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Dementia & Neuropsychologia

Print version ISSN 1980-5764

Dement. neuropsychol. vol.7 no.1 São Paulo Jan./Mar. 2013 


Hippocampal sclerosis dementia: An amnesic variant of frontotemporal degeneration

Demência com esclerose hipocampal: variante amnéstica da degeneração frontotemporal

Chiadi U. Onyike1 

Olga Pletnikova2 

Kelly L. Sloane1 

Campbell Sullivan3 

Juan C. Troncoso4 

Peter V. Rabins1 

1Division of Geriatric Psychiatry and Neuropsychiatry, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, USA.

2Division of Neuropathology, Department of Pathology, Johns Hopkins School of Medicine, Baltimore, USA.

3Division of Medical Psychology, Department of Psychiatry and Behavioral Sciences, Johns Hopkins School of Medicine, Baltimore, USA.

4Division of Neuropathology, Department of Pathology, Johns Hopkins School of Medicine, Baltimore, USA. Department of Neurology, Johns Hopkins School of Medicine, Baltimore, USA.



To describe characteristics of hippocampal sclerosis dementia.


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.


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 amnesia plus abnormal 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%).


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.

Key words: dementia classification; hippocampal sclerosis; frontotemporal dementia; neuropsychiatry



descrever as características da demência com esclerose hipocampal.


Uma amostra de conveniência de HSD foi recrutada no Johns Hopkins University Brain Resource Center. Vinte e quatro casos com diagnóstico patológico pós-morte de demência com esclerose hipocampal foram revisados para caracterização clínica, utilizando variáveis obtidas de prontuários médicos.


A maioria dos pacientes (79,2%) tinha amnésia no início, e a maioria (54,2%) apresentava comportamento anormal e transtorno psiquiátrico. Aproximadamente 42% apresentavam um estado amnésico e 37,5% amnésia mais conduta anormal e transtorno psiquiátrico. Todos acabaram por desenvolver um distúrbio comportamental ou psiquiátrico. Desorientação, disfunção executiva, afasia, agnosia e apraxia foram incomuns no início. A doença de Alzheimer (AD) foi o diagnóstico clínico inicial em 89% e o diagnóstico clínico final em 75%. A maior parte dos casos mostrou atrofia e perda neuronal localizada no hipocampo, amígadale cortex entorrinal.


HSD apresenta características patológicas de DFT e as características clínicas que mimetizam AD. Os resultados, colocadas no contexto do trabalho anterior, suporta a ideia de que HSD pertence à família FTD, em que pode ser identificada como uma variante amnésica.

Palavras-chave: classificação demência; esclerose hipocampal; demência frontotemporal; neuropsiquiatria

Texto completo disponível apenas em PDF.

Full text available only in PDF format.


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Received: November 25, 2012; Accepted: February 02, 2013

Chiadi U. Onyike. Division of Geriatric Psychiatry and Neuropsychiatry Johns Hopkins University - 600 N. Wolfe Street, Meyer 279 Baltimore, MD 21287 - USA. E-mail:

Disclosure: The authors report no conflicts of interest.

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