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A retrospective study on clinical features of childhood moyamoya disease

      Abstract

      Background

      Childhood moyamoya disease(MMD)can lead to progressive and irreversible neurological impairment. Early age at onset is likely associated with a worst prognosis of the disease. The study aims to summarize the clinical characteristics of childhood MMD for supporting the diagnosis and treatment of early MMD.

      Methods

      A retrospective study was conducted on children aged 0-16 years who were diagnosed with MMD in the department of Neurology and neurosurgery of our hospital from October 2016 to April 2020. The clinical characteristics of children with MMD were summarized for analysis, and the distribution of sex and initial attacks type among different age groups were determined by data comparison.

      Results

      The study surveyed 114 children (the male to female sex ratio of 1:1.07) with MMD and 6.1% of them had family history. The mean age of onset was 7.15±3.30 years old and the peak age of onset was 5 to 8 years old. The most commonly initial attacks type was transient ischemic attack (TIA) (62 cases, 54.4%) with limb weakness. The incidence of the initial attacks type in the three age groups was varied (P < 0.05). The result of overall prognosis was good in 86 cases (89.6%).

      Conclusions

      In this study, MMD cases were mainly ischemic type and TIA was the most commonly initial attacks type. Infant group was more prone to have cerebral infarction, while preschool and school-age groups tended to have TIA. The treatments and prognosis of the studied MMD cases were achieved with good outcomes.

      Keywords

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      References

        • Kim J.E.
        • Jeon J.S.
        An update on the diagnosis and treatment of adult Moyamoya disease taking into consideration controversial issues.
        Neurol Res. 2014; 36: 407-416
        • Amlie-Lefond C.
        • Ellenbogen R.G.
        Factors associated with the presentation of moyamoya in childhood.
        J Stroke Cerebrovasc Dis. 2015; 24: 1204-1210
        • Giustini A.J.
        • Stone S.A.
        • Ramamoorthy C.
        Moyamoya disease in children and its anesthetic implications: A review.
        Paediatr Anaesth. 2020; 30: 1191-1198
      1. Research Committee On The Pathology And Treatment Of Spontaneous Occlusion Of The Circle Of Willis HLSR. Guidelines for diagnosis and treatment of moyamoya disease (spontaneous occlusion of the circle of Willis). 2012.

        • Zheng J.
        • Yu L.B.
        • Dai K.F.
        • et al.
        Clinical Features, Surgical Treatment, and Long-Term Outcome of a Multicenter Cohort of Pediatric Moyamoya.
        Front Neurol. 2019; 10: 14
        • Appireddy R.
        • Ranjan M.
        • Durafourt B.
        • et al.
        Surgery for Moyamoya Disease in Children.
        J Child Neurol. 2019; 34: 517-529
        • Fullerton H.J.
        • Wintermark M.
        • Hills N.K.
        • et al.
        Risk of Recurrent Arterial Ischemic Stroke in Childhood: A Prospective International Study.
        Stroke. 2016; 47: 53-59
        • Dlamini N.
        • Muthusami P.
        • Amlie-Lefond C.
        Childhood Moyamoya: Looking Back to the Future.
        Pediatr Neurol. 2019; 91: 11-19
        • Currie S.
        • Raghavan A.
        • Batty R.
        • et al.
        Childhood moyamoya disease and moyamoya syndrome: a pictorial review.
        Pediatr Neurol. 2011; 44: 401-413
        • Zhao M.
        • Lin Z.
        • Deng X.
        • et al.
        Clinical Characteristics and Natural History of Quasi-Moyamoya Disease.
        J Stroke Cerebrovasc Dis. 2017; 26: 1088-1097
        • Lee S.
        • Rivkin M.J.
        • Kirton A.
        • et al.
        Moyamoya Disease in Children: Results From the International Pediatric Stroke Study.
        J Child Neurol. 2017; 32: 924-929
        • Kim J.S.
        Moyamoya Disease: Epidemiology, Clinical Features, and Diagnosis.
        J Stroke. 2016; 18: 2-11
        • Kim T.
        • Lee H.
        • Bang J.S.
        • et al.
        Epidemiology of Moyamoya Disease in Korea: Based on National Health Insurance Service Data.
        J Korean Neurosurg Soc. 2015; 57: 390-395
        • Shang S.
        • Zhou D.
        • Ya J.
        • et al.
        Progress in moyamoya disease.
        Neurosurg Rev. 2020; 43: 371-382
        • Sato Y.
        • Kazumata K.
        • Nakatani E.
        • et al.
        Characteristics of Moyamoya Disease Based on National Registry Data in Japan.
        Stroke. 2019; 50: 1973-1980
        • Kainth D.
        • Chaudhry S.A.
        • Kainth H.
        • et al.
        Epidemiological and clinical features of moyamoya disease in the USA.
        Neuroepidemiology. 2013; 40: 282-287
        • Guey S.
        • Tournier-Lasserve E.
        • Herve D.
        • et al.
        Moyamoya disease and syndromes: from genetics to clinical management.
        Appl Clin Genet. 2015; 8: 49-68
        • Ball A.J.
        • Steinberg G.K.
        • Elbers J.
        Quality of Life in Pediatric Moyamoya Disease.
        Pediatr Neurol. 2016; 63: 60-65
        • Bao X.Y.
        • Duan L.
        • Yang W.Z.
        • et al.
        Clinical features, surgical treatment, and long-term outcome in pediatric patients with moyamoya disease in China.
        Cerebrovasc Dis. 2015; 39: 75-81
        • Zhao M.
        • Zhang D.
        • Wang S.
        • et al.
        Adolescents with moyamoya disease: clinical features, surgical treatment and long-term outcomes.
        Acta Neurochir (Wien). 2017; 159: 2071-2080
        • Zhang Y.
        • Bao X.
        • Duan L.
        • et al.
        Encephaloduroarteriosynangiosis for pediatric moyamoya disease: long-term follow-up of 100 cases at a single center.
        J Neurosurg Pediatr. 2018; 22: 173-180
        • Ng J.
        • Thompson D.
        • Lumley J.P.
        • et al.
        Surgical revascularisation for childhood moyamoya.
        Childs Nerv Syst. 2012; 28: 1041-1048
        • Guzman R.
        • Lee M.
        • Achrol A.
        • et al.
        Clinical outcome after 450 revascularization procedures for moyamoya disease. Clinical article.
        J Neurosurg. 2009; 111: 927-935
        • Appireddy R.
        • Ranjan M.
        • Durafourt B.A.
        • et al.
        Surgery for Moyamoya Disease in Children.
        J Child Neurol. 2019; 34: 517-529
        • Imai H.
        • Miyawaki S.
        • Ono H.
        • et al.
        The importance of encephalo-myo-synangiosis in surgical revascularization strategies for moyamoya disease in children and adults.
        World Neurosurg. 2015; 83: 691-699
        • Zhao Y.
        • Lu J.
        • Yu S.
        • et al.
        Comparison of Long-Term Effect Between Direct and Indirect Bypass for Pediatric Ischemic-Type Moyamoya Disease: A Propensity Score-Matched Study.
        Front Neurol. 2019; 10: 795
        • Shen W.
        • Xu B.
        • Li H.
        • et al.
        Enlarged Encephalo-Duro-Myo-Synangiosis Treatment for Moyamoya Disease in Young Children.
        World Neurosurg. 2017; 106: 9-16
        • Kamada F.
        • Aoki Y.
        • Narisawa A.
        • et al.
        A genome-wide association study identifies RNF213 as the first Moyamoya disease gene.
        J Hum Genet. 2011; 56: 34-40
        • Baek J.W.
        • Jo K.I.
        • Park J.J.
        • et al.
        Prevalence and clinical implications of renal artery stenosis in pediatric moyamoya disease.
        Eur J Paediatr Neurol. 2016; 20: 20-24
        • Fukushima H.
        • Takenouchi T.
        • Kosaki K.
        Homozygosity for moyamoya disease risk allele leads to moyamoya disease with extracranial systemic and pulmonary vasculopathy.
        Am J Med Genet A. 2016; 170: 2453-2456
        • Singhi P.
        • Choudhary A.
        • Khandelwal N.
        Pediatric moyamoya disease: clinical profile, literature review and sixteen year experience from a tertiary care teaching institute.
        Indian J Pediatr. 2013; 80: 1015-1020
        • Liu P.
        • Han C.
        • Li D.S.
        • et al.
        Hemorrhagic Moyamoya Disease in Children: Clinical, Angiographic features, and Long-Term Surgical Outcome.
        Stroke. 2016; 47: 240-243
        • Gaillard J.
        • Klein J.
        • Duran D.
        • et al.
        Incidence, clinical features, and treatment of familial moyamoya in pediatric patients: a single-institution series.
        J Neurosurg Pediatr. 2017; 19: 553-559
        • Tho-Calvi S.C.
        • Thompson D.
        • Saunders D.
        • et al.
        Clinical features, course, and outcomes of a UK cohort of pediatric moyamoya.
        Neurology. 2018; 90: e763-e770
        • Cai-Xia W.
        • Qing-Yi W.
        • Yi-Ming Z.
        • et al.
        Causes, clinical and imaging characteristics of moyamoya disease in children.
        Biomedical Research. 2018; 29: 1154-1159
        • Ge P.
        • Zhang Q.
        • Ye X.
        • et al.
        Clinical Features, Surgical Treatment, and Long-Term Outcome in Children with Hemorrhagic Moyamoya Disease.
        J Stroke Cerebrovasc Dis. 2018; 27: 1517-1523