Dysexecutive mild cognitive impairment associated to frontal atrophy: Case report

Balthazar, Marcio Luiz Figueredo; Damasceno, Benito Pereira

doi:10.1590/S1980-57642009DN20100015

Abstract

Non-amnestic mild cognitive impairment (MCI) evolving to neurodegenerative diseases other than Alzheimer's disease (AD) is rarely well documented. We report a case of a 49 year-old woman who presented a slowly progressive attentional/dysexecutive syndrome sparing other cognitive domains and without significant impairment of daily life activities. Her mother had Parkinsonism and her brother, a psychotic syndrome. Brain CT/MRI showed frontal atrophy while brain SPECT showed moderate cortical hypoperfusion, mainly in the frontal lobes. Our case is an example of non-memory MCI whose neuropsychological data and brain imaging indicating high likelihood of progression to a non-AD dementia.

Key words:
mild cognitive impairment; executive function; Alzheimer's disease; frontotemporal dementia

Resumo

Comprometimento cognitivo leve não-amnéstico evoluindo para uma doença neurodegenerativa outra que não doença de Alzheimer é raramente documentado. Nós descrevemos o caso de uma mulher de 49 anos de idade que apresenta uma síndrome atencional/disexecutiva lentamente progressiva, sem acometimento de outros domínios cognitivos ou comprometimento significativo de atividades de vida diária, com antecedente familiar de parkinsonismo (sua mãe) e psicose (seu irmão). Tomografia e ressonância magnética cerebral mostram atrofia frontal e o SPECT, hipoperfusão cortical moderada, principalmente em lobos frontais. Nosso caso é um exemplo de comprometimento cognitivo leve cujo perfil neuropsicológico e de neuroimagem sugere que pode haver probabilidade maior de evoluir para uma doença neurodegenerativa não-DA.

Palavras-chave:
comprometimento cognitivo leve; função executiva; doença de Alzheimer; demência frontotemporal

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References

¹
Winblad B, Palmer K, Kivipelto M, et al. Mild cognitive impairment-beyond controversies, towards a consensus: report of the International Working Group on Mild Cognitive Impairment. J Intern Med 2004; 256:240-246.
²
Portet F, Ousset PJ, Visser PJ et al. Mild cognitive impairment (MCI) in medical practice: a critical review of the concept and new diagnostic procedure. Report of the MCI Working Group of the European Consortium on Alzheimer's disease. J Neurol Neurosurg Psychiatry 2006; 77: 714-718.
³
Petersen RC. Mild cognitive impairment as a diagnostic entity. J Intern Med 2004; 256: 183-194.
⁴
Petersen RC, Doody R, Kurz A, Mohs RC, Morris JC, Rabins PV et al. Current concepts in Mild Cognitive Impairment. Arch Neurol 2001; 58: 1985-1992.
⁵
Brucki SMD, Nitrini R, Caramelli P, Bertolucci PHF, Okamoto IH. Sugestões para o uso do mini-exame do estado mental no Brasil. Arq Neuropsiquiatr 2003; 61(3-B):777-781.
⁶
Christensen A-L. Luria's Neuropsychological Investigation, (2nd ed.) Copenhagen: Munksgaard, 1979.
⁷
Lezak MD. Neuropsychological Assessment, (3rd ed.). New York: Oxford University Press, 1995.
⁸
Dubois B, Slachevsky A, Litvan I, Pillon B. The FAB: A Frontal Assessment Battery at bedside. Neurology 2000; 55: 1621-1626.
⁹
Beato RG, Nitrini R, Formigoni AP, Caramelli P. Brazilian version of the Frontal Assessment Battery (FAB). Dement Neuropsychol 2007; 1: 59-65.
¹⁰
Wechsler D. Wechsler Memory Scale - Revised: Manual. USA: The Psychological Corporation, Hartcourt Brace Jovanovich Inc., 1987
¹¹
Beck AT, Ward CH, Mendelson M, Mock J, Erbaugh J. An inventory for measuring depression. Arch Gen Psychiatry 1961; 4: 561-571.
¹²
Organização Mundial da Saúde. Classificação Estatística Internacional de Doenças e Problemas Relacionados à Saúde. (CID-10). São Paulo: Universidade de São Paulo; 1997.
¹³
American Psychiatric Association. Diagnostic and statistical manual of mental disorders, 4th ed. (DSM-IV). Washington, DC: American Psychiatric Association; 1994.
¹⁴
McKann GM, Albert MS, Grossman M, Miller, B, Dickson D, Trojanowski JQ. Clinical and Pathological Diagnosis of Frontotemporal Dementia. Report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol 2001; 58: 1803-1809.
¹⁵
Busse A, Hensel A, Güne U, Angermeyer MC, Riedel-Heller SG. Mild cognitive impairment, long-term course of four clinical subtypes. Neurology 2006; 67: 2176-2185.
¹⁶
Robert M, Maturanath PS. Tau and tauopathies. Neurology India 2007; 1: 11-16.
¹⁷
Leverenz JB, Yu CE, Montine TJ et al. A novel progranulin mutation associated with variable clinical presentation and tau, TDP43 and alpha-synuclein pathology. Brain 2007; 130: 1360-1374.
¹⁸
Davion S, Johnson N, Weintraub S, et al. Clinicopathologic correlation in PGRN mutations. Neurology 2007; 69: 1113-1121.

Publication Dates

Publication in this collection
Jan-Mar 2008

History

Received
08 Jan 2008
Reviewed
02 Mar 2008
Accepted
16 Feb 2008

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

[1] ¹
Winblad B, Palmer K, Kivipelto M, et al. Mild cognitive impairment-beyond controversies, towards a consensus: report of the International Working Group on Mild Cognitive Impairment. J Intern Med 2004; 256:240-246.

[2] ²
Portet F, Ousset PJ, Visser PJ et al. Mild cognitive impairment (MCI) in medical practice: a critical review of the concept and new diagnostic procedure. Report of the MCI Working Group of the European Consortium on Alzheimer's disease. J Neurol Neurosurg Psychiatry 2006; 77: 714-718.

[3] ³
Petersen RC. Mild cognitive impairment as a diagnostic entity. J Intern Med 2004; 256: 183-194.

[4] ⁴
Petersen RC, Doody R, Kurz A, Mohs RC, Morris JC, Rabins PV et al. Current concepts in Mild Cognitive Impairment. Arch Neurol 2001; 58: 1985-1992.

[5] ⁵
Brucki SMD, Nitrini R, Caramelli P, Bertolucci PHF, Okamoto IH. Sugestões para o uso do mini-exame do estado mental no Brasil. Arq Neuropsiquiatr 2003; 61(3-B):777-781.

[6] ⁶
Christensen A-L. Luria's Neuropsychological Investigation, (2nd ed.) Copenhagen: Munksgaard, 1979.

[7] ⁷
Lezak MD. Neuropsychological Assessment, (3rd ed.). New York: Oxford University Press, 1995.

[8] ⁸
Dubois B, Slachevsky A, Litvan I, Pillon B. The FAB: A Frontal Assessment Battery at bedside. Neurology 2000; 55: 1621-1626.

[9] ⁹
Beato RG, Nitrini R, Formigoni AP, Caramelli P. Brazilian version of the Frontal Assessment Battery (FAB). Dement Neuropsychol 2007; 1: 59-65.

[10] ¹⁰
Wechsler D. Wechsler Memory Scale - Revised: Manual. USA: The Psychological Corporation, Hartcourt Brace Jovanovich Inc., 1987

[11] ¹¹
Beck AT, Ward CH, Mendelson M, Mock J, Erbaugh J. An inventory for measuring depression. Arch Gen Psychiatry 1961; 4: 561-571.

[12] ¹²
Organização Mundial da Saúde. Classificação Estatística Internacional de Doenças e Problemas Relacionados à Saúde. (CID-10). São Paulo: Universidade de São Paulo; 1997.

[13] ¹³
American Psychiatric Association. Diagnostic and statistical manual of mental disorders, 4th ed. (DSM-IV). Washington, DC: American Psychiatric Association; 1994.

[14] ¹⁴
McKann GM, Albert MS, Grossman M, Miller, B, Dickson D, Trojanowski JQ. Clinical and Pathological Diagnosis of Frontotemporal Dementia. Report of the Work Group on Frontotemporal Dementia and Pick's Disease. Arch Neurol 2001; 58: 1803-1809.

[15] ¹⁵
Busse A, Hensel A, Güne U, Angermeyer MC, Riedel-Heller SG. Mild cognitive impairment, long-term course of four clinical subtypes. Neurology 2006; 67: 2176-2185.

[16] ¹⁶
Robert M, Maturanath PS. Tau and tauopathies. Neurology India 2007; 1: 11-16.

[17] ¹⁷
Leverenz JB, Yu CE, Montine TJ et al. A novel progranulin mutation associated with variable clinical presentation and tau, TDP43 and alpha-synuclein pathology. Brain 2007; 130: 1360-1374.

[18] ¹⁸
Davion S, Johnson N, Weintraub S, et al. Clinicopathologic correlation in PGRN mutations. Neurology 2007; 69: 1113-1121.

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