Paraneoplastic limbic encephalitis associated with lung cancer

Shen, Kaini; Xu, Yuanfu; Guan, Hongzhi; Zhong, Wei; Chen, Minjiang; Zhao, Jing; Li, Longyun; Wang, Mengzhao

doi:10.1038/s41598-018-25294-y

Cited by 30 publications

(40 citation statements)

References 29 publications

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“…For all antibody-defined AIE subgroups combined, we could identify 116 publications that matched our search criteria and contained relevant information for the intended analyses. Ten were identified for AIE with AMPAR antibodies (7–16), 15 for CASPR2 (16–30), eight for DPPX (31–38), four for GABA A R (15, 39–41) with patients reported by the Pettingil et al being excluded as these were not tested for GABA A R antibodies in CSF and represented a different phenotype (42), 15 for GABA B R antibodies (12, 15, 16, 43–54), 15 for GAD antibodies (19, 22, 55–67), five for GlyR antibodies (68–72), 14 for IgLON5 antibodies (73–86), 25 for LGI1 antibodies (5, 6, 15, 16, 19–22, 30, 52, 77, 87–100), and finally 27 for NMDAR antibodies (4, 15, 16, 19, 22, 52, 62, 88, 89, 101–118). The history of the data search and the results are depicted in Figure 1.…”

Section: Resultsmentioning

confidence: 99%

Cerebrospinal Fluid Findings in Patients With Autoimmune Encephalitis—A Systematic Analysis

2019

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Autoimmune encephalitides (AIE) comprise a group of inflammatory diseases of the central nervous system (CNS), which can be further characterized by the presence of different antineuronal antibodies. Recently, a clinical approach for diagnostic criteria for the suspected diagnosis of AIE as well as definitive AIE were proposed. These are intended to guide physicians when to order the antineuronal antibody testing and/or facilitate early diagnosis even prior to the availability of the specific disease-confirming test results to facilitate prompt treatment. These diagnostic criteria also include the results of basic cerebrospinal fluid (CSF) analysis. However, the different antibody-defined AIE subtypes might be highly distinct with regard to their immune pathophysiology, e.g., the pre-dominance of specific IgG subclasses, IgG1, or IgG4, or frequency of paraneoplastic compared to idiopathic origin. Thus, it is conceivable that the results of basic CSF analysis might also be very different. However, this has not been explored systematically. Here, we systematically reviewed the literature about the 10 most important AIE subtypes, AIE with antibodies against NMDA, AMPA, glycine, GABA A , and GABA B receptors as well as DPPX, CASPR2, LGI1, IgLON5, or glutamate decarboxylase (GAD), with respect to the reported basic CSF findings comprising CSF leukocyte count, total protein, and the presence of oligoclonal bands (OCB) restricted to the CSF as a sensitive measure for intrathecal IgG synthesis. Our results indicate that these basic CSF findings are profoundly different among the 10 different AIE subtypes. Whereas, AIEs with antibodies against NMDA, GABA B , and AMPA receptors as well as DPPX show rather frequent inflammatory CSF changes, in AIEs with either CASPR2, LGI1, GABA A , or glycine receptor antibodies CSF findings were mostly normal. Two subtypes, AIEs defined by either GAD, or IgLON5 antibodies, did not fit into this general pattern. In AIE with GAD antibodies, positive OCBs in the absence of other changes were typical, while the CSF in IgLON5 antibody-positive AIE was characterized by elevated protein.

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Section: Resultsmentioning

confidence: 99%

Cerebrospinal Fluid Findings in Patients With Autoimmune Encephalitis—A Systematic Analysis

2019

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“…Onconeural antibodies associated with paraneoplastic LE are Ma, Hu, Ri, CRMP5, and SOX1 [ 66 , 145 ], with Ma2 being the major intracellular antibody causing paraneoplastic LE [ 146 ]. In paraneoplastic LE associated with lung cancer, Hu and cell surface GABA-BR antibodies were the most frequently encountered antibodies, whereas in paraneoplastic LE associated with testicular tumor, Ma2 antibody is the most frequent antibody [ 147 , 148 ].…”

Section: Intracellular Antibodiesmentioning

confidence: 99%

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

Kao

Lin²,

Weng

et al. 2020

IJMS

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Limbic encephalitis (LE) is a rare cause of encephalitis presenting as an acute and subacute onset of neuropsychiatric manifestations, particularly with memory deficits and confusion as core features, along with seizure occurrence, movement disorders, or autonomic dysfunctions. LE is caused by neuronal antibodies targeting the cellular surface, synaptic, and intracellular antigens, which alter the synaptic transmission, especially in the limbic area. Immunologic mechanisms involve antibodies, complements, or T-cell-mediated immune responses in different degree according to different autoantibodies. Sensitive cerebrospinal fluid markers of LE are unavailable, and radiographic findings may not reveal a typical mesiotemporal involvement at neurologic presentations; therefore, a high clinical index of suspicions is pivotal, and a neuronal antibody testing is necessary to make early diagnosis. Some patients have concomitant tumors, causing paraneoplastic LE; therefore, tumor survey and treatment are required in addition to immunotherapy. In this study, a review on the molecular and immunologic aspects of LE was conducted to gain awareness of its peculiarity, which we found quite different from our knowledge on traditional psychiatric illness.

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“…The diagnosis must be supported by the identification of specific neuronal autoantibodies [6]. The management is complex and based on a multidisciplinary and combined approach of immunotherapy and specific anticancer treatment [7–26]. The prognosis is usually poor and strictly related to the subtype of neuronal autoantibodies, clinical patient’s status and comorbidities, and cancer characteristics and stage [24–26].…”

Section: Introductionmentioning

confidence: 99%

A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report

et al. 2019

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BackgroundParaneoplastic limbic encephalitis (PLE) is a rare autoimmune neurological syndrome observed in cancer patients. PLE is difficult to diagnose and presents a variable response to treatment, depending on the characteristics of the tumor and neuronal autoantibodies.Case presentationA 64-year-old, Caucasian, non-smoker man presented with a rapidly developing cognitive impairment, personality change, spatial disorientation, and short-term memory loss associated with anorexia and cervical and inguinal lymph nodes. The 18F-FDG PET scan documented intensely hypermetabolic lymph nodes, which histologically corresponded to a metastasis from a small cell neuroendocrine carcinoma. The brain MRI revealed a high T2-weighted FLAIR signal of the hippocamps, consisted with a PLE. The presence of anti-neuronal Hu antibodies confirmed the diagnosis. The patient underwent plasmapheresis, associated to a systemic chemotherapy resulting in a partial and temporary improvement of the neurological symptoms. Four cycles of intravenous immunoglobulins were also necessary. After six cures of chemotherapy, the lymph node metastases regressed. However, a new anorectal lesion was detected and was histologically confirmed as a primary small cell neuroendocrine carcinoma, which was treated with concomitant chemoradiotherapy. At the end of this treatment, the patient showed a rapid tumor progression leading to his death.ConclusionsThis case highlights the rare entity, PLE, which is difficult to diagnose and manage. In addition, this is the first published case of PLE associated with an anorectal small cell neuroendocrine carcinoma, which appeared after completion of systemic chemotherapy.

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Paraneoplastic limbic encephalitis associated with lung cancer

Cited by 30 publications

References 29 publications

Cerebrospinal Fluid Findings in Patients With Autoimmune Encephalitis—A Systematic Analysis

Cerebrospinal Fluid Findings in Patients With Autoimmune Encephalitis—A Systematic Analysis

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

A paraneoplastic limbic encephalitis from an anorectal small cell neuroendocrine carcinoma: a case report

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