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Case Report Overlapping of Paraneoplastic Limbic Encephalitis and Lambert-Eaton Myasthenic Syndrome in a Patient with Small Cell Lung Cancer: a Rare Case Report

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Case Report Overlapping of Paraneoplastic Limbic Encephalitis and Lambert-Eaton Myasthenic Syndrome in a Patient with Small Cell Lung Cancer: a Rare Case Report Int J Clin Exp Pathol 2016;9(4):4917-4920 www.ijcep.com /ISSN:1936-2625/IJCEP0023660 Case Report Overlapping of paraneoplastic limbic encephalitis and Lambert-Eaton myasthenic syndrome in a patient with small cell lung cancer: a rare case report Wei Hou1*, Xue Hou1*, Libo Wang1, Shudong Wang2, Yuyu Zhang1, Lijuan Ding1, Lihua Dong1 Departments of 1Radiation Oncology, 2Cardiovascular Center, The First Hospital of Jilin University, Changchun, China. *Equal contributors. Received January 9, 2016; Accepted March 22, 2016; Epub April 1, 2016; Published April 15, 2016 Abstract: Paraneoplastic neurologic syndrome (PNS) is a rare group of disorders caused by nervous system dam- ages in the setting of cancer unrelated to the tumor sites. It may affect one or more regions of the nervous system and occurred in less than 1 per 10,000 patients diagnosed with a malignancy. Patients with PNS may present symptoms or damages in the central and peripheral nervous system, the neuromuscular junction and muscle, which usually results in severely disabling. Nowadays, paraneoplastic limbic encephalitis (PLE) and Lambert-Eaton myasthenic syndrome (LEMS) are the most commonly reported types of PNS compared with the others. PLE is a rare disorder featured by personality change, seizure, irritability, and depression due to involvement of the limbic system secondary to an autoimmune response to neurons of the brain provoked by the antibodies. LEMS is well known as a classical paraneoplastic syndrome when the neuromuscular junction is affected. In this case, we report a case of paraneoplastic limbic encephalitis (PLE) and Lambert-Eaton myasthenic syndrome (LES) in an elderly Asian male with small cell lung cancer (SCLC). Keywords: Paraneoplastic limbic encephalitis, Lambert-Eaton myasthenic syndrome, small-cell lung cancer, para- neoplastic neurologic syndrome Introduction reported an overlap of PLE and LEMS second- ary to small cell lung cancer (SCLC). Paraneoplastic neurologic syndrome (PNS) is a rare group of disorders caused by nervous sys- Case presentation tem damages in the setting of cancer unrelated A 65-year-old male patient presented to the to the tumor sites. Patients with PNS may pres- Emergency Department of our hospital in March ent symptoms or damages in the central and 2014 with suspected seizure. His family mem- peripheral nervous system, the neuromuscular bers reported the patient showed intermittent junction and muscle, which usually results in episodes of anxiety, confusion and memory severely disabling. Nowadays, paraneoplastic loss, as well as personality change and irritabil- limbic encephalitis (PLE) and Lambert-Eaton ity within 3 weeks before admission. No focal or myasthenic syndrome (LEMS) are the most global neurologic deficits like numbness, weak- commonly reported types of PNS compared ness or cranial nerve abnormalities were identi- with the others. PLE is a rare disorder featured fied. Computed tomography (CT) on brain by personality change, seizure, irritability, and revealed no abnormalities. Brain MRI demon- depression due to involvement of the limbic strated aberrant signals in the left basal gan- system secondary to an autoimmune response glia, based on which chronic lacunar infarction to neurons of the brain provoked by the anti- was considered. Twenty-four hour electroen- bodies [1-3]. LEMS is well known as a classical cephalogram indicated multiple, irregular paraneoplastic syndrome when the neuromus- sharp-slow waves and sharp waves in the mid- cular junction is affected [4-6]. In this case, we dle line of forehead, especially the right side. Overlapping of PLE and LEMS in a cancer patient cell count, 15×106/L; apocyte, 0.07; monocyte, 0.93. On this basis, the patient was diagnosed with limbic encephalitis, and he showed com- plete symptomatic relief after medication of antiepileptics. Two months later, the patient visited our hospital for the routine physical check. Chest computerized tomography (CT) confirmed a right hilar mass with extensive mediastinal involvement (Figure 1), but no dis- tant metastases were identified. The patient was diagnosed with small cell lung cancer (SCLC) according to the transbronchial biopsy (Figure 2). Taken together, we confirmed that Figure 1. CT images of mediastina (A) and the lung the patient suffered from PLE. (B). High density shadows were observed at the infe- rior lobe of right lung in proximity to the pulmonary Subsequently, the patient received one cycle of hilum. The margins were clear and the lobes were chemotherapy (EP regimen). The second cycle distinguished. Soft tissue density was observed in of chemotherapy was performed 3 weeks (June the mediastina. Besides, high density shadows in 2015) later using topotecan. In July, the patient strip-like and nodular patterns were noticed in the showed asthenia, systematic fatigue and dis- inferior lobe of right lung. Further, multiple swollen lymph nodes were identified in the mediastina. ability. Physical examination at the time of admission revealed diminished ability and diffi- culty to sustain upward gaze for more than 30 seconds. It was difficult for the patient to sus- tain hold against gravity of the upper extremi- ties. However, a transient recovery, such as standing up from the bed or seat, was noticed after moderate exercise. Electromyogram was performed, which revealed a decrease of com- pound muscle action potential (56%) upon the stimulation of right ulnar nerve under a fre- quency of 3 Hz. In addition, a 7.3-fold increase was observed in the compound muscle action potential upon the stimulation with a frequency of 20 Hz (Table 1). Taken together, the patient was diagnosed with LEMS. As the diagnosis of SCLC was definite, sequen- tial radiotherapy was performed subsequently with total doses of 5400cGy/27F in pGTVnd and 4860cGy/27F in PTV respectively. Subsequent CT scanning confirmed a posi- tive response to treatment with an elimina- tion of mediastinal metastasis. No ventilation dysfunction was found in lung base throughout the hospitalization fortunately. His cognition and confusion were markedly improved; the Figure 2. Histology from a lung biopsy showing infil- weakness of muscle was not progressed. tration of the right lobe with neoplastic cells, consis- tent with small cell lung cancer (H&E, 100×). Discussion The incidence of PNS is extremely lower with a The cerebrospinal fluid (CSF) analysis results rate of less than 1/10000 [1]. In the majority of were as follows: protein, 0.38 g/L; glucose, these cases, several regions of the nervous 4.17 mmol/L; Cl, 130.5 mmol/L; white blood system were affected including cerebrum, 4918 Int J Clin Exp Pathol 2016;9(4):4917-4920 Overlapping of PLE and LEMS in a cancer patient Table 1. The result of neurophysiological tests with adequate repeti- mediated neurologic disor- tive stimulation: compound motor action potentials (CMAP) ampli- der. The orthodox autoimm- tude was up to a 56% decrement at low frequency repetitive stimu- une antibodies in CSF were lation (3 Hz supramaximal stimulation) and up to 729% increment tested, including anti-HU, an- at high frequency stimulation (20 Hz supramaximal stimulation) ti-R1, and anti-YO. The anti- HU is commonly found in Repetitive electrical stimulation Event Frequency Time Amplitude approximately 50% of the Musculi abductor digiti quinti 1 10@2 Hz 14:05:24 -11.7 PLE cases. Meanwhile, anti- 2 10@2 Hz 14:05:34 -19.4 R1 antibodies are less com- 3 10@3 Hz 14:05:42 -38.4 monly detected, but it sup- 4 10@3 Hz 14:05:49 -56.0 ports the diagnoses of PLE 5 10@5 Hz 14:05:58 -18.6 most peculiarly [7, 8]. Elec- 6 10@5 Hz 14:06:06 -13.3 troencephalography can be a 7 30@20 Hz 14:06:15 729 useful tool to support the diagnosis of PLE, as it dem- onstrate focal or generalized spine, neuromuscular junction and muscle [7]. slow and/or sharp wave predominantly in the To date, PNS is considered as a disease not temporal regions [8, 9]. In a previous study, related to tumor, metastasis, or metabolites Lawn et al summarized the clinical, MRI and due to the presence of anti-neural antibody in electroencephalographic features of PLE (10), these patients [2]. which were cognitive dysfunction (92%), sei- zures (58%) and psychiatric symptoms (50%). Nowadays, the final diagnosis of PNS is largely Serum paraneoplastic neuronal antibodies depend on the essential components, including were identified in a majority (64%) of these clinical symptoms, cancer diagnosed within 4 patients. Abnormal CSF was found in 78% of years after the onset of neurologic symptoms, these patients. In this case, the patient was exclusion of other causes, as well as any of the diagnosed with SCLC, coupled with a distinctive followings: brain MRI showed a lesion in tempo- history of altered personality, cognitive disorder ral lobe and/or brain stem, cerebrospinal fluid and seizure activity that strongly suggested analysis indicating inflammation without posi- PLE. CSF sample was obtained, the results indi- tive cytology, and/or epileptic discharge in tem- cated negative results and no serum neuronal poral lobes by ECG analysis [2]. In this case, the antibodies, and however, such facts could not patient was finally confirmed with PLE and exclude the PLE. LEMS due to the presence of all standards above mentioned. Lambert-Eaton myasthenic syndrome (LEMS) is The most common types of neoplasms associ- a rare but well-known paraneoplastic disorder ated with PLE are SCLC, testicular tumor, thy- featured by fatigability and muscle weakness moma, Hodgkin’s lymphoma and breast cancer predominantly involving the proximal extremity. [3]. Limbic system consisted of hippocampus, In addition, it has been acknowledged as an amygdala, nucleus anterior thalamic and limbic autoimmune disease that harasses the normal- cortex, is essential for the memory, learning, ly credible neurotransmission at the neuromus- and higher emotion. Typically, PLE manifests cular junction. Such disruption is thought to with seizures and changes in memory, mood result from an auto antibody-mediated removal and personality.
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