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[Syringomyelia, scoliosis and idiopathic Arnold-Chiari malformations: a common etiology].
Rev Neurol. 1996 Aug; 24(132):937-59.RN

Abstract

INTRODUCTION

The major theories of the pathogenesis of idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID) and the Arnold-Chiari malformation (ARCH) are reviewed. A new theory involving a common pathology for SMI, ESCID and ARCH is proposed. Its confirmation depends on the presence of an abnormally low position of the conus medullaris (CM) in patients with SMI. Material and method. Of 292 patients with syringomyelia (SM), 231 patients with SMI were selected, and 55 of these were chosen in whom the level of the conus medullaris (NCM) could be determined, together with figures for SMI, ESCID and ARCH by magnetic resonance (RM). The position of the conus medullaris in 50 patients who did not have SM, ESCID nor ARCH on cervical and lumbar RM was determined.

RESULTS

96.54% of patients with SMI showed some descent of the cerebellar tonsils. 72.97% of the SMI patients also had ESCID: 74.07% of the patients in whom the conus medullaris was seen had scoliosis and a low position of the conus medullaris. 6% of the control group had a conus medullaris at the level of the body of L1, whilst 84.21% of the patients with SMI had a partial or complete image of the conus medullaris at this level. In these patients, an unusually low position of the conus medullaris was confirmed.

CONCLUSIONS

The unusually low position of the conus medullaris in SMI and its close relationship to ARCH and ESCID make it likely that the same pathogenic mechanism is shared by them all. Abnormal asynchromy of growth of the notochord and the spinal cord gives rise to a distinct disorder which shows as different syndromes.

Authors+Show Affiliations

Servicio de Neurocirugia, Clinica Tres Torres, Barcelona. nroyo@idgrup.ibernet.com

Pub Type(s)

Comparative Study
English Abstract
Journal Article
Review

Language

spa

PubMed ID

8755356

Citation

Royo-Salvador, M B.. "[Syringomyelia, Scoliosis and Idiopathic Arnold-Chiari Malformations: a Common Etiology]." Revista De Neurologia, vol. 24, no. 132, 1996, pp. 937-59.
Royo-Salvador MB. [Syringomyelia, scoliosis and idiopathic Arnold-Chiari malformations: a common etiology]. Rev Neurol. 1996;24(132):937-59.
Royo-Salvador, M. B. (1996). [Syringomyelia, scoliosis and idiopathic Arnold-Chiari malformations: a common etiology]. Revista De Neurologia, 24(132), 937-59.
Royo-Salvador MB. [Syringomyelia, Scoliosis and Idiopathic Arnold-Chiari Malformations: a Common Etiology]. Rev Neurol. 1996;24(132):937-59. PubMed PMID: 8755356.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - [Syringomyelia, scoliosis and idiopathic Arnold-Chiari malformations: a common etiology]. A1 - Royo-Salvador,M B, PY - 1996/8/1/pubmed PY - 1996/8/1/medline PY - 1996/8/1/entrez SP - 937 EP - 59 JF - Revista de neurologia JO - Rev Neurol VL - 24 IS - 132 N2 - INTRODUCTION: The major theories of the pathogenesis of idiopathic syringomyelia (SMI), idiopathic scoliosis (ESCID) and the Arnold-Chiari malformation (ARCH) are reviewed. A new theory involving a common pathology for SMI, ESCID and ARCH is proposed. Its confirmation depends on the presence of an abnormally low position of the conus medullaris (CM) in patients with SMI. Material and method. Of 292 patients with syringomyelia (SM), 231 patients with SMI were selected, and 55 of these were chosen in whom the level of the conus medullaris (NCM) could be determined, together with figures for SMI, ESCID and ARCH by magnetic resonance (RM). The position of the conus medullaris in 50 patients who did not have SM, ESCID nor ARCH on cervical and lumbar RM was determined. RESULTS: 96.54% of patients with SMI showed some descent of the cerebellar tonsils. 72.97% of the SMI patients also had ESCID: 74.07% of the patients in whom the conus medullaris was seen had scoliosis and a low position of the conus medullaris. 6% of the control group had a conus medullaris at the level of the body of L1, whilst 84.21% of the patients with SMI had a partial or complete image of the conus medullaris at this level. In these patients, an unusually low position of the conus medullaris was confirmed. CONCLUSIONS: The unusually low position of the conus medullaris in SMI and its close relationship to ARCH and ESCID make it likely that the same pathogenic mechanism is shared by them all. Abnormal asynchromy of growth of the notochord and the spinal cord gives rise to a distinct disorder which shows as different syndromes. SN - 0210-0010 UR - https://www.unboundmedicine.com/medline/citation/8755356/[Syringomyelia_scoliosis_and_idiopathic_Arnold_Chiari_malformations:_a_common_etiology]_ L2 - http://www.diseaseinfosearch.org/result/6465 DB - PRIME DP - Unbound Medicine ER -