Idiopathic Hypertrophic Pachymeningitis: Does Earlier Treatment Improve Outcome?

Children

Ming Lim¹  Ata Siddiqui²  Emilia Rizzo³  AilsaElizabeth Ritchie⁴  Vinay Shivamurthy⁵ 

[1] Children’s Neurosciences, Evelina London Children’s Hospital at Guy’s and St Thomas’ NHS Foundation Trust, King’s Health Partners Academic Health Science Centre, London SE1 7RS, UK;Department of Neuroradiology, King’s College Hospital NHS Foundation Trust, London SE5 9RS, UK;Medical Doctor, University of Catania, Piazza dell’Università 2, 95124 Catania, Italy;Paediatric Ophthalmology, Evelina London Children’s Hospital at Guy’s and St Thomas’ NHS Foundation Trust, London SE1 7EH, UK;Paediatric Rheumatology, Evelina London Children’s Hospital at Guy’s and St Thomas’ NHS Foundation Trust, London SE1 7EH, UK;

关键词: dural thickening;    cranial neuropathy;    immunotherapy;    sarcoid;    steroids;    visual failure;   

DOI  :  10.3390/children8010011

来源: DOAJ

【 摘 要 】

Background/goal: Hypertrophic pachymeningitis is a rare chronic inflammatory disorder characterized by marked fibrous thickening of the cerebral and/or spinal dura mater. This condition has largely been reported in adults, but there are very few reports in children. Methods: We describe a 14-year-old boy with idiopathic hypertrophic pachymeningitis, who presented with deteriorating vision on a background of severe headache. We evaluated pediatric cases of hypertrophic pachymeningitis and compared treatments and their relation to outcomes. Results: There are only eleven pediatric cases of hypertrophic pachymeningitis reported in the literature. In the patients treated with steroids either at presentation or subsequent relapses, a good response was reported. In the cases with delayed initiation of steroid treatment, this was often related to an incomplete recovery. In our patient, this delay may have contributed to his poor visual outcome. Conclusions: Early initiation of steroid treatment in children with idiopathic hypertrophic pachymeningitis may improve outcomes.

【 授权许可】

Unknown