Introduction: Moyamoya disease is a rare angiopathy characterized by a progressive stenosis of the intracranial internal carotid arteries (ICA). First described in 1957, its pathophysiological mechanisms are still not well understood. Its association with various systemic diseases is termed moyamoya syndrome
Observation: A 21-year-old female patient, with a family history of hypothyroidism, was admitted initially with stroke. The clinical examination revealed hemiparesis of the right hemisphere and dysarthria, in addition to a moderate goiter and tachycardia. Cerebral CT scan with contrast showed bilateral stenosis of the intracranial ICA with 78% in the left and 30% in the right ICA, confirming the diagnosis of moyamoya angiopathy. Biological analysis on admission and prior to the imaging procedure revealed suppressed TSH level and 10-fold increase in serum FT4. The diagnosis of Graves disease was made upon the presence of anti-TSH receptor antibodies: 5 IU/l (< 2 IU/l) and high thyroidal technetium-99 m pertechnetate uptake. Thyroid peroxidase antibodies were also positive and 32-fold elevated (1600 IU/ml). The patient received 30 mg qd of thiamazole in addition to rehabilitative management with improvement of its poststroke hemiparesis.
Discussion: Moyamoya syndrome represents one-third of moyamoya angiopathy and is associated with well-recognized conditions like neurofibromatosis type 1, Down syndrome, sickle cell disease but also autoimmune diseases. Autoimmune thyroiditis are increasingly reported in the literature in patients with moyamoya angiopathy suggesting a possible role of the immune process in the pathogenesis of this disease.
21 May 2022 - 24 May 2022