AI Article Synopsis
- SPOAN is a hereditary condition characterized by congenital optic atrophy, early-onset spastic paraplegia, and peripheral neuropathy, leading to significant disability.
- A study involving 61 individuals demonstrated that the modified Barthel index is more effective than specific spastic paraplegia scales in assessing disease progression.
- The study found spasticity varied widely among participants, and hand grip strength decreased with age, highlighting the severity of the disabilities caused by SPOAN.
Article Abstract
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.
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| http://dx.doi.org/10.1590/s0004-282x2010000100002 | DOI Listing |
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