Sylaja P N, Cherian P J, Das C K, Radhakrishnan V V, Radhakrishnan K
Department of Neurology, Sree Chitra Tirunal Institute for Medical Sciences and Technology, Trivandrum, Kerala, India.
Neurol India. 2002 Mar;50(1):53-9.
Idiopathic hypertrophic cranial pachymeningitis is a rare form of fibrosing chronic inflammatory process of unknown etiology, which causes thickening of the intracranial dura mater. We present four patients with hypertrophic cranial pachymeningitis who presented with chronic headache and cranial nerve palsies. The diagnosis of idiopathic hypertrophic cranial pachymeningitis was based on neuroimaging findings of thickened enhancing dura, exclusion of known causes and histopathologic findings compatible with nonspecific inflammation in the meningeal biopsies. Corticosteroid therapy was effective in all cases in inducing a complete or partial remission of the neurologic symptoms and signs. We describe the clinical, radiological and pathological features of idiopathic hypertrophic cranial pachymeningitis and discuss the relationship of this entity with other inflammatory fibrosclerotic disorders to explain the pathogenesis. A high index of suspicion, prompt confirmation of the diagnosis by meningeal biopsy, and early institution and long-term maintenance of steroid therapy may help to prevent irreversible neurologic sequelae, especially blindness.
特发性肥厚性硬脑膜炎是一种病因不明的罕见的纤维性慢性炎症过程，可导致颅内硬脑膜增厚。我们报告了4例肥厚性硬脑膜炎患者，他们均表现为慢性头痛和颅神经麻痹。特发性肥厚性硬脑膜炎的诊断基于硬脑膜增厚强化的神经影像学表现、排除已知病因以及脑膜活检中与非特异性炎症相符的组织病理学表现。皮质类固醇治疗在所有病例中均有效，可使神经症状和体征完全或部分缓解。我们描述了特发性肥厚性硬脑膜炎的临床、放射学和病理学特征，并讨论了该疾病与其他炎症性纤维硬化性疾病的关系，以解释其发病机制。高度的怀疑指数、通过脑膜活检迅速确诊以及早期应用和长期维持类固醇治疗可能有助于预防不可逆的神经后遗症，尤其是失明。
