Khalid Aisha, Uche Enoch O
Research, Harvard Medical School, Boston, USA.
Neurological Surgery, University of Nigeria Teaching Hospital, Enugu, NGA.
Cureus. 2022 Aug 27;14(8):e28466. doi: 10.7759/cureus.28466. eCollection 2022 Aug.
Idiopathic hypertrophic cranial pachymeningitis (IHCP) is a rare chronic inflammatory disease characterized by diffuse thickening of the dura mater. Although IHCP mostly presents as a diffuse lesion, it may also occur as focal tumour-like lesions. Here we present the first reported case of IHCP associated with a Chiari type I malformation (CMI). A 65-year-old man presented with a one-year history of chronic headache and vertigo exacerbated by standing and neck flexion. The neurological examination was unremarkable except for tongue wasting and fasciculations. MRI demonstrated features of CMI and findings suggestive of IHCP. Posterior fossa decompression resulted in significant symptomatic improvement and the diagnosis of IHCP was confirmed on histopathology. Though there is no consensus about the management of IHCP in this case, we advocate surgical decompression with prolonged steroid therapy.
特发性肥厚性硬脑膜炎（IHCP）是一种罕见的慢性炎症性疾病，其特征为硬脑膜弥漫性增厚。尽管IHCP大多表现为弥漫性病变，但也可能以局灶性肿瘤样病变的形式出现。在此，我们报告首例与I型Chiari畸形（CMI）相关的IHCP病例。一名65岁男性，有1年慢性头痛病史，站立及颈部屈曲时眩晕加重。神经系统检查除舌肌萎缩和肌束震颤外无异常。MRI显示CMI特征及提示IHCP的表现。后颅窝减压术后症状显著改善，组织病理学确诊为IHCP。尽管对于该病例中IHCP的治疗尚无共识，但我们主张手术减压并延长类固醇治疗。
