Moyamoya disease is a progressive chronic neurological disease due to multiple narrowing of the carotid arteries. It can be seen in association with atherosclerosis, vasculitis, haematological conditions, connective tissue disease, neurocutaneous syndromes and certain autoimmune diseases. Very few cases have been reported in association with Graves disease and the majority being in the East Asian population. We present a rare case of this association in a young Caucasian lady.
A 25 years old lady admitted under the stroke team with expressive dysphasia and right sided facial weakness, 3 weeks prior to that she had low grade fever, weight loss and she went down two dress sizes. There were no other dysthyroid symptoms. She smoked, had used illicit drugs in the past and had a history of depression.
On examination, she had expressive dysphasia, drooping of the right corner of the mouth and deviation of the tongue to the right. Her right plantar was upgoing and had no sensory neglect or inattention.
CT head confirmed a Right MCA infarct. She was investigated for causes of stroke −negative vasculitic and autoimmune screen, normal Echocardiogram, glucose and lipid profile. However, she was found to have a raised fT4 of 52.4 and undetectable TSH level. On thyroid examination, she had no signs of thyroid eye disease, had a diffuse palpable goitre with a regular pulse.
She then had an MRI brain which showed an acute infarct in the left putamen with few surrounding lesions of infarct within the left MCA territory. There was marked narrowing of the supraclinoid internal carotid arteries bilaterally characteristic of Moyamoya disease.
She did well on titration of Carbimazole and was seen in endocrine clinic with no residual neurological deficit. It is important to maintain euthyroid status as the return of elevated thyroid levels has been reported with worse neurological outcomes.