颅颈肥厚性硬脑膜炎
Craniocervical hypertrophic pachymeningitis.
作者信息
Mancilha Murilo Dos Santos, Andreão Filipi Fim, Costa Januário Bárbara de Ávila, Ribeiro Filipe Virgilio, Nepomuceno Fernandes Matheus, Fabrini Paleare Luis Flavio, da Silva Bruno Pasqualino Aguilar, Felippe Marcelo Medeiros, Quadros Danilo Gomes
机构信息
Department of Neurosurgery, Anhembi Morumbi University, São José dos Campos, São Paulo, Brazil.
Department of Neurosurgery, Federal University of Rio de Janeiro, Rio de Janeiro, Brazil.
出版信息
Surg Neurol Int. 2025 May 16;16:179. doi: 10.25259/SNI_179_2025. eCollection 2025.
BACKGROUND
Hypertrophic pachymeningitis (HP) is a rare neurological disorder characterized by dural thickening. Here, we discuss the diagnosis and surgical management of a 38-year-old whose myelopathy was attributed to dorsally compressive HP extending from the lower cerebellar fossa to C3.
CASE DESCRIPTION
A 38-year-old male with Sjögren's syndrome presented with cervical pain, upper limb paresis, dysphagia, and left-sided tongue/palate paralysis. The cervical magnetic resonance (MR) showed a dorsally compressive lower cerebellar fossa to C3 lesion. When the biopsy revealed HP, and once conservative treatment failed, the patient successfully underwent a posterior surgical decompression, lesion debulking, and craniocervical fusion.
CONCLUSION
Cervical HPs should be diagnosed early on MR, and those with significant myelopathy, aggressively surgically treated.
背景
肥厚性硬脑膜炎（HP）是一种以硬脑膜增厚为特征的罕见神经系统疾病。在此，我们讨论一名38岁患者的诊断及手术治疗情况，该患者的脊髓病归因于从下小脑窝延伸至C3的背侧压迫性HP。
病例描述
一名患有干燥综合征的38岁男性，出现颈部疼痛、上肢轻瘫、吞咽困难及左侧舌/腭麻痹。颈椎磁共振成像（MR）显示下小脑窝至C3存在背侧压迫性病变。活检显示为HP，保守治疗失败后，患者成功接受了后路手术减压、病变减积及颅颈融合术。
结论
颈椎HP应早期通过MR诊断，对于有明显脊髓病的患者，应积极进行手术治疗。
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