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

• Main Author: Graciani,Zodja
• Publication Date: 2010
• Other Authors: Santos,Silvana, Macedo-Souza,Lucia Inês, Monteiro,Carlos Bandeira de Mello, Veras,Maria Isabel, Amorim,Simone, Zatz,Mayana, Kok,Fernando
• Format: Article
• Language: eng
• Source: Arquivos de neuro-psiquiatria (Online)
• Download full: http://old.scielo.br/scielo.php?script=sci_arttext&pid=S0004-282X2010000100002
• topic: hereditary spastic paraplegia; peripheral nervous system disorder; optic atrophy; scales; psychomotor performance

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.