Okimura Y, Tanno H, Karasudani H, Suda S, Ono J, Isobe K
Department of Neurosurgery, Kimitsu Central Hospital, Kisarazu, Japan.
No Shinkei Geka. 1991 Mar;19(3):259-62.
A case of hypertrophic cranial pachymeningitis was reported. A 58-year-old female presented the symptoms of headache and vomiting. At the age of 27, she had suffered from tuberculosis. Neurological examination on admission revealed bilateral papilledema, bilateral hearing disturbance, right hypoglossal nerve palsy, ataxic gait, and bilateral intentional tremor. CT scan showed dilatation of the lateral and third ventricles, and compression of the fourth ventricle with marked enhancement of cerebellar tentorium. A ventriculoperitoneal shunt was installed bringing about improvement in bilateral papilledema, ataxic gait, and bilateral intentional tremor. One month later, ataxic gait and bilateral intentional tremor recurred, and monoparesis of the left upper extremity developed. MRI demonstrated hypertrophic dura mater in the posterior fossa and compressed cervical spinal cord. Decompressive surgery was performed bringing about remarkable clinical improvement. The pathological specimen showed thickening of the dura mater with concentric layers of dense fibrous tissue infiltrated with plasma cells. A diagnosis of hypertrophic cranial pachymeningitis was established. Three years later, the clinical features were found unchanged, but contrast enhancement of cerebellar tentorium had progressed markedly. Hypertrophic pachymeningitis is a uncommon disease. But it should be noted that intracranial involvement is very rare. The etiology, symptomatology, neuroradiology, and treatment are discussed and the literature is reviewed.
报告了一例肥厚性硬脑膜炎病例。一名58岁女性出现头痛和呕吐症状。她27岁时曾患结核病。入院时神经学检查发现双侧视乳头水肿、双侧听力障碍、右侧舌下神经麻痹、共济失调步态和双侧意向性震颤。CT扫描显示侧脑室和第三脑室扩张,第四脑室受压,小脑幕明显强化。进行了脑室腹腔分流术,双侧视乳头水肿、共济失调步态和双侧意向性震颤有所改善。一个月后,共济失调步态和双侧意向性震颤复发,左上肢出现单瘫。MRI显示后颅窝硬脑膜肥厚,颈髓受压。进行了减压手术,临床症状明显改善。病理标本显示硬脑膜增厚,有同心圆状致密纤维组织层,伴有浆细胞浸润。确诊为肥厚性硬脑膜炎。三年后,临床特征无变化,但小脑幕的强化明显进展。肥厚性硬脑膜炎是一种罕见疾病。但应注意的是,颅内受累非常罕见。本文讨论了其病因、症状学、神经放射学及治疗方法,并对相关文献进行了综述。