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

Autor:	Cláudia R. C. Galvão, Priscilla M. A. Cavalcante, Ricardo Olinda, Zodja Graciani, Mayana Zatz, Fernando Kok, Silvana Santos, Selma Lancman
Jazyk:	angličtina
Rok vydání:	2019
Předmět:	Spastic paraplegia Optic atrophy Rare diseases Longitudinal survey Neurology. Diseases of the nervous system RC346-429
Zdroj:	BMC Neurology, Vol 19, Iss 1, Pp 1-7 (2019)
Druh dokumentu:	article
ISSN:	1471-2377
DOI:	10.1186/s12883-019-1465-5
Popis:	Abstract 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
Databáze:	Directory of Open Access Journals
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