Prognostic factors in anti-neuronal antibody positive patients

Aydın, Çağla; Çelik, Şenay Yıldız; İçöz, Sema; Ulusoy, Canan; Gündüz, Tuncay; Demir, Gülşen Akman; Kürtüncü, Murat; Tüzün, Erdem

doi:10.29399/npa.23033

Cited by 7 publications

(7 citation statements)

References 21 publications

(38 reference statements)

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“…To the best of our knowledge, no studies have reported that GTCS is a protective or a risk factor regarding the prognosis of encephalitis; however, we found that the "complete recovery" group had a higher incidence of GTCS, which needs further verification. In anti-NMDAR encephalitis, de Montmollin et al found that the increased white blood cells in CSF indicated good neurological outcomes, but the study was performed in patients requiring ICU admission (38); others found a correlation between abnormal CSF (only pleocytosis and increased protein levels) and poor outcomes based on univariate analyses, but this result barely reached significance with a p-value of 0.049, and the results of their multivariate regression analysis were not described (36); and other reports described that the OB-included CSF findings had no relationships with clinical outcomes in patients with anti-NMDAR encephalitis or anti-neuronal antibody-associated encephalitis (39,40). The "unhealed" group of patients also had a higher incidence of psychiatric disturbance (p = 0.053) than the "complete recovery" group, however, there have been no studies focusing on this factor.…”

Section: Discussionmentioning

confidence: 98%

Clinical Characteristics and Long-Term Prognosis of Anti-LGI1 Encephalitis: A Single-Center Cohort Study in Beijing, China

Zhang

Wang

et al. 2021

Front. Neurol.

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Background: This study aimed to analyze the clinical characteristics of anti-leucine-rich glioma-inactivated protein 1 (LGI1) encephalitis patients and investigate prognostic factors by using a large-sample and long-term follow-up cohort.Methods: The clinical data of 45 patients (29 males; mean age, 57.0 years) from May 2014 to August 2019 were collected. All patients were followed up by face-to-face interviews in the third month after discharge and then by telephone and/or face-to-face interviews every 6 months until November 2020. We evaluated each patient's response to the initial treatments at the first interview and divided them into “responders” and “nonresponders.” Relapses were recorded. At the end of follow-up, each patient was evaluated and reclassified into “complete recovery” or “unhealed” groups. Intergroup differences were assessed.Results: All patients presented with seizures at the initial consultation. Other common manifestations included cognitive dysfunction (82.2%), psychiatric disturbance (66.7%), sleep disorder (54.5%), and hyponatremia (66.7%). During the follow-up period (32.8 ± 13.5 months), six patients experienced relapse within 6–37 months. We observed that the patients who did not respond to the initial treatments and those who relapsed all had a poor long-term prognosis. The patients in the “unhealed” group were older (p = 0.009), had a lower incidence of generalized tonic–clonic seizures (p = 0.041), and had a higher probability of cerebrospinal fluid (CSF) abnormalities (p = 0.024) than those in the “complete recovery” group.Conclusion: Anti-LGI1 encephalitis was characterized by seizures, cognitive impairment, psychiatric disturbance, and sleep disorders and was often accompanied by hyponatremia. Patients who responded poorly to the initial treatments and those patients who relapsed had dismal long-term prognoses. Advanced age and CSF abnormalities may be risk factors for poor prognosis, but these still need to be verified.

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Section: Discussionmentioning

confidence: 98%

Clinical Characteristics and Long-Term Prognosis of Anti-LGI1 Encephalitis: A Single-Center Cohort Study in Beijing, China

Zhang

Wang

et al. 2021

Front. Neurol.

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“…Lung cancer has also been linked to SPSSD. This has been reported in nine studies, involving six SPSSD subtypes, including classic SPS ( 66 – 69 ), subacute sensory neuronopathy, subacute cerebellar degeneration ( 60 ), paraneoplastic neurologic syndromes ( 70 ), and PERM ( 71 – 73 ) ( Table 2 ). Sinha et al reported that thymoma coexists with lung cancer and SPSSD ( 76 ).…”

Section: Clinical Characteristics Of Malignant Tumor-related Spssdsmentioning

confidence: 77%

“…Breast cancer is the most common carcinoma linked to SPSSDs. Table 1 shows that from 29 studies on breast cancer, six SPSSD subtypes, including classic SPS ( 21 , 30 , 37 – 47 , 50 , 63 , 64 ), SLS ( 57 , 58 , 65 ), paraneoplastic cerebellar degeneration ( 59 ), subacute sensory neuronopathy, subacute cerebellar degeneration ( 60 ), and PERM ( 61 ), among which classic SPS is the major SPSSD subtype, were found to be involved. Patients with breast cancer and PSSD were determined to have other carcinomas, such as colon cancer, non-Hodgkin lymphoma, thymoma and lymphoma, and malignant melanoma ( 21 , 34 , 39 , 41 ).…”

Section: Clinical Characteristics Of Malignant Tumor-related Spssdsmentioning

confidence: 99%

An update on malignant tumor-related stiff person syndrome spectrum disorders: clinical mechanism, treatment, and outcomes

Peng,

Yang,

Xue

et al. 2023

Front. Neurol.

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Stiff person syndrome (SPS) is a rare central nervous system disorder associated with malignancies. In this review, we retrieved information from PubMed, up until August 2023, using various search terms and their combinations, including SPS, stiff person syndrome spectrum disorders (SPSSDs), paraneoplastic, cancer, and malignant tumor. Data from peer-reviewed journals printed in English were organized to explain the possible relationships between different carcinomas and SPSSD subtypes, as well as related autoantigens. From literature searching, it was revealed that breast cancer was the most prevalent carcinoma linked to SPSSDs, followed by lung cancer and lymphoma. Furthermore, classic SPS was the most common SPSSD subtype, followed by stiff limb syndrome and progressive encephalomyelitis with rigidity and myoclonus. GAD65 was the most common autoantigen in patients with cancer and SPSSDs, followed by amphiphysin and GlyR. Patients with cancer subtypes might have multiple SPSSD subtypes, and conversely, patients with SPSSD subtypes might have multiple carcinoma subtypes. The first aim of this review was to highlight the complex nature of the relationships among cancers, autoantigens, and SPSSDs as new information in this field continues to be generated globally. The adoption of an open-minded approach to updating information on new cancer subtypes, autoantigens, and SPSSDs is recommended to renew our database. The second aim of this review was to discuss SPS animal models, which will help us to understand the mechanisms underlying the pathogenesis of SPS. In future, elucidating the relationship among cancers, autoantigens, and SPSSDs is critical for the early prediction of cancer and discovery of new therapeutic modalities.

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“…Although there is a robust anti‐Zic4 association with PNS in SCLC, 6 scarce case‐reports of anti‐Zic4 positivity exist in relation to breast and ovarian cancer, 8 with pure anti‐Zic4 PNS in endometrial cancer being oncologically unique 9 …”

Section: Discussionmentioning

confidence: 99%

“…Cases of cerebellar dysfunction mixed with other neurological deficits have only been described in patients with the concurrent presence of anti-Zic4 and other onconeural antibodies (anti-Hu, -Yo, -CRMP5). 6 Although there is a robust anti-Zic4 association with PNS in SCLC, 6 scarce case-reports of anti-Zic4 positivity exist in relation to breast and ovarian cancer, 8 with pure anti-Zic4 PNS in endometrial cancer being oncologically unique. 9 What is most intriguing from the neuroimmunological perspective is the recent history of CNS CMV infection, and its possible contribution to immunogenicity.…”

mentioning

confidence: 99%

Anti‐Zic4 encephalitis in endometrial cancer: Autoimmunity triggered by PD‐1 inhibitor or recent CMV encephalitis?

Filippakopoulou

Karachalia

Gryllia

et al. 2022

Clinical & Exp Neuroim

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Background: Autoimmune encephalitis has been well described in oncological patients treated with immune checkpoint inhibitors (ICIs), as a paraneoplastic neurological syndrome. Central nervous system (CNS) infections from herpes viruses are also known to trigger secondary neurologic autoimmunity.Case Presentation: We hereby describe a patient with a recent history of cytomegalovirus (CMV) CNS infection and ongoing immunotherapy with the programmed-celldeath-protein-1(PD-1) inhibitor pembrolizumab for metastatic endometrial cancer.The patient presented with progressive encephalopathy along with cerebellar signs, rapidly evolving to status epilepticus requiring mechanical ventilation and advanced care unit (ACU) support. A serum paraneoplastic antibody panel revealed strongly positive anti-Zic4 antibodies. Despite first-line immunotherapy and aggressive anticonvulsant treatment, the patient died a few days postintubation. Conclusion:In anti-Zic4 encephalitis, both PD-1 inhibitor treatment and recent CMV viral insult may contribute to the development of neurological autoimmunity. Further research is needed to identify ways of predicting neurological damage in oncological patients treated with ICIs.

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Prognostic factors in anti-neuronal antibody positive patients

Abstract: NMDAR, Hu and Ma2-antibodies were the most prevalent ANA in this first antibody screening study in a Turkish cohort. Antibody type was determined to be the foremost prognostic factor in ANA seropositive cases.

Cited by 7 publications

References 21 publications

Clinical Characteristics and Long-Term Prognosis of Anti-LGI1 Encephalitis: A Single-Center Cohort Study in Beijing, China

Clinical Characteristics and Long-Term Prognosis of Anti-LGI1 Encephalitis: A Single-Center Cohort Study in Beijing, China

An update on malignant tumor-related stiff person syndrome spectrum disorders: clinical mechanism, treatment, and outcomes

Anti‐Zic4 encephalitis in endometrial cancer: Autoimmunity triggered by PD‐1 inhibitor or recent CMV encephalitis?

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