Chiari decompression for syringomyelia in the setting of IgG4-related hypertrophic pachymeningitis: A case-based update

被引:0
作者
Moskalik, A. [1 ]
Kercher, M. [1 ]
Vitt, J. [1 ]
Lee, H. S. [2 ]
Waldau, B. [1 ]
机构
[1] Univ Calif Davis, Dept Neurol Surg, 4860 Y St,Suite 3740, Sacramento, CA 95616 USA
[2] Univ Calif Davis, Dept Pathol, Davis, CA 95616 USA
关键词
Pachymeningitis; Syringomyelia; IgG4-related disease; Chiari decompression; FEATURES;
D O I
10.1016/j.neuchi.2022.02.007
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Hypertrophic pachymeningitis can lead to clinical brainstem and cervical spinal cord compression leading to neurologic deficits. IgG4-related hypertrophic pachymeningitis (IgG4-RHP) is one recently recognized etiology of previously idiopathic cases. A 34-year-old right-handed female presented with slowly pro-gressive neurologic symptoms and worsening radiographic syringomyelia. She successfully underwent Chiari decompression and excision of her pachymeningitis with improvement in her radiographic find-ings. Extensive clinical workup has led to a diagnosis of IgG4-RHP and treatment with steroids. IgG4-RHP is a rare cause of spinal cord compression and on our review of the literature this is the first description of significant syringomyelia associated with this condition. This remains a challenging entity to treat and neurology and rheumatology referrals should be placed early to investigate IgG4-RHP as an etiology for idiopathic cases. Treatment of this disease is likely to evolve with further research.(c) 2022 Elsevier Masson SAS. All rights reserved.
引用
收藏
页码:E97 / E100
页数:4
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