Steroid-Responsive Encephalopathy Associated With Autoimmune Thyroiditis

• DOI: 10.1001/archneur.63.2.197
• Publication Date: 2006-02-13T21:33:17Z
• Authors: 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
• Keywords: Autoimmune thyroiditis

Background: Steroid-responsive encephalopathy associated with autoimmune thyroiditis (SREAT), often termed Hashimoto encephalopathy, is a poorly understood and misdiagnosed entity.Objective: To characterize the clinical, laboratory, radiologic findings in patients SREAT to potentially improve recognition of this treatable entity.Design: Retrospective analysis clinical features diagnostic test data.Setting: Two affiliated tertiary care referral institutions.Patients: Twenty consecutive (6 male) diagnosed as having from 1995 2003.Main Outcome Measures: Clinical ancillary SREAT. Results:The median age at disease onset was 56 years (range, 27-84 years).The most frequent were tremor 16 (80%), transient aphasia myoclonus 13 (65%), gait ataxia seizures 12 (60%), sleep abnormalities 11 (55%).All assigned an alternative misdiagnosis presentation, commonly viral encephalitis (n=5), Creutzfeldt-Jakob (n=3), or degenerative dementia (n=4).The laboratory increased liver enzyme levels 11, serum sensitive thyroid-stimulating hormone erythrocyte sedimentation rate 5.In only 5 (25%) did cerebrospinal fluid suggest inflammatory process.Magnetic resonance imaging believed be related present (26%). Conclusions:The are more varied than previously reported.Misdiagnosis presentation common.This syndrome should considered even if level normal, profile does not process, neuroimaging results normal.Until pathophysiologic mechanism other encephalopathies better characterized, we believe that descriptive terms reflect association rather causation appropriate for syndrome.