Document Detail

Steroid-responsive encephalopathy associated with autoimmune thyroiditis.
MedLine Citation:
PMID:  16476807     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), often termed Hashimoto encephalopathy, is a poorly understood and often misdiagnosed entity. OBJECTIVE: To characterize the clinical, laboratory, and radiologic findings in patients with SREAT to potentially improve recognition of this treatable entity. DESIGN: Retrospective analysis of clinical features and diagnostic test data. SETTING: Two affiliated tertiary care referral institutions. PATIENTS: Twenty consecutive (6 male) patients diagnosed as having SREAT from 1995 to 2003. MAIN OUTCOME MEASURES: Clinical features and ancillary test findings associated with SREAT. RESULTS: The median age at disease onset was 56 years (range, 27-84 years). The most frequent clinical features were tremor in 16 (80%), transient aphasia in 16 (80%), myoclonus in 13 (65%), gait ataxia in 13 (65%), seizures in 12 (60%), and sleep abnormalities in 11 (55%). All patients were assigned an alternative misdiagnosis at presentation, most commonly viral encephalitis (n = 5), Creutzfeldt-Jakob disease (n = 3), or a degenerative dementia (n = 4). The most frequent laboratory abnormalities were increased liver enzyme levels in 11, increased serum sensitive thyroid-stimulating hormone levels in 11, and increased erythrocyte sedimentation rate in 5. In only 5 patients (25%) did cerebrospinal fluid abnormalities suggest an inflammatory process. Magnetic resonance imaging abnormalities believed to be related to the encephalopathy were present in 5 patients (26%). CONCLUSIONS: The clinical, laboratory, and radiologic findings associated with SREAT are more varied than previously reported. Misdiagnosis at presentation is common. This treatable syndrome should be considered even if the serum sensitive thyroid-stimulating hormone level and erythrocyte sedimentation rate are normal, the cerebrospinal fluid profile does not suggest an inflammatory process, and neuroimaging results are normal. Until the pathophysiologic mechanism of this and other autoimmune encephalopathies is better characterized, we believe that descriptive terms that reflect an association rather than causation are most appropriate for this syndrome.
Pablo Castillo; Bryan Woodruff; Richard Caselli; Steven Vernino; Claudia Lucchinetti; Jerry Swanson; John Noseworthy; Allen Aksamit; Jonathan Carter; Joseph Sirven; Gene Hunder; Vahab Fatourechi; Bahram Mokri; Daniel Drubach; Sean Pittock; Vanda Lennon; Brad Boeve
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Publication Detail:
Type:  Journal Article; Research Support, N.I.H., Extramural; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Archives of neurology     Volume:  63     ISSN:  0003-9942     ISO Abbreviation:  Arch. Neurol.     Publication Date:  2006 Feb 
Date Detail:
Created Date:  2006-02-14     Completed Date:  2006-02-28     Revised Date:  2007-11-14    
Medline Journal Info:
Nlm Unique ID:  0372436     Medline TA:  Arch Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  197-202     Citation Subset:  AIM; IM    
Department of Neurology, Mayo Clinic College of Medicine, Rochester, Minn 55905, USA.
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MeSH Terms
Age of Onset
Aged, 80 and over
Brain Diseases / drug therapy*,  etiology*,  pathology
Hashimoto Disease / complications*
Liver / enzymology
Magnetic Resonance Imaging
Middle Aged
Retrospective Studies
Steroids / therapeutic use
Grant Support
Reg. No./Substance:
Comment In:
Arch Neurol. 2006 Feb;63(2):175-6   [PMID:  16476805 ]

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