Salunke Pravin, Aggarwal Ashish, Gupta Kirti, Agrawal Pallavi, Ahuja Chirag Kamal, Vasishta Rakesh Kumar
Department of Neurosurgery, Postgraduate Institute of Medical Education & Research, Chandigarh, India.
Br J Neurosurg. 2012 Aug;26(4):490-8. doi: 10.3109/02688697.2012.657269. Epub 2012 Mar 9.
OBJECT/ BACKGROUND: Large demyelinating lesions (LDLs) may present with unusual features like seizures and significant mass effect and often masquerade a tumour. Even radiological features are confusing. With clinical signs of increased intra-cranial pressure (ICP), decompressive surgery becomes life-saving. However, resection of the involved nervous tissue is unnecessary and may lead to permanent residual deficits that otherwise can be avoided.
We present a series of eight patients with focal deficits and/or raised pressure symptoms wherein a diagnosis of tumour was made preoperatively. The clinico-radiological picture and outcome has been described.
Clinically, all these patients had focal deficits and five had raised ICP. Three patients had seizures. Two patients had long standing visual deterioration in one eye. Radiology showed irregular enhancement in two and concentric rings in one. The deep grey matter was involved in one and cortex in four. Biopsy/decompressive surgery and resection of lesion improved the sensorium in all, but focal deficits persisted. Two patients died after being discharged in a conscious state, and one died in hospital.
High index of suspicion is required to diagnose demyelination prior to surgery. Unexplained long standing clinical features, radiology that has contrast enhancement patterns and mass effect (dissociation between contrast enhancement and mass effect) that is unusual for glioma should raise the suspicion of such non-neoplastic lesions. For patients with minimal mass effect with focal deficits, open/stereotactic biopsy from multiple areas of the lesion is preferable for diagnosis. Those presenting with mass effect, decompressive craniectomy and biopsy from the lesion is preferable than attempting complete resection especially in and around the eloquent areas. A second look surgery to resect the lesion can always be undertaken once histopathology suggests a neoplastic etiology and rules out a demyelinating lesion.
目的/背景:大型脱髓鞘病变(LDLs)可能表现出不寻常的特征,如癫痫发作和显著的占位效应,常伪装成肿瘤。即使是放射学特征也容易混淆。伴有颅内压(ICP)升高的临床体征时,减压手术可挽救生命。然而,切除受累神经组织并无必要,且可能导致永久性残留缺陷,而这些缺陷原本是可以避免的。
我们报告了一系列8例有局灶性缺损和/或压力升高症状的患者,术前均诊断为肿瘤。描述了临床放射学表现及结果。
临床上,所有这些患者均有局灶性缺损,5例颅内压升高。3例患者有癫痫发作。2例患者单眼长期视力下降。放射学检查显示2例不规则强化,1例呈同心圆状强化。1例累及深部灰质,4例累及皮质。活检/减压手术及病变切除术后所有患者的意识均有改善,但局灶性缺损持续存在。2例患者在神志清醒出院后死亡,1例在医院死亡。
术前诊断脱髓鞘需要高度怀疑。无法解释的长期临床特征、具有对比增强模式和占位效应(对比增强与占位效应分离)的放射学表现(这对于胶质瘤来说不常见)应引起对这类非肿瘤性病变的怀疑。对于占位效应轻微且有局灶性缺损的患者,从病变的多个区域进行开放/立体定向活检更有利于诊断。对于有占位效应的患者,减压性颅骨切除术及病变活检比试图完全切除更可取,尤其是在明确功能区及其周围。一旦组织病理学提示肿瘤病因并排除脱髓鞘病变,可随时进行二次手术切除病变。