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.
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: Neil J Scolding; Fady Joseph; Patricia A Kirby; Ingrid Mazanti; Françoise Gray; Jacqueline Mikol; David Ellison; David A Hilton; Timothy L Williams; James M MacKenzie; John H Xuereb; Seth Love Journal: Brain Date: 2005-01-19 Impact factor: 13.501
Authors: Shyam Prabhakaran; Matthew Bramlage; Mark A Edgar; Betty Diamond; John A Hardin; Bruce T Volpe Journal: J Rheumatol Date: 2005-09 Impact factor: 4.666
Authors: I G McKeith; D Galasko; K Kosaka; E K Perry; D W Dickson; L A Hansen; D P Salmon; J Lowe; S S Mirra; E J Byrne; G Lennox; N P Quinn; J A Edwardson; P G Ince; C Bergeron; A Burns; B L Miller; S Lovestone; D Collerton; E N Jansen; C Ballard; R A de Vos; G K Wilcock; K A Jellinger; R H Perry Journal: Neurology Date: 1996-11 Impact factor: 9.910
Authors: Yasha Kadkhodayan; Abdulrahman Alreshaid; Christopher J Moran; DeWitte T Cross; William J Powers; Colin P Derdeyn Journal: Radiology Date: 2004-10-21 Impact factor: 11.105
Authors: R J Caselli; B W Scheithauer; C A Bowles; M R Trenerry; F B Meyer; J S Smigielski; M Rodriguez Journal: Ann Neurol Date: 1991-07 Impact factor: 10.422
Authors: R J Caselli; B W Scheithauer; J D O'Duffy; G C Peterson; B F Westmoreland; P A Davenport Journal: Mayo Clin Proc Date: 1993-09 Impact factor: 7.616