CT/MRI-Cranial Nerves and Vessels: Moyamoya disease

What is it?

Moyamoya disease is a cerebrovascular disorder caused by blocked arteries near the basal ganglia.  It affects the Circle of Willis and surrounding arteries.    Moyamoya disease is very rare and progressive.  The disease was first discovered in the 1960's in Japan and primarily affects children, but can occur in adults.  Moyamoya means "puff of smoke" in Japanese. Moyamoya disease has a strong family history and  an inherited genetic abnormality. 



Symptoms

medscape.org

Symptoms vary with children and adults.  The first symptoms of children are often times stroke or TIAs.  Children have muscle weakness, paralysis of one size, or sometimes seizures.  Most adults experience a hemorrhagic stroke due to constant blood clots in the affected brain vessels. 

Individuals may experience:

• speech deficits
• sensory and cognitive impairments
• disturbed consciousness
• involuntary movements
• vision problems

(http://www.ninds.nih.gov/disorders/moyamoya/moyamoya.htm)


Diagnosis & Treatments

Magnetic resonance imaging (MRI) or cat scan (CT) can be used to identify Moyamoya disease.  MRI exams are the gold standard because of the detail.  "There are several types of revascularization surgery that can restore blood flow to the brain by opening narrowed blood vessels or by bypassing blocked arteries" (http://www.ninds.nih.gov/disorders/moyamoya/moyamoya.htm). Children normally respond better to surgery than adults.  After surgery most patients have no more strokes or other problems related to the diease.  

medscape.org

More information:
http://www.ninds.nih.gov/disorders/moyamoya/moyamoya.htm
or
http://emedicine.medscape.com/article/1180952-overview

Ashli