Indian Journal of Physical Medicine and Rehabilitation

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VOLUME 29 , ISSUE 1 ( 2018 ) > List of Articles

ORIGINAL ARTICLE

Clinicoradiological Profile of Childhood Moyamoya Disease: Indian Study of 30 Children with Literature Review

Satish A Lahoti, BimanKanti Ray

Keywords : Early diagnosis, Myriad presentations, Neuroradiological spectrum,Childhood moyamoya disease

Citation Information : Lahoti SA, Ray B. Clinicoradiological Profile of Childhood Moyamoya Disease: Indian Study of 30 Children with Literature Review. Indian J Phy Med Rehab 2018; 29 (1):13-19.

DOI: 10.5005/jp-journals-10066-0020

License: CC BY-ND 3.0

Published Online: 00-03-2018

Copyright Statement:  Copyright © 2018; Jaypee Brothers Medical Publishers (P) Ltd.


Abstract

Introduction: Moyamoya disease is an uncommon cerebrovascular disease characterized by progressive steno-occlusive changes in terminal internal carotid arteries (ICAs) and their main branches, associated with development of moyamoya vessels.1 We present the largest case series of childhood moyamoya disease from India. Materials and methods: Thirty patients of childhood Moyamoya disease, whose diagnosis was confirmed by magnetic resonance imaging (MRI), MR angiography (MRA), and digital subtraction angiography (DSA), were studied for various spectrum of clinical and radiological manifestations. Results: Of the 30 patients evaluated (mean age: 6.71 years; F:M ratio 1.15:1), majority (96.66%) presented with ischemic symptoms, whereas only one (3.33%) had hemorrhagic stroke. Ischemic stroke with hemiparesis was the most common presenting feature (66.66%); rarer manifestations of moyamoya disease like headache, seizures, cognitive decline, visual loss, and bihemispherical transient ischemic attacks (TIAs) were also seen as presenting features. Radiologically, other than ischemic and hemorrhagic stroke, normal parenchyma with abnormal flow voids was the uncommon pattern seen on MRI brain, seen in 23.33%; 1 patient had isolated corpus callosal infarct. On angiography, 21 (70%) had bilateral ICA disease, 5 (16.66%) had unilateral ICA disease, and 4 (13.33%) had bilateral ICA with associated posterior circulation involvement. Conclusion: Other than seizures and strokes, headache, cognitive decline, bihemispherical TIAs and isolated corpus callosal involvement were the unusual presentations found in this study. Radiologically, unilateral affection and posterior circulation involvement were also found. It is important to be familiar with the usual and unusual clinical manifestations and MRI/MRA findings in moyamoya disease to make an early diagnosis leading to a good prognosis.


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  1. Takanashi J. Moyamoya disease in children. Brain Dev 2011 Mar;33(3):229-234.
  2. Takeuchi K, Shimizu K. Hypoplasia of the bilateral internal carotid arteries. Brain Nerve 1957;9:37-43.
  3. Suzuki J, Takaku A. Cerebrovascular “moyamoya” disease: disease showing abnormal net-like vessels in the base of brain. Arch Neurol 1969 Mar;20(3):288-299.
  4. Scott RM, Smith JL, Robertson RL, Madsen JR, Soriano SG, Rockoff MA. Long-term outcome in children with moyamoya syndrome after cranial revascularization by pial synangiosis. J Neurosurg 2004 Feb;100(2 Suppl):142-149.
  5. Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, Kojima M, Lin Y, Ohno Y. Epidemiological features of moyamoya disease in Japan: finding from a nationwide survey. Clin Neurol Neurosurg 1997 Oct;99 (Suppl 2):S1-S5.
  6. Baba T, Houkin K, Kuroda S. Novel epidemiological features of moyamoya disease. J Neurol Neurosurg Psychiatry 2008 Aug;79(8):900-904.
  7. Yonekawa Y, Ogata N, Kaku Y, Taub E, Imhof HG. Moyamoya disease in Europe, past and present status. Clin Neurol Neurosurg 1997 Oct;99 (Suppl 2):S58-S60.
  8. Smith ER, Scott RM. Surgical management of moyamoya syndrome. Skull Base 2005 Feb;15(1):15-26.
  9. Smith JL. Understanding and treating moyamoya disease in children. Neurosurg Focus 2009 Apr;26(4):E4.
  10. Kitahara T, Ariga N, Yamaura A, Makino H, Maki Y. Familial occurrence of moya-moya disease: report of 3 Japanese families. J Neurol Neurosurg Psychiatry 1979 Mar;42(3):208-214.
  11. Søgaard I, Jørgensen J. Familial occurrence of bilateral intracranial occlusion of the internal carotid arteries (Moya Moya). Acta Neurochir 1975;31(3-4):245-252.
  12. Soriano SG, Sethna NF, Scott RM. Anesthetic management of children with moyamoya syndrome. Anesth Analg 1993 Nov;77(5):1066-1070.
  13. Nagaraja D, Verma A, Taly AB, Kumar MV, Jayakumar PN. Cerebrovascular disease in children. Acta Neurol Scand 1994 Oct;90(4):251-255.
  14. Han DH, Nam DH, Oh CW. Moyamoya disease in adults: characteristics of clinical presentation and outcome after encephalo-duro-arterio-synangiosis. Clin Neurol Neurosurg 1997;99(Suppl 2):S151-S155.
  15. Jea A, Smith ER, Robertson R, Scott RM. Moyamoya syndrome associated with Down syndrome: outcome after surgical revascularization. Pediatrics 2005 Nov;116(5):e694-e701.
  16. Doherty MJ, Jayadev S, Watson NF, Konchada RS, Hallam DK. Clinical implications of splenium magnetic resonance imaging signal changes. Arch Neurol 2005 Mar;62(3):433-437.
  17. Suzuki J, Kodama N. Moyamoya disease—a review. Stroke 1983;14:104-109.
  18. Mugikura S, Takahashi S, Higano S, Shirane R, Kurihara N, Furuta S, Ezura M, Takahashi A. The relationship between cerebral infarction and angiographic characteristics in childhood moyamoya disease. AJNR Am J Neuroradiol 1999 Feb;20(2):336-343.
  19. Shoukat S, Itrat A, Taqui AM, Zaidi M, Kamal KA. Moyamoya disease: a clinical spectrum, literature review and case series from a tertiary care hospital in Pakistan. BMC Neurol 2009 Apr 15;9:15.
  20. Garg AK, Suri A, Sharma BS. Ten-year experience of 44 patients with moyamoya disease from a single institution. J Clin Neurosci 2010 Apr;17(4):460-463.
  21. Singhi P, Choudhary A, Khandewal N. Pediatric moyamoya disease: clinical profile, literature review and sixteen year experience from a tertiary care teaching institute. Indian J Pediatr 2013 Dec;80(12):1015-1020.
  22. Chinchure SD, Pendharkar HS, Gupta AK, Bodhey N, Harsha KJ. Adult onset moyamoya disease: institutional experience. Neurol India 2011 Sep-Oct;59(5):733-738.
  23. Srivastava T, Sannegowda RB, Mittal RS, Jain RS, Tejwani S, Jain R. An institutional experience of 26 patients with Moyamoya disease: a study from Northwest India. Ann Indian Acad Neurol 2014 Apr;17:182-186.
  24. Lefond AC, Zaidat OO, Lew SM. Moyamoya disease in early infancy: case report and literature review. Pediatr Neurol 2011 Apr;44(4):299-302.
  25. Sribnick EA, Goldblatt MR, Campbell JW, Roberts JR. Moyamoya disease in a four-month-old: a case study. Clin Pediatr (Phila) 2003 Apr;42(3):281-284.
  26. Duan L, Bao XY, Yang WZ, Shi WC, Li DS, Zhang ZS, Zong R, Han C, Zhao F, Feng J. Moyamoya disease in China its clinical features and outcomes. Stroke 2012 Jan;43(1):56-60.
  27. Yamauchi T, Houkin K, Tada M, Abe H. Familial occurrence of moyamoya disease. Clin Neurol Neurosurg 1997 Oct;99 (Suppl 2):S162-S167.
  28. Fukui M, Kono S, Sueishi K, Ikezaki K. Moyamoya disease. Neuropathology 2000 Sep;20(Suppl):S61-S64.
  29. Nanba R, Kuroda S, Tada M, Ishikawa T, Houkin K, Iwasaki Y. Clinical features of familial moyamoya disease. Childs Nerv Syst 2006 Mar;22(3):258-262.
  30. Ikeda H, Sasaki T, Yoshimoto T, Fukui M, Arinami T. Mapping of a familial moyamoya disease gene to chromosome 3p24.2- p26. Am J Hum Genet 1999 Feb;64(2):533-537.
  31. Nanba R, Tada M, Kuroda S, Houkin K, Iwasaki Y. Sequence analysis and bioinformatics analysis of chromosome 17q25 in familial moyamoya disease. Childs Nerv Syst 2005 Jan;21(1):62-68.
  32. Inoue TK, Ikezaki K, Sasazuki T, Matsushima T, Fukui M. Linkage analysis of moyamoya disease on chromosome 6. J Child Neurol 2000 Mar;15(3):179-182.
  33. Inoue TK, Ikezaki K, Sasazuki T, Matsushima T, Fukui M. Analysis of class II genes of human leukocyte antigen in patients with moyamoya disease. Clin Neurol Neurosurg 1997 Oct;99(Suppl 2):S234-S237.
  34. Han H, Pyo CW, Yoo DS, Huh PW, Cho KS, Kim DS. Associations of Moyamoya patients with HLA class I and class II alleles in the Korean population. J Korean Med Sci 2003 Dec;18(6):876-880.
  35. Sakurai K, Horiuchi Y, Ikeda H, Ikezaki K, Yoshimoto T, Fukui M, Arinami T. A novel susceptibility locus for moyamoya disease on chromosome 8q23. J Hum Genet 2004;49(5):278-281.
  36. Kodama N, Suzuki J. Cerebrovascular Moyamoya disease. III Report—The study on the aging of the perforating branches and the possibility of collateral pathway. Neurol Med Chir 1974;14 Pt 1:55-67.
  37. Iwama T, Morimoto M, Hashimoto N, Goto Y, Todaka T, Sawada M. Mechanism of intracranial rebleeding in moyamoya disease. Clin Neurol Neurosurg 1997 Oct;99 (Suppl 2): S187-S190.
  38. Irikura K, Miyasaka Y, Kurata A, Tanaka R, Fujii K, Yada K, Kan S. A source of haemorrhage in adult patients with moyamoya disease: the significance of tributaries from the choroidal artery. Acta Neurochir (Wien) 1996;138(11): 1282-1286.
  39. Kawaguchi S, Sakaki T, Morimoto T, Kakizaki T, Kamada K. Characteristics of intracranial aneurysms associated with moyamoya disease: a review of 111 cases. Acta Neurochir (Wien) 1996;138(11):1287-1294.
  40. Kuroda S, Houkin K, Kamiyama H, Abe H. Effects of surgical revascularization on peripheral artery aneurysms in moyamoya disease: report of three cases. Neurosurgery 2001 Aug;49(2):463-467.
  41. Seol HJ, Wang KC, Kim SK, Hwang YS, Kim KJ, Cho BK. Headache in pediatric moyamoya disease: a review of 204 consecutive cases. J Neurosurg 2005 Nov;103(5 Suppl): 439-442.
  42. Olesen J, Friberg L, Olsen TS, Andersen AR, Lassen NA, Hansen PE, Karle A. Ischaemia-induced (symptomatic) migraine attacks may be more frequent than migraine-induced ischaemic insults. Brain 1993 Feb;116(Pt 1):187-202.
  43. Kossorotoff M. Cognitive decline in moyamoya: influence of chronic cerebral hypoxia, history of stroke, or comorbid conditions? Dev Med Child Neurol 2012 Jan;54(1):4-7.
  44. Hogan AM, Pit-ten Cate IM, Vargha-Khadem F, Prengler M, Kirkham FJ. Physiological correlates of intellectual function in children with sickle cell disease: hypoxaemia, hyperaemia and brain infarction. Dev Sci 2006 Jul;9(4):379-387.
  45. Ibrahimi D, Tamargo RJ, Ahn ES. Moyamoya disease in children. Childs Nerv Syst 2010 Oct;26(10):1297-1308.
  46. Pandey P, Stephens TB, Steinberg GK. Patients with moyamoya disease presenting with movement disorder. J Neurosurg Pediatr 2010 Dec;6(6):559-566.
  47. Zheng W, Wanibuchi M, Onda T, Liu H, Koyanagi I, Fujimori K, Houkin K. A case of moyamoya disease presenting with chorea. Childs Nerv Syst 2006 Mar;22(3):274-278.
  48. Jayakumar PN, Vasudev MK, Srikanth SG. Posterior circulation abnormalities in moyamoya disease: a radiological study. Neurol India 1999 Jun;47(2):112-117.
  49. Kelly ME, Bell-Stephens TE, Marks MP, Do HM, Steinberg GK. Progression of unilateral moyamoya disease: a clinical series. Cerebrovasc Dis 2006;22(2-3):109-115.
  50. Houkin K, Abe H, Yoshimoto T, Takahashi A. Is “unilateral” moyamoya disease different from moyamoya disease? J Neurosurg 1996 Nov;85(5):772-776.
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