|Year : 2022 | Volume
| Issue : 5 | Page : 764-766
Moyamoya syndrome associated with graves' disease: A case report
Shalesh Rohatgi1, Varsha Rangankar2, Prajwal Rao1, Satish Nirhale3, Pravin Naphade1
1 Department of Neurology, Dr. D. Y. Patil Medical College, Hospital and Research Center, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India
2 Department of Radiodiagnosis, Dr. D. Y. Patil Medical College, Hospital and Research Center, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India
3 Department of Medicine, Dr. D. Y. Patil Medical College, Hospital and Research Center, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra, India
|Date of Submission||30-Nov-2021|
|Date of Decision||15-Mar-2022|
|Date of Acceptance||15-Mar-2022|
|Date of Web Publication||06-Oct-2022|
Dr. Shalesh Rohatgi
Dr. D. Y. Patil Medical College, Hospital and Research Center, Dr. D. Y. Patil Vidyapeeth, Pune, Maharashtra
Source of Support: None, Conflict of Interest: None
We are reporting a case of 28-year-old young lady who presented with sensory stroke. Magnetic resonance imaging (MRI) of brain showed bilateral watershed infarcts in middle and anterior cerebral territories. Brain angiography showed stenosis of supraclinoid portion of both internal carotid arteries (ICAs) without any moyamoya vessels (collaterals). Contrast MRI of brain showed enhancement of vessel wall in supraclinoid portion of both ICAs suggestive of inflammation. She was a known case of hyperthyroidism on irregular treatment. Work up for vasculitis was negative. Temporal artery biopsy was normal. She was diagnosed as a case of moyamoya syndrome associated with Graves' disease. She showed good response to immunosuppressants, aspirin, and treatment of hyperthyroidism. She had no ischemic events for 6 months but was later lost to follow-up. After 1 year she again presented with two episodes of transient ischemic attacks. Repeat imaging showed reduction in the wall thickening and enhancement and no new infarcts.
Keywords: Internal carotid arteries, moyamoya syndrome, thyroid disorders
|How to cite this article:|
Rohatgi S, Rangankar V, Rao P, Nirhale S, Naphade P. Moyamoya syndrome associated with graves' disease: A case report. Med J DY Patil Vidyapeeth 2022;15:764-6
|How to cite this URL:|
Rohatgi S, Rangankar V, Rao P, Nirhale S, Naphade P. Moyamoya syndrome associated with graves' disease: A case report. Med J DY Patil Vidyapeeth [serial online] 2022 [cited 2022 Nov 26];15:764-6. Available from: https://www.mjdrdypv.org/text.asp?2022/15/5/764/357778
| Introduction|| |
Moyamoya disease and moyamoya syndrome (also known as quasi moyamoya disease) are two different but closely related entities. Moyamoya disease is usually idiopathic and in 10% of cases, it may be familial. Moyamoya syndrome occurs secondary to systemic diseases such as atherosclerosis or autoimmune diseases especially Graves' disease.
The radiological features of moyamoya disease are concentric luminal narrowing around circle of Willis, none or only minimal enhancement of vessel wall along with collateral sprouting of vessels. In moyamoya syndrome, usually there is stenosis of supraclinoid portion of internal carotid arteries (ICAs), no or very few sprouting of collaterals and vessel wall enhancement may be seen when it is associated with Grave's disease.
| Case Report|| |
A 28-year-old lady presented with acute onset of numbness and paraesthsia on the right side of the body including the face since 1 month. She was a known case of hyperthyroidism on irregular treatment.
There was no history of hypertension, diabetes mellitus, constitutional symptoms, headache, transient neurological deficit, redness of eyes, mucosal ulcers, skin rashes, joint pains, or jaw claudication. Clinical examination revealed that she was normotensive. Peripheral pulses including temporal artery pulsations were well felt and there was no tenderness on temporal arteries. She had no exophthalmos, resting tachycardia, or finger tremors. There was no skin rash or mucosal ulcers. Neurological and opthalmological examination were normal. Hemoglobin was 12 gm/dl, erythrocyte sedimentation rate was 48 mm fall. T3/T4/TSH were 1.89/14.06/<0.01, respectively. Anti-thyroid peroxidase (TPO) antibodies were 100 IU/ml (positive).
Thyrotropin receptor antibodies, antinuclear antibodies, antinuclear cytoclastic antibodies, CSF varicella-zoster IgM, and cryglobulins were all negative. CSF proteins were marginally raised (71.80 mg/dl) with normal glucose and no cells. Urine analysis was normal. Temporal artery doppler and biopsy was normal.
MRI brain showed acute watershed infarct between middle cerebral and anterior cerebral arterial territories bilaterally [Figure 1] which raised suspicion of bilateral ICA stenosis. MR angiography of brain revealed lack of flow-related enhancement in the clinoid and supraclinoid segments of bilateral ICAs [Figure 2]a Pre and post-contrast enhanced T1-weighted fat-saturated dark blood images (b–d) showed diffuse concentric vessel wall thickening and reduced caliber in bilateral clinoid and supraclinoid ICAs (left > right) with post-contrast enhancement [Figure 2]b, [Figure 2]c, [Figure 2]d which was subsequently confirmed on digital subtraction angiography [Figure 3]. However, there was no evidence of collateral vessel sprouting (moyamoya vessels). T1 contrast MRI showed enhancement of vessel wall of bilateral ICAs in supra-clinoid portion suggestive of inflammation [Figure 3]. Ultrasound and biopsy of the temporal artery was normal.
|Figure 1: Diffusion weighted and ADC images of MRI brain showing acute infarcts in the watershed areas of both middle cerebral and anterior cerebral arteries (arrows)|
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|Figure 2: (a–b) 3-D time of flight MR angiography (a) reveals lack of flow related enhancement in the clinoid and supraclinoid segments of bilateral ICAs. Pre- and post-contrast enhanced T1-weighted fat-saturated dark blood images (b–d) showing diffuse concentric vessel wall thickening and reduced caliber in bilateral clinoid and supraclinoid ICAs (left >right) with post contrast enhancement (white arrows)|
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|Figure 3: Digital substraction angiography shows high grade stenosis of supraclinoid portion of both ICAs, left >right (arrows). There is no sprouting of collaterals seen|
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In view of imaging findings and raised CSF proteins possibility of angitis of ICAs was kept and patient was started on steroids and cyclophosphamide along with aspirin and dose of carbimazole was increased. Patient did not have fresh episode of any ischemic event for 6 months and then was lost to follow-up. She again presented with repeated transient ischemic attacks after 1 year and repeat imaging showed reduction in contrast enhancement of vessel wall and increase in the lumen of distal ICAs compared to the previous study.
| Discussion|| |
Stenosis of terminal portion of ICAs along with the sprouting of collaterals (moyamoya vessels) is seen in moyamoya disease which is primarily idiopathic or genetic. Similar radiological findings can occur in other diseases like atherosclerosis or autoimmune diseases called moya moya syndrome which is usually not associated with sprouting of collaterals.
Involvement of ICAs can occur in Takayasu disease and Giant cell arteritis also.
There are few case reports of moyamoya syndrome association with Graves disease.,,,,
The exact cause of the relationship between moyamoya syndrome and thyroid autoimmune disease is unknown, possibly some immune-mediated inflammation of vessels is hypothesized. Isolated involvement of intracranial vessels without moyamoya vessels is known., Till 2014, only 30 cases of coexisting Graves' disease and moyamoya syndrome have been described.
Our patient was a young lady who was a known case of hyperthyroidism, had elevated thyroid hormones (T3, T4) and anti-TPO antibodies were positive. She had isolated involvement of clinoid and supraclinoid portions of both ICAs without any moyamoya vessels.
She showed good response to immunosuppression and antithyroid treatment.
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Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2], [Figure 3]