Cognitive Functions in Children and Adults with Moyamoya Vasculopathy: A Systematic Review and Meta-Analysis

Overview

Journal J Stroke

Date 2018 Oct 13

PMID 30309228

Citations 25

Authors

Annick Kronenburg

Esther van den Berg

Monique M van Schooneveld

Kees P J Braun

Lionel Calviere

Albert van der Zwan

Catharina J M Klijn

Affiliations

Soon will be listed here.

Abstract

Background And Purpose: Patients with moyamoya vasculopathy (MMV) may experience cognitive impairment, but its reported frequency, severity, and nature vary. In a systematic review and metaanalysis, we aimed to assess the presence, severity, and nature of cognitive impairments in children and adults with MMV.

Methods: We followed the MOOSE guidelines for meta-analysis and systematic reviews of observational studies. We searched Ovid Medline and Embase for studies published between January 1, 1969 and October 4, 2016. Independent reviewers extracted data for mean intelligence quotient (IQ) and standardized z-scores for cognitive tests, and determined percentages of children and adults with cognitive deficits, before and after conservative or surgical treatment. We explored associations between summary measures of study characteristics and cognitive impairments by linear regression analysis.

Results: We included 17 studies (11 studies reporting on 281 children, six on 153 adults). In children, the median percentage with impaired cognition was 30% (range, 13% to 67%); median IQ was 98 (range, 71 to 107). Median z-score was -0.39 for memory, and -0.43 for processing speed. In adults, the median percentage with impaired cognition was 31% (range, 0% to 69%); median IQ was 95 (range, 94 to 99). Median z-scores of cognitive domains were between -0.9 and -0.4, with multiple domains being affected. We could not identify determinants of cognitive impairment.

Conclusion: s A large proportion of children and adults with MMV have cognitive impairment, with modest to large deficits across various cognitive domains. Further studies should investigate determinants of cognitive deficits and deterioration, and the influence of revascularization treatment on cognitive functioning.

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References

Lei Y, Su J, Jiang H, Guo Q, Ni W, Yang H . Aberrant regional homogeneity of resting-state executive control, default mode, and salience networks in adult patients with moyamoya disease. Brain Imaging Behav. 2016; 11(1):176-184. DOI: 10.1007/s11682-016-9518-5. View

Weinberg D, Rahme R, Aoun S, Batjer H, Bendok B . Moyamoya disease: functional and neurocognitive outcomes in the pediatric and adult populations. Neurosurg Focus. 2011; 30(6):E21. DOI: 10.3171/2011.3.FOCUS1150. View

Herzog R, Alvarez-Pasquin M, Diaz C, Del Barrio J, Estrada J, Gil A . Are healthcare workers' intentions to vaccinate related to their knowledge, beliefs and attitudes? A systematic review. BMC Public Health. 2013; 13:154. PMC: 3602084. DOI: 10.1186/1471-2458-13-154. View

Kuroda S, Houkin K, Ishikawa T, Nakayama N, Ikeda J, Ishii N . Determinants of intellectual outcome after surgical revascularization in pediatric moyamoya disease: a multivariate analysis. Childs Nerv Syst. 2004; 20(5):302-8. DOI: 10.1007/s00381-004-0924-4. View

Kronenburg A, Braun K, van der Zwan A, Klijn C . Recent advances in moyamoya disease: pathophysiology and treatment. Curr Neurol Neurosci Rep. 2013; 14(1):423. DOI: 10.1007/s11910-013-0423-7. View

Ohtaki M, Uede T, Morimoto S, Nonaka T, Tanabe S, Hashi K . Intellectual functions and regional cerebral haemodynamics after extensive omental transplantation spread over both frontal lobes in childhood moyamoya disease. Acta Neurochir (Wien). 1998; 140(10):1043-53; discussion 1052-3. DOI: 10.1007/s007010050213. View

Festa J, Schwarz L, Pliskin N, Cullum C, Lacritz L, Charbel F . Neurocognitive dysfunction in adult moyamoya disease. J Neurol. 2009; 257(5):806-15. DOI: 10.1007/s00415-009-5424-8. View

Karzmark P, Zeifert P, Bell-Stephens T, Steinberg G, Dorfman L . Neurocognitive impairment in adults with moyamoya disease without stroke. Neurosurgery. 2011; 70(3):634-8. DOI: 10.1227/NEU.0b013e3182320d1a. View

Kazumata K, Tha K, Narita H, Kusumi I, Shichinohe H, Ito M . Chronic ischemia alters brain microstructural integrity and cognitive performance in adult moyamoya disease. Stroke. 2014; 46(2):354-60. DOI: 10.1161/STROKEAHA.114.007407. View

10.

Kronenburg A, Esposito G, Fierstra J, Braun K, Regli L . Combined Bypass Technique for Contemporary Revascularization of Unilateral MCA and Bilateral Frontal Territories in Moyamoya Vasculopathy. Acta Neurochir Suppl. 2014; 119:65-70. DOI: 10.1007/978-3-319-02411-0_11. View

11.

Scott R, Smith E . Moyamoya disease and moyamoya syndrome. N Engl J Med. 2009; 360(12):1226-37. DOI: 10.1056/NEJMra0804622. View

12.

Stroup D, Berlin J, Morton S, Olkin I, Williamson G, Rennie D . Meta-analysis of observational studies in epidemiology: a proposal for reporting. Meta-analysis Of Observational Studies in Epidemiology (MOOSE) group. JAMA. 2000; 283(15):2008-12. DOI: 10.1001/jama.283.15.2008. View

13.

Calviere L, Ssi Yan Kai G, Catalaa I, Marlats F, Bonneville F, Larrue V . Executive dysfunction in adults with moyamoya disease is associated with increased diffusion in frontal white matter. J Neurol Neurosurg Psychiatry. 2012; 83(6):591-3. DOI: 10.1136/jnnp-2011-301388. View

14.

Matsushima Y, Aoyagi M, Koumo Y, Takasato Y, Yamaguchi T, Masaoka H . Effects of encephalo-duro-arterio-synangiosis on childhood moyamoya patients--swift disappearance of ischemic attacks and maintenance of mental capacity. Neurol Med Chir (Tokyo). 1991; 31(11):708-14. DOI: 10.2176/nmc.31.708. View

15.

Su S, Hai J, Zhang L, Yu F, Wu Y . Assessment of cognitive function in adult patients with hemorrhagic moyamoya disease who received no surgical revascularization. Eur J Neurol. 2013; 20(7):1081-7. DOI: 10.1111/ene.12138. View

16.

Calviere L, Catalaa I, Marlats F, Viguier A, Bonneville F, Cognard C . Correlation between cognitive impairment and cerebral hemodynamic disturbances on perfusion magnetic resonance imaging in European adults with moyamoya disease. Clinical article. J Neurosurg. 2010; 113(4):753-9. DOI: 10.3171/2010.4.JNS091808. View

17.

Karasawa J, Touho H, Ohnishi H, Miyamoto S, Kikuchi H . Long-term follow-up study after extracranial-intracranial bypass surgery for anterior circulation ischemia in childhood moyamoya disease. J Neurosurg. 1992; 77(1):84-9. DOI: 10.3171/jns.1992.77.1.0084. View

18.

Tagawa T, ITAGAKI Y, Mimaki T, Tanaka J, Ito N, Suzuki Y . [Intelligence and regional cerebral blood flow in children with Moyamoya disease]. No To Hattatsu. 1989; 21(1):9-13. View

19.

Williams T, Westmacott R, Dlamini N, Granite L, Dirks P, Askalan R . Intellectual ability and executive function in pediatric moyamoya vasculopathy. Dev Med Child Neurol. 2011; 54(1):30-7. DOI: 10.1111/j.1469-8749.2011.04144.x. View

20.

Karzmark P, Zeifert P, Tan S, Dorfman L, Bell-Stephens T, Steinberg G . Effect of moyamoya disease on neuropsychological functioning in adults. Neurosurgery. 2008; 62(5):1048-51. DOI: 10.1227/01.neu.0000325866.29634.4c. View