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Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population
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| Zeitschriftentitel: | Arquivos de Neuro-Psiquiatria |
|---|---|
| Personen und Körperschaften: | , |
| In: | Arquivos de Neuro-Psiquiatria, 77, 2019, 1, S. 10-18 |
| Format: | E-Article |
| Sprache: | Unbestimmt |
| veröffentlicht: |
FapUNIFESP (SciELO)
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| author_facet |
Ortega, Roberta Paiva Magalhães Rosemberg, Sérgio Ortega, Roberta Paiva Magalhães Rosemberg, Sérgio |
|---|---|
| author |
Ortega, Roberta Paiva Magalhães Rosemberg, Sérgio |
| spellingShingle |
Ortega, Roberta Paiva Magalhães Rosemberg, Sérgio Arquivos de Neuro-Psiquiatria Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population Neurology Neurology (clinical) |
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ortega, roberta paiva magalhães |
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Ortega, Roberta Paiva Magalhães Rosemberg, Sérgio 1678-4227 0004-282X FapUNIFESP (SciELO) Neurology Neurology (clinical) http://dx.doi.org/10.1590/0004-282x20180153 <jats:p>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.</jats:p> Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population Arquivos de Neuro-Psiquiatria |
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| title |
Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
| title_unstemmed |
Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
| title_full |
Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
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Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
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Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
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Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
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hereditary spastic paraplegia: a clinical and epidemiological study of a brazilian pediatric population |
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Neurology Neurology (clinical) |
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http://dx.doi.org/10.1590/0004-282x20180153 |
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2019 |
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10-18 |
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<jats:p>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.</jats:p> |
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| author | Ortega, Roberta Paiva Magalhães, Rosemberg, Sérgio |
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| description | <jats:p>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.</jats:p> |
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| spelling | Ortega, Roberta Paiva Magalhães Rosemberg, Sérgio 1678-4227 0004-282X FapUNIFESP (SciELO) Neurology Neurology (clinical) http://dx.doi.org/10.1590/0004-282x20180153 <jats:p>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.</jats:p> Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population Arquivos de Neuro-Psiquiatria |
| spellingShingle | Ortega, Roberta Paiva Magalhães, Rosemberg, Sérgio, Arquivos de Neuro-Psiquiatria, Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population, Neurology, Neurology (clinical) |
| title | Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
| title_full | Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
| title_fullStr | Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
| title_full_unstemmed | Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
| title_short | Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
| title_sort | hereditary spastic paraplegia: a clinical and epidemiological study of a brazilian pediatric population |
| title_unstemmed | Hereditary spastic paraplegia: a clinical and epidemiological study of a Brazilian pediatric population |
| topic | Neurology, Neurology (clinical) |
| url | http://dx.doi.org/10.1590/0004-282x20180153 |