Introduction: Moyamoya disease is a rare, progressive cerebrovascular disorder characterized by the narrowing or blockage of the internal carotid arteries at the base of the brain. As these arteries become constricted, small, fragile blood vessels develop to compensate for the reduced blood flow. These tiny vessels appear like a “puff of smoke” on imaging scans.“Moyamoya” is a Japanese term that means “hazy”.
This disease reduces blood flow to the brain, causing strokes, transient ischemic attacks, headaches,and seizures. Moyamoya can affect both children and adults.
Causes of Moyamoya disease:
The exact cause of Moyamoya disease is still unknown, but several factors contribute to its development. These include:
- Genetic Factors:
Moyamoya disease often runs in families, suggesting a hereditary component.
Mutations in the RNF213 gene have been related to the disease.
- Idiopathic (Primary Moyamoya Disease):
In many cases, there is no identifiable underlying cause.
- Associated Conditions (Secondary Moyamoya Syndrome):
Moyamoya-like changes can occur with other medical conditions, referred to as Moyamoya syndrome.
These conditions include:
-Neurofibromatosis type 1 (NF1)
- Sickle cell disease
- Down syndrome
- Autoimmune diseases (like systemic lupus erythematosus)
- Thyroid disorders (especially Graves’ disease)
- Cranial radiation therapy (especially in childhood)
- Inflammation or Infections:
In rare cases, Chronic inflammation with infections affects blood vessels.
Moyamoya disease results from a combination of genetic susceptibility and environmental or physiological triggers.
Types of Moyamoya Disease:
Moyamoya disease can be classified into two main types based on different causes:
- Primary Moyamoya Disease called Idiopathic Moyamoya: This is the classic form of the disease with no underlying condition. It occurs on both sides of the brain.
- Secondary Moyamoya Syndrome called Moyamoya syndrome: This form affects only one side of the brain.
Associated with Neurofibromatosis type 1 (NF1)
- Sickle cell disease
- Down syndrome
- Thyroid disorders (e.g., Graves’ disease)
- Systemic lupus erythematosus (SLE)
Symptoms of Moyamoya Disease:
Moyamoya disease symptoms result from reduced blood flow to the brain, which varies in children and adults. Common symptoms include:
In Children:
- Transient Ischemic Attacks (TIAs)
- Seizures
- Headaches
- Developmental delays or difficulty in learning
- Weakness or numbness on one side of the body
- Involuntary movements – Especially in the limbs.
In Adults:
- Strokes or TIAs
- Memory and concentration problems
- Severe headaches
- Speech difficulties
- Visual disturbances
- Seizures
- Hemorrhagic stroke
- Mood changes or behavioral disturbances.
Symptoms may be triggered or worsened by activities that lower blood pressure, such as crying, hyperventilation, or physical exertion.
General Approach to Moyamoya Disease & Its Side Effects
General Approach (Management & Treatment):
The main goal in managing Moyamoya disease is to improve blood flow to the brain and prevent strokes or neurological deterioration. The approach includes:
- Medical Management:
Antiplatelet therapy (e.g., aspirin): To reduce the risk of stroke.Managing blood pressure, cholesterol and avoiding dehydration or overexertion.
- Surgical Treatment:
Surgery aims to restore blood flow to the brain by creating new pathways:
- Direct revascularization: Connecting a scalp artery (usually the superficial temporal artery) directly to a brain artery (middle cerebral artery)
- Indirect revascularization: Placing tissue near the brain surface to encourage the growth of new vessels over time (e.g., EDAS – encephaloduroarteriosynangiosis).
- Supportive Therapy:
- Physical and occupational therapy for patients recovering from strokes or dealing with weakness.
- Speech therapy If language or swallowing is affected.
- Psychological support: For cognitive or emotional issues.
Side Effects/Complications of Moyamoya Disease:
In severe cases, Moyamoya disease can lead to Neurological Complications:
- Stroke or recurrent TIAs
- Seizures
- Memory loss, poor concentration, and learning difficulties
- Paralysis or weakness
- Speech or language problems
- Surgical Complications:
Infection and Bleeding occur in post-operative stroke
Hyperperfusion syndrome – Sudden increase in blood flow post-surgery that can cause brain swelling or seizures
- Psychosocial Effects:
- Anxiety or depression
- Academic or work challenges
- Dependency in daily activities
Homeopathic Approach to Moyamoya Disease
Homeopathy focuses on stimulating the body’s self-healing ability using individualized remedies based on a person’s physical, emotional, and mental state. While homeopathy cannot reverse Moyamoya disease or replace medical/surgical treatment, it may be used as supportive therapy for symptom management and well-being.
Homeopathic Medicines for Supportive Care in Moyamoya Disease:
- Calcarea Phosphorica: This medicine is well indicated for mental dullness, developmental delays, headaches and weakness of limbs in children due to reduced brain oxygenation.
- Baryta Carb: This medicine is suitable for mental retardation, memory issues, difficulty in speech, shyness or delayed development.
- Belladonna: This remedy is used for sudden heat in the head with headaches and high blood pressure.
- Cicuta Virosa: This remedy is used for convulsions, epilepsy, violent spasms, and post-seizure exhaustion.
- Natrum Muriaticum: This medicine works well for headaches, emotional stress, weakness and forgetfulness
Dietary Guidelines:
A diet chart for Moyamoya disease focuses on promoting brain health, supporting blood circulation, and preventing risk factors like high blood pressure, high cholesterol, or stroke.
Small and frequent meals can be preferred. Staying well-hydrated helps.
Avoid excessive salt, sugar, and processed foods.
Daily Diet Chart:
- Morning (7:00–8:00 AM)
-Warm water with lemon or soaked almonds (4–5)
-Herbal tea or green tea.
- Breakfast (8:30–9:30 AM)
-Oats porridge with fruits and flaxseeds
-Multigrain toast with peanut butter and a boiled egg.
-Vegetable poha or upma with seeds (chia, flax)
- Mid-morning snack (11:00 AM)
-Fresh fruit (banana, apple, or orange).
-Handful of walnuts or pumpkin seeds
- Lunch (1:00–2:00 PM)
-Brown rice or whole wheat roti
-Grilled or steamed vegetables (broccoli, spinach, carrots, etc.)
-Lentils/dal or lean protein (chicken/fish or tofu)
and Fresh salad with olive oil
-Buttermilk or curd (low-fat)
- Afternoon Snack (4:00–5:00 PM)
-Coconut water or fresh juice (no added sugar)
-Roasted chickpeas or makhana (fox nut)
- Evening (6:00–6:30 PM)
-Green tea with 2–3 digestive biscuits
-Light soup (vegetable or bone broth)
- Dinner (7:30–8:30 PM)
-Quinoa, millet or soft chapati with Light with vegetable curry or steamed fish.
– Small bowl of lentils or soup and Light salad.
- Before Bed (9:30–10:00 PM)
-Warm milk with a pinch of turmeric
or Herbal tea (like chamomile or ashwagandha-based)
- Foods to Include:
-Omega-3 rich foods: Fatty fish (like salmon), walnuts, flaxseeds
-Iron & B-vitamins: Leafy greens, eggs, whole grains
-Antioxidants: Berries, turmeric, green tea
-Magnesium & potassium: Bananas, avocados, legumes
- Foods to Avoid:
-High-sodium foods (chips, pickles, processed meats)
-Sugary drinks and junk food
-Excess caffeine
-Saturated fats and trans fats (deep-fried foods, fast food)
Frequently Asked Questions (FAQs) about Moyamoya Disease:
- Who is at risk of developing Moyamoya disease?
Children aged 5–10 years and adults aged 30–50 are most commonly affected. People with a family history of Moyamoya or related conditions are at higher risk.
- How is Moyamoya disease diagnosed?
It is diagnosed through imaging tests, such as:
MRI and MRA, CT angiography, Cerebral angiography
- Is there a cure for Moyamoya disease?
There is no permanent cure, but surgery can significantly improve blood flow and reduce the risk of strokes. Long-term management helps patients live normally.
- What treatments are available?
-Surgical revascularization (direct or indirect)
-Medications: Aspirin or other antiplatelets
-Lifestyle changes and rehabilitation
-Supportive therapies (e.g., physiotherapy, speech therapy)
- Can Moyamoya disease be fatal?
If left untreated, recurrent strokes or brain hemorrhages can be life-threatening. Early diagnosis and proper treatment improve the condition of the patient.
Conclusion
It is a challenging syndrome, but by intervention in the early stages, good management, and combined treatments such as homeopathy, patients can improve their quality of life. Dr. Vaseem Choudhary and his experienced team of professionals at Homeo Care Clinic are homeopathic treatment specialists who treat children afflicted with neurological disorders by providing them with natural but effective individualized medicines.
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