Sinha M K, Garg R K, Bhatt M L B, Chandra A
Department of Neurology, Chhatrapati Shahuji Maharaj Medical University, Lucknow, 226 003, India.
J Postgrad Med. 2010 Apr-Jun;56(2):146-9. doi: 10.4103/0022-3859.65292.
Tumefactive demyelinating lesion, a variant of multiple sclerosis, is a solitary large demyelinating lesion, which mimics cerebral neoplasm. Distinguishing tumefactive lesions from other etiologies of intracranial space-occupying lesions is essential to avoid inadvertent surgical or toxic chemotherapeutic interventions. We report two unusual cases of tumefactive demyelinating lesion. The first patient presented with recurrent right focal tonic-clonic seizures with secondary generalization of three-month duration. Her neurological examination was normal. Another patient presented with right homonymous hemianopia. In this patient, the diagnosis was established after biopsy of the lesion, which revealed perivascular lymphocytic infiltrate and aggregates of foam cells in white matter with relatively uninvolved grey matter, suggestive of tumefactive demyelinating lesion. Administration of intravenous methylprednisolone resulted in rapid clinical improvement in both the patients and the brain lesion decreased in size. Both, epilepsy and homonymous hemianopia, are unusual manifestations of tumefactive demyelinating lesions. In our cases, cerebral tumors were initial diagnoses. Presence of an open ring or incomplete ring lesions and other magnetic resonance characteristics helped in differentiating demyelinating lesions from other neoplastic and infective diseases of the brain. Differential diagnosis of tumefactive demyelinating lesions, at times, may prove to be a challenging task for the treating physician.
瘤样脱髓鞘病变是多发性硬化的一种变体，是一种孤立的大型脱髓鞘病变，可模拟脑肿瘤。将瘤样病变与颅内占位性病变的其他病因区分开来，对于避免意外的手术或毒性化疗干预至关重要。我们报告了两例不寻常的瘤样脱髓鞘病变病例。首例患者出现持续三个月的复发性右侧局灶性强直阵挛性发作并继发全身性发作。她的神经系统检查正常。另一例患者出现右侧同向性偏盲。在该患者中，病变活检后确诊，活检显示白质中有血管周围淋巴细胞浸润和泡沫细胞聚集，灰质相对未受累，提示瘤样脱髓鞘病变。静脉注射甲基强的松龙后，两名患者的临床症状均迅速改善，脑部病变体积减小。癫痫和同向性偏盲都是瘤样脱髓鞘病变的不寻常表现。在我们的病例中，最初诊断为脑肿瘤。开放环或不完全环病变的存在以及其他磁共振特征有助于将脱髓鞘病变与脑部其他肿瘤性和感染性疾病区分开来。有时，瘤样脱髓鞘病变的鉴别诊断对治疗医生来说可能是一项具有挑战性的任务。
