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: Farrah J Mateen, Keith A Josephs, Joseph E Parisi, Daniel A Drubach, Richard J Caselli, Kejal Kantarci, Clifford Jack Jr, Bradley F Boeve
• Journal: Neurocase (Neurocase) Vol. 18 Issue 1 Pg. 1-12 (Feb 2012) ISSN: 1465-3656 [Electronic] England
• PMID: 21714739 (Publication Type: Case Reports, Journal Article, Research Support, N.I.H., Extramural)

Chemical References

• Steroids

Topics

• Aged
• Alzheimer Disease (complications, pathology, physiopathology)
• Brain (pathology)
• Brain Diseases (drug therapy, etiology, physiopathology)
• Fatal Outcome
• Female
• Humans
• Magnetic Resonance Imaging (methods)
• Male
• Middle Aged
• Steroids (therapeutic use)