Idiopathic Spinal Cord Herniation: Report of Eight Cases and Review of the Literature

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Study Design.A case series of eight patients with idiopathic spinal cord herniation and a review of the literature.Objective.To report on this rare entity, provide insight on its natural history, and propose an optimal management strategy.Summary of Background Data.Idiopathic spinal cord herniation is a rare disease with 50 cases reported before the current study.Methods.Eight cases (follow-up 1 month to 8 years) are reported using available information from patient charts, interviews, and assessments. All imaging studies are reviewed. The review of the literature was performed using PUBMED.Results.Four patients, followed without surgical intervention, have not progressed. Of the three patients who underwent surgical repair by one of the authors, two improved and one was unchanged. A fourth patient, who was initially treated by another surgeon who failed to identify the dural defect and herniation, had a poor outcome.Conclusion.The pathophysiology of the dural defect is still uncertain. The typical presentation is Brown–Séquard syndrome. Microsurgical repair in cases with progression of neurologic deficits is usually successful in achieving recovery of function or arrest of progression.

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