Chronic Inflammatory Neuropathy Research Articles

P.060 Provider and patient perspectives on outcome measure use in clinical care for chronic inflammatory neuropathy

Background: The use of patient reported and functional outcome measures in routine practice enhances shared decision making and supports patient-centred care. This study compared the perspectives of Chronic Inflammatory Neuropathy (CIN) patients and providers regarding their experience using an outcome measure panel. Methods: A one year study was conducted to evaluate a nine measure outcome set in routine clinical practice for CIN. The panel included patient-reported outcome measures (e.g., I-RODS and EQ-5D-5L) and functional measures (e.g., grip strength). At the conclusion of the study, participants and providers completed an online questionnaire on their experience. Results: 25 patients and five providers completed the questionnaire. Both patients and providers reported benefit in tracking disease progression, supporting treatment-related decisions, and broadening views of health. Both groups agreed patient involvement in care was enhanced. Preference for specific measures, frequency, and data presentation differed. Providers emphasized integration into electronic medical records and streamlining processes. 100% of providers and 80% of patients wanted to continue completing outcome measures. Conclusions: CIN patients and providers recognize the value of integrating outcome measures into routine care. To effectively implement these measures in clinical settings, it is important to understand the patient and provider perspective and prevent unnecessary burdens to ensure sustainability of use.

Neuromyelitis Optica Spectrum Disorder (NMOSD) or Chronic-Relapsing Inflammatory Optic Neuropathy (CRION)?: A Challenge in Diagnosis and Treatment

Abstract
 Introduction : Neuromyelitis optica spectrum disorder (NMOSD) is a chronic inflammatory, immune-mediated central nervous system disease targeting the AQP4-water channel membrane protein of the optic nerve and the spinal cord. This case report aims to present a case of seroconverting NMOSD which was initially diagnosed as chronic relapsing inflammatory optic neuropathy (CRION).
 Case Illustration : 15-year-old female with recurrent, steroid-dependent optic neuritis of the LE. During the first attack, brain MRI showed optic nerve inflammation with normal brain findings. Initial test for AQP4-IgG, MOG-Ab and ANA profile were negative. The diagnosis of CRION was made. Treatment with corticosteroid and immunosuppressant agent was maintained for one year. After treatment cessation, however, LE optic neuritis recurred with bilateral lower limb weakness. Spinal MRI showed signs of longitudinal extensive transverse myelitis. Re-testing for AQP4-IgG showed positive result. Diagnosis of NMOSD was made, and the patient was treated with high-dose intravenous corticosteroid followed by oral dose, and immunosuppressant. Final visual acuity of the LE was 1.0, with minimal residual visual field defect and good motor function.
 Discussion : During the initial attack, the patient did not meet the 2015 NMOSD international consensus diagnostic criteria. The AQP4-IgG was negative and no other corresponding signs and symptoms. The criteria was met one year after: optic neuritis, LETM and positive seroconversion of AQP4-IgG.
 Conclusion : Diagnosing NMOSD is at times challenging especially during initial attack. Retesting of the AQP4- IgG is at times necessary in seronegative patients, especially in children showing ADEM-like event with optic neuritis and LETM to achieve diagnosis and proper treatment strategy.

Evaluating yield and utilization of ganglioside antibody testing in clinical practice

Background and objectivesGanglioside antibodies can help diagnose distinct acute and chronic inflammatory neuropathies including axonal variants of Guillain-Barre syndrome, Miller-Fisher syndrome (MFS), multifocal motor neuropathy, and chronic sensory ataxic neuropathies. Because ganglioside antibody testing may be routinely ordered in patients with suspected inflammatory neuropathy, we sought to evaluate its yield and utilization in clinical practice. MethodsWe performed a retrospective chart review of all patients at London Health Sciences Centre who underwent ganglioside antibody testing between April 2019 and August 2023. The disease phenotype was determined for each patient, and the proportion of all tests that yielded a true-positive result was calculated. Ganglioside antibody positivity was classified as a true-positive result if the disease phenotype was robustly associated with the detected ganglioside antibody and there was no other more likely diagnosis. ResultsWe identified 92 patients who underwent ganglioside antibody testing. One patient (1%) was classified as having a true-positive result; this patient had GQ1b-IgG positivity with MFS. Among 92 patients tested, 20 patients (22%) had a disease phenotype that was considered to be robustly associated with ganglioside antibody positivity. ConclusionsThe yield of ganglioside antibody testing in clinical practice is low. We found that this testing is frequently ordered in patients with disease phenotypes that are not robustly associated with ganglioside antibody positivity, indicating that suboptimal test utilization is a primary contributor to its low yield. Restricting ganglioside antibody testing to patients with characteristic disease phenotypes would be valuable to improving yield and utilization of this testing.

Nodopathy: clinic, diagnosis, treatment. Clinical description

AIM: Evaluation of the significance and possibilities of laboratory-instrumental diagnostic methods in establishing the diagnosis and selection of targeted therapy in patients with nodopathies.
 MATERIALS AND METHODS: System analysis of data from foreign and domestic literature with the presentation of a clinical case.
 RESULTS: Polyneuropathies are classified as demyelinating or axonal based on electrophysiological studies. However, in 2015, in addition to axonal and demyelinating neuropathies, it was proposed to distinguish a separate pathophysiological group — nodopathies. The pathogenesis of nodopathies may differ depending on the type of ion channels involved in the process, but always leads to a loss of excitability of the axon membrane; in the nodal region the membrane becomes inexcitable. Such neuropathies are characterized by transient conduction blocks followed by the development of axonal degeneration. Typical examples of nodopathies are acute motor axonal neuropathy, as well as multifocal motor neuropathy. Current pathophysiological understanding of specialized nodal regions (nodes of Ranvier) and associated axoglial proteins is growing. Hypotheses have been put forward about their role in the pathogenesis of immune-mediated attack on the peripheral myelinated axon. Recently, high titers of antibodies directed against a number of key adhesion molecules have been identified in both acute and chronic inflammatory neuropathies. These facts add to the differences in differential diagnosis between axonal and demyelinating peripheral neuropathies. New disease classification schemes based on seropositivity, improved electrophysiological and ultrasound classification, and identification of putative underlying pathological targets and mechanisms are being rapidly developed.
 CONCLUSION: Using our clinical example, we demonstrated the capabilities of laboratory and instrumental diagnostic methods in establishing a diagnosis in a patient with one of the forms of nodopathies — multifocal motor neuropathy.

Optic neuritis in Turkish children and adolescents: A multicenter retrospective study

BackgroundVarious etiologies may underlie optic neuritis, including autoantibody-mediated disorders described in the last decade. We re-examined demographic, clinical, laboratory features and prognostic factors in pediatric patients with autoimmune optic neuritis according to current knowledge. MethodsCases of pediatric ON from 27 centers in Türkiye diagnosed between 2009 and 2022 were included for retrospective evaluation. ResultsThe study included 279 patients, 174 females and 105 males, with a female-to-male ratio of 1.65. The average age at onset was 12.8 ± 3.4 years, and mean follow-up, 2.1 years (range: 1–12.1 years). Patients <10 years old were grouped as "prepubertal" and those ≥10 years old as "others”. The diagnoses made at the end of follow-up were multiple sclerosis associated optic neuritis (n = 90, 32.3 %), single isolated optic neuritis (n = 86, 31 %), clinically isolated syndrome (n = 41, 14.7 %), myelin oligodendrocyte glycoprotein antibody associated optic neuritis (n = 22, 7.9 %), and relapsing isolated optic neuritis (n = 18, 6.5 %). Predominant diagnoses were myelin oligodendrocyte glycoprotein antibody associated optic neuritis and acute disseminated encephalomyelitis associated optic neuritis in the prepubertal group and multiple sclerosis associated optic neuritis in the older group. Recurrences were observed in 67 (24 %) patients, including 28 with multiple sclerosis associated optic neuritis, 18 with relapsing isolated optic neuritis, 11 with myelin oligodendrocyte glycoprotein antibody associated optic neuritis, 8 with aquaporin-4 antibody related optic neuritis, and 2 with chronic relapsing inflammatory optic neuropathy. Recurrences were more common among female patients. Findings supporting the diagnosis of multiple sclerosis included age of onset ≥ 10 years (OR=1.24, p = 0.027), the presence of cranial MRI lesions (OR=26.92, p<0.001), and oligoclonal bands (OR=9.7, p = 0.001). Treatment in the acute phase consisted of intravenous pulse methylprednisolone (n = 46, 16.5 %), pulse methylprednisolone with an oral taper (n = 212, 76 %), and combinations of pulse methylprednisolone, plasmapheresis, or intravenous immunoglobulin (n = 21, 7.5 %). Outcome at 12 months was satisfactory, with 247 out of 279 patients (88.5 %) demonstrating complete recovery. Thirty-two patients exhibited incomplete recovery and further combination treatments were applied. Specifically, patients with relapsing isolated optic neuritis and aquaporin-4 antibody related optic neuritis displayed a less favorable prognosis. ConclusionOur results suggest optic neuritis is frequently bilateral in prepubertal and unilateral in peri‑ or postpubertal patients. Age of onset 10 or older, presence of oligoclonal bands, and brain MRI findings reliably predict the development of multiple sclerosis. The risk of developing multiple sclerosis increases mostly during the second and third years of follow-up. Relapsing isolated optic neuritis remains a separate group where the pathogenesis and outcome remain unclear. Investigation of predisposing and diagnostic biomarkers and long follow-up could help to define this group.

Epidemiology of demyelinating diseases in Mexico: A registry-based study.

Demyelinating diseases (DD) are a group of chronic neurological diseases associated with loss and injury of brain or spinal cord regions. These conditions could trigger impairment of neurological functions and disability from earlier stages of life. Epidemiological data on DD remains insufficient for decision-making in the Mexican healthcare system. This study aims to describe the epidemiology of DD based on data from Mexico's National Registry of Demyelinating Diseases. A cross-sectional, registry-based, observational study was performed. We analyzed 408 reports of multiple sclerosis (331, 81%), neuromyelitis optica spectrum disorder (67, 16%), chronic recurrent inflammatory optic neuropathy (5, 1%), clinically isolated syndrome (4, 0.9%), and autoimmune encephalitis (1, 0.2%) reported across 2021. The time from first symptoms to diagnosis of any DD was about 3 years. A treatment failure history was detected in 40% of patients. It was estimated that NMOSD accounts for 20% of all disorders. There was evidence that the use of brand-name and generic IFN drug products lead to increased therapeutic failures. Our research team suggests reinforcing educational programs and activities based on diagnosis and clinical management improvement to first-contact physicians and specialty doctors and promoting awareness in the whole population.

Сhronic relapsing inflammatory optic neuropathy: diagnostic strategies and treatment (literature review)

The article presents a review and analysis of scientific works on the problem of chronic relapsing inflammatory optic neuropathy, its diagnostic criteria and treatment methods. Chronic relapsing inflammatory optic neuropathy, first described in 2003, is an autoimmune inflammatory disease of the optic nerve of unknown etiology, accompanied by frequent painful episodes of optic neuritis, with a clear response to steroid therapy and relapses after its withdrawal. An analysis of foreign works published in the E-Library, CrossRef, PubMed, Web of Science, Scopus databases was carried out in order to systematize data on the etiology, pathogenesis, clinical manifestations, diagnostic criteria, differential diagnosis and treatment of this pathological condition. It has been established that chronic recurrent inflammatory optic neuropathy is a rare, recurrent, corticosteroid-dependent optic neuropathy in which there is no other neurological deficit, no etiological factor is identified and it is a diagnosis of exclusion. It is important to identify such patients because the appointment of appropriate immunosuppressive therapy causes remission of the disease.

Neuropathic Tremor in Guillain-Barré Syndrome.

Neuropathic Tremor (NT) is a postural/kinetic tremor of the upper extremity, often encountered in patients with chronic neuropathies such as paraprotein-associated and hereditary neuropathies. To describe the clinical and electrophysiological features of NT in a previously underrecognized setting- during recovery from Guillain-Barré Syndrome (GBS). Patients with a documented diagnosis of GBS in the past, presenting with tremor were identified from review of clinical records. Participants underwent structured, videotaped neurological examination, and electrophysiological analysis using tri-axial accelerometry-surface electromyography. Tremor severity was assessed using the Fahn-Tolosa-Marin Tremor Rating Scale. We describe the clinical and electrophysiological features of 5 patients with GBS associated NT. Our cohort had a fine, fast, and slightly jerky postural tremor of frequency ranging from 8 to 10 Hz. Dystonic posturing and overflow movements were noted in 4/5 patients. Tremor appeared 3 months-5 years after the onset of GBS, when patients had regained near normal muscle strength and deep tendon jerks were well elicitable. Electrophysiological analysis of tremor strongly suggested the presence of a central oscillator in all patients. NT is not limited to chronic inflammatory or hereditary neuropathies and may occur in the recovery phase of GBS. The tremor is characterized by a high frequency, jerky postural tremor with dystonic posturing. Electrophysiological evaluation suggests the presence of a central oscillator, hypothetically the cerebellum driven by impaired sensorimotor feedback.

P.029 Relevance and home completion rate of patient reported outcome measures set in chronic inflammatory neuropathies

Background: Patient-centred care is important in the management of chronic inflammatory neuropathies (CIN) given the heterogeneity in disease course and treatment response. Patient Reported Outcome Measures (PROMs) support value-based healthcare by aligning treatment goals with what matters most to patients. This study evaluated the relevance of PROMs to patients and the feasibility of use in clinical management. Methods: PROMs assessing quality of life, pain, fatigue, and overall disability were collected prospectively from 32 patients with CIN every three months over a 12-month period. Patients provided feedback on relevance of the measures. PROMs were sent electronically prior to the visit. Results: Completion rate was 92%. Home vs. in-clinic completion increased from 56% to 85% over the course of the study. There was an association between completion of the panel and perceived relevance. The PROMs were consistently rated as highly relevant, with disability and fatigue measures rated highest. Conclusions: PROMs are appraised as highly relevant among patients with CIDP and MMN. Patients require support initially but adapt to electronically delivered home completion of questionnaires. We recommend inclusion of PROMs into routine clinical practice as a means of capturing aspects of health that are not easily assessed in a clinic visit.

Clinical Implications of Specific Autoantibodies in Chronic Inflammatory Neuropathies (P11-8.013)

<h3>Objective:</h3> A large-scale, in-depth analysis of clinical, electrophysiological characteristics and treatment response of autoantibody associated immune-mediated neuropathies. <h3>Background:</h3> Chronic inflammatory neuropathies (CINs) can have varied clinical presentations. In the past two decades several autoantibodies have been identified in CIN patients. While the literature suggests specific clinical phenotypes with some of these autoantibodies, the full range of possible presentations and the associated clinical syndromes of each antibody requires additional investigation. <h3>Design/Methods:</h3> We reviewed the electronic medical records of adult patients undergoing evaluation for immune mediated neuropathies between 01/01/2012 and 04/01/2022 at our institution who were assessed for autoantibodies. The clinical presentations, laboratory work-up, electrodiagnostic studies, and treatment responses were reviewed for this ongoing project. <h3>Results:</h3> This analysis resulted in a total of 1168 patients who were evaluated for CIN-associated autoantibodies. Anti-trisulfated-heparin-disaccharide (TS-HDS) autoantibodies were present in 266 (22.8%) patients, anti-fibroblast-growth-factor-receptor-3 (FGFR3) autoantibodies were present in 200 (17.1%) patients and 33 (2.8%) patients were positive for anti-neurofascin (NF)-140/155 autoantibodies. Other autoantibodies were rare (&lt;1%), and multiple autoantibodies were detected in some patients.. Interestingly, some patients with anti-NF presented with focal sensorimotor dysfunction prior to subsequent bilateral involvement. About 20% of patients with anti-NF autoantibodies had predominant demyelinating features in electrodiagnostic studies. Anti-FGFR3 autoantibodies were often associated with sensory involvement, but sometimes motor dysfunction was also present. Anti-TS-HDS-associated CIN had a more varied clinical presentation. There were differences in therapeutic response based on the associated autoantibodies; however, the therapeutic decision was provider-specific, and a direct comparison was not feasible. <h3>Conclusions:</h3> Autoantibodies associated with CINs can have a variety of often overlapping motor and sensory presentations. In a subset of patients, their presence can help gauge therapeutic responsiveness. However, in some cases, they may represent an epiphenomenon, and further studies are warranted. <b>Disclosure:</b> Dr. Bushlyar has nothing to disclose. Dr. Zubair has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for The MedNet. Dr. Cardenas Castillo has nothing to disclose. Kevin O’Connor has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Kevin O’Connor has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Roche. Kevin O’Connor has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Genentech. Kevin O’Connor has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Viela Bio. Kevin O’Connor has received stock or an ownership interest from Cabaletta. Dr. DiCapua has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Argenx. Dr. DiCapua has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Argenx. Dr. Desai has nothing to disclose. Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion . Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for argenx. Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Momenta . Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Immunovant . Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cabaletta Bio . Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Viela Bio. Dr. Nowak has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cour Pharma. The institution of Dr. Nowak has received research support from Ra Pharma. The institution of Dr. Nowak has received research support from Alexion . The institution of Dr. Nowak has received research support from Momenta . The institution of Dr. Nowak has received research support from Immunovant . Dr. Nowak has received research support from argenx. Dr. Nowak has received research support from Viela Bio . Dr. Nowak has a non-compensated relationship as a Member of the Board of Directors with Myasthenia Gravis Foundation of America (MGFA) that is relevant to AAN interests or activities. Dr. Tseng has nothing to disclose. Dr. Roy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion Pharmaceuticals. Dr. Roy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda Pharmaceuticals. Dr. Roy has received personal compensation in the range of $500-$4,999 for serving as a Consultant for argenx. Dr. Roy has stock in Cabaletta bio. . The institution of Dr. Roy has received research support from Martin Shubik Fund for IBM at Yale University.

Chronic inflammatory neuropathies and their impact on activities and participation.

Chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy (MMN) are inflammatory neuropathies that can lead to considerable limitations in daily activities and in social participation. However, systematic evaluation of these self-reported limitations is lacking in the currently available studies. Understanding the impact of these diagnoses on patients' life is important to optimize management strategies. To systematically assess the self-reported limitations in activities and participation and determine associated factors. A survey study was conducted in 2021 in a cohort of patients with CIDP (n = 257) and MMN (n = 148) from a university hospital. The survey included the Rasch-built Overall Disability Scale and the Utrecht Scale for Evaluation of Rehabilitation-Participation, questions addressing personal and disease-related factors and treatment. Multivariate linear regression analysis was used to determine associations with disease-related and personal factors. A total of 147 CIDP and 103 MMN patients responded. Limitations in activities were reported by 70.7% CIDP and 52.2% MMN patients with moderate to severe limitations in 22.4% and 5.9% patients, respectively. Participation restrictions were reported by 50% of CIDP and 40% of MMN patients, nevertheless satisfaction with participation was high. Fatigue, pain and resilience were independently associated with limitations in activities and satisfaction with participation in CIDP patients. Activity limitations and restrictions in participation are common in CIDP patients and to a lesser extent in MMN patients. Fatigue, pain and resilience independently contributed to perceived limitations in CIDP patients. Referral to a rehabilitation physician is warranted to address these limitations appropriately.

Risk of disease relapse, safety and tolerability of SARS-CoV-2 vaccination in patients with chronic inflammatory neuropathies.

The aim was to evaluate the risk of relapse after severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination, and its safety and tolerability, in patients with chronic inflammatory neuropathies. In this multicenter, cohort and case-crossover study, the risk of relapse associated with SARS-CoV-2 vaccination was assessed by comparing the frequency of relapse in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) patients who underwent or did not undergo vaccination. Frequency of relapse in the 3 months prior to and after vaccination, and safety and tolerability of SARS-CoV-2 vaccination, were also assessed. In all, 336 patients were included (278 CIDP, 58 MMN). Three hundred and seven (91%) patients underwent SARS-CoV-2 vaccination. Twenty-nine patients (9%) did not undergo vaccination. Mild and transient relapses were observed in 16 (5%) patients (13 CIDP, 3 MMN) after SARS-CoV-2 vaccination and in none of the patients who did not undergo vaccination (relative risk [RR] 3.21, 95% confidence interval [CI] 0.19-52.25). There was no increase in the specific risk of relapse associated with type of vaccine or diagnosis. Comparison with the 3-month control period preceding vaccination revealed an increased risk of relapse after vaccination (RR 4.00, 95% CI 1.35-11.82), which was restricted to CIDP patients (RR 3.25, 95% CI 1.07-9.84). The safety profile of SARS-CoV-2 vaccination was characterized by short-term, mild-to-moderate local and systemic adverse events. Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccination in CIDP and MMN patients does not seem to be associated with an increased risk of relapse at the primary end-point, although a slightly increased risk in CIDP patients was found compared to the 3 months before vaccination.

P-41 The impact of the SARS-CoV-2-pandemic on patients with chronic inflammatory neuropathies: Results from the German INHIBIT register

P-41 The impact of the SARS-CoV-2-pandemic on patients with chronic inflammatory neuropathies: Results from the German INHIBIT register

Luteolin: A promising natural agent in management of pain in chronic conditions.

Pain due to chronic conditions is a frequent and insufficiently addressed problem. Current drug options for pain management (either in cases of chronic inflammatory conditions or neuropathy) do not adequately treat pain. Moreover, they are associated with important adverse events in long term use. Luteolin is a flavonoid widely present in the plant kingdom and its sources have been assembled in a comprehensive list of this paper. Luteolin has shown in several research studies a range of pharmacological properties; anti-inflammatory, antioxidant, neuroprotective, and analgesic. In this article, we summarize the effects and potential benefits from introducing luteolin as an adjuvant agent in established protocols for pain management. We review the most indicative in vivo and in vitro evidence of how luteolin can target the molecular pathways involved in pathogenesis of chronic inflammatory and neuropathic pain. The data reviewed strongly support luteolin's promising benefits in pain management and raise the need for further clinical trials that can establish its role in clinical practice.

The impact of the SARS-CoV-2-pandemic on patients with chronic inflammatory neuropathies: results from the German INHIBIT register

IntroductionSARS-CoV-2 pandemic is especially compromising for patients with autoimmune diseases with or without immunomodulatory treatment. This study aimed to investigate the longitudinal changes in the health care of patients with immune-mediated neuropathies during the COVID-19 pandemic.MethodsWe performed a longitudinal study using questionnaires in a prospective cohort of patients with immune-mediated neuropathies at two timepoints of the pandemic: May–July 2021 and May–July 2022.ResultsThe cohort consisted of 73 patients (55 male), mean age 62 years, 68 patients with CIDP, 5 with other immune neuropathies. In 2021, 19.2% of the patients reported a reduced number of physician–patient-contacts, while 13.7% reported this in 2022. Nevertheless, the overall health-care situation worsened from 2021 to 2022: 15.1% reported reduced overall healthcare in 2021, 26.0% in 2022. In 2021, 29.4% of patients reported absence of physio-/occupational therapy, while 34.4% reported this in 2022. Switching immunomodulatory treatment and stretching of treatment intervals occurred more often in 2022 (38.4%) than in 2021 (27.4%). 12 COVID-19-infections occurred overall, with typical only mild symptoms. The rate of fully vaccinated patients was 61.6% and 98.6% in May–July 2021 and 2022, respectively. Only minor side-effects after vaccination were reported.ConclusionDespite mitigation of COVID-19 restrictions from 2021 to 2022, the health-care situation of patients worsened in this time. Reasons could be the international shortage of immunoglobulins during the pandemic and reduced physio/ergotherapy due to lingering regulatory restrictions. Vaccination rate was high in our cohort of patients compared to the general German population and CIDP did not seem to be a risk factor for severe SARS-CoV-2 infections.

Clinical spectrum of neuro‐ophthalmological manifestations associated in MOG antibodies in children

AbstractIntroduction: The discovery of the role of MOG antibodies over the last decade has tremendously modified our comprehension of inflammatory disorders of the central nervous system (CNS), especially in children.Objective: To describe the clinical spectrum of neuro‐ophthalmological manifestations associated in MOG antibodies in children.Methods: Literature review using Pubmed and Google Scholar and chosen clinical case series.Results: Three long known clinical entities affecting the optic nerve in children have been recently proven to be largely associated with MOG antibodies: optic neuritis in the context of acute disseminated encephalomyelitis (ADEM); monophasic optic neuritis; chronic relapsing inflammatory optic neuropathy (CRION). ADEM is a child's specificity. CRION often starts during late childhood. These entities can occur after one another in a same child.Conclusions: The absence of overlap between multiple sclerosis, aquaporine 4 antibodies (associated with neuromyelitis optica only) and MOG antibodies, allows for a new, clearer delineation of inflammatory disorders of the CNS in children, closer to the precise classical clinical descriptions than to the semi‐recent criteria‐based “spectra”.

Paediatric optic neuritis: experience from a tertiary referral centre in Malaysia

Background: Optic neuritis typically presents with acute or subacute onset of mild or profound blurring of vision. There are very limited reports regarding optic neuritis in the paediatric population compared to adults from the South Asian region. We report a series of 7 cases of paediatric optic neuritis.&#x0D; Methods: All optic neuritis cases aged less than 12 years old in 2016 were studied retrospectively.&#x0D; Results: Out of 44 patients with optic neuritis, 7 of them were of paediatric age. The mean age was 9.9 years. All patients had sudden onset profound vision loss (range 3/60 to hand movement). Four patients had bilateral involvement, all had reduced colour vision. Three had underlying acute disseminated encephalomyelitis. All were treated with high-dose intravenous corticosteroids. Five patients made full recovery, one patient had partial recovery, and one patient had no visual recovery. Interestingly, none of them had multiple sclerosis (MS) or neuromyelitis optica (NMO) at the time of diagnosis.&#x0D; Conclusion: Prognosis is generally good for isolated cases of paediatric optic neuritis. Outcome of cases secondary to ADEM depends on the degree and extent of demyelination. A diagnosis of chronic relapsing inflammatory optic neuropathy can be considered in recurrent cases that are steroid responsive. None of our cases had underlying MS or NMO during the study period.

Optical Coherence Tomography in Chronic Relapsing Inflammatory Optic Neuropathy, Neuromyelitis Optica and Multiple Sclerosis: A Comparative Study.

Purpose: To examine the optical coherence tomography (OCT) features of the retina in patients with chronic relapsing inflammatory optic neuropathy (CRION) and compare them with those of neuromyelitis optica spectrum disorder (NMOSD), relapsing-remitting multiple sclerosis (RRMS) with and without optic neuritis (ON), and healthy controls (HC). Methods: In this retrospective cross-sectional study, we used spectral domain OCT to evaluate the retinal structure of 14 participants with CRION, 22 with NMOSD, 40 with RRMS with unilateral ON, and 20 HC. The peripapillary retinal nerve fiber layer (pRNFL), total macular volume (TMV), and papillomacular bundle (PMB) were measured, and intra-retinal segmentation was performed to obtain the retinal nerve fiber (RNFL), ganglion cell (GCL), inner plexiform (IPL), inner nuclear (INL), outer plexiform (OPL) and outer nuclear (ONL) layer volumes. Results: The global pRNFL [39.33(±1.8) µm] and all its quadrants are significantly thinner in CRION compared with all other groups (p < 0.05). CRION patients have decreased volumes of TMV, RNFL, GCL, and IPL compared with all other groups (p < 0.05). Conclusion: Severe thinning in pRNFL and thinning in intra-retinal segments of IPL, GCL, RNFL, and TMV could be helpful in differentiating CRION from NMOSD and RRMS.

Application and Research Progress of High Frequency Ultrasound in the Diagnosis of Chronic Inflammatory Neuropathies

In recent years, clinicians have gradually improved their understanding of multiple neuropathy and have done some studies about chronic inflammatory neuropathies, for example, chronic inflammatory demyelinating polyneuropathy, multifocal motor neuropathy, and Lewis-Sumne syndrome. The early diagnosis is very important for the next step treatment and long-term prognosis. At present, the disease mainly depends on clinical and neural electrophysiological examination, but imaging studies are few. In recent years, with the rapid development of high frequency ultrasound, it could clearly show the morphology of the nerve, and it has been an emerging diagnosis tool of polyneuropathies. This article mainly reviews the application and the latest research progress of high frequency ultrasound in these diseases.

Neurofilament light chain and total tau in the differential diagnosis and prognostic evaluation of acute and chronic inflammatory polyneuropathies.

Background and PurposeTo investigate the diagnostic and prognostic value of axonal injury biomarkers in patients with inflammatory polyneuropathies.MethodsNeurofilament light chain (NfL) and total tau (T‐tau) were measured in the cerebrospinal fluid (CSF) and plasma in 41 patients with Guillain–Barré syndrome (GBS), 32 patients with chronic inflammatory demyelinating polyneuropathy (CIDP), 10 with paraproteinemia‐related demyelinating polyneuropathy (PDN), and 8 with multifocal motor neuropathy (MMN), in comparison with 39 disease‐free controls and 59 other controls. Outcome was measured with the GBS‐disability score (GBS‐ds) or Inflammatory Neuropathy Cause and Treatment (INCAT) disability score.ResultsNeurofilament light chain levels in CSF and plasma were higher in GBS, CIDP, and PDN vs. disease‐free controls. Patients with MMN had higher NfL levels in plasma vs. disease‐free controls, but lower levels in CSF and plasma vs. patients with amyotrophic lateral sclerosis (ALS).T‐tau levels in plasma were higher in GBS, CIDP, PDN, and MMN vs. all control groups.Neurofilament light chain levels in CSF and plasma in patients with GBS correlated with GBS‐ds, as higher levels were associated with inability to run after 6 and 12 months. NfL levels in CSF and plasma in CIDP did not correlate significantly with outcome.ConclusionsAcute and chronic inflammatory neuropathies are associated with an increase in levels of NfL in CSF and plasma, but NfL is validated as a prognostic biomarker only in GBS. NfL could be used in differentiating patients with MMN from ALS. T‐tau in plasma is a novel biomarker that could be used in a diagnostic assessment of patients with acute and chronic inflammatory polyneuropathies.