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Moyamoya disease

Moyamoya disease is an extremely rare disorder in most parts of the world except in Japan. The pathogenesis of Moyamoya disease is unknown. Moyamoya disease is characterized by progressive intracranial vascular stenoses of the circle of Willis, resulting in successive ischemic events. Hemmorhagic events can also occur. The condition leads to irreversible blockage of the main blood vessels-the carotid arteries- to the brain as they enter into the skull. It is a disease that tends to affect children and adults in the third to fourth decades of life. In children it tends to cause strokes or seizures. In adults it tends to cause bleeding or strokes. more...

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The clinical features are cerebral ischaemia (strokes), recurrent transient ischaemic attacks (TIA's or "mini strokes"), sensorimotor paralysis (numbness in the extremeties), convulsions and/or migraine-like headaches.

The process of blockage (vascular occlusion) once it begins tends to continue despite any known medical management unless treated with surgery. The repeated strokes can lead to severe functional impairment or even death so it is important to recognize these lesions and treat them early on. Without treatment, there is progressive deterioration of neurologic function and re-hemorrhage.

The diagnosis is initially suggested by CT, MRI, or angiogram. Contrast-enhanced T1-weighted images are better than FLAIR images for depicting the leptomeningeal ivy sign in moyamoya disease. MRI and MRA should be performed for the diagnosis and follow-up of moyamoya disease. Diffusion-weighted imaging can also be used for following the clinical course of children with moyamoya disease, in whom new focal deficits are highly suspicious of new infarcts.

Often nuclear medicine studies such as SPECT (single photon emission computerized tomography) are used to demonstrate the decreased blood and oxygen supply to areas of the brain involved with the Moyamoya disease. Conventional angiography provided the conclusive diagnosis of moyamoya disease in most cases and should be performed before any surgical considerations.

There are many operations that have been developed for the condition, but currently the most favored are: EDAS, EMS, STA-MCA, EC-IC bypass graft, and multiple burr holes. Direct superficial temporal artery to middle cerebral artery bypass is considered the treatment of choice, although it's efficacy, particularly for hemorrhagic disease, remains uncertain. Multiple burr holes have been used in frontal and parietal bones with good neovascularisation achieved.

The EDAS (encephaloduroarteriosynangiosis) procedure requires dissection of a scalp artery over a course of several inches and then making a small temporary opening in the skull directly beneath the artery. The artery is then sutured to the surface of the brain and the bone replaced.

In the EMS (encephalomyosynangiosis) operation, the temporalis muscle, which is in the temple region of the forehead, is dissected and through an opening in the skull placed onto the surface of the brain.

All of these operations have in common the concept of a blood and oxygen "starved" brain reaching out to grasp and develop new and more efficient means of bringing blood to the brain and bypassing the areas of blockage. The modified direct anastomosis and encephalo-myo-arterio-synagiosis play a role in this improvement by increasing CBF after the operation. A significant correlation is found between the postoperative effect and the stages of preoperative angiograms. It is crucial for surgery that the anesthesiologist have experience in managing these children as the type of anesthesia they require is very different from the standard anesthetic children get for almost any other type of neurosurgical procedure.

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Quantitative evaluation of cerebral metabolites and cerebral blood flow in patients with carotid stenosis
From Neurological Research, 9/1/01 by Uno, Masaaki

We examined the metabolic and hemodynamic status of patients with severe carotid stenosis and evaluated the effectiveness of carotid endarterectomy (CEA) by comparing pre- and post-operative results of quantitative proton magnetic resonance spectroscopy (^sup 1^H-MRS) and single-photon emission computed tomography (SPECT). Quantitative ^sup 1^H-MRS and SPECT were performed in 17 patients with severe carotid stenosis before CEA; in 10 patients the examinations were repeated after CEA. There was a significant correlation between the degree of internal carotid artery (ICA) stenosis and the N-acetyl-aspartate (NAA) concentration, and between CBF and NAA in the basal ganglia. In 10 of the 17 patients (58.8%) we noted a decrease of NAA on ^sup 1^H-MRS. After CEA in these patients, NAA was significantly increased, and in 7 of 8 patients whose pre-operative SPECT had indicated impairment of cerebral vasoreactivity, it was improved. Quantitative ^sup 1^H-MRS and CBF measurements can indicate the severity of metabolic and hemodynamic impairment in patients with severe carotid stenosis, and can help to assess the effectiveness of CEA. [Neurol Res 2001; 23: 573-580]

Keywords: Carotid endarterectomy; carotid stenosis; proton magnetic resonance spectroscopy; single photon emission computed tomography

INTRODUCTION

CONCLUSION

Measurement of absolute concentration of NAA and the CBF using Patlak plot method can indicate the neuronal and hemodynamic status in patients with severe carotid stenosis which could not be demonstrated by the MRI and clinical symptoms. The NAA concentration, determined by quantitative measurement, may be an indicator for evaluating the effectiveness of CEA.

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6 Harada M, Miyoshi K, Uno M, Okada T, Hisaoka S, Hori A, Nishitani H. Neuronal impairment of adult moyamoya disease detected by quantified proton MRS and comparison with cerebral perfusion by SPECT with Tc-99m HM-PAO: A trial of clinical quantification of metabolites. J Magn Reson Imaging 1999; 10: 124-179

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Masaaki Uno, Masafumi Harada* and Shinji Nagahiro

Department of Neurological Surgery, *Department of Radiology, School of Medicine, The University of Tokushima, Tokushima, Japan

Correspondence and reprint requests to: Masaaki Uno, MD, Department of Neurological Surgery, School of Medicine, The University of Tokushima, Tokushima, Japan. [neuros@clin.med.tokushima-u.ac.jp]

Accepted for publication December 2000.

Copyright Forefront Publishing Group Sep 2001
Provided by ProQuest Information and Learning Company. All rights Reserved

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