Motor and functional evaluation of patients with spastic paraplegia, optic atrophy, and neuropathy (SPOAN)

Arquivos de Neuro-Psiquiatria

Zodja Graciani²  Silvana Santos¹  Lucia Inês Macedo-souza¹  Carlos Bandeira De Mello Monteiro¹  Maria Isabel Veras¹  Simone Amorim¹  Mayana Zatz¹  Fernando Kok¹ 

[1] ,University of São Paulo School of Medicine Department of Neurology São Paulo SP ,Brazil

关键词: hereditary spastic paraplegia;    peripheral nervous system disorder;    optic atrophy;    scales;    psychomotor performance;    paraplegia espástica hereditária;    doença do sistema nervoso periférico;    atrofia óptica;    escalas;    performance psicomotora;   

DOI  :  10.1590/S0004-282X2010000100002

来源: SciELO

【 摘 要 】

Spastic paraplegia, optic atrophy, and neuropathy (SPOAN) is an autosomal recessive complicated form of hereditary spastic paraplegia, which is clinically defined by congenital optic atrophy, infancy-onset progressive spastic paraplegia and peripheral neuropathy. In this study, which included 61 individuals (age 5-72 years, 42 females) affected by SPOAN, a comprehensive motor and functional evaluation was performed, using modified Barthel index, modified Ashworth scale, hand grip strength measured with a hydraulic dynamometer and two hereditary spastic paraplegia scales. Modified Barthel index, which evaluate several functional aspects, was more sensitive to disclose disease progression than the spastic paraplegia scales. Spasticity showed a bimodal distribution, with both grades 1 (minimum) and 4 (maximum). Hand grip strength showed a moderate inverse correlation with age. Combination of early onset spastic paraplegia and progressive polyneuropathy make SPOAN disability overwhelming.

【 授权许可】

CC BY   
 All the contents of this journal, except where otherwise noted, is licensed under a Creative Commons Attribution License