Impact of disease duration on functional status of patients with spinocerebellar ataxia type 2

Thiago R. Padilha Amarante; Sibele Y. M. Takeda; Hélio A. G. Teive; Marise Bueno Zonta

doi:10.1590/0004-282X20170146

Arquivos de Neuro-Psiquiatria, Table of Contents

CC BY-NC-ND 4.0 · Arq Neuropsiquiatr 2017; 75(11): 773-777
DOI: 10.1590/0004-282X20170146

Article

Impact of disease duration on functional status of patients with spinocerebellar ataxia type 2

Repercussões do tempo de evolução sobre o perfil funcional de pacientes com ataxia espinocerebelar do tipo 2

Thiago R. Padilha Amarante

¹Universidade Federal do Paraná, Programa de Residência Integrada Multiprofissional em Cuidados Hospitalares - Saúde para Adultos e Idosos, Curitiba PR, Brasil;

,

Sibele Y. M. Takeda

²Universidade Federal do Paraná, Departamento de Prevenção e Reabilitação de Fisioterapia, Curitiba PR, Brasil;

,

Hélio A. G. Teive

³Universidade Federal do Paraná, Hospital de Clínicas, Departamento de Medicina Interna, Curitiba PR, Brasil;

⁴Universidade Federal do Paraná, Unidade dos Distúrbios do Movimento, Curitiba PR, Brasil

,

Marise Bueno Zonta

¹Universidade Federal do Paraná, Programa de Residência Integrada Multiprofissional em Cuidados Hospitalares - Saúde para Adultos e Idosos, Curitiba PR, Brasil;

› Author Affiliations

Abstract

ABSTRACT

Objective: To correlate disease duration in spinocerebellar ataxia type 2 (SCA2) with disease severity, balance and functionality.

Method: Sixteen SCA2 patients were analyzed for: disease duration, disease severity (SARA score), balance (Berg balance scale score) and functionality (FIM and Lawton scores).

Results: Greater severity was correlated with worse functionality (Lawton: r = −0.0561, FIM: r = −0.6402) and balance (r = −0.7188). Longer disease duration was correlated with greater severity (p = 0.0002) and reduced functionality (FIM: p = 0.005; Lawton: p = 0.0402) and balance (p = 0.0036). A year increase in disease duration corresponded to a 0.8-point increase on the SARA scale, a 1.38-point decrease in FIM score, a 2.30-point decrease on the Berg balance scale and a 0.45-point decrease on the Lawton scale.

Conclusion: Longer disease duration in this series of SCA2 patients was correlated with greater disease severity, worse balance and greater functional dependency.

RESUMO

Objetivo: Correlacionar com o tempo de evolução da doença em pacientes com ataxia espinocerebelar do tipo 2 (SCA2) com a gravidade, equilíbrio e funcionalidade.

Método: Foram considerados dados de 16 indivíduos: tempo de evolução, nível de gravidade (SARA) e escores de equilíbrio (BERG) e funcionalidade (MIF e LAWTON).

Resultados: A maior gravidade esteve relacionada aos piores escores de funcionalidade (LAWTON: r = −0,0561, MIF: r = −0,6402) e de equilíbrio (r = −0,7188). O maior tempo de evolução esteve relacionado à maior gravidade (p = 0,0002) e menor funcionalidade (MIF: p = 0,005; Lawton (p = 0,0402) e equilíbrio (p = 0,0036) sendo que para o aumento de um ano no tempo de evolução, espera-se um aumento de 0,8 pontos no escore da SARA, e decréscimo de 1,38 pontos na MIF, 2,30, na Berg e 0,45 na Lawton.

Conclusão: O tempo de evolução dos sintomas está associado a maior gravidade da doença, pior equilíbrio e maior dependência funcional em pacientes comSCA2.

Keywords:

spinocerebellar ataxias - severity of illness index - postural balance

Palavras-chave:

ataxia espinocerebelar - índice de gravidade da doença - equilíbrio postural

Full Text

References

References
1 Teive HAG, Ashizawa T. Primary and secondary ataxias. Curr Opin Neurol. 2015;28(4):413-22. https://doi.org/10.1097/WCO.20170146201701460227
2 Sun YM, Lu C, Wu ZY. Spinocerebellar ataxia: relationship between phenotype and genotype: a review. Clin Genet. 2016;90(4):305-14. https://doi.org/10.1111/cge.12808
3 Magaña JJ, Velázquez-Pérez L, Cisneros B. Spinocerebellar ataxia type 2: clinical presentation, molecular mechanisms, and therapeutic perspectives. Mol Neurobiol. 2013;47(1):90-104. https://doi.org/10.1007/s12035-012-8348-8
4 Luo L, Wang J, Raymond YL, Figueroa KP Pulst SM, Kuo PH et al. The initial symptom and motor progression in spinocerebellar ataxias. Cerebellum. 2017;16(3):615-22. https://doi.org/10.1007/s12311-016-0836-3
5 Araujo MJL, Cardoso PL, Silva LC, Oliveira DA. A atuação da fisioterapia neurofuncional na doença de José-Machado: relato de caso. Neurobiologia. 2010;73(1):75-83.
6 Jacobi H, Montcel ST, Bauer P Giunti P, Cook A, Labrum R et al. Long-term disease progression in spinocerebellar ataxia types 1, 2, 3, and 6: a longitudinal cohort study. Lancet Neurol. 2015;14(11):1101-8. https://doi.org/10.1016/S1474-4422(15)00202-1
7 Lee YC, Liao YC, Wang PS, Lee IH, Lin KP, Soong BW. Comparison of cerebellar ataxias: a three-year prospective longitudinal assessment. Mov Disord. 2011;26(1 1):2081-7. https://doi.org/10.1002/mds.23809
8 Berg KO, Wood-Dauphinee SL, Williams JI, Maki BE. Measuring balance in the elderly: validation of an instrument. Can J Public Health. 1992;83 Suppl2:S7-1.
9 Assis CS, Batista LC, Wolosker N, Zerati AE, Gengo RC. Functional independence measure in patients with intermittent claudication. Rev Esc Enferm USP. 2015;49(5):756-61. http://dx.doi.org/10.1590/S0080-623420150000500007
10 Lawton MP, Brody EM. Assesment of older people: self-maintaining and instrumental activities of daily living. Gerontologist. 1969;9(3):179-86. https://doi.org/10.1093/gerontZ9.3_Part_1.179
11 FhonJR, Diniz MA, Leonardo KC, Kusumota L, Haas VJ, Partezani RA. Frailty syndrome related to disability in the elderly. Acta Paul Enferm. 2012;25(4):589-94. http://dx.doi.org/10.1590/S0103-21002012005000016
12 Riberto M, Miyazaki MH, Jucá SSH, Sakamoto H, Pinto PPN, Battistella LR. Validação da versão brasileira da Medida de Independência Funcional. Acta Fisiatr. 2004;11(2):72-6. http://dx.doi.org/10.5935/0104-7795.20040003
13 Miyamoto ST, Lombardi J, Berg KO, Ramos LR, Natour J. Brazilian version of the Berg balance scale. Braz J Med Biol Res 2004;37(9):1411-21. https://doi.org/10.1590/S0100-879X2004000900017
14 Santos RL, Junior JS. [Reliability of the Brazilian version of the Scale of Instrumental Activities of Daily Living[. RBPS. 2008;21(4):290-6. Portuguese. https://doi.org/10.5020/18061230.2008.p290
15 Braga Neto P Godeiro Junior C, Dutra LA, Pedroso JL, Barsottini OGP Translation and validation into Brazilian version of the Scale of the Assessment and Rating of Ataxia (SARA). Arq Neuropsiquiatr. 2010;68(2):228-30. https://doi.org/10.1590/S0004-282X2010000200014
16 Weyer A, Abele M, Schmitz-Hubsch T, Schoch B, Frings M, Timmann D et al. Reliability and validity of the scale for the assessment and rating of ataxia: a study in 64 ataxia patients. Mov Disord. 2007(22):1633-7. https://doi.org/10.1002/mds.21544
17 Velázquez-Pérez L, Rodríguez-Labrada R, García-Rodríguez JC, Almaguer-Mederos LE, Cruz-Marino T, Laffita-Mesa JM. A comprehensive review of Spinocerebellar ataxia type 2 in Cuba. Cerebellum. 2011;10(2):184-98. https://doi.org/10.1007/s12311-011-0265-2
18 Leonardi MM, Lopes GJ, Bezerra PP, Borges AP. [Impact of static and dynamic imbalance in the risk of falls in paients with spinocerebellar ataxia]. Rev Neurocienc. 2008:17(2):178-82. Portuguese.
19 Kembhavi G, Darrah J, Magill-Evans J, Loomis J. Using the berg balance scale to distinguish balance abilities in children with cerebral palsy. Pediatr Phys Ther. 2002;14(2):92-9. https://doi.org/10.1097/00001577-200214020-00005
20 Aizawa CY, Pedroso JL, Braga Neto P Callegari MR, Barsottini OG. Patients with autosomal dominant spinocerebellar ataxia have more risk of falls, important balance impairment, and decreased ability to function. Arq Neuropsiquiatr 2013;71(8):508-11. https://doi.org/10.1590/0004-282X20130094
21 Torriani C, Mota EPO, Gomes CS, Batista C, Costa MC, Vieira EM et al. Avaliação comparativa do equilíbrio dinâmico em diferentes pacientes neurológicos por meio do teste Get Up And Go. Rev Neurocienc. 2006;14(3):135-9.
22 Edwards DF, Hahn M, Baum C, Dromerick AW. The impact of mild stroke on meaningful activity and life satisfaction. J Stroke Cerebrovasc Dis. 2006;5(4):151-7. https://doi.org/10.1016/jjstrokecerebrovasdis.2006.04.001
23 León-Carrión J, Machuca-Murga F, Murg- Sierra M, Domínguez-Morales R. Eficacia de un programa de tratamiento intensivo, integral y multidisciplinar de pacientes con traumatismo craneoencefálico: valores médico legales. Rev Neurol. 2001;33(4),377-83.
24 Lana R, Alvares L, Nasciutti-Prudente C, Goulart F, Teixeira-Salmela L, Cardoso F. Percepção da qualidade de vida de indivíduos com doença de Parkinson através do PDQ-39. Rev Bras Fisioter. 2007;1 1(5):397-402. https://doi.org/10.1590/S1413-35552007000500011