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Arquivos de Neuro-Psiquiatria

Print version ISSN 0004-282X

Abstract

GRACIANI, Zodja et al. Motor and functional evaluation of patients with spastic paraplegia, optic atrophy, and neuropathy (SPOAN). Arq. Neuro-Psiquiatr. [online]. 2010, vol.68, n.1, pp.03-06. ISSN 0004-282X.  http://dx.doi.org/10.1590/S0004-282X2010000100002.

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.

Keywords : hereditary spastic paraplegia; peripheral nervous system disorder; optic atrophy; scales; psychomotor performance.

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