Case 6
History:
25 year old male with right arm weakness
Findings:
CT Angiogram of the head and neck shows absence of the bilateral MCA with promience of the small perforrator vessels. The sagittal views of the neck show diffuse, smooth narrowing of the bilateral ICA indicative of chronic/severe stenosis of the intra cranial compartment.
Diagnosis:
Moyamoya
Discussion:
Moyamoya disease is a rare, progressive cerebrovascular disorder secondary to occlusion or stenosis of distal/supraclinoid portion of the internal carotid arteries, followed by development of collateral network. First described in Japan in the 1960’s. The exact cause is unknown. The disease primarily affects children, but it can also occur in adults. In children, the first symptom is often stroke, or recurrent TIAs, headaches or seizures. Adults most often experience a hemorrhagic stroke, subarachnoid or intraventricular.
References:
- Mark S. Greenberg. Handbook of neurosurgery, 5nd edition. Thieme. 2001
