title: Steroid-responsive encephalopathy subsequently associated with Alzheimer's disease pathology: a case series.
abstract: BACKGROUND
  Steroid-responsive encephalopathies can be considered vasculitic or non-vasculitic. Clinicopathological studies of non-vasculitic steroid-responsive encephalopathy are unusual, but can explain the range of diagnoses consistent with a steroid-responsive presentation in life.
  
  
  OBJECTIVE
  To extend the range of clinical features and pathological findings consistent with steroid-responsive encephalopathy. Design, methods, and patients: A clinicopathological case series of four patients (two women, ages 54-71 years) with steroid-responsive encephalopathy followed at this institution until the time of death.
  
  
  RESULTS
  Clinical features were suggestive of Creutzfeld-Jakob disease (CJD), dementia with Lewy bodies (DLB), and parkinsonism, but pathological examination revealed only Alzheimer's disease-related findings without evidence of Lewy bodies or prion disease in all cases. All patients demonstrated marked, sustained improvement following steroid treatment, based on clinical, magnetic resonance imaging, and/or electroencephalogram studies. Alzheimer's disease was not diagnosed in life due to the atypical clinical features, lack of hippocampal atrophy on brain imaging, and a dramatic symptomatic response to steroids.
  
  
  CONCLUSIONS
  Steroid-responsive encephalopathy is the clinical presentation of some patients with Alzheimer's disease-related pathology at autopsy, and can be consistent with the clinical diagnoses of parkinsonism, DLB, or CJD disease in life.
authors.affiliation: Department of Neurology, Johns Hopkins University, Baltimore, MD, USA.
authors.forename: Farrah J
authors.initials: FJ
authors.lastname: Mateen
authors.forename: Keith A
authors.initials: KA
authors.lastname: Josephs
authors.forename: Joseph E
authors.initials: JE
authors.lastname: Parisi
authors.forename: Daniel A
authors.initials: DA
authors.lastname: Drubach
authors.forename: Richard J
authors.initials: RJ
authors.lastname: Caselli
authors.forename: Kejal
authors.initials: K
authors.lastname: Kantarci
authors.forename: Clifford
authors.initials: C
authors.lastname: Jack
authors.forename: Bradley F
authors.initials: BF
authors.lastname: Boeve
mesh_terms: D000368:Aged; D000544:Alzheimer Disease / Q000150:complications* / Q000473:pathology* / Q000503:physiopathology; D001921:Brain / Q000473:pathology; D001927:Brain Diseases / Q000188:drug therapy* / Q000209:etiology* / Q000503:physiopathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging / Q000379:methods; D008297:Male; D008875:Middle Aged; D013256:Steroids / Q000627:therapeutic use*
chemical_list: D013256:Steroids