J Cerebrovasc Endovasc Neurosurg.  2022 Jun;24(2):160-165. 10.7461/jcen.2021.E2021.07.003.

Acute cerebral infarction combined with a thyroid storm in a patient with both Moyamoya syndrome and Graves’ disease

• Gill JH
• Nam TK
• Jung HK
• Jang KM
• Choi HH
• Park YS
• Kwon JT

• KMID: 2531659
• DOI: http://doi.org/10.7461/jcen.2021.E2021.07.003

Abstract

Moyamoya syndrome (MMS) associated with hyperthyroidism, such as Graves’ disease, is a rare condition that causes ischemic stroke with thyrotoxicity. A 43-year-old woman with symptoms of right hemiparesis was admitted. Brain magnetic resonance imaging revealed a small cerebral infarction in the left frontal lobe. Cerebral angiography revealed multi-vessel intracranial occlusive disease. Several days later, neurologic deterioration and aggravation of cerebral infarction developed due to a thyroid storm. A thyroid function test revealed the following: thyroid-stimulating hormone (TSH) ＜0.01 μunits/mL (reference range, 0.55–4.78 μunits/mL); triiodo-thyronine ＞8.0 ng/mL (reference range, 0.77–1.81 ng/mL); free thyroxine (T4) of 9.47 pmol/L (reference range, 11.4–22.6 pmol/L); and TSH receptor antibody of 37.10 U/L (reference range, 0–10 U/L). For thyroid storm management, we initiated treatment with methimazole, Gemstein’s solution, and hydrocortisone. Finally, the thyroid disease was controlled, and neurologic deficits improved. We describe a case of acute cerebral infarction combined with a thyroid storm in a patient with Moyamoya syndrome and Graves’ disease. Hyperthyroidism such as Graves’ disease should be considered in the differential diagnosis for patho-etiologic mechanisms associated with MMS. A cerebrovascular disease with a thyroid storm can lead to severe mortality and morbidity. Prompt diagnosis and strict treatment are important.

Keyword

Moyamoya Syndrome; Graves’ Disease; Cerebral Infarction; Thyroid Storm

Figure

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  Fig. 1. Brain magnetic resonance imaging. (A) initial diffusion image, (B) aggravated diffusion image. Cerebral infarction territory at both frontal lobes was increased.

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  Fig. 2. Cerebral angiography. (A) Right internal cerebral artery (ICA), (B) Left ICA, (C) Left vertebral artery. Steno-occlusion of bilateral distal ICA with moyamoya vessels.

Reference

1. Clinical practice. Graves’ disease. N Engl J Med. 2008; Jun. 358(24):2594–605.

2. Burch HB, Cooper DS. Management of Graves disease: a review. JAMA. 2015; Dec. 314(23):2544–54.

3. Burch HB, Wartofsky L. Life-threatening thyrotoxicosis: thyroid storm. Endocrinol Metab Clin North Am. 1993; Jun. 22(2):263–77.

4. Golomb MR, Biller J, Smith JL, Edwards-Brown M, Sanchez JC, Nebesio TD, et al. A 10-year-old girl with coexistent moyamoya disease and Graves’ disease. J Child Neurol. 2005; Jul. 20(7):620–4.

5. Im S-H, Oh CW, Kwon O-K, Kim JE, Han DH. Moyamoya disease associated with Graves disease: special considerations regarding clinical significance and management. J Neurosurg. 2005; Jun. 102(6):1013–7.

6. Ito H, Yokoi S, Yokoyama K, Asai T, Uda K, Araki Y. Progressive stenosis and radiological findings of vasculitis over the entire internal carotid artery in moyamoya vasculopathy associated with Graves’ disease: a case report and review of the literature. BMC Neurol. 2019; Mar. 19(34):

7. Jang SA, Baek YH, Park TS, Lee KA. A case of acute cerebral infarction and thyroid storm associated with Moyamoya disease. Int J Thyroidol. 2017; 10(1):56–60.

8. Seong G-H, Bae JS, Lee J-H, Kim Y. Acute ischemic stroke in Moyamoya syndrome associated with thyrotoxicosis. J Neurocrit Care. 2018; Sep. 11(2):129–33.

9. Kumar P, Panigrahi I, Sankhyan N, Ahuja C, Goyadi PK. Down syndrome with moyamoya disease: A case series. J Pediatr Neurosci. 2018; Apr-Jun. 13(2):201–4.

10. Kushima K, Satoh Y, Ban Y, Taniyama M, Ito K, Sugita K. Graves’ thyrotoxicosis and Moyamoya disease. Can J Neurol Sci. 1991; May. 18(2):140–2.

11. Russman AN, Katramados AM, Silver B, Mitsias PD. Moyamoya syndrome associated with Graves’ disease: a case report and review of the literature. J Stroke Cerebrovasc Dis. 2011; Nov. 20(6):528–36.

12. Menconi F, Marcocci C, Marinò M. Diagnosis and classification of Graves’ disease. Autoimmun Rev. 2014; Apr-May. 13(4-5):398–402.

13. Nakamura K, Yanaka K, Ihara S, Nose T. Multiple intracranial arterial stenoses around the circle of Willis in association with Graves’ disease: report of two cases. Neurosurgery. 2003; Nov. 53(5):1210–5.

14. Ngo SY, Chew HC. When the storm passes unnoticed--a case series of thyroid storm. Resuscitation. 2007; Jun. 73(3):485–90.

15. Nikiforov YE, Biddinger PW, Thompson LDR. Diagnostic Pathology and Molecular Genetics of the Thyroid. 2nd ed. Philadelphia: Wolters Kluwer Health/Lippincott Williams & Wilkins;2012. p. X.

16. Noh BH, Cho S-W, Ahn SY. Simultaneous occurrence of diabetic ketoacidosis, thyroid storm, and multiple cerebral infarctions due to Moyamoya disease. J Pediatr Endocrinol Metab. 2016; Feb. 29(2):221–5.

17. Ohba S, Nakagawa T, Murakami H. Concurrent Graves’ disease and intracranial arterial stenosis/occlusion: special considerations regarding the state of thyroid function, etiology, and treatment. Neurosurg Rev. 2011; Jul. 34(3):297–304. discussion 304.

18. Ono Y, Ono S, Yasunaga H, Matsui H, Fushimi K, Tanaka Y. Factors associated with mortality of thyroid storm: analysis using a national inpatient database in Japan. Medicine (Baltimore). 2016; Feb. 95(7):e2848.

19. Peerless SJ. Risk factors of moyamoya disease in Canada and the USA. Clin Neurol Neurosurg. 1997; Oct. 99 Suppl 2:S45–8.

20. Ren S-C, Gao B-Q, Yang W-L, Feng W-X, Xu J, Li S-W, et al. Von Willebrand factor and coagulation factor VIII in Moyamoya disease associated with Graves’ disease: A case report. Exp Ther Med. 2016; Oct. 12(5):3195–200.

21. Sasaki T, Nogawa S, Amano T. Co-morbidity of moyamoya disease with Graves’ disease. Report of three cases and a review of the literature. Intern Med. 2006; 45(9):649–53.

22. Shen A, Ryu S, Lin S. Concurrent moyamoya disease and Graves’ thyrotoxicosis: case report and literature review. Acta Neurol Taiwan. 2006; Jun. 15(2):114–9.

23. Swee DS, Chng CL, Lim A. Clinical characteristics and outcome of thyroid storm: a case series and review of neuropsychiatric derangements in thyrotoxicosis. Endocr Pract. 2015; Feb. 21(2):182–9.

24. Ueki K, Meyer FB, Mellinger JF. Moyamoya disease: the disorder and surgical treatment. Mayo Clin Proc. 1994; Aug. 69(8):749–57.