MOYAMOYA DISEASE

MOYAMOYA DISEASE:  A disease that progressively blocks the cerebral vasculature flow in the brain, in particular the Circle of Willis and the feeding vessels.
SYMPTOMS: 
Children:  Hemiparesis, monoparesis, sensory impairment, involuntary movements, headaches, dizziness, or seizures, menatal retardation and neurological deficits may be present. Tend to suffer cerebral ischemic events than adults.
Adults:  Symptoms similar to children with possible intraventricular, subarachnoid, or intracerebral hemorrhage. Tend to suffer hemorrhage more than children.
DIFFERENTIAL DIAGNOSIS:
Stroke
Clots
Cerebral aneurysms
Blood discrasias
Cavernous sinus syndromes
Intracranial hemorrhage
DIAGNOSIS
Labs:  protein s, protein c, antithrombin III, homocysteine, factor V Leiden, ESR, TFTs, thyroid autoantibodies
Imaging studies:  MRA, cerebral angiography
TREATMENT:  No definite treatment as for medication is available. Treatment and therapy are directed towards the complications of the disease.
Conservative Approach:
Intracerebral hemorrhage:  manage HTN
Severe stroke:  ICU monitoring until patient stable
Ischmeic stroke:  anticoagulation (coumadin or heparin) therapy to prevent further strokes
Consult with experienced neurologist for guidance
Angiogram to determine stenosis or occlusion
Surgical Approach:
STA-MCA--superficial temporal artery-middle cerebral artery anastomosis
EDAS--encephaloaduroarteriosynangiosis
EDAMS--encephaloduroarteriomyosynangiosis, pial synangiosis, and omental transplantation
CONSULTATIONS:
Neurological consult absolute must
Neuroradiology consult to determine what testing is needed
PROGNOSIS:
Outcome--dependent upon the extent and nature of hemorrhage
Prognosis--dependent upon the recurrence of the attacks
REFERENCES:
Hoch, D. U. S. National Library of Medicine, (2010). Circle of willis Bethesda, MD: Retrieved from http://www.nlm.nih.gov/medlineplus/ency/imagepages/18009.htm

Junath, M. (2009, December 19). Moyamoya disease [Web log message]. Retrieved from http://manju-imagingxpert.blogspot.com/2009/12/moyamoya-disease.html
Sekhar, L., & Kim, L. (2011). Neurological surgery clinical services moyamoya syndrome. Manuscript submitted for publication, Department of Neurological Surgery, University of Washington, Washington. Retrieved from http://neurosurgery.washington.edu/patientcare/clinicalservices/moyamoya.asp.
Suchoeiki, R., Kao, A. , & , Initials. (2011, Marc 23). Moyamoya disease. Retrieved from http://emedicine.medscape.com/article/1180952-overview.



Angiogram





MRI T1 weighted