Objective: To characterize the clinical, laboratory and radiologic findings as well as prognosis of Hashimoto encephalopathy (HE) presenting with cerebellar ataxia

Background: HE is a rare condition presenting with a variety of neurologic and psychiatric features. Cerebellar ataxia has also been reported as one of the common manifestations of HE. However, the clinical characteristics of ataxic variant of HE has not been described.

Methods: We retrospectively reviewed clinical charts of 204 unexplained encephalopathic patients. Fourteen HE patients were identified by the proposed diagnostic criteria by Graus and colleagues in 2016.(1)

Results: Cerebellar ataxia was found in 7 HE patients (50%) in this cohort. The median age at onset was 46 years (range, 22-77 years). There was a preponderance of female patients (71%). Four patients (57%) had cerebellar ataxia as the first manifestation. The median onset of encephalopathy was 4.5 weeks (range, 2-10 weeks) after ataxia. Two patients (29%) had the onsets of cerebellar ataxia and encephalopathy at the same time. Encephalopathy preceded ataxia in only 1 patient (14%). Serum anti-thyroperoxidase (anti-TPO) and anti-thyroglobulin (anti-TG) antibodies were positive in 6 (86%) and 4 (57%), respectively. Two patients (29%) had positive results for both antibodies. All of patients were euthyroidism. Regarding cerebrospinal fluid (CSF), only 2 patients (29%) had elevated protein level and pleocytosis. Six patients had abnormal magnetic resonance imaging (MRI) in cerebellum and its connections. Increased signal on T2-weighted and fluid-attenuated inversion recovery (FLAIR) images in cerebellar white and gray matter were observed in 3 (43%) and 2 (29%), respectively. One patient (14%) had cerebellar atrophy. All patients received high-dose intravenous methylprednisolone for 3-5 days, then oral prednisolone which was tapered down very slowly. Median (range) of the Modified Rankin Scale (MRS) at presentation, discharge, and 3-month follow up were 4 (1-5), 2 (0-4), and 1 (0-2), respectively.

Conclusions: Cerebellar ataxia is a common presentation of HE which is a treatable condition. Cerebellar ataxia preceded the encephalopathic features in most of the case. Therefore, identification of HE by anti-TPO and anti-TG testing should be highly considered as a part of the cerebellar ataxia work up.

References: 1. Graus F, Titulaer MJ, Balu R, Benseler S, Bien CG, Cellucci T, et al. A clinical approach to diagnosis of autoimmune encephalitis. The Lancet Neurology. 2016;15(4):391-404.

« Back to 2018 International Congress

MDS Abstracts - https://www.mdsabstracts.org/abstract/cerebellar-ataxia-as-a-manifestation-of-hashimoto-encephalopathy/