Hippocampal sclerosis dementia: An amnesic variant of frontotemporal degeneration

Onyike, Chiadi U.; Pletnikova, Olga; Sloane, Kelly L.; Sullivan, Campbell; Troncoso, Juan C.; Rabins, Peter V.

doi:10.1590/S1980-57642013DN70100013

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 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%).

Conclusion:

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

RESUMO

Objetivo:

descrever as características da demência com esclerose hipocampal.

Métodos:

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.

Resultados:

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.

Conclusão:

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

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REFERENCES

Attems J, Jellinger KA. Hippocampal sclerosis in Alzheimer disease and other dementias. Neurology 2006;66:775.
Amador-Ortiz C, Ahmed Z, Zehr C, Dickson DW. Hippocampal sclerosis dementia differs from hippocampal sclerosis in frontal lobe degeneration. Acta Neuropathol 2006;113:245-252.
Josephs KA, Dickson DW. Hippocampal sclerosis in tau-negative frontotemporal lobar degeneration. Neurobiol Aging 2007;28:1718-1722.
Pao WC, Dickson DW, Crook JE, Finch NA, Rademakers R, Graff-Radford NR. Hippocampal Sclerosis in the Elderly: Genetic and Pathologic Findings, Some Mimicking Alzheimer Disease Clinically. Alzheimer Dis Assoc Disord 2011;25:364-368.
DeJesus-Hernandez M, Mackenzie IR, Boeve BF, et al. Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS. Neuron 2011;72: 245-256.
Renton AE, Majounie E, Waite A, et al. A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD. Neuron2011;72:257-268.
Murray ME, DeJesus-Hernandez M, Rutherford NJ, et al. Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72. Acta Neuropathol2011; 122:673-690.
Bigio EH, Weintraub S, Rademakers R, et al. Frontotemporal lobar degeneration with TDP-43 proteinopathy and chromosome 9p repeat expansion in C9ORF72: clinicopathologic correlation. Neuropathology 2012; doi: 10.1111/j.1440-1789.2012.01332.x.
Beach TG, Sue L, Scott S, Layne K, et al. Hippocampal sclerosis dementia with tauopathy. Brain Pathology 2006;13:263-278.
Cairns NJ, Bigio EH, Mackenzie IRA, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol2007;114:5-22.
Mackenzie I, Neumann M, Bigio EH, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol2009 2010;119:1-4.
Probst A, Taylor KI, Tolnay M. Hippocampal sclerosis dementia: a reappraisal. Acta Neuropathol2007;114:335-345.
Zarow C, Sitzer TE, Chui HC. Understanding hippocampal sclerosis in the elderly: epidemiology, characterization, and diagnostic issues. Curr Neurol Neurosci Rep 2008;8:363-370.
Dickson DW, Davies P, Bevona C, et al. Hippocampal sclerosis: a common pathological feature of dementia in very old (> or = 80 years of age) humans. Acta Neuropathol 1994;88:212-221.
Rasmusson DX, Brandt J, Steele C, Hedreen JC, Troncoso JC, Folstein M. Accuracy of Clinical Diagnosis of Alzheimer Disease and Clinical Features of Patients with Non-Alzheimer Disease. Neuropathology Alzheimer Dis Assoc Disord1996;10:180-186.
Barker WW, Luis CA, Kashuba A, et al. Relative frequencies of Alzheimer disease, Lewy body, vascular and frontotemporal dementia, and hippocampal sclerosis in the State of Florida Brain Bank. Alzheimer Dis Assoc Disord2002;16:203-212.
White L, Petrovitch H, Hardman J, et al. Cerebrovascular Pathology and Dementia in Autopsied Honolulu-Asia Aging Study Participants. Ann N Y Acad Sci 2002;977:9-23.
Leverenz JB, Agustin CM, Tsuang D, et al. Clinical and neuropathological characteristics of hippocampal sclerosis: a community-based study. Arch Neurol 2002;59:1099-1106.
Ala TA, Beh GO, William H Frey I. Pure hippocampal sclerosis: a rare cause of dementia mimicking Alzheimer's disease. Neurology2000;54: 843-848.
Jellinger K. Pure hippocampal sclerosis. a rare cause of dementia mimicking Alzheimer's disease. Neurology2000;55:739-740.
Blass DM, Hatanpaa K, Brandt J, et al. Dementia in hippocampal sclerosis resembles frontotemporal dementia more than Alzheimer disease. Neurology2004;63:492-497.
Hatanpaa K, Blass DM, Pletnikova O, Crain B, Bigio EH, Hedreen J, et al. Most cases of dementia with hippocampal sclerosis may represent frontotemporal dementia. Neurology2004 15;63:538-542.
McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan E. Clinical diagnosis of Alzheimer"s disease: report of the NINCDS-ADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer"s Disease. Neurology1984;34: 939-944.
Corey-Bloom J, Sabbagh MN, Bondi MW, et al. Hippocampal sclerosis contributes to dementia in the elderly. Neurology1997;48:154-160.
Constantinidis J, Richard J, Tissot R. Pick's disease. Histological and clinical correlations. Eur Neurol 1974;11:208-217.
Lippa CF, Dickson DW: Hippocampal sclerosis dementia: expanding the phenotypes of frontotemporal dementias? Neurology 2004;63: 414-415.
Beekly D, Ramos E, Lee W, et al. The National Alzheimer's Coordinating Center (NACC) database: the Uniform Data Set. Alzheimer Dis Assoc Disord2007;21:249-258.

Publication Dates

Publication in this collection
Jan-Mar 2013

History

Received
25 Nov 2012
Accepted
02 Feb 2013

This is an open-access article distributed under the terms of the Creative Commons Attribution License

[1] Attems J, Jellinger KA. Hippocampal sclerosis in Alzheimer disease and other dementias. Neurology 2006;66:775.

[2] Amador-Ortiz C, Ahmed Z, Zehr C, Dickson DW. Hippocampal sclerosis dementia differs from hippocampal sclerosis in frontal lobe degeneration. Acta Neuropathol 2006;113:245-252.

[3] Josephs KA, Dickson DW. Hippocampal sclerosis in tau-negative frontotemporal lobar degeneration. Neurobiol Aging 2007;28:1718-1722.

[4] Pao WC, Dickson DW, Crook JE, Finch NA, Rademakers R, Graff-Radford NR. Hippocampal Sclerosis in the Elderly: Genetic and Pathologic Findings, Some Mimicking Alzheimer Disease Clinically. Alzheimer Dis Assoc Disord 2011;25:364-368.

[5] DeJesus-Hernandez M, Mackenzie IR, Boeve BF, et al. Expanded GGGGCC Hexanucleotide Repeat in Noncoding Region of C9ORF72 Causes Chromosome 9p-Linked FTD and ALS. Neuron 2011;72: 245-256.

[6] Renton AE, Majounie E, Waite A, et al. A Hexanucleotide Repeat Expansion in C9ORF72 Is the Cause of Chromosome 9p21-Linked ALS-FTD. Neuron2011;72:257-268.

[7] Murray ME, DeJesus-Hernandez M, Rutherford NJ, et al. Clinical and neuropathologic heterogeneity of c9FTD/ALS associated with hexanucleotide repeat expansion in C9ORF72. Acta Neuropathol2011; 122:673-690.

[8] Bigio EH, Weintraub S, Rademakers R, et al. Frontotemporal lobar degeneration with TDP-43 proteinopathy and chromosome 9p repeat expansion in C9ORF72: clinicopathologic correlation. Neuropathology 2012; doi: 10.1111/j.1440-1789.2012.01332.x.

[9] Beach TG, Sue L, Scott S, Layne K, et al. Hippocampal sclerosis dementia with tauopathy. Brain Pathology 2006;13:263-278.

[10] Cairns NJ, Bigio EH, Mackenzie IRA, et al. Neuropathologic diagnostic and nosologic criteria for frontotemporal lobar degeneration: consensus of the Consortium for Frontotemporal Lobar Degeneration. Acta Neuropathol2007;114:5-22.

[11] Mackenzie I, Neumann M, Bigio EH, et al. Nomenclature and nosology for neuropathologic subtypes of frontotemporal lobar degeneration: an update. Acta Neuropathol2009 2010;119:1-4.

[12] Probst A, Taylor KI, Tolnay M. Hippocampal sclerosis dementia: a reappraisal. Acta Neuropathol2007;114:335-345.

[13] Zarow C, Sitzer TE, Chui HC. Understanding hippocampal sclerosis in the elderly: epidemiology, characterization, and diagnostic issues. Curr Neurol Neurosci Rep 2008;8:363-370.

[14] Dickson DW, Davies P, Bevona C, et al. Hippocampal sclerosis: a common pathological feature of dementia in very old (> or = 80 years of age) humans. Acta Neuropathol 1994;88:212-221.

[15] Rasmusson DX, Brandt J, Steele C, Hedreen JC, Troncoso JC, Folstein M. Accuracy of Clinical Diagnosis of Alzheimer Disease and Clinical Features of Patients with Non-Alzheimer Disease. Neuropathology Alzheimer Dis Assoc Disord1996;10:180-186.

[16] Barker WW, Luis CA, Kashuba A, et al. Relative frequencies of Alzheimer disease, Lewy body, vascular and frontotemporal dementia, and hippocampal sclerosis in the State of Florida Brain Bank. Alzheimer Dis Assoc Disord2002;16:203-212.

[17] White L, Petrovitch H, Hardman J, et al. Cerebrovascular Pathology and Dementia in Autopsied Honolulu-Asia Aging Study Participants. Ann N Y Acad Sci 2002;977:9-23.

[18] Leverenz JB, Agustin CM, Tsuang D, et al. Clinical and neuropathological characteristics of hippocampal sclerosis: a community-based study. Arch Neurol 2002;59:1099-1106.

[19] Ala TA, Beh GO, William H Frey I. Pure hippocampal sclerosis: a rare cause of dementia mimicking Alzheimer's disease. Neurology2000;54: 843-848.

[20] Jellinger K. Pure hippocampal sclerosis. a rare cause of dementia mimicking Alzheimer's disease. Neurology2000;55:739-740.

[21] Blass DM, Hatanpaa K, Brandt J, et al. Dementia in hippocampal sclerosis resembles frontotemporal dementia more than Alzheimer disease. Neurology2004;63:492-497.

[22] Hatanpaa K, Blass DM, Pletnikova O, Crain B, Bigio EH, Hedreen J, et al. Most cases of dementia with hippocampal sclerosis may represent frontotemporal dementia. Neurology2004 15;63:538-542.

[23] McKhann G, Drachman D, Folstein M, Katzman R, Price D, Stadlan E. Clinical diagnosis of Alzheimer"s disease: report of the NINCDS-ADRDA Work Group under the auspices of Department of Health and Human Services Task Force on Alzheimer"s Disease. Neurology1984;34: 939-944.

[24] Corey-Bloom J, Sabbagh MN, Bondi MW, et al. Hippocampal sclerosis contributes to dementia in the elderly. Neurology1997;48:154-160.

[25] Constantinidis J, Richard J, Tissot R. Pick's disease. Histological and clinical correlations. Eur Neurol 1974;11:208-217.

[26] Lippa CF, Dickson DW: Hippocampal sclerosis dementia: expanding the phenotypes of frontotemporal dementias? Neurology 2004;63: 414-415.

[27] Beekly D, Ramos E, Lee W, et al. The National Alzheimer's Coordinating Center (NACC) database: the Uniform Data Set. Alzheimer Dis Assoc Disord2007;21:249-258.

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