🧠 Moyamoya Disease

Moyamoya disease is a rare, progressive narrowing of the internal carotid arteries and their main branches at the base of the brain. To compensate for poor blood flow, the brain forms a network of small collateral vessels that look like “puffs of smoke” (moyamoya in Japanese) on angiography.

Who Gets It

• Children (5–10 years) and adults (30–50 years) most commonly affected

• More common in females and people of Asian descent

• May occur with other disorders (Moyamoya syndrome):

  • Sickle cell disease

  • Neurofibromatosis type 1

  • Down syndrome

  • Marfan syndrome
    
    

Causes (Possible)

• Genetic mutations (familial cases reported)

• Chronic inflammation or infection of cerebral vessels

• Trauma (rarely)
  
  

Symptoms

Due to decreased oxygen delivery to brain tissue:

• Stroke or TIA-like episodes (transient weakness or numbness)

• Speech disturbance

• Headache

• Involuntary movements (chorea)

• Seizures

• Vision or cognitive problems
  
  

Diagnosis

• MRI/MRA or CT Angiogram: shows vessel narrowing and collateral network

• Digital Subtraction Angiography (DSA): gold standard for diagnosis

• Genetic testing: may be considered in familial cases
  
  

Treatment

• Medical therapy:

  • Aspirin to reduce stroke risk

  • Antiepileptic drugs for seizure control

  • Analgesics for headaches

• Surgical treatment (Definitive):

  • Revascularization procedures to restore cerebral blood flow

    • Direct bypass: STA–MCA anastomosis

    • Indirect bypass: EDAS, EMS, or multiple burr hole surgeries
      
      

Post-Surgery and Lifestyle Care

• Take medications as prescribed

• Avoid dehydration and smoking

• Avoid activities that cause sudden pressure changes (e.g., scuba diving) or head trauma

• Maintain good hydration and follow-up imaging
  
  

Emergency Signs — Act FAST

If symptoms of stroke appear:

• Balance loss

• Eye/vision changes

• Face drooping

• Arm weakness

• Speech difficulty

• Time to call emergency services immediately