Hahn Lewis D, Fulbright Robert, Baehring Joachim M
Department of Medicine, Yale University School of Medicine, New Haven, CT 06510, United States.
Department of Radiology, Yale University School of Medicine, New Haven, CT 06510, United States.
J Neurol Sci. 2016 Aug 15;367:278-83. doi: 10.1016/j.jns.2016.06.024. Epub 2016 Jun 11.
Hypertrophic Pachymeningitis (HP) denotes inflammation and thickening of the dura mater that can be idiopathic or secondary to a wide variety of conditions. Clinically, HP can present as debilitating headaches and cranial nerve defects but in other cases may be completely asymptomatic. We aimed to determine the relative incidence of different etiologies of HP and compare their associated imaging findings. Additionally, we sought to compare the clinical features of the underlying syndromes. We retrospectively examined twenty-two consecutive cases of HP seen in a single practitioner neurology practice over a ten-year time period. The most common etiologies were idiopathic HP and neurosarcoidosis. No imaging features were completely specific to any etiology. Nonetheless, idiopathic HP typically demonstrated diffuse regular enhancement whereas neurosarcoidosis was more likely to display a nodular enhancement pattern. Headache and cranial neuropathies were the most common clinical presentation. HP symptoms were often responsive to steroids but complete responses were rare. HP is a diagnostic challenge without specific findings on minimally or non-invasive diagnostic studies. Biopsy is often required and serves as the basis for effective therapy.
肥厚性硬脑膜炎（HP）是指硬脑膜的炎症和增厚，可为特发性或继发于多种疾病。临床上，HP可表现为使人衰弱的头痛和脑神经缺损，但在其他情况下可能完全无症状。我们旨在确定HP不同病因的相对发病率，并比较其相关的影像学表现。此外，我们试图比较潜在综合征的临床特征。我们回顾性研究了在一位神经科医生的诊所中连续十年间见到的22例HP病例。最常见的病因是特发性HP和神经结节病。没有任何影像学特征对任何病因具有完全特异性。尽管如此，特发性HP通常表现为弥漫性规则强化，而神经结节病更可能表现为结节状强化模式。头痛和脑神经病变是最常见的临床表现。HP症状通常对类固醇有反应，但完全缓解很少见。HP是一项诊断挑战，在微创或非侵入性诊断研究中没有特异性发现。通常需要进行活检，并作为有效治疗的基础。
