Said Sarmad, Cooper Chad J, Reyna Edgar, Alkhateeb Haider, Diaz Jesus, Nahleh Zeina
Department of Internal Medicine, Paul L. Foster School of Medicine, Texas Tech University Health Sciences Center, El Paso, TX, U.S.A.
Am J Case Rep. 2013 Oct 1;14:391-4. doi: 10.12659/AJCR.889560. eCollection 2013.
Female, 59 FINAL DIAGNOSIS: Paraneoplastic limbic encephalitis Symptoms: Seizure • memory changes • decreased concentration
Chemotherapy Clinical Procedure: Cerebral images Specialty: Hematology • Oncology.
Challenging differential diagnosis.
Paraneoplastic neurological disorders (PND) are defined as remote effects on the nervous system that are not caused directly by the tumor, its metastases, or metabolic disruptions. This syndrome occurs in less than 1 per 10,000 patients diagnosed with a malignancy. Many antibodies are found in the central nervous system in PND, the most well known are Anti-Hu, Tr, CV2 Ta, Yo, Ri and amphiphysin. Paraneoplastic limbic encephalitis occurs due to involvement of the limbic system secondary to an autoimmune response to neurons of the brain provoked by the antibodies. PATIENTs, thus, present with seizures, changes in mood, memory, and personality.
Fifty-nine years-old female patient presented with seizures, decreased concentration and memory changes. Laboratory workup was remarkable for hyponatremia. Further workup included brain computerized tomography (CT) and magnetic resonance imaging (MRI), which suggested a diagnosis of encephalitis for limbic encephalitis. Anti-Hu, anti-Ma and NMDA-receptor antibodies were requested of which Anti Hu antibodies were positive. Transbronchial biopsy was obtained which confirmed the diagnosis of small cell lung cancer.
A very high index of suspicion should thus be present when patients present with paraneoplastic abnormalities. It must be emphasized that limbic encephalitis (LE) occurs at an early stage of the disease development and therefore the detection of paraneoplastic LE can lead to a quicker identification of the underlying malignancy and a better outcome.
女性,59岁
副肿瘤性边缘叶脑炎
癫痫发作、记忆力改变、注意力不集中
化疗
脑部影像
血液学、肿瘤学
具有挑战性的鉴别诊断
副肿瘤性神经系统疾病(PND)被定义为并非由肿瘤、其转移灶或代谢紊乱直接引起的对神经系统的远距离影响。这种综合征在每10000名被诊断患有恶性肿瘤的患者中发生率不到1例。在PND患者的中枢神经系统中发现了许多抗体,其中最著名的是抗Hu、Tr、CV2/Ta、Yo、Ri和抗 amphiphysin抗体。副肿瘤性边缘叶脑炎是由于抗体引发的针对脑神经元的自身免疫反应导致边缘系统受累而发生的。因此,患者会出现癫痫发作、情绪、记忆和性格改变。
一名59岁女性患者出现癫痫发作、注意力不集中和记忆力改变。实验室检查发现低钠血症明显。进一步检查包括脑部计算机断层扫描(CT)和磁共振成像(MRI),提示边缘叶脑炎的诊断。检测了抗Hu、抗Ma和NMDA受体抗体,其中抗Hu抗体呈阳性。进行了经支气管活检,确诊为小细胞肺癌。
因此,当患者出现副肿瘤性异常时,应高度怀疑。必须强调的是,边缘叶脑炎(LE)发生在疾病发展的早期阶段,因此检测到副肿瘤性LE可导致更快地识别潜在的恶性肿瘤并获得更好的治疗结果。