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Previous Volume 360:1226-1237 March 19, 2009 Number 12 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

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The moyamoya syndrome is a cerebrovascular condition that predisposes affected patients to stroke in association with progressive stenosis of the intracranial internal carotid arteries and their proximal branches. Reduced blood flow in the major vessels of the anterior circulation of the brain leads to compensatory development of collateral vasculature by small vessels near the apex of the carotid, on the cortical surface, leptomeninges, and branches of the external carotid artery supplying the dura and the base of the skull. In rare cases, this process also involves the posterior circulation, including the basilar and posterior cerebral arteries.

First described in 1957 . . . [Full Text of this Article]

Epidemiologic Features

Presentation

Age-Related and Geographic Differences in Presentation

Ischemic Symptoms

Hemorrhage

Headache and Other Symptoms

Associated Conditions

Pathophysiological Features

Analysis of Pathological Findings

Genetic Studies

Angiogenesis and Extracellular-Matrix–Related Peptides

Natural History and Prognosis

Diagnosis

Computed Tomography

Magnetic Resonance Imaging

Angiography

Other Diagnostic Techniques

Screening

Treatment

Medical Therapy

Surgery

Treatment of Acute Symptoms

Conclusions

Source Information

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

Address reprint requests to Dr. Smith at the Department of Neurosurgery, Children's Hospital Boston, 300 Longwood Ave., Boston, MA 02115, or at edward.smith@childrens.harvard.edu.

Related Letters:

Moyamoya Disease and Moyamoya Syndrome
Derdeyn C. P., Pollak L., Scott R. M., Smith E. R.
Extract | Full Text | PDF  
N Engl J Med 2009; 361:97-98, Jul 2, 2009. Correspondence

This article has been cited by other articles:

• Sandhu, G., Ranade, A., Narayanswami, G. (2009). Fatal massive cerebral infarctions in a young patient with diabetic ketoacidosis--a rare case of atherosclerotic moyamoya disease. QJM 0: hcp161v1-hcp161 [Full Text]  
• Derdeyn, C. P., Pollak, L., Scott, R. M., Smith, E. R. (2009). Moyamoya Disease and Moyamoya Syndrome. NEJM 361: 97-98 [Full Text]