title: Diagnostic and therapeutic aspects of Hashimoto's encephalopathy.
abstract: OBJECTIVE
  To share our experience on clinical presentation and management of patients diagnosed with Hashimoto's Encephalopathy (HE) at Vanderbilt Medical Center between 1999 and 2012.
  
  
  BACKGROUND
  HE is a rare disorder characterized by encephalopathy and central nervous system (CNS) dysfunction, elevated antithyroid antibodies, the absence of infection or structural abnormalities in the CNS, and a response to treatment with steroids. The relationship between thyroid antibodies and encephalopathy has remained unresolved.
  
  
  METHODS
  Retrospective chart review.
  
  
  RESULTS
  We identified 13 patients who met the criteria for the diagnosis of HE. The median age was 49 years (range, 2-66) and all except one were women. Encephalopathy in the form of altered mental status, stroke-like symptoms or seizures, with prompt resolution of symptoms upon receiving steroids, was the commonest presentation, seen in 7 patients. The second commonest presentation was subacute progressive decrease in cognitive function, which reversed within days to weeks after steroid therapy, seen in 4 patients. Electroencephalogram (EEG) was available in 12 patients and was abnormal in 8, showing nonspecific cerebral dysfunction in all 8 and epileptiform activity in 3. Treatment consisted of steroids in the acute phase for 12 of 13 patients with rapid improvement in symptoms. Maintenance therapy was rituximab in 7 patients, intravenous immunoglobulin (IVIg) in 7, azathioprine in 4, mycophenolate mofetil in 3, and methotrexate in 1 (some patients received sequential therapy with different agents). There was complete or near complete resolution of symptoms in 12 of the 13 patients.
  
  
  CONCLUSIONS
  We present a cohort of patients in whom CNS dysfunction was associated with elevated antithyroid antibodies and reversal of disease followed immunomodulatory therapies.
authors.affiliation: Vanderbilt University, Department of Neurology, Nashville, TN 37232, USA.
authors.forename: Inan
authors.initials: I
authors.lastname: Olmez
authors.forename: Harold
authors.initials: H
authors.lastname: Moses
authors.forename: Subramaniam
authors.initials: S
authors.lastname: Sriram
authors.forename: Howard
authors.initials: H
authors.lastname: Kirshner
authors.forename: Andre H
authors.initials: AH
authors.lastname: Lagrange
authors.forename: Siddharama
authors.initials: S
authors.lastname: Pawate
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies; D001379:Azathioprine / Q000627:therapeutic use; D001927:Brain Diseases / Q000175:diagnosis* / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D004569:Electroencephalography; D004660:Encephalitis; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D005260:Female; D050031:Hashimoto Disease / Q000175:diagnosis* / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use; D008297:Male; D008727:Methotrexate / Q000627:therapeutic use; D008875:Middle Aged; D012189:Retrospective Studies; D013256:Steroids / Q000627:therapeutic use
chemical_list: D001323:Autoantibodies; D004791:Enzyme Inhibitors; D016756:Immunoglobulins, Intravenous; D013256:Steroids; D001379:Azathioprine; D008727:Methotrexate
keywords: Altered mental status; Anti-thyroid antibodies; Mycophenolate mofetil; Nonvasculitic autoimmune meningoencephalitis (NAIM); Rituximab; Steroid responsive encephalopathy with antithyroid antibodies (SREAT)