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

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dc.contributor.authorGalvao C.R.C.
dc.contributor.authorCavalcante P.M.A.
dc.contributor.authorOlinda R.
dc.contributor.authorGraciani Z.
dc.contributor.authorZatz M.
dc.contributor.authorKok F.
dc.contributor.authorSantos S.
dc.contributor.authorLancman S.
dc.date.accessioned2024-03-12T23:54:52Z
dc.date.available2024-03-12T23:54:52Z
dc.date.issued2019
dc.description.abstract© 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.
dc.description.issuenumber1
dc.description.volume19
dc.identifier.doi10.1186/s12883-019-1465-5
dc.identifier.issn1471-2377
dc.identifier.urihttps://dspace.mackenzie.br/handle/10899/35359
dc.relation.ispartofBMC Neurology
dc.rightsAcesso Aberto
dc.subject.otherlanguageLongitudinal survey
dc.subject.otherlanguageOptic atrophy
dc.subject.otherlanguageRare diseases
dc.subject.otherlanguageSpastic paraplegia
dc.titleMotor impairment in a rare form of spastic paraplegia (Spoan syndrome): A 10-year follow-up
dc.typeArtigo
local.scopus.citations1
local.scopus.eid2-s2.0-85074126866
local.scopus.subjectAdolescent
local.scopus.subjectAdult
local.scopus.subjectAge of Onset
local.scopus.subjectBrazil
local.scopus.subjectChild
local.scopus.subjectChild, Preschool
local.scopus.subjectDisease Progression
local.scopus.subjectFemale
local.scopus.subjectFollow-Up Studies
local.scopus.subjectHereditary Sensory and Motor Neuropathy
local.scopus.subjectHumans
local.scopus.subjectLongitudinal Studies
local.scopus.subjectMale
local.scopus.subjectMiddle Aged
local.scopus.subjectMotor Disorders
local.scopus.subjectOptic Atrophy
local.scopus.subjectParaplegia
local.scopus.subjectProspective Studies
local.scopus.subjectYoung Adult
local.scopus.updated2024-05-01
local.scopus.urlhttps://www.scopus.com/inward/record.uri?partnerID=HzOxMe3b&scp=85074126866&origin=inward
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