Motor impairment in a rare form of spastic paraplegia (Spoan syndrome): A 10-year follow-up

Data de publicação
BMC Neurology
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Galvao C.R.C.
Cavalcante P.M.A.
Olinda R.
Graciani Z.
Zatz M.
Kok F.
Santos S.
Lancman S.
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© 2019 The Author(s).Background: Spastic paraplegia, optic atrophy and neuropathy (Spoan syndrome) is an autosomal recessive disease with approximately 70 cases recorded in Brazil and Egypt. Methods: This is a prospective longitudinal study performed with 47 patients affected with Spoan syndrome of seven communities of Rio Grande do Norte (Brazil) to investigate changes in motor function based on comparative data obtained from a 10-year follow-up. Results: The mean age of the participants was 47.21 ± 12.42 years old, and the mean age at loss of ambulation and hand function were 10.78 ± 5.55 and 33.58 ± 17.47 years old, respectively. Spearman's correlation analysis between the score on the Modified Barthel Index and the investigated variables evidenced statistical significance for age (p < 0.001) and right- A nd left-hand grip strength (p = 0.042 and p = 0.021, respectively). Statistical significance was not evidenced for the remainder of the variables, including age at onset of symptoms (p = 0.634), age at loss of ambulation (p = 0.664) and age at loss of hand function (p = 0.118). Conclusions: Our analysis allows asserting that the participants exhibited slight dependence until age 35. The greatest losses occurred from ages 35 to 41, and starting at 50, practically all patients become completely dependent. These findings are relevant for determining the prognosis as well as suitable treatment, rehabilitation and assistive technology for these individuals.
Assuntos Scopus
Adolescent , Adult , Age of Onset , Brazil , Child , Child, Preschool , Disease Progression , Female , Follow-Up Studies , Hereditary Sensory and Motor Neuropathy , Humans , Longitudinal Studies , Male , Middle Aged , Motor Disorders , Optic Atrophy , Paraplegia , Prospective Studies , Young Adult
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