Positive oligoclonal bands and CSF pleocytosis in narcolepsy type 1: A case report supporting the immune-mediated hypothesis

Moreno‐Estébanez, Ana; Villabeitia, I. Bilbao; Guibert, Teresa Echeverria; Bilbao, Mar Mendibe; Durán, Sabas Boyero; Martinez, Laura; González‐Pinto, Tirso; Beitia, Garazi Agirre; Eizaguirre, Amaia González; Rodríguez‐Antigüedad, Alfredo

doi:10.1016/j.jneuroim.2019.577111

Cited by 4 publications

(3 citation statements)

References 29 publications

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“…Interestingly, the CSF pleocytosis gradually disappeared after treatment, but CFS hcrt-1 levels remained unchanged. 79 Among 4 NT1 children with undetectable CSF hypocretin-1, treated with IVIG for 6 months within 1 year from onset, only one case showed a significant reduction of EDS and cataplexy frequency whereas in the others no persistent clinical changes were noted. 80 The short-lived benefit induced by therapy was confirmed by three further pediatric cases, treated close to onset 81 , 82 and in 4 idiopathic adult cases with various disease durations.…”

Section: Immunotherapiesmentioning

confidence: 91%

Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder

Giannoccaro¹,

Liguori²,

Plazzi³

et al. 2021

NSS

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Narcolepsy type 1 (NT1) is a lifelong sleep disorder, primarily characterized clinically by excessive daytime sleepiness and cataplexy and pathologically by the loss of hypocretinergic neurons in the lateral hypothalamus. Despite being a rare disorder, the NT1related burden for patients and society is relevant due to the early onset and chronic nature of this condition. Although the etiology of narcolepsy is still unknown, mounting evidence supports a central role of autoimmunity. To date, no cure is available for this disorder and current treatment is symptomatic. Based on the hypothesis of the autoimmune etiology of this disease, immunotherapy could possibly represent a valid therapeutic option. However, contrasting and limited results have been provided so far. This review discusses the evidence supporting the use of immunotherapy in narcolepsy, the outcomes obtained so far, current issues and future directions.

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Section: Immunotherapiesmentioning

confidence: 91%

Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder

Giannoccaro¹,

Liguori²,

Plazzi³

et al. 2021

NSS

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“…Due to the high affinity between DQA1*0102 and DQBI*0602, there is a strong linkage imbalance, and their DQα and DQβ chains are easily bound to form HLA-DQ dimers, which are susceptible to narcolepsy ( 9 ). DQB1*0602 can increase the risk of NT1 by 200 times ( 10 ), and DQA1*0102, a homologous allele, also has a similar risk ( 11 ). In various races (Japan, South Korea, Caucasians, African Americans), this disease is closely related to the HLA-DQ dimer ( 12–15 ).…”

Section: Introductionmentioning

confidence: 99%

Correlation analysis between HLA-DQA10102/DQB10602 genotypes and narcolepsy patients in China

Zhao,

Zhang,

Yan

et al. 2024

Front. Neurol.

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Background and objectiveAt present, the etiology of narcolepsy is not fully understood, and it is generally believed to be an autoimmune reaction caused by interactions between environmental and genetic factors. Human leukocyte antigen (HLA) class II genes are strongly associated with this gene, especially HLA-DQB1*0602/DQA1*0102. In this study, we mainly analyzed the correlation between different genotypes of HLA-DQB1*0602/DQA1*0102 and clinical manifestations in Chinese patients with narcolepsy.Experimental methodNarcolepsy patients who were treated at the Department of Neurology, The First Affiliated Hospital of Shandong First Medical University from January 2021 to September 2023 were selected. General information, sleep monitoring data, cerebrospinal fluid (CSF) orexin levels, and human leukocyte antigen gene typing data were collected. The statistical analysis was performed using SPSS 26.0, and the graphs were drawn using GraphPad Prism 9.5.Main resultsA total of 78 patients were included in this study. The DQA1 and DQB1 gene loci were detected in 54 patients, and only the DQB1 gene locus was detected in 24 narcoleptic patients. The most common allele at the HLA-DQB1 locus was *0602 (89.7%), and the most common genotype at this locus was *0602*0301 (19.2%), followed by *0602*0602 (17.9%). The most common phenotype of the HLA-DQA1 locus is *0102 (92.6%), and the most common genotype of this locus is *0102*0102 (27.8%), followed by *0102*0505 (14.8%). There were significant differences (p < 0.05) between HLA-DQB1*0602-positive and HLA-DQB1*0602-negative patients in terms of orexin-A levels, presence or absence of cataplexy, UNS, PSG sleep latency, REM sleep latency, N1 sleep percentage, oxygen depletion index, and average REM latency on the MSLT. The HLA-DQA1*0102-positive and HLA-DQA1*0102-negative patients showed significant differences (p < 0.05) in disease course, presence or absence of sudden onset, PSG REM sleep latency, N1 sleep percentage, and average REM latency on the MSLT. There were significant differences in the average REM latency of the MSLT between HLA-DQB1*0602/DQA1*0102 homozygous and heterozygous patients p < 0.05, and no differences were found in the baseline data, orexin-A levels, scale scores, or other sleep parameters.ConclusionDifferent genotypes of HLA-DQA1*0102/DQB1*0602 are associated with symptoms of cataplexy in Chinese narcoleptic patients. Homozygous individuals have a shorter mean REM latency in the MSLT, greater genetic susceptibility, and relatively more severe sleepiness.

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“…[32] The HLA allele DQAI*01:02 is in strong linkage disequilibrium with DQBI*06:02 and carries a similar risk. [33] DPBI*05:01 also increases the risk of NT1, although the results have a smaller impact. [34] In contrast, some other class II alleles had the greatest effect on narcolepsy risk, but 2 studies showed a small and independent effect of class I alleles.…”

Section: Introductionmentioning

confidence: 99%

TCM syndrome differentiation and treatment of narcolepsy based on neurobiological mechanism: A review

2022

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Narcolepsy is a relatively rare brain disorder caused by the selective loss of orexin neurons. Narcolepsy is divided into Narcolepsy Type 1 (NT1) and Narcolepsis Type 2 (NT2). The pathogenesis of NT1 has been well established due to the severe loss of orexin neurons, while NT2 is still poorly understood, and little is known about its underlying neurobiological mechanisms. human leukocyte antigen alleles have been found to strongly influence the development of narcolepsy, with more than 90% of NT1 patients carrying the human leukocyte antigen II allele DQB1*06:02. In addition to the genetic evidence for the DQBI*06:02 allele, some other evidence suggests that a T cell-mediated immune mechanism destroys the orexin neurons of NT1, with CD4 + T cells being key. For this disease, traditional Chinese medicine (TCM) therapy has its own characteristics and advantages, especially the combination of acupuncture and medicine in the treatment of this disease in TCM, which has made considerable and gratifying progress. The purpose of this review is to introduce the frontier progress of neurobiology of narcolepsy, and to explore the syndrome differentiation and treatment of narcolepsy with the combined use of TCM and Western medicine combined with TCM.

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Positive oligoclonal bands and CSF pleocytosis in narcolepsy type 1: A case report supporting the immune-mediated hypothesis

Cited by 4 publications

References 29 publications

Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder

Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder

Correlation analysis between HLA-DQA10102/DQB10602 genotypes and narcolepsy patients in China

TCM syndrome differentiation and treatment of narcolepsy based on neurobiological mechanism: A review

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Positive oligoclonal bands and CSF pleocytosis in narcolepsy type 1: A case report supporting the immune-mediated hypothesis

Cited by 4 publications

References 29 publications

Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder

Reviewing the Clinical Implications of Treating Narcolepsy as an Autoimmune Disorder

Correlation analysis between HLA-DQA1*0102/DQB1*0602 genotypes and narcolepsy patients in China

TCM syndrome differentiation and treatment of narcolepsy based on neurobiological mechanism: A review

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Correlation analysis between HLA-DQA10102/DQB10602 genotypes and narcolepsy patients in China