Moyamoya Disease Clinical Presentation

Updated: Nov 09, 2018
  • Author: Roy Sucholeiki, MD; Chief Editor: Amy Kao, MD  more...
  • Print

History and Physical Examination


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. Adults experience hemorrhage more commonly; cerebral ischemic events are more common in children.

Children may have hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures. Mental retardation 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.

Physical examination

Physical examination findings depend on the location and severity of the hemorrhage or ischemic insult. There may be findings that indicate a condition associated with moyamoya syndrome such as skin abnormalities or dysmorphisms.



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. [10]