Published online Mar 5, 2026. doi: 10.4331/wjbc.v17.i1.117645
Revised: January 15, 2026
Accepted: February 2, 2026
Published online: March 5, 2026
Processing time: 83 Days and 15 Hours
The diagnosis of immunoglobulin G4 (IgG4)-related autoimmune neuropathies relies on a combination of clinical evaluation, imaging, and biological analyses, including serum and cerebrospinal fluid assessments. Several IgG4 autoantibodies have been described in these disorders, including muscle-specific kinase IgG4, leucine-rich glioma-inactivated 1 IgG4, nodo-paranodal IgG4, Ig-like domain-containing protein 5, anti-dipeptidyl-peptidase-like protein-6 antibodies, and contactin-associated protein-like 2 IgG4. Accurate identification of these autoan
Core Tip: Human leukocyte antigen (HLA) class II molecules present peptides derived from neuronal and glial membrane proteins to CD4+ T cells, particularly follicular helper T cells and interleukin (IL)-10-producing regulatory subsets. In the IL-10-rich milieu associated with chronic antigen exposure, B cells undergo immunoglobulin G4 (IgG4) class switching and differentiate into high-affinity plasma cells and memory cells, thereby driving the production of pathogenic IgG4 neuro-autoantibodies. These antibodies disrupt protein-protein interactions rather than inducing classical inflammatory responses. IgG4 neuro-autoantibodies are mainly detected in serum and cerebrospinal fluid using cell-based assays, although these assays are labor-intensive and require specialized cell culture and transfection facilities. HLA typing may have clinical relevance in patients with intrathecal IgG4 antibodies and may help explain specific phenotypic associations in anti-Ig-like domain-containing protein 5 disease, including the increased frequency of sleep and bulbar manifestations. Distinct HLA associations also underlie the divergent clinical profiles observed in leucine-rich glioma-inactivated 1 and contactin-associated protein-like 2 antibody diseases.
- Citation: Bouayad A. Clinical utility of human leukocyte antigen genotyping and immunoglobulin G4 autoantibody testing in autoimmune neurological diseases: A focused minireview. World J Biol Chem 2026; 17(1): 117645
- URL: https://www.wjgnet.com/1949-8454/full/v17/i1/117645.htm
- DOI: https://dx.doi.org/10.4331/wjbc.v17.i1.117645
Autoimmune neuropathies arise from a breakdown of immunological tolerance to components of the central nervous system (CNS) and the peripheral nervous system (PNS), including myelin, Schwann cells, axons, and motor or autonomic ganglionic neurons[1-3]. In a subset of these disorders, compelling evidence supports antibody-mediated autoimmunity driven by immunoglobulin G4 (IgG4) autoantibodies that target extracellular neuronal and glial antigens[4,5]. IgG4 is the least abundant IgG subclass in serum, accounting for approximately 1%-4% of total circulating IgG[6]. Notably, patients with IgG4-mediated autoimmune neuropathies typically lack the characteristic features of IgG4-related disease (IgG4-RD), such as marked serum IgG4 elevation, tissue fibrosis, and infiltration by IgG4-positive plasma cells[4,7]. The detection of increased IgG4 levels and IgG4 oligoclonal bands in cerebrospinal fluid (CSF) may reflect blood-brain barrier dysfunction or intrathecal immune activation[8,9]. However, these markers lack sufficient specificity and sensitivity as diagnostic markers for IgG4-mediated autoimmune neurological disorders, where diagnosis primarily relies on detecting antigen-specific autoantibodies rather than on CSF IgG4 patterns[10]. Therefore, diagnosis of IgG4-mediated autoimmune neurological disorders indeed relies more heavily on detecting specific pathogenic IgG4 autoantibodies rather than CSF IgG4 patterns.
Pathogenic IgG4 autoantibodies have been identified against muscle-specific kinase (MuSK)[11,12], leucine-rich glioma-inactivated 1 (LGI1)[13], contactin-1 (CNTN1)[14], neurofascin-155 (NF155)[15], contactin-associated protein 1 (Caspr1)[16], contactin-associated protein-like 2 (CASPR2)[17], Ig-like domain-containing protein 5 (IgLON5)[18], and dipeptidyl-peptidase-like protein-6 (DPPX)[19,20] in various autoimmune neuropathies. Both human leukocyte antigen (HLA) class II loci[21-24] and environmental factors, including infections and alterations in the gut microbiota[20,25], have been implicated in the pathogenesis of these disorders. However, the mechanisms underlying these associations remain unclear. HLA class II molecules typically bind peptides derived from extracellular neuronal and glial antigens and present them to follicular helper T (Tfh) cells, which play a central role in promoting IgG4 autoantibody responses[26,27]. Several studies have demonstrated that dysregulated Tfh cell activity, particularly within the Tfh2 subset, promotes plasmablast differentiation and IgG4 class switching in IgG4-RD[28,29]. Nevertheless, the direct contribution of autoreactive B cells to the pathogenesis of IgG4-mediated autoimmune neuropathies remains poorly defined and requires further investigation.
Detection of IgG4 autoantibodies has become central to the diagnosis and stratification of neurological disorders, particularly in patients refractory to standard anti-inflammatory therapies, including intravenous immunoglobulin (IVIg), immunosuppressants, and plasmapheresis[5]. While neural autoantibody testing does not replace comprehensive evaluation, including clinical, imaging, electrophysiological, and CSF assessments, it can, when properly applied and interpreted, significantly enhance diagnostic precision in autoimmune neurological disorders[30].
In light of the rapidly advancing understanding of autoantibody-mediated mechanisms in neurological disease, this minireview summarizes current knowledge regarding the role of HLA alleles in regulating IgG4 autoantibody pro
Myasthenia gravis (MG) is a heterogeneous disorder of the neuromuscular junction. Approximately 7% of patients with acetylcholine receptor-negative MG have IgG4 autoantibodies targeting MuSK, a postsynaptic protein. These antibodies reflect defects in both central and peripheral B-cell tolerance, allowing autoreactive B cells to persist and differentiate into plasma cells[31]. The circulating B-cell repertoire in MuSK MG is markedly distorted[32]. It originates from an abnormal naive B-cell pool characterized by biased immunoglobulin heavy chain (IGHV) gene usage, marked by reduced IGHV3 and increased IGHV1 and IGHV4 expression, shaped by defective tolerance checkpoints, and subsequently expands into the memory compartment after activation[32]. IgG4 antibodies can undergo fragment antigen-binding (Fab) arm exchange, leading to bispecific antibodies[32]. The pivotal importance of class-switched, high-affinity IgG4 autoantibodies in the pathophysiology of MuSK MG implies cooperation between autoreactive B cells and CD4+ T lymphocytes[11]. Activation of autoreactive CD4+ T cells necessitates recognition of neural-specific peptides presented by HLA class II molecules to T-cell receptors, thereby implicating HLA class II alleles as key susceptibility factors (Figure 1). This hypothesis is supported by genetic studies across diverse ethnic populations, which have identified specific HLA-DRB1 and DQB1 genes as risk factors for the development of IgG4 autoantibodies. The higher IgG4 titres of MuSK auto
IgLON5 is a neuronal cell adhesion molecule that plays key roles in cell adhesion and signaling through interactions with cytoskeletal proteins. Antibodies targeting IgLON5 are predominantly of the IgG4 subclass, as demonstrated by immunostaining of rat hippocampal tissue[38]. These autoantibodies constitute the immunological hallmark of the disease and are typically detected in both serum and CSF[39].
Anti-IgLON5 antibodies have been significantly associated with the HLA-DRB1*10:01 allele[40,41]. HLA class II alleles may present IgLON5-derived peptides with specific binding affinities that enhance antibody production, or present them in distinct temporal or contextual settings, resulting in stronger antibody responses (Figure 1). In silico predictions identified two IgLON5 peptides (LRLLAAAAL and IVHVPARIV) potentially presented by HLA-DRB1 molecules, particularly HLA-DRB1*10:01, to T cells, thereby initiating or sustaining the autoimmune response in anti-IgLON5 disease[23]. Although the helper T cell and the B cell must interact with parts of the same antigen, they do not need to recognize the same epitope, a phenomenon known as linked recognition[42]. Furthermore, anti-IgLON5 disease shows a strong association with specific HLA-DQ alleles, particularly DQB1*05:01-DQA1*01:01, DQB1*05:01-DQA1*01:05, and DQB1*05:03-DQA1*01:04, implicating HLA-DQ-mediated antigen presentation and T-cell activation as key mechanisms in the initiation of autoimmunity[41,43,44]. HLA-DQB1*05 subtypes may mediate risk more directly than DRB1*10:01 due to LD[43]. Patients with the HLA-DRB1*10:01 allele showed increased serum anti-IgLON5 IgG levels[39]. Interestingly, Koneczny et al[45] demonstrated that patients carrying the HLA-DRB1*10:01 allele may exhibit enhanced antibody production within the brain and spinal cord (intrathecal synthesis) in anti-IgLON5 disease. Therefore, HLA typing may be clinically relevant in patients with intrathecal IgG4 anti-IgLON5 antibodies. Individuals lacking CSF antibodies are more likely to develop a progressive supranuclear palsy-like phenotype and less frequently carry the HLA-DRB1*10:01- DQB1*05:01 haplotype[23]. This rare haplotype, present in less than 3% of the general population, therefore supports the diagnosis of anti-IgLON5 disease[23].
Anti-IgLON5 disease is characterized by progressive brainstem dysfunction, gait instability, sleep disturbances, cognitive decline, and movement disorders[40]. Sleep abnormalities in this condition typically include parasomnias, stridors, and obstructive apnea[46]. The HLA-DRB1*10:01 allele may explain associations with certain clinical features of anti-IgLON5 disease, such as a higher frequency of sleep and bulbar symptoms[23]. Unlike most other autoimmune disorders, anti-IgLON5 disease predominantly affects older individuals, with onset before the age of 50 being uncommon[18]. Moreover, patients carrying specific HLA-DQ risk alleles tend to develop the disease later in life[43].
Expression of CASPR2 and LGI1 occurs in both the CNS and PNS, including the dorsal root ganglia and peripheral nerves. Anti-LGI1 is one of the common neuronal-surface autoantibodies in autoimmune encephalitis affecting adults over 40 years of age, with an incidence of 0.8 per million per year in the Dutch population[47]. Patients with anti-LGI1 and anti-CASPR2 antibodies exhibit marked clinical heterogeneity. Genome-wide and replication studies have shown a strong association between anti-LGI1 encephalitis and the HLA-DRB1*07:01-DQA1*02:01-DQB1*02:02 haplotype[48]. Similarly, anti-LGI1 encephalitis has been linked to the DRB1*07:01-DQB1*02:02 haplotype in an independent cohort[24]. In addition, another study, besides replicating the above-mentioned HLA association with LGI1-mediated encephalitis, described an additional association between the HLA-DRB1*11:01-DQA1*05:01-DQB1*03:01 haplotype and anti-CASPR2 encephalitis[22]. Antibodies against LGI1 are most commonly linked to epilepsy and limbic encephalitis, whereas antibodies against CASPR2 are associated with Morvan syndrome, neuropathic pain, and neuromyotonia[5]. These differences in the clinical heterogeneity of patients with anti-LGI1 and CASPR2 antibodies may depend on the HLA genotype[44]. Recent evidence suggests that HLA genetics may help stratify clinical subtypes of CASPR2 autoimmunity, with HLA-DRB1*11:01 allele enriched specifically among patients with limbic encephalitis[44,49,50]. In silico analyses revealed distinct CASPR2-derived peptides with a high likelihood of presentation by the overrepresented HLA-DQA1*05:01-DQB1*03:01 heterodimer[22].
Together, these data indicate that distinct HLA associations underlie the clinical divergence of LGI1- and CASPR2-antibody diseases: HLA-DRB1*07:01 is linked to LGI1 encephalitis, whereas HLA-DRB1*11:01 specifically enriches CNS forms of CASPR2 autoimmunity, highlighting allele-specific immune pathways that drive phenotype variation.
IgG4 anti-NF155 antibodies are predominantly detected in a subset of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) known as autoimmune nodopathy[51]. These autoantibodies target the glial isoform of NF155, which is expressed in the paranodal loops of Schwann cells[27,52]. The prevalence of these autoantibodies ranges from 1% to 21% among CIDP patients, depending on the population studied and the detection method used[27]. Anti-NF155-associated autoimmune nodopathy primarily affects young men and is characterized by cerebellar-like tremor, distal motor involvement, and ataxia[53-55]. Patients often exhibit a poor response to IVIg therapy[56].
The mechanisms underlying the production of IgG4 anti-NF155 antibodies remain incompletely understood. Their presence in serum reflects impaired central and peripheral B-cell tolerance, allowing the persistence of autoreactive memory B cells and plasma cells that continuously secrete pathogenic antibodies[2] (Figure 1). Recent single-cell analyses suggest that impaired development of naïve B cells in NF155-mediated autoimmune nodopathy may result from defective BCR signaling and dysregulated CD4+ T cell function[57]. Circulating autoreactive B cells are detectable in patients with antibodies targeting nodal and paranodal proteins, including CNTN1, CASPR1, and most prominently NF155[2]. Several studies have also reported associations between anti-NF155 IgG4 autoantibodies and HLA class II alleles, specifically DRB1*15 and the DRB1*15-DQB1*06 haplotype, across different ethnic populations[58-60]. These findings suggest that cooperation between autoreactive B cells and CD4+ T lymphocytes may drive the generation of IgG4 anti-NF155 antibodies (Figure 1). Presentation of NF155 peptides by HLA class II molecules, particularly DRB1*15:01, DRB1*15:02, DQA1*01:02-DQB1*06:02, and DQA1*01:03-DQB1*06:01, activates naïve T cells and promotes Tfh2/Th1 cell-differentiation (Figure 1)[27]. Tfh2 lymphocytes promote IgG4 class-switch recombination through interleukin (IL)-4, IL-13, and IL-10 secretion[27]. The NF155 epitope with the highest affinity for presentation by HLA class II molecules corresponds to the peptide spanning amino acids 336-344[59].
IgG4 autoantibodies do not activate the complement system or engage activating Fcγ receptors on immune cells. IgG4 neuro-autoantibodies cause autoimmune neurological diseases primarily through functional disruption of protein-protein interactions, rather than complement activation or classical inflammation[61]. Their pathophysiological effects fall into three main categories (Figure 2):
Autoantibodies targeting trans-synaptic protein complexes impair synaptic transmission through distinct but interconnected mechanisms. Anti-MuSK antibodies interfere with the MuSK-low-density lipoprotein receptor-related protein 4 (LRP4) interaction, preventing agrin-mediated clustering of acetylcholine receptors at the neuromuscular junction[62-64]. This disruption leads to disorganized postsynaptic architecture and reduced responsiveness to acetylcholine[64,65]. Similarly, anti-LGI1 antibodies disrupt the LGI1-ADAM22/ADAM23 complex, impairing postsynaptic anchoring of AMPA receptors and altering presynaptic Kv1 potassium channel function, thereby contributing to limbic encephalitis[66,67]. Finally, anti-CASPR2 antibodies inhibit the interaction between Caspr2 and contactin-2[17], leading to aberrant Kv1 channel distribution and neuronal hyperexcitability[68], which clinically manifests as Morvan’s syndrome or peripheral nerve hyperexcitability.
IgG4 anti-NF155 antibodies target neurofascin-155 in nerve terminals and roots where the blood-nerve barrier is absent or permeable[69]. These antibodies prevent the interaction between glial NF155 and the axonal contactin-1/Caspr1 complex that normally forms a septate-like junction anchoring myelin loops to the axon[26]. This binding leads to the disassembly of the paranodal junctions and disruption of the paranodal architecture[26]. The disruption leads to loss of ion channel segregation, paranode dismantling, and Schwann cell terminal loop detachment from axons[70]. The pathogenic effect is characterized by reduced Nfasc155 protein levels and impaired formation of the paranodal complex in neonatal animals[69].
Anti-IgLON5 antibodies bind persistently to neuronal cell-surface IgLON5, predominantly expressed in the hypothalamic and brainstem regions, triggering acute neuronal hyperactivity[38]. Prolonged neuronal dysfunction may subsequently lead to tau hyperphosphorylation and accumulation as a downstream neurodegenerative process, particularly in hypothalamic and brainstem neurons, resulting in the characteristic sleep, bulbar, and motor dysfunctions of anti-IgLON5 disease[71-73].
The detection of anti-MuSK antibodies primarily relies on cell-based immunofluorescence assays (CBA-IFA), which are considered viable alternatives to the traditional radioimmunoprecipitation assay (RIA) due to their commercial availability, high sensitivity, specificity, and ability to detect conformationally preserved epitopes[74,75]. Fixed CBA-IFA can serve as a first-step diagnostic test, whereas live CBA-IFA is useful for confirming serum-negative cases identified by RIA and fixed CBA-IFA[76]. However, these assays are time-consuming because they require cell culture facilities and the use of transfected or genetically engineered cell lines. Enzyme-linked immunosorbent assay (ELISA) can be used for the quantitative measurement of MuSK-IgG in patient serum[77]. This immunoassay offers several advantages, including wide accessibility, low cost, simple laboratory procedures, and high specificity, making it easier to implement in routine clinical laboratories, especially when RIA or cell-based assays (CBA) is not available[75]. The use of IgG subclass-specific secondary antibodies in CBA allows identification of IgG4 predominance, which is diagnostically significant in MuSK-MG[78]. Anti-MuSK antibodies primarily recognize the Ig-like domain 1 of the MuSK epitope[12]. Functional assays, including the solid-phase binding assay and the tyrosine phosphorylation assay, are essential for demonstrating the pathogenic activity of MuSK autoantibodies. The solid-phase binding assay directly assesses the inhibition of the MuSK-Lrp4 interaction by IgG4 antibodies, while the tyrosine phosphorylation assay evaluates the blockade of agrin-induced MuSK phosphorylation[12].
IgLON5 autoantibodies are usually detected in serum and CSF using both CBA and immunohistochemistry (IHC)[71]. This approach provides optimal diagnostic sensitivity and specificity[79]. The CBA, employing indirect immunofluorescence on HEK293 cells expressing full-length human IgLON5, provides high specificity and enables subclass analysis, confirming the predominance of IgG4 antibodies[80]. IgLON5 antibodies recognize non-glycosylated epitopes in the Ig-like domain 2 of IgLON5 antigen[80]. IHC on rat hippocampal sections provides confirmatory diagnostic evidence by demonstrating neuronal surface binding of patient antibodies[18]. IgG4 binds to IgLON5 but does not induce internalization, as it is functionally monovalent[80].
The detection of IgG4 subclass of LGI1 and CASPR2 autoantibodies primarily relies on live CBA-IFA[81]. Commercial CBAs offer a rapid, specific, and reproducible diagnostic tool, with strong inter-observer agreement[82,83]. However, these assays have some limitations, including false-negative results and reduced sensitivity for certain autoantibodies, particularly in CSF samples[79,84]. Most studies indicate that LGI1 and CASPR2 antibodies are generally more readily detected in serum than in CSF[13,47,81,85]. Nevertheless, serum antibody positivity may not always correlate with clinical outcomes, underscoring the importance of interpreting results in the context of clinical presentation[86]. IHC on rodent brain tissue may be used as a screening test, showing characteristic hippocampal staining patterns, but it lacks subclass specificity[87].
Detection of anti-NF155 antibodies requires assays that maintain the native conformation structure of the glial isoform. CBA using HEK293 or COS7 cells expressing human NF155 are considered the most reliable diagnostic tools[88]. These assays enable IgG subclass determination and reduce cross-reactivity with other neurofascin isoforms, including NF140 and NF186[89]. Immunofluorescence using teased nerve fibers or paranodal regions of peripheral nerve tissue can confirm antibody localization but should be interpreted in conjunction with CBA results. ELISA can be used as a confirmatory method to quantify antibody titers and identify IgG subclasses[53,60]. Anti-NF155 antibody titers are informative for tracking disease progression, with studies demonstrating their association with clinical severity scores and serum neurofilament levels[60].
First-line treatment of IgG4-mediated neurological autoimmune diseases primarily consists of glucocorticoids (GCs), IVIg, and plasma exchange (PE)[90]. Short courses of high-dose GCs represent the most commonly used first-line acute therapy for MuSK-MG and anti-LGI1/anti-CASPR2 autoimmune encephalitis, often producing rapid initial clinical improvement[91,92]. However, their effectiveness is limited by significant adverse effects, including weight gain, hypertension, hyperglycaemia, and opportunistic infections, as well as by the development of refractory disease in a subset of patients[93].
IVIg has proven to be an effective anti-inflammatory and immunomodulatory therapy in anti-LGI1/anti-CASPR2 autoimmune encephalitis[92] and in DPPX antibody-mediated disease[94]. IVIg is frequently administered in combination with GCs as part of first-line treatment[95]. However, IVIg appears to be less effective in anti-NF155 autoimmune nodopathy[53,60] and in MuSK-MG[91]. This limited efficacy likely reflects the fact that IVIg targets inflammatory mechanisms largely irrelevant to IgG4 pathology and cannot efficiently neutralize or eliminate IgG4 autoantibodies[96].
PE is typically used following GCs and IVIg[90]. Patients receiving first-line immunotherapy combining PE with GCs, with or without IVIg, demonstrate higher recovery rates compared with those receiving regimens without GCs[90]. PE is a well-established and effective treatment for myasthenic exacerbations and crises[91] and appears to be more effective than IVIg in MuSK-MG[97]. PE is also effective in treating LGI1- and CASPR2-associated encephalitis[98].
Second-line treatment of IgG4-mediated neurological autoimmune diseases primarily consists of anti-CD20 agents and cyclophosphamide (CYC), although oral antimetabolite agents such as mycophenolate mofetil and azathioprine may also be considered[90,95,96]. The central role of autoreactive B cells and their cooperation with Tfh cells in driving IgG4 autoantibody production further supports the use of B-cell-targeted therapies in cases of refractory or relapsing disease. Rituximab (RTX) suppresses short-lived plasma cells and their CD20+ precursors involved in IgG4 production, leading to marked reductions in autoantibody titers across several IgG4 autoantibody-mediated neurological disorders, including MuSK-MG, CIDP with anti-NF155 antibodies, and autoimmune encephalitis associated with antibodies targeting the Ranvier paranode and juxtaparanode[99-101]. Clinical guidelines recommend early use of RTX when initial standard immunosuppressive therapy fails to achieve rapid remission[102]. Although the initial cost of RTX is high, this may be partially offset by reductions in long-term treatment costs. Notably, quantitative measurement of anti-NF155 antibody titers is useful for monitoring disease activity in autoimmune nodopathy and may help guide the duration and intensity of RTX therapy[60]. HLA typing may eventually enable risk stratification and personalized treatment strategies based on genetic susceptibility profiles. CYC is primarily used as a second-line immunosuppressive agent in severe or refractory autoimmune encephalitis, including anti-LGI1, anti-CASPR2, and DPPX-associated diseases[95,103,104]. However, CYC is used less frequently than RTX in MuSK-MG and anti-NF155 autoimmune nodopathy[105,106].
Third-line therapies for IgG4-mediated neurological autoimmune diseases include plasma cell-depleting agents and cytokine-targeting treatments[90]. Daratumumab, a monoclonal antibody targeting CD38, can deplete autoreactive long-lived plasma cells and has been shown to reduce anti-CASPR2 antibody levels in the CSF of patients unresponsive to RTX, thereby overcoming the limitations of therapies that target only CD20+ B cells[107]. Tocilizumab, a monoclonal antibody targeting the IL-6 receptor, has demonstrated clinical benefit in anti-LGI1- and CASPR2-associated autoimmune encephalitis, improving clinical outcomes and reducing antibody levels even in cases resistant to first-line therapy and RTX[108,109].
Recognizing IgG4-mediated neurological disorders as a distinct disease spectrum separate from systemic IgG4-related disease enhances diagnostic precision and facilitates the development of rational, targeted therapeutic strategies for these increasingly recognized conditions. These disorders are characterized by distinct pathophysiological mechanisms involving functional disruption of protein-protein interactions rather than classical inflammation; the absence of typical systemic features, such as fibrosis and marked serum IgG4 elevation; the presence of diverse IgG4 autoantibodies targeting antigens in the central and peripheral nervous systems; and specific HLA associations.
Integrating HLA class II typing with precise IgG4 autoantibody detection represents an emerging paradigm for the diagnosis and management of these disorders. However, many current assays require specialized cell-culture facilities and are time-consuming, limiting their availability in routine clinical laboratories. Commercial cell-based assays also show variable performance, particularly in CSF samples, which may contribute to inconsistent results between laboratories and hinder standardization. Future advances should therefore prioritize harmonizing detection methods, developing rapid point-of-care testing, and combining genetic and serological markers to guide personalized immunotherapies tailored to the distinct pathophysiological mechanisms of these increasingly recognized diseases. Additionally, future applications of liquid chromatography-tandem mass spectrometry technology may enable the dissection of the molecular architecture and glycosylation of IgG4 autoantibodies, unlocking novel diagnostic and prognostic biomarkers for autoimmune neurological disorders.
Current evidence for the treatment of IgG4-mediated neurological disorders remains limited, relying primarily on case reports, case series, and a small number of controlled studies. Well-designed studies are needed to establish evidence-based therapeutic strategies.
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