A 9-year-old boy presented with an episode of syncope, and MR imaging revealed bilateral internal carotid artery stenosis with moyamoya vessel formation. He had had prophylactic cranial irradiation at a total dose of 24 Gy for the treatment of acute lymphocytic leukemia at the age of 4. Following this, he was in a complete state of remission for 6 years. During an observation period of a year after the onset of syncope, MR imaging showed development of multiple ischemic lesions in both hemispheres. He developed a transient ischemic attack of mild motor weakness in his arm and an indirect anastomosis was performed on the severely affected side at the age of 10. Radiation-induced vasculopathies are known to be associated with primary diseases of intracranial tumors, but the frequency is unclear. Ours is the third case in whom prophylactic cranial irradiation for a hematological disorder might have induced cerebral vasculopathies.

1.
Beyer RA, Paden P, Sobel DF, Flynn FG: Moyamoya pattern of vascular occlusion after radiotherapy for glioma of the optic chiasm. Neurology 1986;36:1173–1178.
2.
Bitzer M, Topka H: Progressive cerebral occlusive disease after radiation therapy. Stroke 1995;26:131–136.
3.
Peiffer J: Encephalomyelitis and demyelinating diseases in patients with extracerebral malignant tumors. J Neuroimmunol 1988;20:253–257.
4.
al-Amro A, Schultz H: Moyamoya vasculopathy after cranial irradiation – a case report. Acta Oncol 1995;34:261–263.
5.
Hilal SK, Solomon GE, Gold AP, Carter S: Primary cerebral arterial occlusive disease in children. II. Neurocutaneous syndromes. Radiology 1971;99:87–94.
6.
Inoue T, Matsushima T, Fujii K, Fukui M, Hasuo K, Matsuo H: Akin moyamoya disease in children (in Japanese). No Shinkei Geka 1993;21:59–65.
7.
Ishikawa T, Houkin K, Yoshimoto T, Abe H: Vasoreconstructive surgery for radiation-induced vasculopathy in childhood. Surg Neurol 1997;48:620–626.
8.
Ishiyama K, Tomura N, Kato K, Takahashi S, Watarai J, Sasajima T, Mizoi K: A patient with Moyamoya-like vessels after radiation therapy for a tumor in the basal ganglia (in Japanese). No To Shinkei 2001;53:969–973.
9.
Kestle JR, Hoffman HJ, Mock AR: Moyamoya phenomenon after radiation for optic glioma. J Neurosurg 1993;79:32–35.
10.
Lee KF, Hodes PJ: Intracranial ischemic lesions. Radiol Clin North Am 1967;5:353–360.
11.
Maruyama K, Mishima K, Saito N, Fujimaki T, Sasaki T, Kirino T: Radiation-induced aneurysm and moyamoya vessels presenting with subarachnoid haemorrhage. Acta Neurochir (Wien) 2000;142:139–143.
12.
Mori K, Takeuchi J, Ishikawa M, Handa H, Toyama M, Yamaki T: Occlusive arteriopathy and brain tumor. J Neurosurg 1978;49:22–35.
13.
Nishizawa S, Ryu H, Yokoyama T, Ninchoji T, Shimoyama I, Yamamoto S, Uemura K: Post-irradiation vasculopathy of intracranial major arteries in children – Report of two cases. Neurol Med Chir (Tokyo) 1991;31:336–341.
14.
Okuno T, Prensky AL, Gado M: The moyamoya syndrome associated with irradiation of an optic glioma in children: Report of two cases and review of the literature. Pediatr Neurol 1985;1:311–316.
15.
Omura M, Aida N, Sekido K, Kakehi M, Matsubara S: Large intracranial vessel occlusive vasculopathy after radiation therapy in children: Clinical features and usefulness of magnetic resonance imaging. Int J Radiat Oncol Biol Phys 1997;38:241–249.
16.
Rajakulasingam K, Cerullo LJ, Raimondi AJ: Childhood moyamoya syndrome. Postradiation pathogenesis. Childs Brain 1979;5:467–475.
17.
Servo A, Puranen M: Moyamoya syndrome as a complication of radiation therapy. Case report. J Neurosurg 1978;48:1026–1029.
18.
Sinsawaiwong S, Phanthumchinda K: Progressive cerebral occlusive disease after hypothalamic astrocytoma radiation therapy. J Med Assoc Thai 1997;80:338–342.
19.
Hata M, Ogino I, Aida N, Saito K, Omura M, Kigasawa H, Toyoda Y, Tachibana K, Matsubara S, Inoue T: Prophylactic cranial irradiation of acute lymphoblastic leukemia in childhood: Outcomes of late effects on pituitary function and growth in long-term survivors. Int J Cancer 2001;96(suppl):117–124.
20.
Waber DP, Shapiro BL, Carpentieri SC, Gelber RD, Zou G, Dufresne A, Romero I, Tarbell NJ, Silverman LB, Sallan SE: Excellent therapeutic efficacy and minimal late neurotoxicity in children treated with 18 grays of cranial radiation therapy for high-risk acute lymphoblastic leukemia: A 7-year follow-up study of the Dana-Farber Cancer Institute Consortium Protocol 87-01. Cancer 2001;92:15–22.
21.
Foreman NK, Laitt RD, Chambers EJ, Duncan AW, Cummins BH: Intracranial large vessel vasculopathy and anaplastic meningioma 19 years after cranial irradiation for acute lymphoblastic leukaemia. Med Pediatr Oncol 1995;24:265–268.
22.
Kagan AR, Bruce DW, Di Chiro G: Fatal foam cell arteritis of the brain after irradiation for Hodgkin’s disease: Angiography and pathology. Stroke 1971;2:232–238.
23.
Painter MJ, Chutorian AM, Hilal SK: Cerebrovasculopathy following irradiation in childhood. Neurology 1975;25:189–194.
24.
Laitt RD, Chambers EJ, Goddard PR, Wakeley CJ, Duncan AW, Foreman NK: Magnetic resonance imaging and magnetic resonance angiography in long term survivors of acute lymphoblastic leukemia treated with cranial irradiation. Cancer 1995;76:1846–1852.
25.
Cramer SC, Robertson RL, Dooling EC, Scott RM: Moyamoya and Down syndrome. Clinical and radiological features. Stroke 1996;27:2131–2135.
26.
Dobson SR, Holden KR, Nietert PJ, Cure JK, Laver JH, Disco D, Abboud MR: Moyamoya syndrome in childhood sickle cell disease: A predictive factor for recurrent cerebrovascular events. Blood 2002;99:3144–3150.
27.
Akgun D, Yilmaz S, Senbil N, Aslan B, Gurer YY: Moyamoya syndrome with protein S deficiency. Eur J Paediatr Neurol 2000;4:185–188.
28.
Andeejani AM, Salih MA, Kolawole T, Gader AM, Malabarey TO, al Damegh S, al Boukai A: Moyamoya syndrome with unusual angiographic findings and protein C deficiency: Review of the literature. J Neurol Sci 1998;159:11–16.
29.
Charuvanij A, Laothamatas J, Torcharus K, Sirivimonmas S: Moyamoya disease and protein S deficiency: A case report. Pediatr Neurol 1997;17:171–173.
30.
Cohen N, Berant M, Simon J: Moyamoya and Fanconi’s anemia. Pediatrics 1980;65:804–805.
31.
Hiyama H, Kusano R, Muragaki Y, Miura N: Moyamoya disease associated with thrombotic thrombocytopenic purpura (TTP) (in Japanese). No Shinkei Geka 1994;22:567–572.
32.
Holz A, Woldenberg R, Miller D, Kalina P, Black K, Lane E: Moyamoya disease in a patient with hereditary spherocytosis. Pediatr Radiol 1998;28:95–97.
33.
Lin HC, Chen RL, Wang PJ: Paroxysmal nocturnal hemoglobinuria presenting as moyamoya syndrome. Brain Dev 1996;18:157–159.
34.
Matsuda M, Enomoto T, Yanaka K, Nose T: Moyamoya disease associated with hemophilia A. Case report. Pediatr Neurosurg 2002;36:157–160.
35.
Pavlakis SG, Verlander PC, Gould RJ, Strimling BC, Auerbach AD: Fanconi anemia and moyamoya: Evidence for an association. Neurology 1995;45:998–1000.
36.
Tokunaga Y, Ohga S, Suita S, Matsushima T, Hara T: Moyamoya syndrome with spherocytosis: Effect of splenectomy on strokes. Pediatr Neurol 2001;25:75–77.
37.
Mitchell WG, Fishman LS, Miller JH, Nelson M, Zeltzer PM, Soni D, Siegel SM: Stroke as a late sequela of cranial irradiation for childhood brain tumors. J Child Neurol 1991;6:128–133.
Copyright / Drug Dosage / Disclaimer
Copyright: All rights reserved. No part of this publication may be translated into other languages, reproduced or utilized in any form or by any means, electronic or mechanical, including photocopying, recording, microcopying, or by any information storage and retrieval system, without permission in writing from the publisher.
Drug Dosage: The authors and the publisher have exerted every effort to ensure that drug selection and dosage set forth in this text are in accord with current recommendations and practice at the time of publication. However, in view of ongoing research, changes in government regulations, and the constant flow of information relating to drug therapy and drug reactions, the reader is urged to check the package insert for each drug for any changes in indications and dosage and for added warnings and precautions. This is particularly important when the recommended agent is a new and/or infrequently employed drug.
Disclaimer: The statements, opinions and data contained in this publication are solely those of the individual authors and contributors and not of the publishers and the editor(s). The appearance of advertisements or/and product references in the publication is not a warranty, endorsement, or approval of the products or services advertised or of their effectiveness, quality or safety. The publisher and the editor(s) disclaim responsibility for any injury to persons or property resulting from any ideas, methods, instructions or products referred to in the content or advertisements.
You do not currently have access to this content.