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Controversies in Neurology
January 2001

Surgery for Moyamoya Syndrome?Yes

Author Affiliations

From the Department of Pediatric Neurosurgery, The Children's Hospital, and Harvard Medical School, Boston, Mass.



Arch Neurol. 2001;58(1):128-130. doi:10.1001/archneur.58.1.128

THE CLINICAL presentation of most patients with moyamoya syndrome is cerebral ischemia. In my own series of 100 patients younger than 21 years [unpublished data, 1985-1999], 66 presented with stroke and 18 with transient ischemic attacks (TIAs). Cerebral hypoperfusion in these patients can be documented by a variety of imaging studies including diffusion-weighted magnetic resonance imaging (MRI), xenon flow studies using computed tomography (CT) or positron emission tomography (PET), and single photon emission computed tomography (SPECT) studies with acetazolamide challenge. One of the most striking observations in these patients is that TIAs can be precipitated by hyperventilation, an indication of how marginal the cerebral blood flow can become owing to the basal arterial stenoses seen in this syndrome. As the moyamoya inevitably progresses to complete occlusion of the internal carotid artery,1 the clinical syndrome also progresses unless the spontaneous leptomeningeal or transdural collateral blood supply is sufficient to supply the cerebrum distal to the occlusive process. If the development of the collateral blood supply is insufficient or too slow, the patient becomes symptomatic. While observing pediatric patients with the syndrome for more than 2 decades, I have never seen a patient in whom the arteriographic process did not progress and in whom the clinical syndrome did not similarly evolve while the patient was untreated, although the rate of progression of the clinical syndrome and arteriographic process is unpredictable.2 Even though there are some reports in the literature from Japan indicating that ischemic symptoms tend to decline or resolve over time,3 in many of these series the follow-up was limited and the authors noted significant concomitant deterioration in cognitive function or activities of daily living. Other Japanese reports indicate that when untreated, moyamoya syndrome has a poor natural history.4

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