title: Hashimoto's encephalopathy as a treatable adult-onset cerebellar ataxia mimicking spinocerebellar degeneration.
abstract: BACKGROUND
  Hashimoto's encephalopathy (HE) presents with a variety of neurologic and neuropsychiatric features. In this study, we investigated the clinical and immunological profiles of the cerebellar ataxic form of HE.
  
  
  METHODS
  The clinical features, treatments, laboratory features, brain imaging, and serum anti-NH(2)-terminal of α-enolase autoantibodies (anti-NAE Abs), a useful diagnostic marker for HE, were investigated in 13 patients who presented with sporadic adult-onset cerebellar ataxia and fulfilled the HE diagnostic criteria (antithyroid Abs and responsiveness to immunotherapy).
  
  
  RESULTS
  All of the patients presented with truncal ataxia, but nystagmus was uncommon (17%). Eight patients had an insidious onset that mimicked spinocerebellar degeneration (SCD), but brain imaging showed little or no cerebellar atrophy in all of the patients. Those patients with serum anti-NAE Abs (n = 8) did not have nystagmus and tended to respond better to immunotherapy than the anti-NAE Ab-negative patients.
  
  
  CONCLUSIONS
  The present study suggests that insidious adult-onset and truncal ataxia are common in the cerebellar ataxic form of HE, which mimics SCD, but that nystagmus and severe cerebellar atrophy are uncommon. Antithyroid and anti-NAE Abs may be useful for diagnosing cerebellar ataxic HE.
authors.affiliation: Second Department of Internal Medicine, Faculty of Medical Sciences, University of Fukui, Fukui, Japan.
authors.forename: Akiko
authors.initials: A
authors.lastname: Matsunaga
authors.forename: Masamichi
authors.initials: M
authors.lastname: Ikawa
authors.forename: Akihiro
authors.initials: A
authors.lastname: Fujii
authors.forename: Yasunari
authors.initials: Y
authors.lastname: Nakamoto
authors.forename: Masaru
authors.initials: M
authors.lastname: Kuriyama
authors.forename: Makoto
authors.initials: M
authors.lastname: Yoneda
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001323:Autoantibodies / Q000097:blood; D001927:Brain Diseases / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D002524:Cerebellar Ataxia / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D003937:Diagnosis, Differential; D004660:Encephalitis; D005260:Female; D050031:Hashimoto Disease / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D010751:Phosphopyruvate Hydratase / Q000276:immunology; D013132:Spinocerebellar Degenerations / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology
chemical_list: D001323:Autoantibodies; D010751:Phosphopyruvate Hydratase