Case Presentation: A 24-year-old Hispanic female with a past medical history of diabetes mellitus and hyperthyroidism presented with slurred speech for one day that was associated with left-sided upper and lower extremity weakness. She did not use tobacco, alcohol, or illicit drugs. She had sinus tachycardia at 170 beats per minute and was febrile with a temperature of 101º F. Physical examination revealed a drowsy female who opened her eyes spontaneously and exhibited a right gaze preference. She had marked weakness in her left upper and lower extremities. Laboratory results were significant for elevated white blood cell count of 18 k/uL. Thyroxine and free T3 were elevated at at 3.8 ng/dL 10.8 pg/mL respectively while TSH was suppressed at less than 0.01 uIU/mL. Cerebral imaging revealed bilateral stenosis of multiple intracranial arteries including internal carotid arteries, anterior divisions of the middle cerebral arteries (M2, M3, M4 branches), and left anterior cerebral arteries (A1, pericallosal and callosal marginal branches), with watershed and embolic cerebral infarction. She was admitted to the medical intensive care unit with diagnoses of acute stroke and thyroid storm. She was treated with propranolol, glucocorticoids, and propylthiouracil. Thrombolytic drugs and intravascular interventions were withheld as she presented outside the therapeutic window. Unfortunately, the patient exhibited progressive intracranial swelling leading to herniation of the brain and brain death. Care was withdrawn as per family’s wishes and she expired soon after.

Discussion: Moyamoya disease (MMD)is a rare, poorly understood disease, which can lead to an increased incidence of stroke. Incidence of MMD varies among different ethnic groups, with the highest incidence in Asians. MMD is associated with risk factors such as atherosclerosis, infections, hematologic conditions, autoimmune diseases, and endocrine abnormalities such as diabetes. Studies have shown an increasing association between MMD and hyperthyroidism, with a higher incidence of ischemic stroke in this group. Anti-thyroperoxidase antibody, anti-thyroglobulin antibody and anti-thyrotropin receptor antibody have been found to be elevated in patients presenting with acute stroke and features of MMD on imaging. The presence of thyroid antibodies and hyperthyroidism, both of which were present in our patient, may play an important role in the development of adult MMD

Conclusions: MMD is a rare cerebrovascular disease which can present with transient ischemic attack, ischemic or hemorrhagic stroke, or epilepsy. MMD is associated with risk factors such as atherosclerosis, infections, hematologic conditions, and endocrine abnormalities such as diabetes and thyroid disorders such as Grave’s disease. Recent studies show an association between MMD and hyperthyroidism.

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IMAGE 2: FATAL ISCHEMIC STROKE AND THYROID STORM IN A YOUNG PATIENT WITH MOYAMOYA DISEASE.