Preprint Article Version 1 NOT YET PEER-REVIEWED

Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected from a Syndrome of Caudal Regression

Version 1 : Received: 25 November 2016 / Approved: 27 November 2016 / Online: 27 November 2016 (04:35:36 CET)

A peer-reviewed article of this Preprint also exists.

Devesa, J.; Alonso, A.; López, N.; García, J.; Puell, C.I.; Pablos, T.; Devesa, P. Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome. Int. J. Mol. Sci. 2017, 18, 230. Devesa, J.; Alonso, A.; López, N.; García, J.; Puell, C.I.; Pablos, T.; Devesa, P. Growth Hormone (GH) and Rehabilitation Promoted Distal Innervation in a Child Affected by Caudal Regression Syndrome. Int. J. Mol. Sci. 2017, 18, 230.

Journal reference: Int. J. Mol. Sci. 2017, 18, 230
DOI: 10.3390/ijms18010230

Abstract

Caudal regression syndrome (CRS) is a congenital abnormality characterized by an incomplete development of the spinal cord (SC) and other abnormalities. We studied a 9-months old CRS child presenting: interruption of SC at L2-L3 level, sacral agenesis, lack of innervation of the inferior limbs (flaccid paraplegia) and neurogenic bladder and bowel. Given the effects of growth hormone (GH) on the proliferation, differentiation and migration of neural stem cells (NSCs), we treated him with GH and rehabilitation, trying to induce the recovery of main sequelae. GMFM-88 test score was 12.31%. After a blood analysis, GH treatment (0.3 mg/day, 5 days/week, 3 months and then 15 days without GH) and rehabilitation commenced. This protocol was followed during 5 years, being the last GH dose 1 mg/day. Blood analysis and physical exams were performed every 3 months initially and every 6 months later. Six months after commencing the treatment 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 any muscle over the knees and controlled his sphincters. Three years later he walked with the help of canes, there was plantar flexion and GMFM-88 score was 78.48%. In summary, GH plus rehabilitation may be useful for innervating distal territories, below the level of the incomplete spinal cord in CRS. Most likely, GH acts on ependymal SC NSCs, as the hormone does in the neurogenic niches in the brain.

Subject Areas

GH; syndrome of caudal regression; sacral agenesis; physiotherapy; neurogenic bladder; flaccid paraplegia

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