with steep neurologic decline. […] It is estimated that up to two-thirds of people with moya-moya disease have symptomatic progression that cannot be halted by medical treatment alone. […] The initial neurologic status of an individual is the best predictor of the disease course. Early diagnosis coupled with close follow-up and intervention when appropriate are the major determinants of a favorable long-term outcome.
  • #1 Progression of disease in unilateral moyamoya syndrome – PubMed
    https://pubmed.ncbi.nlm.nih.gov/18275294/
    Progression of vasculopathy associated with moyamoya syndrome is extremely variable. […] Of 235 surgically treated patients with moyamoya syndrome, 33 (14%) presented with unilateral disease (4 adults and 29 children). […] During this period, 10 (30%) of 33 patients progressed to bilateral disease. […] Of patients with unilateral moyamoya syndrome, 30% will have progression of arteriopathy during long-term follow-up. […] Several factors, including contralateral abnormalities on initial imaging, congenital cardiac anomaly, previous cranial irradiation, Asian ancestry, and familial moyamoya syndrome, were associated with an increased risk of progression. […] Patients with known unilateral angiographic disease should undergo continued monitoring by using MR imaging and MR angiography at regular intervals.
  • #1 Frontiers | Potential predictors for progression of moyamoya disease: A systematic review and meta-analysis
    https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2023.1128338/full
    Background: 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. […] Results: A total of 842 patients from 12 studies were included. 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. Subgroup analyses indicated that the same three factors were associated with the progression of unilateral to bilateral MMD. [...] Conclusions: 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.
  • #1 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
    https://www.nature.com/articles/s41598-023-47984-y
    Moyamoya disease is characterized as a condition in which progressive stenosis and occlusion are observed in the terminal portion of the internal carotid artery (ICA), accompanied by the development of an abnormal vascular network around it. These pathological features are not stagnant; they continuously cause vascular changes and progress, leading to ischemic and hemorrhagic strokes. The progression rate of moyamoya vessels is known to be approximately 20% in the non-surgical group. Furthermore, the rebleeding risk in patients presenting with hemorrhage is 30-65%, and even asymptomatic patients have a 20-40% risk of stroke and progression. […] Elevated triglyceride levels (200) were significantly associated with higher likelihood of end-point events (HR: 2.292, CI 1.004-4.979, P=0.03). Severe decreased rCVR findings on Diamox SPECT were also significantly associated with end-point events (HR: 3.431, CI 1.254-9.389, P=0.02). Increased CRP levels and white blood cell (WBC) count were significantly associated with moyamoya disease progression.
  • #1 Moyamoya Disease: Causes, Symptoms, Stages, and Treatment Options
    https://www.maxhealthcare.in/blogs/know-about-moyamoya-disease
    Vision problems: Blurred vision, loss of vision in one eye, or other visual disturbances might occur due to reduced blood flow to the eyes. […] Weakness or paralysis: Sudden weakness or paralysis on one side of the body, known as hemiparesis or hemiplegia, can occur during strokes. […] Moyamoya disease typically progresses through stages characterized by the degree of vascular changes and the associated risk of developing symptoms or complications. The stages of Moyamoya disease are often categorized as follows: […] During this stage, individuals might begin experiencing symptoms due to reduced blood flow to the brain. Symptoms can include transient ischemic attacks (TIAs), often referred to as mini-strokes. These TIAs are brief episodes of neurological dysfunction, manifesting as temporary weakness, numbness, speech difficulties, or visual disturbances.
  • #1 Moyamoya Disease: Causes, Symptoms, Stages, and Treatment Options
    https://www.maxhealthcare.in/blogs/know-about-moyamoya-disease
    As the disease progresses, the risk of more severe strokes increases. Ischemic strokes, resulting from reduced blood flow, can lead to more pronounced and lasting neurological deficits, such as weakness, paralysis, speech difficulties, or other neurological impairments. […] In this advanced stage, the risk of severe and recurrent strokes might persist. Individuals may experience a higher frequency of ischemic or hemorrhagic strokes, leading to more significant and lasting neurological impairment. […] Early diagnosis and intervention are critical to potentially slow or halt disease progression and reduce the risk of developing more severe symptoms or complications. […] Long-term effects can vary, but they might include cognitive impairment, recurrent strokes, neurological deficits, and, in severe cases, permanent disability. Effective management and treatment can help mitigate long-term effects.
  • #1
    https://www.mountelizabeth.com.sg/conditions-diseases/moyamoya-disease/symptoms-causes
    Moyamoya disease is a progressive cerebrovascular disorder characterised by the narrowing or blockage of the carotid arteries in the brain. This condition can result in recurrent strokes and transient ischaemic attacks (TIAs). The symptoms of Moyamoya disease can vary depending on the age of the patient and the severity of the condition. Common symptoms include: Sudden weakness or paralysis, particularly on one side of the body, indicating a stroke or a TIA. Recurrent, severe headaches. Seizures, particularly seen in children. Cognitive decline, including difficulty with thinking, memory, or learning. Vision problems, including sudden vision loss or disturbances. Speech difficulties: Trouble speaking or understanding speech. Involuntary movements such as chorea or dystonia. […] Due to blood vessels progressively narrowing or being blocked in the brain, several serious complications can occur, including: Recurrent TIAs. Ischaemic stroke, otherwise known as cerebral infarct. Haemorrhagic stroke. Cognitive and developmental delays, particularly in children. Weakness or paralysis. Speech difficulties. Seizures. Emotional and behavioural changes such as depression, anxiety, and personality changes.
  • #1 Moyamoya Disease Clinical Presentation: History and Physical Examination, Complications
    https://emedicine.medscape.com/article/1180952-clinical
    Children and adults with moyamoya disease may have different clinical presentations. The symptoms and clinical course vary widely, with the disease ranging from being asymptomatic to manifesting as transient events to causing severe neurologic deficits. […] Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Intellectual disability or persistent neurologic deficits may be present. […] Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults. […] A meta-analysis published in 2018 showed that approximately 30% of children and adults with moyamoya disease or syndrome have cognitive impairment, even without occurrence of stroke, suggesting that chronic hypoperfusion is a contributing factor.
  • #1 Moyamoya Disease – PsychDB
    https://www.psychdb.com/cl/moyamoya-disease
    Moyamoya Disease is a cerebrovascular disorder characterized by progressive unilateral or bilateral stenosis of the distal internal carotid arteries (often extending to the anterior and middle cerebral arteries). Up to a quarter of patients may present with heterogenous psychiatric symptoms that may be misdiagnosed as a behavioural (especially in children), mood, or psychotic disorder. […] The core clinical feature is ischemia secondary to hypoperfusion, which can cause repeated transient ischemic attacks (TIAs) or stroke in the cerebral cortex (e.g. – frontal, parietal, and temporal lobes). Hemorrhagic stroke can also occur from rupture of fragile collateral vessels or aneurysms with intraventricular, intraparenchymal, and subarachnoid distributions. The disruption of the anterior circulation can result in hemiparesis, speech disturbance, and hemisensory impairments. Headache is a common presenting and persisting symptom even with treatment. Cognitive impairment is also common, with impairments in processing speed, verbal memory, verbal fluency, and executive function.
  • #1 How Moyamoya disease affects children: a… | Riley Children’s Health
    https://www.rileychildrens.org/connections/how-moyamoya-disease-affects-children-a-comprehensive-guide
    Moyamoya disease symptoms are caused by reduced blood flow to the brain and weakened blood vessels. Common symptoms include: […] Alongside the physical symptoms, children with Moyamoya disease may experience cognitive challenges such as difficulties with memory, attention and learning. Emotional and psychological factors may include anxiety or depression. Socially, your child may experience feelings of isolation. […] “Moyamoya also greatly impacts children’s everyday routine,” Dr. Qaiser said. “Patients have to be mindful of contact sports, and they also need to make sure they stay well hydrated, given that dehydration can lead to strokes.” […] Ultimately, moyamoya is a complex condition significantly affecting children, and understanding its impacts is crucial to managing the disease.
  • #1 Moyamoya – Neurosurgeon
    https://www.vicneurosurgery.com.au/moyamoya/
    Dilated moyamoya compensatory vessels within the inner part of the brain known as the basal ganglia have been implicated in the development of abnormal jerking or writhing (choreiform) movements in children. These may be very disabling and difficult to treat, though often reported to resolve following revascularisation surgery. […] Seen within more than half the moyamoya patient population, this may be due to either an accumulation of multiple small strokes, or simply from chronic cerebral ischaemia.
  • #1 Moyamoya Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK535455/
    In addition to TIA or ischemic strokes, adults also frequently present with hemorrhagic stroke. Hemorrhage mainly results from a rupture of fragile moyamoya collaterals and seen in deep areas of the brain, such as basal ganglia, periventricular deep white matter. Intraventricular hemorrhage is common due to the close proximity of the primary site of intracerebral hemorrhage. […] Seizures are common complications of either ischemic or hemorrhagic events. […] Migraine-like headaches are common in both, children and/or adults and occur probably from the stimulation of dural nociceptor by dilated trans-dural collaterals. […] The overall prognosis is variable. Two-thirds of patients with Moyamoya disease have a symptomatic progression over five years with poor outcomes. Progression of the occlusive process continues regardless of symptom severity, ongoing treatment, age, sex, type and location of the disease. However, data from the North American series shows 13.3% and 1.7% of ischemic and hemorrhagic strokes, respectively. Factors that may show a poor prognosis could be but not limited to: hemorrhagic strokes at presentation, female gender, familial form of onset and pediatric age of onset. Concomitant thyroid disorder and smoking impacts negatively on overall prognosis in MMD. Early surgical revascularization has a preferable prognosis.
  • #1 Moyamoya Disease Symptoms and Treatment | Froedtert & MCW
    https://www.froedtert.com/moyamoya
    Symptoms of moyamoya are similar to a stroke. Symptoms can include weakness or numbness in an arm or leg, difficulty speaking or paralysis affecting one side of the body. Other symptoms include seizures or changes in memory or recall. A severe headache can occur along with the other stroke symptoms that develop. […] Without surgery, individuals with moyamoya disease may experience progressive mental decline and multiple strokes as the arteries continue to narrow. Without treatment, moyamoya disease can be fatal as the result of intracerebral hemorrhage (bleeding within the brain).
  • #1 Moyamoya Disease: Practice Essentials, Background, Etiology
    https://emedicine.medscape.com/article/1180952-overview
    Children and adults with moyamoya disease may have different clinical presentations. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children. […] 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).
  • #1 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
    Moyamoya disease is usually diagnosed by a doctor who specializes in conditions of the brain, called a neurologist. […] 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. […] 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. […] Without surgery, moyamoya disease can cause cognitive decline due to narrowing blood vessels.
  • #1 Moyamoya disease, Moya Moya | Mayfield Brain & Spine
    https://mayfieldclinic.com/pe-moyamoya.htm
    The progression of Moyamoya follows a typical course and can be classified into stages based on angiography findings. Once the process of blockage (vascular occlusion) begins, it tends to continue. No known medication can reverse the blockage. Once a major stroke or bleeding has occurred, the patient may be left with permanent loss of function. It is important, therefore, to treat this condition promptly. […] Scientists have estimated that among untreated patients with moyamoya, 50 to 66 percent will experience progression of the disease and will suffer poor outcomes. By contrast, a large analysis of surgically treated pediatric patients found disease progression in an estimated 2.6 percent of patients. A more recent review of untreated patients who had no symptoms found an annual stroke rate of 3.2 percent and disease progression in 80 percent. […] If left untreated, the disease will invariably progress, producing clinical deterioration and potentially irreversible neurological deficits over time.
  • #1 Moyamoya Disease | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/moyamoya-disease
    The most common symptom that brings children to the doctor is a sudden loss of function that resembles a stroke, such as difficulty speaking or weakness of one side of the body. […] Sometimes these symptoms are short-lived, resolving over a matter of hours, and no tissue damage is evident on an MRI or CT scan. Doctors call this a transient ischemic attack (TIA). Sometimes the symptoms are more long-lasting or more severe. In these cases, there may be actual tissue damage seen on a brain MRI or CT scan, which would indicate there has been a stroke and not just a TIA. When there has been a stroke, the effects are more long-lasting (days to weeks) or may even be permanent. […] The majority of children receiving the combination of medical and surgical therapy for moyamoya disease have a good outcome, with a low risk of recurrent stroke. Restoring blood flow to the brain is an important step in maximizing their recovery of function. Surgery can be very effective in delivering blood to the brain and improving function.
  • #1 Moyamoya Disease – Child Neurology Foundation
    https://www.childneurologyfoundation.org/disorder/moyamoya-disease/
    Most strokes in children present with the SUDDEN ONSET of symptoms. These symptoms are neurological in nature. […] Common examples include: Weakness of one side of the body is called hemiparesis. Hemiparesis usually occurs in the face, arm, or leg. […] A child may have difficulty making or understanding speech. […] Vision loss occurs in one part of the visual world (e.g. they cannot see one half of the world regardless of which eye they use) or double vision. […] A child may experience poor walking or coordination. […] This includes a feeling of decreased sensation or tingling. […] Children with strokes due to moyamoya may have long-term neurological problems. These can include: Movement problems, Language disorders, Learning, attention, or behavior difficulties, Recurrent seizures (epilepsy). […] In some children, moyamoya disease can be mild. It can be stable over time. These children can be treated with medications to prevent stroke. […] In other children, moyamoya progresses more quickly. A higher risk of stroke may need to be treated with surgery.
  • #2 Moyamoya Disease | Causes, Symptoms & Treatment
    https://www.cincinnatichildrens.org/health/m/moyamoya
    Moyamoya disease is a rare but very serious condition affecting one or both of the internal carotid arteries. These arteries are located at the base of the brain. Moyamoya disease causes them to become narrowed over time. This restricts blood flow to the brain. As a result, blood clots may form, and the risk of having a stroke, mini-stroke or seizure may increase. […] Moyamoya develops over the course of months or years. It is most common during childhood. As the arteries become narrower, the brain forms tiny blood vessels to create new pathways for blood to flow. The tiny blood vessels look like puffs of smoke on a cerebral angiogram. The word moyamoya means puff of smoke in Japanese. […] The first sign of Moyamoya usually is an ischemic stroke. An ischemic stroke happens when a blood clot blocks blood flow to the brain. When brain tissue is cut off from its supply of oxygen for more than three minutes, it begins to die.
  • #2 Moyamoya Disease Symptoms, Surgery and Treatment | UPMC
    https://www.upmc.com/services/neurosurgery/brain/conditions/neurovascular-conditions/conditions/moyamoya
    Moyamoya disease is progressive, meaning the vessel walls will continue to narrow over time. […] Signs and symptoms of Moyamoya disease may include: Aphasia (loss of ability to use or comprehend words), Blurred vision, Cognitive (reasoning or remembering) impairment, Convulsions, Involuntary movements, Numbness in arms and legs, Seizures, Severe headaches. […] Adults with Moyamoya disease also often experience hemorrhagic stroke (a burst blood vessel inside the brain). […] Without surgery, the majority of people with Moyamoya disease will experience mental decline and multiple strokes because of the progressive narrowing of the arteries. If untreated, Moyamoya disease can be fatal.
  • #2 Moyamoya Disease Clinical Presentation: History and Physical Examination, Complications
    https://emedicine.medscape.com/article/1180952-clinical
    Children and adults with moyamoya disease may have different clinical presentations. The symptoms and clinical course vary widely, with the disease ranging from being asymptomatic to manifesting as transient events to causing severe neurologic deficits. […] Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Intellectual disability or persistent neurologic deficits may be present. […] Adults may have symptoms and signs similar to those in children, but intraventricular, subarachnoid, or intracerebral hemorrhage of sudden onset is more common in adults. […] A meta-analysis published in 2018 showed that approximately 30% of children and adults with moyamoya disease or syndrome have cognitive impairment, even without occurrence of stroke, suggesting that chronic hypoperfusion is a contributing factor.
  • #2 Moyamoya Disease – Child Neurology Foundation
    https://www.childneurologyfoundation.org/disorder/moyamoya-disease/
    Moyamoya disease is a common cause of stroke in children. In moyamoya disease, the blood vessels that bring blood to the brain slowly narrow over time. […] As blood flow to the brain decreases over time, a child’s body may form small blood vessels to compensate. These are called collateral vessels. […] Moyamoya’s narrowing of blood vessels can prevent enough blood from getting to the brain. When blood flow to the brain is restricted, a child can have a stroke. Moyamoya can also cause a stroke warning called a transient ischemic attack. […] The primary symptom of moyamoya disease is stroke. Stroke means that part of the brain is damaged due to the lack of blood flow and oxygen to the brain. Caregivers must know how to recognize the signs of stroke and seek immediate medical attention as needed.
  • #2 Moyamoya Disease | Doctor
    https://patient.info/doctor/moyamoya-disease.htm
    In adults, about half of cases first present with intracranial haemorrhage, mainly as the result of a rupture of the fragile, maximally dilated, collateral vessels and occurring mostly in the anterior circulation territory, although arteries from the posterior circulation may also be involved. […] Psychiatric symptoms such as personality changes, psychosis, or depression may occur. […] More general symptoms such as vertigo, dizziness, or headache may be present due to stroke, secondary orthostatic intolerance, or dilation of meningeal collaterals. […] During childhood, disease onset may be triggered by events like coughing or crying that lead to a decrease in carbon dioxide, resulting in vasoconstriction and further cerebral hypoperfusion. […] The prognosis for ischaemic and haemorrhagic MMD depends on the severity and location of the cerebrovascular event.
  • #2 Moyamoya Disease: Symptoms, Stages, and Treatment
    https://www.healthline.com/health/moyamoya-disease
    People with moyamoya disease may experience complications like stroke or bleeding in the brain. […] The symptoms in children can differ from those in adults. […] In children, the symptoms usually occur between 5 and 10 years of age. The first symptom is usually a stroke or ministroke (also called a transient ischemic attack, or TIA). […] Adults with moyamoya disease typically experience symptoms between 30 and 50 years of age. Adults may also experience strokes or TIAs, but more commonly, they experience bleeding in the brain, also called a hemorrhagic stroke. […] The symptoms of moyamoya disease that occur at any age can include: aphasia (difficulties with speaking or understanding others), cognitive or developmental delays, frequent headaches, involuntary movements, paralysis or weakness in your face, arm, or leg, usually on one side of the body, seizures, vision problems.
  • #2 Moyamoya Disease Causes, Symptoms, Treatment, Surgery
    https://www.medicinenet.com/moyamoya_disease/article.htm
    Moyamoya disease can occur in children and adults. There are two times the disease incidence peaks: most commonly, at about 10 years of age in children and between ages 30 to 50 in adults. Moyamoya disease may present differently in adults and children. […] In adults, the most common symptoms include bleeding in the brain (hemorrhagic strokes). Other symptoms of Moyamoya disease in adults include: Lightheadedness or fainting, Visual disturbances, Blurred vision, Loss of vision in one eye, Poor vision in both eyes, Inability to recognize objects, Seizures, Headaches, Unilateral (on one side) weakness, numbness, or paralysis in your face, arm, or leg, Cognitive decline. […] Moyamoya disease in children often results in transient ischemic attacks (TIAs), also called „ministrokes,” and seizures.
  • #2
    https://www.mountelizabeth.com.sg/conditions-diseases/moyamoya-disease/symptoms-causes
    Moyamoya disease is a progressive cerebrovascular disorder characterised by the narrowing or blockage of the carotid arteries in the brain. This condition can result in recurrent strokes and transient ischaemic attacks (TIAs). The symptoms of Moyamoya disease can vary depending on the age of the patient and the severity of the condition. Common symptoms include: Sudden weakness or paralysis, particularly on one side of the body, indicating a stroke or a TIA. Recurrent, severe headaches. Seizures, particularly seen in children. Cognitive decline, including difficulty with thinking, memory, or learning. Vision problems, including sudden vision loss or disturbances. Speech difficulties: Trouble speaking or understanding speech. Involuntary movements such as chorea or dystonia. […] Due to blood vessels progressively narrowing or being blocked in the brain, several serious complications can occur, including: Recurrent TIAs. Ischaemic stroke, otherwise known as cerebral infarct. Haemorrhagic stroke. Cognitive and developmental delays, particularly in children. Weakness or paralysis. Speech difficulties. Seizures. Emotional and behavioural changes such as depression, anxiety, and personality changes.
  • #2 Moyamoya Disease: Causes, Symptoms, Stages, and Treatment Options
    https://www.maxhealthcare.in/blogs/know-about-moyamoya-disease
    The signs and symptoms of Moyamoya disease can vary among individuals and may evolve over time. These may include: […] Transient ischemic attacks (TIAs): Brief episodes of neurological dysfunction, often resembling mini-strokes, leading to temporary symptoms such as weakness, numbness, or paralysis in a limb, difficulty speaking, or vision changes. […] Ischemic strokes: Sudden onset of symptoms such as weakness, paralysis, or sensory disturbances on one side of the body, difficulty speaking, or loss of consciousness due to reduced blood flow to the brain. […] Seizures: Uncontrolled electrical disturbances in the brain that may cause seizures in some individuals with Moyamoya disease. […] Headaches: Frequently recurrent headaches might occur, ranging from mild to severe. […] Cognitive changes: Issues with memory, thinking, and other cognitive functions can develop, particularly in children with the condition.
  • #2 Moyamoya Disease Symptoms & Treatment | NGHS Neurosciences
    https://www.nghs.com/neurosciences/moyamoya
    Some common symptoms of Moyamoya disease include: […] Recurrent strokes […] Seizures […] Cognitive impairment and memory loss […] Headache […] Weakness or numbness in the arms or legs […] Vision problems, including double vision, loss of peripheral vision, or blindness in one eye.
  • #2 Moyamoya Disease: What It Is, Symptoms & Treatment
    https://my.clevelandclinic.org/health/diseases/17244-moyamoya-disease
    A lack of blood supply to your brain can lead to the symptoms of the disease. The first sign of moyamoya disease is often stroke or repeated transient ischemic attacks (TIAs). Healthcare providers may call these mini-strokes. Other moyamoya disease symptoms may include: […] Moyamoya disease treatment involves managing your symptoms with certain medications. Your healthcare provider may recommend or prescribe medications like: […] For most people with moyamoya disease, the condition gets worse over time. They may also experience stroke as blood vessels narrow more and more. Without treatment, moyamoya disease can lead to stroke with severe neurological deficits. But some people remain stable for years without symptoms. Once symptoms start to develop, your provider may suggest bypass surgery. […] With early diagnosis and prompt treatment, people with moyamoya disease can have a normal life expectancy. Without treatment, moyamoya disease can be fatal.
  • #2 Moyamoya disease, Moya Moya | Mayfield Brain & Spine
    https://mayfieldclinic.com/pe-moyamoya.htm
    The progression of Moyamoya follows a typical course and can be classified into stages based on angiography findings. Once the process of blockage (vascular occlusion) begins, it tends to continue. No known medication can reverse the blockage. Once a major stroke or bleeding has occurred, the patient may be left with permanent loss of function. It is important, therefore, to treat this condition promptly. […] Scientists have estimated that among untreated patients with moyamoya, 50 to 66 percent will experience progression of the disease and will suffer poor outcomes. By contrast, a large analysis of surgically treated pediatric patients found disease progression in an estimated 2.6 percent of patients. A more recent review of untreated patients who had no symptoms found an annual stroke rate of 3.2 percent and disease progression in 80 percent. […] If left untreated, the disease will invariably progress, producing clinical deterioration and potentially irreversible neurological deficits over time.
  • #2 Progression of disease in unilateral moyamoya syndrome – PubMed
    https://pubmed.ncbi.nlm.nih.gov/18275294/
    Progression of vasculopathy associated with moyamoya syndrome is extremely variable. […] Of 235 surgically treated patients with moyamoya syndrome, 33 (14%) presented with unilateral disease (4 adults and 29 children). […] During this period, 10 (30%) of 33 patients progressed to bilateral disease. […] Of patients with unilateral moyamoya syndrome, 30% will have progression of arteriopathy during long-term follow-up. […] Several factors, including contralateral abnormalities on initial imaging, congenital cardiac anomaly, previous cranial irradiation, Asian ancestry, and familial moyamoya syndrome, were associated with an increased risk of progression. […] Patients with known unilateral angiographic disease should undergo continued monitoring by using MR imaging and MR angiography at regular intervals.
  • #2 Chemical and perfusion markers as predictors of moyamoya disease progression and complication types | Scientific Reports
    https://www.nature.com/articles/s41598-023-47984-y
    The study found that patients with triglyceride levels of 200 or higher had a significantly higher likelihood of experiencing end-point events, which means any type of clinical stroke and decreased cerebrovascular reserve compared to those with triglyceride levels of less than 150 (HR: 2.292, CI 1.004-4.979, P=0.03). […] The study found that patients with triglyceride levels of 200 or higher had a significantly higher likelihood of experiencing hemorrhagic stroke compared to those with triglyceride levels of less than 150 (HR: 5.180, CI 1.355-19.801, P=0.02). Patients with severely decreased rCVR as assessed by Diamox SPECT demonstrated a significantly higher risk of experiencing ischemic stroke events compared to those with normal SPECT findings (hazard ratio [HR]: 5.939, 95% confidence interval [CI]: 1.616-21.829, P<0.01). [...] 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.
  • #2 Moyamoya disease – Diagnosis and treatment – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/diagnosis-treatment/drc-20355591
    Moyamoya disease is usually diagnosed by a doctor who specializes in conditions of the brain, called a neurologist. […] 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. […] 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. […] Without surgery, moyamoya disease can cause cognitive decline due to narrowing blood vessels.
  • #2 Moyamoya Disease Symptoms and Treatment | Froedtert & MCW
    https://www.froedtert.com/moyamoya
    Symptoms of moyamoya are similar to a stroke. Symptoms can include weakness or numbness in an arm or leg, difficulty speaking or paralysis affecting one side of the body. Other symptoms include seizures or changes in memory or recall. A severe headache can occur along with the other stroke symptoms that develop. […] Without surgery, individuals with moyamoya disease may experience progressive mental decline and multiple strokes as the arteries continue to narrow. Without treatment, moyamoya disease can be fatal as the result of intracerebral hemorrhage (bleeding within the brain).
  • #2 Moyamoya Disease – Child Neurology Foundation
    https://www.childneurologyfoundation.org/disorder/moyamoya-disease/
    Most strokes in children present with the SUDDEN ONSET of symptoms. These symptoms are neurological in nature. […] Common examples include: Weakness of one side of the body is called hemiparesis. Hemiparesis usually occurs in the face, arm, or leg. […] A child may have difficulty making or understanding speech. […] Vision loss occurs in one part of the visual world (e.g. they cannot see one half of the world regardless of which eye they use) or double vision. […] A child may experience poor walking or coordination. […] This includes a feeling of decreased sensation or tingling. […] Children with strokes due to moyamoya may have long-term neurological problems. These can include: Movement problems, Language disorders, Learning, attention, or behavior difficulties, Recurrent seizures (epilepsy). […] In some children, moyamoya disease can be mild. It can be stable over time. These children can be treated with medications to prevent stroke. […] In other children, moyamoya progresses more quickly. A higher risk of stroke may need to be treated with surgery.
  • #2 Moyamoya Disease Treatment
    https://www.rwjbh.org/treatment-care/neuroscience/neurosurgery/conditions-treated/moyamoya-disease/
    Moyamoya is a progressive disease affecting the blood vessels of the brain. It is characterized by a narrowed and/or closed carotid artery that delivers blood to the brain. Because it is blocked, smaller blood vessels (moyamoya vessels) attempt to compensate by opening up to supply blood to the brain. Because this is still insufficient, it leads to a serious reduction in oxygen delivery, and this deprivation causes the Moyamoya symptoms, such as stroke. […] If Moyamoya causes stroke, as it is likely to do because of the inadequate oxygenated blood supply to the brain, the patient may suffer from permanent side effects as a result. For example, many patients have facial paralysis, paralysis of the limbs, and loss of speech. Some temporary neurological function loss may also occur if the patient experiences a transient ischemic attack (also called TIA or a mini stroke), or a full-blown ischemic or hemorrhagic stroke, due to the blockage of oxygenated blood carried to the brain. It may also happen when the tiny blood vessels rupture and leak blood into the brain. […] Moyamoya disease symptoms include: Headaches, Seizures, Weakness of paralysis in the face, arm, or leg on one side of the body, Vision problems, Aphasia (difficulty speaking or understanding speech), Cognitive impairments, Learning disabilities.
  • #2 How Moyamoya disease affects children: a… | Riley Children’s Health
    https://www.rileychildrens.org/connections/how-moyamoya-disease-affects-children-a-comprehensive-guide
    Moyamoya disease symptoms are caused by reduced blood flow to the brain and weakened blood vessels. Common symptoms include: […] Alongside the physical symptoms, children with Moyamoya disease may experience cognitive challenges such as difficulties with memory, attention and learning. Emotional and psychological factors may include anxiety or depression. Socially, your child may experience feelings of isolation. […] “Moyamoya also greatly impacts children’s everyday routine,” Dr. Qaiser said. “Patients have to be mindful of contact sports, and they also need to make sure they stay well hydrated, given that dehydration can lead to strokes.” […] Ultimately, moyamoya is a complex condition significantly affecting children, and understanding its impacts is crucial to managing the disease.
  • #2 Moyamoya – Neurosurgeon
    https://www.vicneurosurgery.com.au/moyamoya/
    Much more common in adults, it is however also described in children. Haemorrhage is typically due to rupture of the fragile compensatory moyamoya vessels, though less commonly may be from an associated brain aneurysm. Most commonly bleeding is within the brain tissue itself (intracerebral haemorrhage, ICH), though maybe within the large fluid cavities of the brain known as ventricles (intraventricular haemorrhage, IVH), or within the smaller fluid spaces overlying the brain (subarachnoid haemorrhage, SAH). […] A common presenting symptom, this may be due to either a stroke, brain haemorrhage or chronic severe lack of blood supply to an area of the brain. […] Headache is a frequent presenting symptom. Typically, it is migraine like in quality and often resistant to standard medical therapy. Unfortunately, headaches may persist even after successful revascularisation surgery. It is thought to be due to dilation of the compensatory vessels causing stretch on the painful outer coverings of the brain, known as the dura, though this is not absolutely clear.
  • #2 Recovery and Long Term Implications of Moyamoya | Stanford Health Care
    https://stanfordhealthcare.org/medical-conditions/brain-and-nerves/moyamoya/faqs/recovery.html
    Will symptoms go away after surgery? It is not uncommon for patients to have persistent symptoms for several weeks after surgery. […] I have headaches. Will these go away? Headaches may or may not be related to moyamoya. Therefore, it’s difficult to say if they will be improved with surgery. They may temporarily be worsened after surgery. […] How long does it take for my strength and energy to come back after surgery? This is also variable from patient to patient. Most patients experience a gradual recovery over the course of a several weeks. It’s not unusual for patients to feel pretty good shortly after surgery, and then to over-exert themselves. This sometimes results in a feeling of low energy. Be patient with yourself and give your body adequate time to recover! […] Can moyamoya patients donate blood? It is recommended that moyamoya patients do not donate blood. Blood donation can deplete the overall blood volume and potentially increase the risk of TIA or stroke.
  • #2 Moyamoya Disease: Practice Essentials, Background, Etiology
    https://emedicine.medscape.com/article/1180952-overview
    Children and adults with moyamoya disease may have different clinical presentations. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children. […] 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).
  • #2 Moyamoya Disease – MD Searchlight
    https://mdsearchlight.com/health/moyamoya-disease/
    Seizures are common complications of either type of stroke. […] Migraine-type headaches are common in both kids and adults and probably occur due to stimulation of pain receptors in the outer coverings of the brain by dilated trans-dural collaterals. […] The future health outlook for patients with Moyamoya disease can differ greatly from person to person. Unfortunately, for two out of every three patients, the condition tends to worsen over five years, leading to poor outcomes. […] Certain factors may contribute to a poorer health outlook, including experiencing a hemorrhagic stroke at the start of the disease, being female, having a family history of the disease, and developing the disease at a young age.
  • #2 Moyamoya Disease: Symptoms, Stages, and Treatment
    https://www.healthline.com/health/moyamoya-disease
    Moyamoya disease is a progressive disease, meaning that it can gradually get worse without treatment. […] If treated, people with moyamoya disease can have an average life expectancy. […] Although moyamoya can be fatal, surgery and medication can prevent moyamoya disease from getting worse. […] When moyamoya disease is left untreated, it can be fatal. […] Without treatment, the brain’s blood supply may decrease over time. As a result, people with moyamoya disease may experience multiple strokes and mental decline. […] Certain surgeries, called revascularizations, can open narrowed blood vessels or bypass blocked vessels and improve blood flow to the brain. […] Certain medications can also help people with moyamoya disease.
  • #2 Orphanet: Moyamoya disease
    https://www.orpha.net/en/disease/detail/2573
    Moyamoya disease (MMD) is a rare intracranial arteriopathy involving progressive stenosis of the cerebral vasculature located at the base of the brain causing transient ischemic attacks or strokes. […] Disease manifestations are highly variable. Some affected individuals remain asymptomatic, some develop transient attacks, and others severe neurologic deficits as a result of infarcts or hemorrhage. Hemorrhage occurs less often in children. Other manifestations include headache, dizziness, seizures, and chorea. Some patients present intellectual disability. The course is also highly variable but generally progressive. […] No specific treatment to stop progression or reverse the intracranial arteriopathy is available for MMD. Drug therapy is mainly used to counter disease complications such as stroke and transient ischemic attacks, to alleviate symptoms, and to prevent cognitive deterioration. […] Early detection and appropriate treatment improve the long-term outcome. The prognosis depends on disease severity. Mortality rates are estimated at about 5% for adults and 2% for children. The main cause of death is hemorrhage.
  • #2 Moyamoya disease // Middlesex Health
    https://middlesexhealth.org/learning-center/diseases-and-conditions/moyamoya-disease
    Moyamoya disease may happen at any age. But symptoms are most common in children between ages 5 and 10 and in adults between ages 30 and 50. Spotting symptoms early is very important to prevent complications such as a stroke. […] Moyamoya disease causes different symptoms in adults and children. In children, the first symptom is usually a stroke or recurrent transient ischemic attack, also called TIA. Adults may experience these symptoms, as well. But adults also may experience bleeding in the brain, known as a hemorrhagic stroke. The bleeding happens because of the way blood vessels in the brain formed. […] Symptoms of moyamoya disease related to reduced blood flow to the brain include: Headache. Seizures. Weakness, numbness or paralysis in the face, arm or leg. This is typically on one side of the body. Vision problems. Trouble speaking or understanding others, known as aphasia. Cognitive or developmental delays. Involuntary movements. […] These symptoms can be triggered by exercise, crying, coughing, straining or a fever. […] Early detection and treatment can help prevent a stroke and serious complications.
  • #2 Moyamoya Disease – StatPearls – NCBI Bookshelf
    https://www.ncbi.nlm.nih.gov/books/NBK535455/
    In addition to TIA or ischemic strokes, adults also frequently present with hemorrhagic stroke. Hemorrhage mainly results from a rupture of fragile moyamoya collaterals and seen in deep areas of the brain, such as basal ganglia, periventricular deep white matter. Intraventricular hemorrhage is common due to the close proximity of the primary site of intracerebral hemorrhage. […] Seizures are common complications of either ischemic or hemorrhagic events. […] Migraine-like headaches are common in both, children and/or adults and occur probably from the stimulation of dural nociceptor by dilated trans-dural collaterals. […] The overall prognosis is variable. Two-thirds of patients with Moyamoya disease have a symptomatic progression over five years with poor outcomes. Progression of the occlusive process continues regardless of symptom severity, ongoing treatment, age, sex, type and location of the disease. However, data from the North American series shows 13.3% and 1.7% of ischemic and hemorrhagic strokes, respectively. Factors that may show a poor prognosis could be but not limited to: hemorrhagic strokes at presentation, female gender, familial form of onset and pediatric age of onset. Concomitant thyroid disorder and smoking impacts negatively on overall prognosis in MMD. Early surgical revascularization has a preferable prognosis.
  • #2 Moyamoya Disease | Children’s Hospital of Philadelphia
    https://www.chop.edu/conditions-diseases/moyamoya-disease
    The most common symptom that brings children to the doctor is a sudden loss of function that resembles a stroke, such as difficulty speaking or weakness of one side of the body. […] Sometimes these symptoms are short-lived, resolving over a matter of hours, and no tissue damage is evident on an MRI or CT scan. Doctors call this a transient ischemic attack (TIA). Sometimes the symptoms are more long-lasting or more severe. In these cases, there may be actual tissue damage seen on a brain MRI or CT scan, which would indicate there has been a stroke and not just a TIA. When there has been a stroke, the effects are more long-lasting (days to weeks) or may even be permanent. […] The majority of children receiving the combination of medical and surgical therapy for moyamoya disease have a good outcome, with a low risk of recurrent stroke. Restoring blood flow to the brain is an important step in maximizing their recovery of function. Surgery can be very effective in delivering blood to the brain and improving function.
  • #3 Moyamoya disease – Symptoms and causes – Mayo Clinic
    https://www.mayoclinic.org/diseases-conditions/moyamoya-disease/symptoms-causes/syc-20355586
    Symptoms of moyamoya disease related to reduced blood flow to the brain include: Headache. Seizures. Weakness, numbness or paralysis in the face, arm or leg. This is typically on one side of the body. Vision problems. Trouble speaking or understanding others, known as aphasia. Cognitive or developmental delays. Involuntary movements. […] These symptoms can be triggered by exercise, crying, coughing, straining or a fever. […] Early detection and treatment can help prevent a stroke and serious complications. […] Most complications from moyamoya disease are linked to the effects of strokes. They include seizures, paralysis and vision problems. Other complications include speech problems, movement disorders and developmental delays. Moyamoya disease can cause serious and permanent damage to the brain.
  • #4 Moyamoya Disease
    https://practicalneurology.com/diseases-diagnoses/stroke/moyamoya-disease/31617/
    Moyamoya disease is a cerebrovascular condition characterized by idiopathic chronic progressive steno-occlusive changes of the terminal portions and proximal branches of the internal carotid arteries (ICAs). These changes reduce blood flow through the anterior circulation of the brain causing progressive cerebral ischemia. […] The clinical presentations of moyamoya are attributed to the changes in intracranial blood flow dynamics and cerebral perfusion. Symptoms can be classified as arising from brain ischemia (eg, strokes, transient ischemic attacks [TIA], and seizures) or as sequelae of the compensatory mechanisms in response to ischemia (eg, hemorrhages from rupture of fragile collateral vessels and headaches from dilated collaterals). […] Symptoms are typically dependent on which brain region is ischemic (eg, frontal, parietal, temporal lobes). Common symptoms include hemiparesis, aphasia, cognitive impairment, seizures, syncope, and visual deficits. Ischemic symptoms may be transient (TIA) or permanent (stroke), and are commonly precipitated in children by hyperventilation (eg, crying, exercise), dehydration, or exertion.