Omerhodžić Ibrahim, Džurlić Almir, Lisica Dino, Mahmutbegović Nevena, Nikšić Maida, Bilalović Nurija, Suljić Enra
Neurosurgery Clinic, Clinical Center of Sarajevo University, Sarajevo, Bosnia and Herzegovina.
Neurology Clinic, Clinical Center of Sarajevo University, Sarajevo, Bosnia and Herzegovina.
Acta Med Acad. 2018 Nov;47(2):193-198. doi: 10.5644/ama2006-124.231.
We present a case of relapsing tumefactive demyelination in a young female patient, that posed a real diagnostic challenge, with a heterogeneous clinical picture, atypical for multiple sclerosis (MS) presentation, and neuroradiological manifestations with a high suspicion of neoplastic diseases.
An 18-year old female patient presented to our Neurosurgical Out-patients' Clinic with symptoms atypical for multiple sclerosis, unremarkable neurological deficit, one tumefactive lesion on MRI, followed by relapse and another two lesions within a period of six months. We decided to perform biopsy of the tumefactive lesion with compressive effect. Serological and clinical data were negative for MS, and the patient did not respond well to corticosteroid therapy. Fresh frozen tumor tissue aroused a strong suspicion of gemistocytic astrocytoma, so total resection was done, but the definitive pathohistological examination confirmed tumefactive demyelination.
For clinicians, it is important to consider demyelinating disease in the differential diagnosis of a tumorlike lesion of the central nervous system, in order to avoid invasive and potentially harmful diagnostic procedures, especially in younger patients.
我们报告一例年轻女性复发性肿胀性脱髓鞘病例，该病例带来了真正的诊断挑战，其临床表现异质性，不符合多发性硬化（MS）的典型表现，神经放射学表现高度怀疑为肿瘤性疾病。
一名18岁女性患者因不符合多发性硬化的非典型症状、无明显神经功能缺损，以及MRI上有一个肿胀性病变前来我们的神经外科门诊就诊，随后在六个月内出现复发及另外两个病变。我们决定对具有压迫效应的肿胀性病变进行活检。MS的血清学和临床数据均为阴性，且患者对皮质类固醇治疗反应不佳。新鲜冰冻肿瘤组织高度怀疑为肥胖型星形细胞瘤，因此进行了全切除，但最终病理组织学检查证实为肿胀性脱髓鞘。
对于临床医生而言，在中枢神经系统肿瘤样病变的鉴别诊断中考虑脱髓鞘疾病很重要，以避免侵入性且可能有害的诊断程序，尤其是在年轻患者中。
