Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population.
Arq Neuropsiquiatr
; 77(1): 10-18, 2019 01.
Article
em En
| MEDLINE
| ID: mdl-30758437
ABSTRACT
AIMS:
To investigate hereditary spastic paraplegia (HSP) in a pediatric Brazilian sample.METHODS:
Epidemiological, clinical, radiological and laboratory data were analyzed in 35 patients.RESULTS:
Simple HSP (HSP-S) was detected in 12 patients, and complicated HSP (HSP-C) was detected in 23 patients. The mean age of onset of symptoms was 2.9 years in HSP-S and 1.6 years in HSP-C (p = 0.023). The disease was more severe in HSP-C. There were no differences in sex, ethnic background, or family history between groups. Intellectual disability was the most frequent finding associated with HSP-C. Peripheral axonal neuropathy was found in three patients. In the HSP-C group, MRI was abnormal in 13 patients. The MRI abnormalities included nonspecific white matter lesions, cerebellar atrophy, thinning of the corpus callosum and the "ear of the lynx sign".CONCLUSIONS:
In children with spastic paraplegia, HSP must be considered whenever similar pathologies, mainly diplegic cerebral palsy, are ruled out.
Texto completo:
1
Coleções:
01-internacional
Base de dados:
MEDLINE
Assunto principal:
Paraplegia Espástica Hereditária
Tipo de estudo:
Observational_studies
Limite:
Adolescent
/
Child
/
Child, preschool
/
Female
/
Humans
/
Infant
/
Male
País/Região como assunto:
America do sul
/
Brasil
Idioma:
En
Revista:
Arq Neuropsiquiatr
Ano de publicação:
2019
Tipo de documento:
Article
País de afiliação:
Brasil