Moyamoya Syndrome as a Rare Complication of Graves' Disease

Abstract

Background: Moyamoya syndrome (MMS) is an occlusive, non-inflammatory, progressive cerebrovascular entity characterized by stenosis of the terminal internal carotid artery and the middle and anterior cerebral arteries. Its early detection is essential to avoid disability. Although its origin is uncertain, it has been associated with autoimmune diseases such as systemic lupus erythematosus, polyarteritis nodosa and, less frequently, Graves' disease (GD), an autoimmune thyroiditis and the most common cause of hyperthyroidism.

Purpose: To present a case of MDS associated with GD in the General Hospital of Medellin, Colombia.

Case presentation: 36-year-old woman with two weeks of myalgias, generalized weakness, right hemiparesis, urinary and fecal incontinence, and weight loss. Cerebral angio-resonance and panangiography evidenced the characteristic “volcano smoke” pattern, confirming SMM. Simultaneously, GD was diagnosed by anti-thyroid stimulating hormone receptor antibodies. He received treatment with methimazole, physical rehabilitation and neurological follow-up, with partial improvement.

Discussion and conclusion: This entity is uncommon, and its diagnosis requires characteristic findings in neuroimaging studies. In young patients, the identification of the angiographic pattern should motivate the search for possible underlying pathologies. Autoimmune diseases are more frequently associated, and to a lesser extent, thyroid pathology. The presence of hyperthyroidism with focal neurological manifestations should raise suspicion of SMM. SMM is infrequent in the national context, due to its low prevalence, low clinical suspicion and limited updated literature. There are still gaps in knowledge about its prognosis and treatment, which hinders an adequate and timely clinical approach.