Background: Myoclonus and tremor are common movement disorder phenomenologies in steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT).Pure ataxia without encephalopathy Fan has rarely been reported.Case report: We report 21- and 40-year-old females who presented with subacute pure ataxia without encephalopathy.
After immunotherapies, both exhibited initial improvement of ataxia, and subsequently remained in plateau phase.Discussion: This treatable disorder should be added to the differential diagnoses of progressive cerebellar ataxia, and anti-thyroid peroxidase and anti-thyroglobulin T-Shirt should be considered as part of the workup.It is crucial not to misdiagnose SREAT presenting with pure cerebellar ataxia as degenerative or spinocerebellar ataxia.