Eguchi K, Kodama Y, Hotta T, Taniguchi E, Hashizume A, Yamasaki F, Yamane T, Kurisu K, Arita K
Department of Neurosurgery, Kure National Hospital, Japan.
No To Shinkei. 1996 May;48(5):475-80.
A case of cranial hypertrophic pachymeningitis of unknown etiology in a patient with 15-year history of headaches, cranial nerve palsies, and gait disturbance is reported. A 77-year-old woman was brought to our institute in a coma. CT revealed intracerebral hemorrhage in the right temporal lobe and thickening of the falx and tentorium. Fifteen years previously the patient had undergone CT scanning because of headaches, cranial nerve palsies, and progressive gait disturbance and a thickened tentorium, mild hydrocephalus and edematous change in the right temporal lobe had been reported. Since the etiology of her symptoms was unclear at the time, she did not receive adequate treatment. Her symptoms gradually progressed thereafter, and her visual acuity and hearing deteriorated. MR imaging in 1994 showed the thickened tentorium as a hypointense area with hyperintense edges on Gd-DTPA enhanced images. Angiography revealed narrowing of posterior portion of the superior sagittal sinus. The patient's condition rapidly deteriorated due to the intracranial hypertension and she subsequently died. Autopsy revealed a thickened tentorium with xanthochromic surface. This hypertrophic change was also seen in the dura mater of the posterior and middle cranial fossa. Microscopic examination of the thickened tentorium revealed extensive fibrous tissue with a chronic inflammatory infiltrate, predominantly of lymphocytes. No specific lesions were revealed by staining with hematoxylin-eosin, PAS, Gram's or Ziehl-Neelsen stains. The patient had no inflammatory or infectious diseases of other organs, and a diagnosis of idiopathic cranial hypertrophic pachymeningitis of unknown etiology was made. Considering the above findings, the thickened tentorium depicted as a hypointense area on the T1- and T2-weighted images and the Gd-enhanced edges of the tentorium are thought to be represent fibrous tissue and inflammatory regions, respectively.
报告了一例病因不明的颅骨肥厚性硬脑膜炎病例，患者有15年头痛、颅神经麻痹和步态障碍病史。一名77岁女性昏迷状态被送至我院。CT显示右侧颞叶脑出血以及大脑镰和小脑幕增厚。15年前，患者因头痛、颅神经麻痹和进行性步态障碍接受CT扫描，报告显示小脑幕增厚、轻度脑积水和右侧颞叶水肿改变。由于当时其症状病因不明，未接受充分治疗。此后其症状逐渐进展，视力和听力下降。1994年的磁共振成像显示，在钆喷酸葡胺增强图像上，增厚的小脑幕呈低信号区，边缘为高信号。血管造影显示上矢状窦后部狭窄。患者因颅内高压病情迅速恶化，随后死亡。尸检发现小脑幕增厚，表面呈黄变。这种肥厚性改变在颅后窝和颅中窝的硬脑膜也可见。对增厚的小脑幕进行显微镜检查，发现广泛的纤维组织伴有慢性炎症浸润，主要为淋巴细胞。苏木精-伊红染色、PAS染色、革兰氏染色或齐-尼氏染色均未发现特异性病变。患者其他器官无炎症或感染性疾病，诊断为病因不明的特发性颅骨肥厚性硬脑膜炎。考虑到上述发现，在T1加权和T2加权图像上显示为低信号区的增厚小脑幕以及小脑幕的钆增强边缘分别被认为代表纤维组织和炎症区域。
