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Moyamoya-like vasculopathy (moyamoya syndrome) in children

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Abstract

Introduction

A large variety of inherited or acquired childhood disorders and conditions may present in conjunction with progressive bilateral stenosis and/or occlusion of the terminal segments of the internal carotid artery. In addition, the development of pathological collateral vessels can be observed in the vicinity of the steno-occlusive changes in these patients. This condition is known as moyamoya-like (MML) vasculopathy. The natural history, the angiographic appearance, and the pathology of MML vasculopathy probably differ from those of definite moyamoya disease. Adequate cerebral vascular imaging should be considered in all pediatric patients with inherited or acquired systemic disorders and symptoms attributable to cerebral ischemia.

Cases and discussion

We present four pediatric cases of MML vasculopathy, and outline the etiology, the current classification, and the therapeutic approaches for this heterogeneous disease entity.

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References

  1. Akgun D, Yilmaz S, Senbil N, Aslan B, Gurer YY (2000) Moyamoya syndrome with protein S deficiency. Eur J Paediatr Neurol 4:185–188

    Article  CAS  PubMed  Google Scholar 

  2. Akutsu H, Sonobe M, Sugita K, Nakai Y, Matsumura A (2003) Familial association of basilar bifurcation aneurysm and moyamoya disease—four case reports. Neurol Med Chir (Tokyo) 43:435–438

    Google Scholar 

  3. Arita K, Uozumi T, Oki S, Kuwabara S, Ohba S, Nakahara T, Muttaqin Z, Kohno H, Yamada K (1992) Moyamoya disease associated with pituitary adenoma—report of two cases. Neurol Med Chir (Tokyo) 32:753–757

    Google Scholar 

  4. Arita K, Kurisu K, Ohba S, Shibukawa M, Kiura H, Sakamoto S, Uozumi T, Nakahara T (2003) Endovascular treatment of basilar tip aneurysms associated with moyamoya disease. Neuroradiology 45:441–445

    Article  CAS  PubMed  Google Scholar 

  5. Aylett SE, Britton JA, De Sousa CM (1996) Down syndrome and moyamoya disease: presentation with subarachnoid hemorrhage. Pediatr Neurol 14:259–261

    Article  CAS  PubMed  Google Scholar 

  6. Barrall JL, Summers CG (1996) Ocular ischemic syndrome in a child with moyamoya disease and neurofibromatosis. Surv Ophthalmol 40:500–504

    CAS  PubMed  Google Scholar 

  7. Berg JM, Armstrong D (1991) On the association of moyamoya disease with Down’s syndrome. J Ment Defic Res 35:398–403

    PubMed  Google Scholar 

  8. Bitzer M, Topka H (1995) Progressive cerebral occlusive disease after radiation therapy. Stroke 26:131–136

    CAS  PubMed  Google Scholar 

  9. Brant-Zawadzki M, Anderson M, DeArmond SJ, Conley FK, Jahnke RW (1980) Radiation-induced large intracranial vessel occlusive vasculopathy. Am J Roentgenol 134:51–55

    CAS  Google Scholar 

  10. Cheng MK (1982) A review of cerebrovascular surgery in the People’s Republic of China. Stroke 13:249–255

    CAS  PubMed  Google Scholar 

  11. Cohen N, Berant M, Simon J (1980) Moyamoya and Fanconi’s anemia. Pediatrics 65:804–805

    CAS  PubMed  Google Scholar 

  12. Cramer SC, Robertson RL, Dooling EC, Scott RM (1996) Moyamoya and Down syndrome. Clinical and radiological features. Stroke 27:2131–2135

    CAS  PubMed  Google Scholar 

  13. Darmody WR, Thomas LM, Gurdjian ES (1967) Postirradiation vascular insufficiency syndrome. Case report. Neurology 17:1190–1192

    CAS  PubMed  Google Scholar 

  14. Debrun G, Lacour P (1974) A new case of moyamoya disease associated with several intracavernous aneurysms. Neuroradiology 7:277–282

    CAS  PubMed  Google Scholar 

  15. DeVeber G, Roach ES, Riela AR, Wiznitzer M (2000) Stroke in children: recognition, treatment, and future directions. Semin Pediatr Neurol 7:309–317

    PubMed  Google Scholar 

  16. Drew JM, Scott JA, Chua GT (1993) General case of the day. Moyamoya syndrome in a child with sickle cell disease. Radiographics 13:483–484

    CAS  PubMed  Google Scholar 

  17. Erickson RP, Woolliscroft J, Allen RJ (1980) Familial occurrence of intracranial arterial occlusive disease (moyamoya) in neurofibromatosis. Clin Genet 18:191–196

    CAS  PubMed  Google Scholar 

  18. Fujiwara S, Miyazono M, Tsuda H, Fukui M (1993) Intraventricular hemorrhage and cerebral ischemic attacks in the presence of lupus anticoagulant mimicking moyamoya disease. J Neurosurg Sci 37:161–164

    CAS  PubMed  Google Scholar 

  19. Fukushima Y, Kondo Y, Kuroki Y, Miyake S, Iwamoto H, Sekido K, Yamaguchi K (1986) Are Down syndrome patients predisposed to moyamoya disease? Eur J Pediatr 144:516–517

    CAS  PubMed  Google Scholar 

  20. Fukuyama Y, Osawa M, Kanai N (1992) Moyamoya disease (syndrome) and the Down syndrome. Brain Dev 14:254–256

    CAS  PubMed  Google Scholar 

  21. Gaggero R, Donati PT, Curia R, De Negri M (1996) Occlusion of unilateral carotid artery in Down syndrome. Brain Dev 18:81–83

    Article  CAS  PubMed  Google Scholar 

  22. Ganesan V, Kirkham FJ (1997) Noonan syndrome and moyamoya. Pediatr Neurol 16:256–258

    Article  CAS  PubMed  Google Scholar 

  23. Ganesan V, Prengler M, McShane MA, Wade AM, Kirkham FJ (2003) Investigation of risk factors in children with arterial ischemic stroke. Ann Neurol 53:167–173

    Article  PubMed  Google Scholar 

  24. Garza-Mercado R (1982) Pseudomoyamoya in sickle cell anemia. Surg Neurol 18:425–431

    CAS  PubMed  Google Scholar 

  25. Gebarski SS, Gabrielsen TO, Knake JE, Latack JT (1983) Posterior circulation intracranial arterial occlusive disease in neurofibromatosis. Am J Neuroradiol 4:1245–1246

    CAS  PubMed  Google Scholar 

  26. Goldstein EM, Singer HS (1990) Moyamoya-like disease in Down’s syndrome. Pediatr Neurosurg 16:14–16

    PubMed  Google Scholar 

  27. Gotze P, Kuhne D (1976) The so-called moyamoya syndrome (hemangiomatous vascular network at the base of the skull) associated with Recklinghausen’s neurofibromatosis. A case report. Nervenarzt 47:34–39

    CAS  PubMed  Google Scholar 

  28. Hanakita J, Kondo A, Ishikawa J, Koyama T, Hazama F (1982) [An autopsy case of moyamoya disease] (in Japanese). No Shinkei Geka 10:531–539

    CAS  PubMed  Google Scholar 

  29. Hattori S, Kiguchi H, Ishii T, Nakajima T, Yatsuzuka H (1998) Moyamoya disease with concurrent von Recklinghausen’s disease and cerebral arteriovenous malformation. Pathol Res Pract 194:363–369

    CAS  PubMed  Google Scholar 

  30. Hirata Y, Matsukado Y, Mihara Y, Kochi M, Sonoda H, Fukumura A (1985) Occlusion of the internal carotid artery after radiation therapy for the chiasmal lesion. Acta Neurochir (Wien) 74:141–147

    Google Scholar 

  31. Horikawa M, Utunomiya H, Hirotaka S, Yamada S, Ohtaki E, Matsuishi T (1997) Case of von Recklinghausen disease associated with cerebral infarction. J Child Neurol 12:144–146

    CAS  PubMed  Google Scholar 

  32. Ichiba N, Murakawa S, Ohtahara S (1984) A case of Down syndrome with “moyamoya” disease: a consideration on fibromuscular dysplasia. No To Hattatsu 16:487–491

    CAS  PubMed  Google Scholar 

  33. Ikeda E, Hosoda Y (1993) Distribution of thrombotic lesions in the cerebral arteries in spontaneous occlusion of the circle of Willis: cerebrovascular moyamoya disease. Clin Neuropathol 12:44–48

    CAS  PubMed  Google Scholar 

  34. Ikezaki K, Han DH, Kawano T, Inamura T, Fukui M (1997) Epidemiological survey of moyamoya disease in Korea. Clin Neurol Neurosurg 99 [Suppl 2]:S6–S10

    Article  PubMed  Google Scholar 

  35. Imaizumi M, Nukada T, Yoneda S, Takano T, Hasegawa K, Abe H (1978) Tuberous sclerosis with moyamoya disease. Case report. Med J Osaka Univ 28:345–353

    CAS  PubMed  Google Scholar 

  36. Imaoka K, Kobayashi S, Fukuda J, Okada K, Tsunematsu T (1990) An association of Shy-Drager syndrome with moyamoya disease—a case report. Rinsho Shinkeigaku 30:560–562

    CAS  PubMed  Google Scholar 

  37. Inoue T, Matsushima T, Fujii K, Fukui M, Hasuo K, Matsuo H (1993) Akin moyamoya disease in children. No Shinkei Geka 21:59–65

    CAS  PubMed  Google Scholar 

  38. Kayama T, Suzuki S, Sakurai Y, Nagayama T, Ogawa A, Yoshimoto T (1986) A case of moyamoya disease accompanied by an arteriovenous malformation. Neurosurgery 18:465–468

    CAS  PubMed  Google Scholar 

  39. Kirkham FJ, Prengler M, Hewes DK, Ganesan V (2000) Risk factors for arterial ischemic stroke in children. J Child Neurol 15:299–307

    CAS  PubMed  Google Scholar 

  40. Kotagal S, Peterson PL, Martens ME, Lee CP, Nigro M, Archer CR (1988) Impaired NADH-CoQ reductase activity in a child with moyamoya syndrome. Pediatr Neurol 4:241–244

    Article  CAS  PubMed  Google Scholar 

  41. Kuchiki H, Katakura K, Kinjo T, Sato K, Kayama T (1998) Transsphenoidal surgery and gamma-knife radiosurgery for a treatment of recurrent craniopharyngioma with moyamoya vessels. No Shinkei Geka 26:273–278

    CAS  PubMed  Google Scholar 

  42. Lamas E, Diez Lobato R, Cabello A, Abad JM (1978) Multiple intracranial arterial occlusions (moyamoya disease) in patients with neurofibromatosis. One case report with autopsy. Acta Neurochir (Wien) 45:133–145

    Google Scholar 

  43. Launay M, Fredy D, Merland JJ, Bories J (1977) Narrowing and occlusion of arteries by intracranial tumors. Review of the literature and report of 25 cases. Neuroradiology 14:117–126

    CAS  PubMed  Google Scholar 

  44. Lichtor T, Mullan S (1987) Arteriovenous malformation in moyamoya syndrome. Report of three cases. J Neurosurg 67:603–608

    CAS  PubMed  Google Scholar 

  45. Likavcan M, Benko J, Papiernikova E, Lindtnerova L (1979) Moyamoya syndrome with hyperlipoproteinemia type IIa. Cesk Neurol Neurochir 42:49–53

    CAS  PubMed  Google Scholar 

  46. Mathew NT, Abraham J, Chandy J (1970) Cerebral angiographic features in tuberculous meningitis. Neurology 20:1015–1023

    CAS  PubMed  Google Scholar 

  47. Matsushima Y, Qian L, Aoyagi M (1997) Comparison of moyamoya disease in Japan and moyamoya disease (or syndrome) in the People’s Republic of China. Clin Neurol Neurosurg 99 [Suppl 2]:S19–S22

    Article  PubMed  Google Scholar 

  48. Merkel KH, Ginsberg PL, Parker JC Jr, Post MJ (1978) Cerebrovascular disease in sickle cell anemia: a clinical, pathological and radiological correlation. Stroke 9:45–52

    CAS  PubMed  Google Scholar 

  49. Mito T, Becker LE (1992) Vascular dysplasia in Down syndrome: a possible relationship to moyamoya disease. Brain Dev 14:248–251

    CAS  PubMed  Google Scholar 

  50. Mori K, Takeuchi J, Ishikawa M, Handa H, Toyama M, Yamaki T (1978) Occlusive arteriopathy and brain tumor. J Neurosurg 49:22–35

    CAS  PubMed  Google Scholar 

  51. Natori Y, Ikezaki K, Matsushima T, Fukui M (1997) ‘Angiographic moyamoya’ its definition, classification, and therapy. Clin Neurol Neurosurg 99 [Suppl 2]:S168–S172

    PubMed  Google Scholar 

  52. Nowak-Gottl U, Gunther G, Kurnik K, Strater R, Kirkham F (2003) Arterial ischemic stroke in neonates, infants, and children: an overview of underlying conditions, imaging methods, and treatment modalities. Semin Thromb Hemost 29:405–414

    Article  PubMed  Google Scholar 

  53. Okuno T, Prensky AL, Gado M (1985) The moyamoya syndrome associated with irradiation of an optic glioma in children: report of two cases and review of the literature. Pediatr Neurol 1:311–316

    Article  CAS  PubMed  Google Scholar 

  54. Outwater EK, Platenberg RC, Wolpert SM (1989) Moyamoya disease in Down syndrome. Am J Neuroradiol 10:S23–S24

    CAS  PubMed  Google Scholar 

  55. Pavlakis SG, Verlander PC, Gould RJ, Strimling BC, Auerbach AD (1995) Fanconi anemia and moyamoya: evidence for an association. Neurology 45:998–1000

    CAS  PubMed  Google Scholar 

  56. Schuster JM, Roberts TS (1999) Symptomatic moyamoya disease and aortic coarctation in a patient with Noonan’s syndrome: strategies for management. Pediatr Neurosurg 30:206–210

    Article  CAS  PubMed  Google Scholar 

  57. Servo A, Puranen M (1978) Moyamoya syndrome as a complication of radiation therapy. Case report. J Neurosurg 48:1026–1029

    CAS  PubMed  Google Scholar 

  58. Sobata E, Ohkuma H, Suzuki S (1988) Cerebrovascular disorders associated with von Recklinghausen’s neurofibromatosis: a case report. Neurosurgery 22:544–549

    CAS  PubMed  Google Scholar 

  59. Steinke W, Tatemichi TK, Mohr JP, Massaro A, Prohovnik I, Solomon RA (1992) Caudate hemorrhage with moyamoya-like vasculopathy from atherosclerotic disease. Stroke 23:1360–1363

    CAS  PubMed  Google Scholar 

  60. Sunder TR (1981) Moyamoya disease in a patient with type I glycogenosis. Arch Neurol 38:251–253

    CAS  PubMed  Google Scholar 

  61. Tsuda H, Hattori S, Tanabe S, Nishioka S, Matsushima T, Ikezaki K, Fukui M, Kinoshita S, Hamasaki N (1997) Thrombophilia found in patients with moyamoya disease. Clin Neurol Neurosurg 99 [Suppl 2]:S229–S233

    Article  PubMed  Google Scholar 

  62. Tsuda H, Hattori S, Tanabe S, Iida H, Nakahara M, Nishioka S, Fujise M, Kinoshita S, Okubo K, Hamasaki N (1999) Screening for aetiology of thrombophilia: a high prevalence of protein S abnormality. Ann Clin Biochem 36:423–432

    PubMed  Google Scholar 

  63. Tsuji N, Kuriyama T, Iwamoto M, Shizuki K (1984) Moyamoya disease associated with craniopharyngioma. Surg Neurol 21:588–592

    Article  CAS  PubMed  Google Scholar 

  64. Tsuruta D, Fukai K, Seto M, Fujitani K, Shindo K, Hamada T, Ishii M (1999) Phakomatosis pigmentovascularis type IIIb associated with moyamoya disease. Pediatr Dermatol 16:35–38

    Article  CAS  PubMed  Google Scholar 

  65. Van Diemen-Steenvoorde R, van Nieuwenhuizen O, de Klerk JB, Duran M (1990) Quasi-moyamoya disease and heterozygosity for homocystinuria in a five-year-old girl. Neuropediatrics 21:110–112

    PubMed  Google Scholar 

  66. Wakai K, Tamakoshi A, Ikezaki K, Fukui M, Kawamura T, Aoki R, Kojima M, Lin Y, Ohno Y (1997) Epidemiological features of moyamoya disease in Japan: findings from a nationwide survey. Clin Neurol Neurosurg 99 [Suppl 2]:S1–S5

    Article  Google Scholar 

  67. Woody RC, Perrot LJ, Beck SA (1992) Neurofibromatosis cerebral vasculopathy in an infant: clinical, neuroradiographic, and neuropathologic studies. Pediatr Pathol 12:613–619

    CAS  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Neurosurgery, Universitätsklinikum Mannheim, Theodor-Kutzer-Ufer 1–3, 68167, Mannheim, Germany

    Peter Horn, Peter Vajkoczy & Peter Schmiedek

  2. Department of Pediatrics, Universitätsklinikum Mannheim, Mannheim, Germany

    Stefan Pfister

  3. Department of Neuroradiology, Universitätsklinikum Mannheim, Mannheim, Germany

    Eva Bueltmann

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  1. Peter Horn
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  2. Stefan Pfister
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  4. Peter Vajkoczy
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Correspondence to Peter Horn.

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Horn, P., Pfister, S., Bueltmann, E. et al. Moyamoya-like vasculopathy (moyamoya syndrome) in children. Childs Nerv Syst 20, 382–391 (2004). https://doi.org/10.1007/s00381-004-0960-0

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