A Co-Operative Study: Clinical Characteristics of 334 Korean Patients with Moyamoya Disease Treated at Neurosurgical Institutes (1976-1994)

Abstract

A co-operative study was conducted to determine the clinical characteristics of patients with moyamoya disease who were diagnosed and treated at neurosurgical institutes in Korea before 1995. Twenty-six hospitals contributed 505 cases and among them, the clinical characteristics of 334 patients with definite moyamoya disease were evaluated. The number of patients began to increase from the late 1980s, and after that approximately 20 patients were treated each year. There were two age peaks: from six to 15 and from 31 to 40 years of age. Haemorrhagic manifestations occurred in approximately 43% of the patients. The major clinical manifestations were haemorrhage in adults (62.4%) and ischaemia in children (61.2%). Overall 54.5% of the patients experienced decreased consciousness levels, mainly due to intracranial haemorrhage or cerebral infarction. In the patients with ischemic manifestations, the adult patients were more likely to have cerebral infarction than the pediatric patients (80% vs. 39%) and the pediatric patients were more likely to have TIA (61% vs. 25%). Thirty eight percent of the patients underwent bypass surgery and 53% of these procedures were performed bilaterally. Treatment policies, including indications for bypass surgery and commonly used drugs, were somewhat different according to the institution. Overall favorable outcome was 73%, and the most significant factor affecting poor outcome was haemorrhagic manifestation. This article describes the characteristics of 334 patients with moyamoya disease, who were diagnosed and treated at neurosurgical institutes in Korea before 1995.

Epidemiological Features of Moyamoya Disease in Japan

“…A report from Korea evaluated 334 patients with definite moyamoya disease. 5) The female-to-male ratio was 1.46. There were two age peaks, at 6-15 years and 31-40 years.…”

“…In contrast, over 300 cases were reported in Korea in 2000. 5) The incidence of moyamoya disease in Korea is similar to that in Japan, but the exact prevalence and incidence ratio of moyamoya disease in Korea are not known. The exact prevalence and incidence of moyamoya disease in China are also unknown.…”

Moyamoya disease in pediatric patients: outcomes of neurosurgical interventions

“…The Research Committee on MMD of the Ministry of Health and Welfare in Japan has identified 4 criteria necessary for the diagnosis of MMD: 1) stenosis or occlusion of the terminal portion of the internal carotid artery; 2) a coexisting abnormal vascular network in the base of the brain or basal ganglia; 3) bilateral vascular involvement; and 4) no other identifiable cause of existing symptoms. 5 These guidelines have been modified on a case-by-case basis at various institutions around the US. Although classically a bilateral disease, MMD can occur unilaterally.…”

Pediatric moyamoya disease presenting with intracerebral hemorrhage—Report of three cases and review of the literature

“…Hemorrhagic type moyamoya disease is frequently seen in adults [1], and quite a low incidence of hemorrhage in patients aged < 16 years has been reported both in Japan [2,15] and Korea [4,14]. In adults, rebleeding is not uncommon and it could worsen the prognosis following hemorrhage [7,27].…”

“…Pediatric patients mainly present with cerebral ischemia and they very rarely bleed [2,4,14,15]. Although the benefits of surgical revascularization for ischemic moyamoya disease have been well recognized, the indications for such treatment in hemorrhagic type to prevent rebleeding have not yet been established [2,6,13,20,22,27].…”