Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome
Caudal regression syndrome (CRS) is a malformation occurring during the fetal period and mainly characterized by an incomplete development of the spinal cord (SC), which is often accompanied by other developmental anomalies. We studied a 9-month old child with CRS who presented interruption of the S...
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MDPI AG
2017-01-01
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| Series: | International Journal of Molecular Sciences |
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| Online Access: | http://www.mdpi.com/1422-0067/18/1/230 |
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| author | Jesús Devesa Alba Alonso Natalia López José García Carlos I. Puell Tamara Pablos Pablo Devesa |
| author_facet | Jesús Devesa Alba Alonso Natalia López José García Carlos I. Puell Tamara Pablos Pablo Devesa |
| author_sort | Jesús Devesa |
| collection | DOAJ |
| description | Caudal regression syndrome (CRS) is a malformation occurring during the fetal period and mainly characterized by an incomplete development of the spinal cord (SC), which is often accompanied by other developmental anomalies. We studied a 9-month old child with CRS who presented interruption of the SC at the L2–L3 level, sacral agenesis, a lack of innervation of the inferior limbs (flaccid paraplegia), and neurogenic bladder and bowel. Given the known positive effects of growth hormone (GH) on neural stem cells (NSCs), we treated him with GH and rehabilitation, trying to induce recovery from the aforementioned sequelae. The Gross Motor Function Test (GMFM)-88 test score was 12.31%. After a blood analysis, GH treatment (0.3 mg/day, 5 days/week, during 3 months and then 15 days without GH) and rehabilitation commenced. This protocol was followed for 5 years, the last GH dose being 1 mg/day. Blood analysis and physical exams were performed every 3 months initially and then every 6 months. Six months after commencing the treatment the GMFM-88 score increased to 39.48%. Responses to sensitive stimuli appeared in most of the territories explored; 18 months later sensitive innervation was complete and the patient moved all muscles over the knees and controlled his sphincters. Three years later he began to walk with crutches, there was plantar flexion, and the GMFM-88 score was 78.48%. In summary, GH plus rehabilitation may be useful for innervating distal areas below the level of the incomplete spinal cord in CRS. It is likely that GH acted on the ependymal SC NSCs, as the hormone does in the neurogenic niches of the brain, and rehabilitation helped to achieve practically full functionality. |
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| language | English |
| last_indexed | 2024-04-12T02:34:55Z |
| publishDate | 2017-01-01 |
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| series | International Journal of Molecular Sciences |
| spelling | doaj.art-f70d3c036d304208bac520cca099d7bd2022-12-22T03:51:37ZengMDPI AGInternational Journal of Molecular Sciences1422-00672017-01-0118123010.3390/ijms18010230ijms18010230Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression SyndromeJesús Devesa0Alba Alonso1Natalia López2José García3Carlos I. Puell4Tamara Pablos5Pablo Devesa6Scientific Direction, Medical Center Foltra, 15886 Teo, SpainChildren Physiotherapy, Medical Center Foltra, 15886 Teo, SpainChildren Physiotherapy, Medical Center Foltra, 15886 Teo, SpainAdults Physiotherapy, Medical Center Foltra, 15886 Teo, SpainPhysical Medicine and Rehabilitation, Medical Center Foltra, 15886 Teo, SpainNeurology, Medical Center Foltra, 15886 Teo, SpainResearch and Development, Medical Center Foltra, 15886 Teo, SpainCaudal regression syndrome (CRS) is a malformation occurring during the fetal period and mainly characterized by an incomplete development of the spinal cord (SC), which is often accompanied by other developmental anomalies. We studied a 9-month old child with CRS who presented interruption of the SC at the L2–L3 level, sacral agenesis, a lack of innervation of the inferior limbs (flaccid paraplegia), and neurogenic bladder and bowel. Given the known positive effects of growth hormone (GH) on neural stem cells (NSCs), we treated him with GH and rehabilitation, trying to induce recovery from the aforementioned sequelae. The Gross Motor Function Test (GMFM)-88 test score was 12.31%. After a blood analysis, GH treatment (0.3 mg/day, 5 days/week, during 3 months and then 15 days without GH) and rehabilitation commenced. This protocol was followed for 5 years, the last GH dose being 1 mg/day. Blood analysis and physical exams were performed every 3 months initially and then every 6 months. Six months after commencing the treatment the GMFM-88 score increased to 39.48%. Responses to sensitive stimuli appeared in most of the territories explored; 18 months later sensitive innervation was complete and the patient moved all muscles over the knees and controlled his sphincters. Three years later he began to walk with crutches, there was plantar flexion, and the GMFM-88 score was 78.48%. In summary, GH plus rehabilitation may be useful for innervating distal areas below the level of the incomplete spinal cord in CRS. It is likely that GH acted on the ependymal SC NSCs, as the hormone does in the neurogenic niches of the brain, and rehabilitation helped to achieve practically full functionality.http://www.mdpi.com/1422-0067/18/1/230GHsyndrome of caudal regressionsacral agenesisphysiotherapyneurogenic bladderflaccid paraplegia |
| spellingShingle | Jesús Devesa Alba Alonso Natalia López José García Carlos I. Puell Tamara Pablos Pablo Devesa Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome International Journal of Molecular Sciences GH syndrome of caudal regression sacral agenesis physiotherapy neurogenic bladder flaccid paraplegia |
| title | Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome |
| title_full | Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome |
| title_fullStr | Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome |
| title_full_unstemmed | Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome |
| title_short | Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome |
| title_sort | growth hormone gh and rehabilitation promoted distal innervation in a child affected by caudal regression syndrome |
| topic | GH syndrome of caudal regression sacral agenesis physiotherapy neurogenic bladder flaccid paraplegia |
| url | http://www.mdpi.com/1422-0067/18/1/230 |
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