Clinical Features of Adult Moyamoya Disease With Special Reference to the Diagnosis

Abstract

Fifteen years of experience with adult moyamoya disease (MMD) in a single department were analyzed to describe the clinical features and to clarify the existence of the disease entity of adult MMD with special reference to its diagnosis. This study included 348 patients treated between 1996 and 2010. Male to female ratio was 128 to 220 with mean age of 39 years. The presence of neurological symptoms and signs during childhood and mean Suzuki stage according to the age were evaluated. Adult MMD was differentiated from other diseases, such as vasculitis, by comparing the laboratory data including common markers and specific markers for such diseases between 22 adult MMD and 21 atherosclerotic disease (ASD) patients. Conventional angiographic data were analyzed to detect progression in 52 unilateral adult MMD patients over a mean follow-up duration of 4.8 years. Only one of 128 patients (0.8%) aged over 40 years showed childhood symptoms and signs. The distribution of the mean Suzuki stage between patients aged 40 years and under and over 40 years was similar. Laboratory tests differentiating the other diseases with the same phenotype with MMD revealed no difference between adult MMD and ASD. The progression of unilateral MMD to bilateral MMD occurred in 6 of 52 patients (11.5%). Adult MMD is not a syndrome, but a distinct disease entity with significant progression of unilateral MMD to bilateral MMD. Diagnostic and therapeutic strategies should be different from pediatric cases when considering adult MMD.

The natural clinical course of hemodynamically stable adult moyamoya disease

“…However, the initial and final clinical outcomes were worst in patients with a hemorrhagic presentation, and all of the deaths resulted from a recurrent hemorrhage. The incidence of hemorrhagic presentation is known to be higher in adult MMD than in pediatric MMD, 9,16,20,23,36 and higher in Asian patients than in Western ones. 2,6,9,10,13,20,32 As reported, the incidence rates are 46%-62.4% in Korea, 9,10 52%-56% in China and Taiwan, 20 21% in Japan, 13,20 13%-29% in United States, 2,6,20,32 and the rate in our study was 25.7%.…”

“…Inclusion criteria were as follows: 1) adult patients (age ≥ 18 years); 2) initial symptoms related to hemorrhage or ischemia or asymptomatic presentation; 3) compatibility with the diagnostic guidelines; 16 4) hemodynamically stable status on single photon emission computed tomography (SPECT); 5) medical records pertaining to evaluation of certain concomitant medical conditions at the first visit and during the follow-up period, such as hypertension, smoking, diabetes mellitus, hyperlipidemia, or cardiovascular or peripheral vascular, endocrinological, or immunological diseases; and 6) follow-up duration of 6 months or more. All patients were initially evaluated with cerebral angiography, CT, and MRI, and SPECT.…”

“…36 Its presentation exhibits a bimodal distribution with age: children mostly exhibit an ischemic presentation, and adults usually present with hemorrhage or ischemia. 6,7,9,16,19,36 Although the pathogenesis and natural course are not yet known, revascularization surgery has been satisfactory for patients with ischemic symptoms. 2,3,5,7,8,18,22,24,34 For pediatric MMD, revascularization surgery is universally recommended, regardless of the severity of MMD, because most symptoms are ischemic, and children's brains are still developing.…”

Long-Term Outcomes After Combined Revascularization Surgery in Adult Moyamoya Disease

“…Medical data were collected prospectively according to our protocol and reviewed retrospectively under approval of the institutional review board. The inclusion criteria were as follows: (1) patients treated by combined revascularization surgery, aged ≥18 years; (2) compatibility with the diagnostic criteria for MMD 23 ; (3) recurrent ischemic symptoms including transient ischemic attack and cerebral infarction, predominantly in the middle cerebral artery territory; (4) significant decrease in basal perfusion and reservoir capacity by brain single photon emission computed tomography (SPECT); and (5) clinical follow-up duration of ≥5 years among patients treated between 2004 and 2008. The patients are generally scheduled to undergo postoperative follow-up 6 months (short-term period) and 5 years (long-term period) after surgery.…”

Sonographic findings associated with stenosis progression and vascular complications in moyamoya disease

“…MMD was diagnosed by digital subtraction angiography according to current diagnostic criteria. 15 To evaluate the factors associated with arterial stenosis, patients who met the following conditions were subjected to further analysis: 1) at least 1 vessel of the distal ICA and the proximal MCA of each hemisphere not occluded; 2) baseline MRI/MRA and TCD evaluations with an interval of less than 2 weeks; 3) favorable temporal windows for TCD and adequate TCD data; 4) follow-up MRI/MRA with an interval of more than 12 months; 5) no other potential causes of moyamoya syndrome; and 6) no surgical treatment for MMD until the follow-up MRI/MRA (because such a surgery might have significantly altered the hemodynamics of basal cerebral arteries). As a result, 19 patients with complete occlusion of both the MCA and the ICA, 26 patients with inadequate follow-up MRI/MRA, and 30 patients who had received surgical treatment were excluded.…”