title: Hippocampal sclerosis dementia: An amnesic variant of frontotemporal degeneration.
abstract: OBJECTIVE
  To describe characteristics of hippocampal sclerosis dementia.
  
  
  METHODS
  Convenience sample of Hippocampal sclerosis dementia (HSD) recruited from the Johns Hopkins University Brain Resource Center. Twenty-four cases with post-mortem pathological diagnosis of hippocampal sclerosis dementia were reviewed for clinical characterization.
  
  
  RESULTS
  The cases showed atrophy and neuronal loss localized to the hippocampus, amygdala and entorrhinal cortex. The majority (79.2%) had amnesia at illness onset, and many (54.2%) showed abnormal conduct and psychiatric disorder. Nearly 42% presented with an amnesic state, and 37.5% presented with amnesiaplusabnormal conduct and psychiatric disorder. All eventually developed a behavioral or psychiatric disorder. Disorientation, executive dysfunction, aphasia, agnosia and apraxia were uncommon at onset. Alzheimer disease (AD) was the initial clinical diagnosis in 89% and the final clinical diagnosis in 75%. Diagnosis of frontotemporal dementia (FTD) was uncommon (seen in 8%).
  
  
  CONCLUSIONS
  HSD shows pathological characteristics of FTD and clinical features that mimic AD and overlap with FTD. The findings, placed in the context of earlier work, support the proposition that HSD belongs to the FTD family, where it may be identified as an amnesic variant.
authors.affiliation: Division of Geriatric Psychiatry and Neuropsychiatry, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, USA.
authors.forename: Chiadi U
authors.initials: CU
authors.lastname: Onyike
authors.affiliation: Division of Neuropathology, Department of Pathology, Johns Hopkins School of Medicine, Baltimore, USA.
authors.forename: Olga
authors.initials: O
authors.lastname: Pletnikova
authors.affiliation: Division of Geriatric Psychiatry and Neuropsychiatry, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, USA.
authors.forename: Kelly L
authors.initials: KL
authors.lastname: Sloane
authors.affiliation: Division of Medical Psychology, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, USA.
authors.forename: Campbell
authors.initials: C
authors.lastname: Sullivan
authors.affiliation: Division of Neuropathology, Department of Pathology, Johns Hopkins School of Medicine, Baltimore, USA. Department of Neurology, Johns Hopkins School of Medicine, Baltimore, USA.
authors.forename: Juan C
authors.initials: JC
authors.lastname: Troncoso
authors.affiliation: Division of Geriatric Psychiatry and Neuropsychiatry, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, USA.
authors.forename: Peter V
authors.initials: PV
authors.lastname: Rabins
keywords: dementia classification; frontotemporal dementia; hippocampal sclerosis; neuropsychiatry