胸腺瘤相关性副肿瘤边缘性脑炎.pptxVIP

Thymoma-associated paraneoplastic encephalitis (TAPE);Up to 50% of patients with thymoma have paraneoplastic neurologic syndromes, the most common being myasthenia gravis.;病例;The patient received anticonvulsants, human intravenous immunoglobulin G (IgG) (400 g/kg daily), and corticosteroids (methylprednisolone 1 gm daily) for 5 days plus a prednisone taper with resolution of neurologic symptoms. Four weeks later, the patient underwent complete, en bloc resection of the mediastinal mass that was invading through the pericardium (type B3, World Health Organization classification,Masaoka stage III). She received adjuvant radiation therapy (5040 cGy) owing to suspected invasion into the pericardial space. Four weeks later, brain MRI showed decrease in size of the brain lesions (Figure 1, B). Two years after the operation, the patient has no evidence of tumor recurrence (Figure 1, D) or neurologic impairment.;Since 1988, when the first case of paraneoplastic encephalitis in the setting of thymic cancer was reported, 28 cases of thymoma-associated paraneoplastic encephalitis (TAPE)have been described (Table 1). 从1988-2010 共报道了28例胸腺瘤相关的副肿瘤脑炎。;;Rickman ;Gesundheit ;The encephalitis can be confined to the limbic system or multifocal and extralimbic. In most patients (82%), neurologic manifestations include memory loss, confusion, and seizures. Seventy percent exhibited bilateral foci of nonenhancing hyperintensity on brain MRI, some appearing weeks after onset of symptoms. The differential diagnosis includes metastases, primary brain tumors, and infection. 这种脑炎可累及边缘系统，边缘系统外结构或是呈多灶损伤。 82%的病人出现的神经症状有记忆丢失，精神错乱和癫痫。 70%的患者在MRI上表现为双侧非强化的高信号，并可以持续数周。 需与转移性、原发性脑肿瘤及感染鉴别。;Although routine analysis of cerebrospinal fluid is nonspecific, sampling of serum and cerebrospinal fluid for antineuronal antibodies (found in 75% of patients withTAPE) will aid in establishing a paraneoplastic pathogenesis. The diagnosis of TAPE depends on the combination of the encephalitis syndrome, MRI find