Objective: To describe two patients with SREAT presenting with progressive cerebellar ataxia.

Background: SREAT or Hashimoto’s encephalopathy is an autoimmune encephalopathy typically presenting with rapidly progressive cognitive decline. Only few cases of ataxia have been reported, and sometimes misdiagnosed as spinocerebellar ataxia.

Methods: Case report and literature review.

Results: We report 21- and 40-year-old women with progressive cerebellar ataxia. The first patient with underlying disease of type 1 diabetes developed worsening walking difficulty over three months. Exam revealed moderate-to-severe truncal and appendicular ataxia without encephalopathy. Work-up for autoimmune ataxia revealed positive anti-thyroid peroxidase (anti-TPO; 1: 1600, normal < 1:100) but negative anti-thyroglobulin (anti-TG; 1:40, normal < 1:20) and anti-GAD antibodies. Thyroid function tests were unremarkable. She received a 3-day course of intravenous methylprednisolone (IVMP) with gradual improvement over a few weeks, followed by a plateau of mild residual ataxia. She had been on oral prednisone which was then tapered down very slowly without exacerbation of her ataxia. The second patient presented with progressive impairment of gait and fine motor coordination of her hands over two years. Her consciousness was normal. Extensive work-up was negative except positive anti-TPO (238.87 IU/ml from another laboratory, normal 0-5.61) and anti-TG (57.35 IU/ml, normal 0-4.11) antibodies. Her ataxia improved slightly after a 3-day course of IVMP, and became plateaued with residual mild-to-moderate ataxia. She continued on oral prednisone which was then tapered down after the initiation of azathioprine. Her ataxia was slightly worse during the bridging period. We review cases of SREAT presenting with pure cerebellar ataxia in the literature. Videos will be demonstrated. 

Conclusions: Identification of SREAT by anti-TPO and anti-TG testing should be considered as part of the work-up of progressive cerebellar ataxia. This disorder is treatable with immunotherapy, and therefore caution is not to misdiagnose this entity as degenerative ataxia.

References: 1. Tang Y, Chu C, Lin MT, Wei G, Zhang X, Da Y, Huang H, Jia J. Hashimoto’s encephalopathy mimicking spinocerebellar ataxia. J Neurol 2011, 258:1705-1707.

2. Nakagawa H, Yoneda M, Fujii A, Kinomoto K, Kuriyama M. Hashimoto encephalopathy presenting with progressive cerebellar ataxia. BMJ Case Rep 2009, 2009.

« Back to 2017 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/steroid-responsive-encephalopathy-with-associated-thyroiditis-sreat-presenting-with-pure-cerebellar-ataxia/