Diagnosis of Moyamoya disease requires bilateral symmetrical stenosis or occlusion of the terminal portion of the internal carotid arterys (ICA)s as well as the presence of dilated collateral vessels at the base of the brain 1). (If unilateral, the diagnosis is considered questionable, 2) and these cases may progress to bilateral involvement).
Other characteristic findings include:
1. stenosis/occlusion starting at the termination of ICA and at origins of ACA and MCA
2. abnormal vascular network in the region of BG (intraparenchymal anastomosis).
3. transdural anastomosis(rete mirabile), AKA “vault moyamoya.”Contributing arteries: anterior falcial, middle meningeal, ethmoidal, occipital, tentorial, STA
4. moyamoya collaterals may also form from the internal maxillary artery via ethmoid sinus to the forebrain in the frontobasal region.
Work-up in suspected cases typically begins with a non-enhanced head CT. Up to 40% of ischemic cases have normal CT. Low-density areas (LDAs) may be seen, usually confined to cortical and subcortical areas (unlike atherosclerotic disease or acute infantile hemiplegia which tend to have LDAs in basal ganglia as well). LDAs tend to be multiple and bilateral, especially in the PCA distribution (poor collaterals), and are more common in children.
MRA usually discloses the stenosis or occlusion of the ICA. Moyamoya vessels appear as flow voids on MRI (especially in basal ganglia) and a fine network of vessels on MRA and are demonstrated better in children than adults. Parenchymal ischemic changes are commonly shown, usually in watershed areas.
MRI images show:
1) diminished blood flow in the internal carotid artery (ICA) and the middle cerebral artery (MCA) and anterior cerebral artery (ACA) and 2) prominent collateral blood flow at the base of the brain. To confirm the diagnosis of moyamoya disease, an angiogram is typically required.
Diagnostic criteria of definitive moyamoya disease include all of the following items based on the conventional angiographic findings.
(1) Stenosis or occlusion of the terminal portion of the intracranial ICA or proximal portions of the anterior cerebral artery (ACA) and/or the middle cerebral artery (MCA).
(2) Development of abnormal vascular networks near the occlusive or stenotic lesions in the arterial phase.
(3) Bilateral lesion 3).
Results demonstrate distinct alterations in the temporal correlations of low-frequency BOLD signals, predominantly in resting-state networks in moyamoya disease. Additionally, Resting state functional magnetic resonance imaging (rs-fMRI) measures were associated with ischemic motor-related symptoms and cognitive performance in the patients. Thus, rs-fMRI may offer a useful non-invasive method of acquiring additional information beyond cerebral perfusion as part of clinical investigations in patients with moyamoya disease 4).
Territorial arterial spin labeling (t-ASL) could reveal comprehensive Moyamoya disease (MMD) cerebral blood perfusion and the vivid perfusion territory shifts fed by the unilateral ICA and ECA and bilateral vertebral arterys (VAs) in a noninvasive, straightforward, nonradioactive, and nonenhanced manner. 3D Time of flight magnetic resonance angiography (3D-TOF-MRA) could subdivide t-ASL perfusion territory shifts according to their shunt arteries. A perfusion territory shift attributable to the secondary collaterals is a potential independent risk factor for preoperative hemorrhage in MMD patients. A perfusion territory shift fed by the primary collaterals may not have a strong effect on preoperative hemorrhage in MMD patients. These findings make the combined modalities of t-ASL and 3D-TOF-MRA a feasible tool for MMD disease assessment, management, and surgical strategy planning 5).
In addition to helping to establish the diagnosis, angiography also identifies suitable vessels for revascularization procedures and unearths associated aneurysms. The angiography-related complication rate is higher than with atherosclerotic occlusive disease. Avoid dehydration prior to and hypotension during the procedure. Six angiographic stages of MMD are described by Suzuki and Takaku 6) that tend to progress up until adolescence and stabilize by age 20.
1 stenosis of suprasellar ICA, usually bilateral
2 development of moyamoya vessels at the base of the brain; ACA MCA & PCA dilated
3 increasing ICA stenosis & prominence of moya-moya vessels (most cases diagnosed at this stage); maximal basal moyamoya
4 entire circle of Willis and PCAs occluded, extracranial collaterals start to appear, moyamoya vessels begin to diminish
5 further progression of stage 4
6 complete absence of moyamoya vessels and major cerebral arteries.
Non-specific in the adult. Juvenile cases: high-voltage slow waves may be seen at rest, predominantly in the occipital and frontal lobes. Hyperventilation produces a normal buildup of monophasic slow waves (delta-bursts) that return to normal 20–60 seconds after hyperventilation. In >50%of cases, after or sometimes continuous with buildup is a second phase of slow waves (this characteristic finding is called “rebuild up”) which are more irregular and slower than the earlier waves, and usually, normalize in ≤10 minutes 7).
CBF is decreased in children with MMD, but relatively normal in adults. There is a shift of CBF from the frontal to the occipital lobes 8) probably reflecting the increasing dependency of CBF on the posterior circulation. Children with MMD have impaired autoregulation of CBF to blood pressure and CO2 (with more impairment of vasodilatation in response to hypercapnia or hypotension than vasoconstriction in response to hypocapnia or hypertension) 9). Xenon (Xe-133) CT can identify areas of low perfusion. Repeating the study after an acetazolamide challenge (which causes vasodilatation) evaluates the reserve capacity of CBF and can identify areas of “steal” which are at high risk of future infarction.