Nationwide survey on quasi-moyamoya disease in Japan.
OBJECTIVE
Moyamoya disease (MMD) is a unique occlusive disease of the bilateral internal carotid arteries with moyamoya vessels. Inherited or acquired disorders and conditions may present in conjunction with MMD. This condition is known as quasi-moyamoya disease. To identify the clinical features of quasi-MMD in Japan, a nationwide survey was conducted.


METHODS
A questionnaire was mailed directly to 241 departments that had acknowledged treating quasi-MMD patients in the primary survey. We ascertained the sex, age, family history, clinical manifestation, radiological findings, treatments, course of the disease, and daily activity of the patients.


RESULTS
A total of 114 departments replied to the questionnaire. The data of 108 patients (66 female and 42 male; female to male ratio 1.57) were registered and analyzed. Mean age was 30.6 years old, with a peak in children. Seven patients (7 %) exhibited familial MMD. The initial clinical manifestation was motor weakness, followed by transient ischemic attack and headache. Their imaging study type included ischemic type in 64 patients (63.4 %), bleeding type in seven (6.9 %), and normal in 27 (26.7 %). Stenoocclusive lesion was seen in the internal carotid artery in more than half of the patients. Development of moyamoya vessels was mild in approximately 40 % of the patients. Almost all cases were accompanied by cerebral hypoperfusion. About half of them were unilateral lesion. Vascular reconstruction was employed for the approximately 60 % patients. The prognosis did not change significantly.


CONCLUSIONS
Clinical features of quasi-MMD were revealed in the nationwide study. In quasi-MMD, unilateral lesion is dominant, and the development of moyamoya vessels and intracranial hemorrhage are less dominant.
Department of Neurosurgery, Nagasaki University School of Medicine, Sakamoto 1-7-1, Nagasaki city, Nagasaki, 852-8501, Japan, kenkuni@nagasaki-u.ac.jp.
Kentaro
K
Hayashi
Nobutaka
N
Horie
Tsuyoshi
T
Izumo
Izumi
I
Nagata
D000293:Adolescent; D000328:Adult; D000368:Aged; D020771:Anterior Cerebral Artery / Q000000981:diagnostic imaging; D058070:Asymptomatic Diseases; D002343:Carotid Artery, Internal / Q000000981:diagnostic imaging; D002533:Cerebral Angiography; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D015897:Comorbidity; D005260:Female; D006801:Humans; D007223:Infant; D020300:Intracranial Hemorrhages / Q000209:etiology*; D002546:Ischemic Attack, Transient / Q000209:etiology*; D007564:Japan / Q000453:epidemiology; D008297:Male; D008875:Middle Aged; D020768:Middle Cerebral Artery / Q000000981:diagnostic imaging; D009072:Moyamoya Disease / Q000150:complications* / Q000175:diagnosis / Q000453:epidemiology; D018908:Muscle Weakness / Q000209:etiology*; D012640:Seizures / Q000209:etiology*; D011795:Surveys and Questionnaires; D055815:Young Adult