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.

Keywords:

• Alzheimer's disease
• Corticosteroids
• Dementia
• Encephalopathy
• Hashimoto's encephalopathy
• Neuropathology

Acknowledgments

Conflicts of interest/financial disclosures: Drs Mateen, Drubach, Parisi, Lennon, Kentarci, Jack, Caselli, and Josephs have no conflicts of interest to report. Dr Boeve has received grant support from Myriad Pharmaceuticals and an honorarium from GE Healthcare. Dr Kantarci has received grant support from the National Institutes of Health (K23 AG030935 (NIH/NIA), R01 AG11378 (NIH/NIA) and P50 AG16574 (NIH/NIA)).