title: Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome.
abstract: 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.
authors.affiliation: Scientific Direction, Medical Center Foltra, 15886 Teo, Spain. devesa.jesus@gmail.com.
authors.forename: Jesús
authors.initials: J
authors.lastname: Devesa
authors.affiliation: Children Physiotherapy, Medical Center Foltra, 15886 Teo, Spain. alba.fisioterapia@foltra.org.
authors.forename: Alba
authors.initials: A
authors.lastname: Alonso
authors.affiliation: Children Physiotherapy, Medical Center Foltra, 15886 Teo, Spain. natalia.fisioterapia@foltra.org.
authors.forename: Natalia
authors.initials: N
authors.lastname: López
authors.affiliation: Adults Physiotherapy, Medical Center Foltra, 15886 Teo, Spain. jose.fisioterapia@foltra.org.
authors.forename: José
authors.initials: J
authors.lastname: García
authors.affiliation: Physical Medicine and Rehabilitation, Medical Center Foltra, 15886 Teo, Spain. cipuell@foltra.org.
authors.forename: Carlos I
authors.initials: CI
authors.lastname: Puell
authors.affiliation: Neurology, Medical Center Foltra, 15886 Teo, Spain. tpablos@foltra.org.
authors.forename: Tamara
authors.initials: T
authors.lastname: Pablos
authors.affiliation: Research and Development, Medical Center Foltra, 15886 Teo, Spain. pdevesap@foltra.org.
authors.forename: Pablo
authors.initials: P
authors.lastname: Devesa
mesh_terms: D002648:Child; D005121:Extremities / Q000294:innervation*; D013006:Growth Hormone / Q000627:therapeutic use*; D006801:Humans; D021621:Imaging, Three-Dimensional; D007223:Infant; D008279:Magnetic Resonance Imaging; D008297:Male; D013118:Spinal Cord Diseases / Q000188:drug therapy* / Q000534:rehabilitation*; D013577:Syndrome
chemical_list: D013006:Growth Hormone
keywords: GH; flaccid paraplegia; neurogenic bladder; physiotherapy; sacral agenesis; syndrome of caudal regression