9177 "Double Trouble: Exploring Moya Moya Syndrome In The Context Of Graves' Disease - A Case Report"

Abstract Disclosure: B. Agrawal: None. V. mehta: None. R. Naseem: None. H. patel: None. Introduction: Moyamoya syndrome, characterized by progressive intimal hyperplasia in the terminal parts of the internal carotid artery and the circle of Willis, leads to stenosis followed by collateralization, resulting in a characteristic "smoky" appearance on angiography, known as "Moya-Moya." This rare vasculopathy has shown an increasing association with Graves' disease. Case Presentation: A 37-year-old female with a medical history of SLE, Graves' disease, Sjogren's syndrome, and hypertension presented with left facial droop and weakness lasting 24 hours. She had a similar episode four months prior, which resolved spontaneously. Initial CT scan revealed scattered areas of parenchymal attenuation in the periventricular white matter. Further imaging with CTA demonstrated steno-occlusive distal intracranial internal carotid artery and bilateral M1 segments, characteristic of Moyamoya syndrome. Concurrently, she had an enlarged multinodular goiter. Laboratory findings showed high t4 and low TSH due to non-compliance to medications. MRI confirmed a multifocal acute/subacute infarct in the right MCA territory. Vasculitis and hypercoagulable work up was negative. Neurosurgical intervention was deferred, and within 24 hours, the patient experienced complete resolution of symptoms with methimazole, aspirin and statin. Discussion Moyamoya syndrome, predominantly affecting Japanese and South Asian populations, often exhibits familial predisposition linked to the RNF213 gene. Typically affecting females with a mean age of 33, Moyamoya in Graves' disease presents with ischemic stroke or TIA during thyrotoxic states, characterized by suppressed TSH and elevated T4, which usually resolves with anti-thyroid therapy. Elevated thyroid autoantibodies are frequently observed in the cases reported so far. However, in contrast to strokes associated with Graves' disease, other autoimmune conditions such as T1DM and neurofibromatosis often manifest with haemorrhagic strokes. Angiography commonly reveals unilateral or asymmetrical vascular lesions, possibly linked to thyrotoxic states increasing cerebral metabolism and inducing multiple stenoses. Conclusion: In female patients presenting with transient ischemic attack (TIA) or ischemic stroke and diagnosed with Moyamoya syndrome on angiography, thyroid disorder screening is crucial. Elevated thyroid antibodies indicate a potential thyroid disorder, warranting initiation of anti-thyroid drugs alongside stroke management protocols to alleviate symptoms. This aims to improve patient outcomes by addressing both vascular and endocrine factors contributing to the pathology of Moyamoya syndrome in this specific demographic. Presentation: 6/1/2024