Zhu Ruixia, He Zhiyi, Ren Yan
Department of Neurology, The First Affiliated Hospital of China Medical University, 155 Nanjing North Street, Shenyang, 110001, China.
Eur Spine J. 2015 May;24 Suppl 4:S633-5. doi: 10.1007/s00586-015-3956-4. Epub 2015 Apr 18.
Hypertrophic craniocervical pachymeningitis (HCP) is a rare disease causing chronic inflammatory hypertrophy of the cranial and spinal dura mater. To increase awareness of this condition, we report the details of a case here.
We reviewed the case of a 78-year-old man presenting with a rare case of HCP and summarized the clinical features, laboratory evaluations and treatment of the case.
In this case, the HCP involved the intracranial dura and high cervical regions, manifesting as lower cranial nerve palsies, headache, and neck pain, developing over 7 months. Magnetic resonance imaging revealed thickening of the dura in the craniocervical region with peripheral enhancement. Steroid therapy was commenced and the symptoms improved rapidly.
HCP can be diagnosed by MRI and laboratory investigations. In this case corticosteroid treatment was effective, although care must be taken when slowly reducing the dose. This case highlights HCP as a cause of progressive cerebellomedullar and cervical spinal cord symptoms.
肥厚性颅颈硬脑膜炎(HCP)是一种罕见疾病,可导致颅和脊髓硬脑膜慢性炎性肥厚。为提高对该病的认识,我们在此报告一例病例详情。
我们回顾了一名78岁男性的罕见HCP病例,并总结了该病例的临床特征、实验室检查及治疗情况。
该病例中,HCP累及颅内硬脑膜和高位颈段区域,表现为低位颅神经麻痹、头痛和颈部疼痛,病程达7个月。磁共振成像显示颅颈区域硬脑膜增厚并伴有外周强化。开始使用类固醇治疗后症状迅速改善。
HCP可通过磁共振成像和实验室检查确诊。在本病例中,皮质类固醇治疗有效,不过在缓慢减药时必须谨慎。该病例突出了HCP作为进行性小脑延髓和颈脊髓症状病因的情况。