Early and severe cognitive impairment in multiple sclerosis

Mendes, Maria Fernanda; Finkelsztejn, Alessandro; Gomes, Sidney; Fragoso, Yára Dadalti

doi:10.1590/S1980-57642012DN06010008

ABSTRACT

Objectives: To report on four new cases of severe cognitive impairment in the early stages of multiple sclerosis (MS) and to review data on the subject since few cases have been reported worldwide.

Methods: Retrospective evaluation of medical records of patients with severe cognitive impairment within the first five years of MS diagnosis. Results on neuropsychological tests and magnetic resonance imaging (MRI) were disclosed.

Results: Four patients from different Brazilian neurological departments in Brazil were evaluated, all presenting with severe cognitive dysfunction classified as rapidly developing dementia. MRI images showed severe brain atrophy and basal ganglia lesions in all patients.

Conclusions: Although rare, severe cognitive impairment in MS represents an important disability and may ultimately constitute another form of the disease.

Key words: multiple sclerosis; cognition; dementia; basal ganglia; brain atrophy

RESUMO

Objetivos: Relatar quatro novos casos de grave comprometimento cognitivo nas fases iniciais da esclerose múltipla (EM). Revisão da literatura no tema, uma vez que muito poucos casos foram descritos no mundo.

Métodos: Avaliação retrospectiva dos prontuários de pacientes com grave comprometimento cognitivo nos cinco primeiros anos de EM. Os resultados de testes neuropsicológicos e de imagens de ressonância magnética (RNM) são apresentados.

Resultados: Quatro pacientes de diferentes departamentos de neurologia brasileiros foram avaliados, todos apresentando disfunção cognitiva muito grave que pode ser classificada como demência de rápida evolução. As imagens na RNM mostraram grave atrofia cerebral e lesões em gânglios da base em todas as pacientes.

Conclusões: Embora muito rara, a alteração cognitiva grave na EM representa uma incapacidade importante e pode, em última análise, ser outra forma da doença.

Palavras-chave: esclerose múltipla; cognição; demência; gânglios da base; atrofia cerebral

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REFERENCES

Feinstein A. The neuropsychiatry of multiple sclerosis. Can J Psychiatry 2004;49:157-163.
Staff NP, Lucchinetti CF, Keegan BM. Multiple sclerosis with predominant, severe cognitive impairment. Arch Neurol 2009;66:1139-1143.
McDonald WI, Compston A, Edan G, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001;50:121-127.
Patti F, Amato MP, Trojano M et al. Cognitive impairment and its relation with disease measures in mildly disabled patients with relapsing-remitting multiple sclerosis: baseline results from the Cognitive Impairment in Multiple Sclerosis (COGIMUS) study. Mult Scler 2009;15:779-788.
Olazarán J, Cruz I, Benito-Léon J, Morales JM, Rivera-Navarro J. Cognitive dysfunction in multiple sclerosis: methods and prevalence from the GEDMA Study. Eur Neurol 2009;61:87-93.
Balsimelli S, Mendes MF, Bertolucci PHF, Tilbery CP. Attention impairment associated with relapsing-remitting multiple sclerosis patients with mild incapacity. Arq Neuropsiquiatr 2007;65:262-267.
Rabinowitz AR, Arnett PA. A longitudinal analysis of cognitive dysfunction, coping, and depression in multiple sclerosis. Neuropsychology 2009;23:581-591.
Figved N, Benedict R, Klevan G, et al. Relationship of cognitive impairment to psychiatric symptoms in multiple sclerosis. Mult Scler 2008;14:1084-1090.
Alajbegović A, Loga N, Tiro N, Alajbegović S, Cindro V, Hozo I. Cognitive and depressive disorders in multiple sclerosis. Acta Clin Croat 2009;48:3-8.
Prakash RS, Snook EM, Lewis JM, Moti RW, Kramer AF. Cognitive impairments in relapsing-remitting multiple sclerosis: a meta-analysis. Mult Scler 2008;14:1250-1261.
Tiemann L, Penner I, Haupts M, Schegel U, Calabrese P. Cognitive decline in multiple sclerosis: impact of topographic lesion distribution on differential cognitive deficit patterns. Mult Scler 2009;15:1164-1174.
Dineen RA, Vilisaar J, Hlinka J, Morgan PS, Constantinescu CS, Auer P. Disconnection as a mechanism for cognitive dysfunction in multiple sclerosis. Brain 2009;132:239-249.
Sánchez MP, Nieto A, Barroso J, Martin V, Hernandez MA. Brain atrophy as a marker of cognitive impairment in mildly disabling relapsing-remitting multiple sclerosis. Eur J Neurol 2008;15:1091-1099.
Calabrese M, Agosta F, Rinaldi F, et al. Cortical lesions and atrophy associated with cognitive impairment in relapsing-remitting multiple sclerosis. Arch Neurol 2009;66:1144-1150.
Valentino P, Cerasa A, Chiriaco C, et al. Cognitive deficits in multiple sclerosis patients with cerebellar symptoms. Mult Scler 2009;15:854-859.
Reisch SG, Grill SE. Corticobasal degeneration. Curr Treat Options Neurol 2009;11:179-185.
Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology 1983;33:1444-1452.

Publication Dates

Publication in this collection
Jan-Mar 2012

History

Received
23 Sept 2011
Accepted
02 Dec 2011

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

[1] Feinstein A. The neuropsychiatry of multiple sclerosis. Can J Psychiatry 2004;49:157-163.

[2] Staff NP, Lucchinetti CF, Keegan BM. Multiple sclerosis with predominant, severe cognitive impairment. Arch Neurol 2009;66:1139-1143.

[3] McDonald WI, Compston A, Edan G, et al. Recommended diagnostic criteria for multiple sclerosis: guidelines from the International Panel on the diagnosis of multiple sclerosis. Ann Neurol 2001;50:121-127.

[4] Patti F, Amato MP, Trojano M et al. Cognitive impairment and its relation with disease measures in mildly disabled patients with relapsing-remitting multiple sclerosis: baseline results from the Cognitive Impairment in Multiple Sclerosis (COGIMUS) study. Mult Scler 2009;15:779-788.

[5] Olazarán J, Cruz I, Benito-Léon J, Morales JM, Rivera-Navarro J. Cognitive dysfunction in multiple sclerosis: methods and prevalence from the GEDMA Study. Eur Neurol 2009;61:87-93.

[6] Balsimelli S, Mendes MF, Bertolucci PHF, Tilbery CP. Attention impairment associated with relapsing-remitting multiple sclerosis patients with mild incapacity. Arq Neuropsiquiatr 2007;65:262-267.

[7] Rabinowitz AR, Arnett PA. A longitudinal analysis of cognitive dysfunction, coping, and depression in multiple sclerosis. Neuropsychology 2009;23:581-591.

[8] Figved N, Benedict R, Klevan G, et al. Relationship of cognitive impairment to psychiatric symptoms in multiple sclerosis. Mult Scler 2008;14:1084-1090.

[9] Alajbegović A, Loga N, Tiro N, Alajbegović S, Cindro V, Hozo I. Cognitive and depressive disorders in multiple sclerosis. Acta Clin Croat 2009;48:3-8.

[10] Prakash RS, Snook EM, Lewis JM, Moti RW, Kramer AF. Cognitive impairments in relapsing-remitting multiple sclerosis: a meta-analysis. Mult Scler 2008;14:1250-1261.

[11] Tiemann L, Penner I, Haupts M, Schegel U, Calabrese P. Cognitive decline in multiple sclerosis: impact of topographic lesion distribution on differential cognitive deficit patterns. Mult Scler 2009;15:1164-1174.

[12] Dineen RA, Vilisaar J, Hlinka J, Morgan PS, Constantinescu CS, Auer P. Disconnection as a mechanism for cognitive dysfunction in multiple sclerosis. Brain 2009;132:239-249.

[13] Sánchez MP, Nieto A, Barroso J, Martin V, Hernandez MA. Brain atrophy as a marker of cognitive impairment in mildly disabling relapsing-remitting multiple sclerosis. Eur J Neurol 2008;15:1091-1099.

[14] Calabrese M, Agosta F, Rinaldi F, et al. Cortical lesions and atrophy associated with cognitive impairment in relapsing-remitting multiple sclerosis. Arch Neurol 2009;66:1144-1150.

[15] Valentino P, Cerasa A, Chiriaco C, et al. Cognitive deficits in multiple sclerosis patients with cerebellar symptoms. Mult Scler 2009;15:854-859.

[16] Reisch SG, Grill SE. Corticobasal degeneration. Curr Treat Options Neurol 2009;11:179-185.

[17] Kurtzke JF. Rating neurologic impairment in multiple sclerosis: an expanded disability status scale (EDSS). Neurology 1983;33:1444-1452.