Immune-mediated rippling muscle disease (iRMD) is a rare myopathy characterized by wavelike muscle contractions (rippling) and percussion-or stretch-induced muscle mounding. A serological biomarker of this disease is lacking.OBJECTIVE To describe a novel autoantibody biomarker of iRMD and report associated clinicopathological characteristics. DESIGN, SETTING, AND PARTICIPANTSThis retrospective cohort study evaluated archived sera from 10 adult patients at tertiary care centers at the Mayo Clinic, Rochester, Minnesota, and Brigham & Women's Hospital, Boston, Massachusetts, who were diagnosed with iRMD by neuromuscular specialists in 2000 and 2021, based on the presence of electrically silent percussion-or stretch-induced muscle rippling and percussion-induced rapid muscle contraction with or without muscle mounding and an autoimmune basis. Sera were evaluated for a common biomarker using phage immunoprecipitation sequencing. Myopathology consistent with iRMD was documented in most patients. The median (range) follow-up was 18 (1-30) months. EXPOSURES Diagnosis of iRMD.MAIN OUTCOMES AND MEASURES Detection of a common autoantibody in serum of patients sharing similar clinical and myopathological features.RESULTS Seven male individuals and 3 female individuals with iRMD were identified (median [range] age at onset, 60 [18-76] years). An IgG autoantibody specific for caveolae-associated protein 4 (cavin-4) was identified in serum of patients with iRMD using human proteome phage immunoprecipitation sequencing. Immunoassays using recombinant cavin-4 confirmed cavin-4 IgG seropositivity in 8 of 10 patients with iRMD. Results for healthy and disease-control individuals (n = 241, including myasthenia gravis and immune-mediated myopathies) were cavin-4 IgG seronegative. Six of the 8 individuals with cavin-4 IgG were male, and the median (range) age was 60 (18-76) years. Initial symptoms included rippling of lower limb muscles in 5 of 8 individuals or all limb muscles in 2 of 8 sparing bulbar muscles, fatigue in 9 of 10, mild proximal weakness in 3 of 8, and isolated myalgia in 1 of 8, followed by development of diffuse rippling. All patients had percussion-induced muscle rippling and half had percussion-or stretch-induced muscle mounding. Four of the 10 patients had proximal weakness. Plasma creatine kinase was elevated in all but 1 patient. Six of the 10 patients underwent malignancy screening; cancer was detected prospectively in only 1. Muscle biopsy was performed in 7 of the 8 patients with cavin-4 IgG; 6 of 6 specimens analyzed immunohistochemically revealed a mosaic pattern of sarcolemmal cavin-4 immunoreactivity. Three of 6 patients whose results were seropositive and who received immunotherapy had complete resolution of symptoms, 1 had mild improvement, and 2 had no change. CONCLUSIONS AND RELEVANCEThe findings indicate that cavin-4 IgG may be the first specific serological autoantibody biomarker identified in iRMD. Depletion of cavin-4 expression in muscle biopsies of patients with iRMD suggests the potential role ...

We present a review of the efficacy and safety profile of eptinezumab (also known by the brand name Vyepti), a calcitonin gene-related peptide (CGRP) monoclonal antibody (mAb) developed by Lundbeck Seattle BioPharmaceuticals, Inc., that received its first approval in the USA on 21 February 2020 for the preventive treatment of migraine in adults. It is administered by an intravenous infusion at a 100 mg dose every 3 months and shows no drug interactions.Studies have shown that eptinezumab is an effective preventative medication in migraine which starts showing its effect from day 1 of its administration, which maintains a consistent level of efficacy through a year of its treatment at doses 100 mg and 300 mg. It was found to be effective at reducing time to headache pain freedom during acute migraine attacks as well.Eptinezumab is a relatively safe drug for the prevention of migraines with treatment-related adverse events occurring at a low frequency. They bear a safe profile in patients with comorbidities like obesity and type 1 diabetes. The most frequent adverse events observed were nasopharyngitis, upper respiratory tract infections (URTIs), and sinusitis and were usually mild. The development of anti-drug antibodies was common, but they declined to undetectable levels with continued dosing and did not appear to impact the overall safety profile of the drug. Further studies are needed to assess long-term safety, use in different patient populations, and to compare its efficacy to other drugs of its class.

Objective: To report the clinical presentations and outcomes of patients with seizure and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD). Methods:We retrospectively reviewed the electronic medical records for clinical and paraclinical features among patients with seizures and MOG-IgG (immunoglobulin G) seropositivity. Results:We identified 213 patients with MOG-IgG seropositivity who fulfilled criteria for MOGAD. Seizures attributed to central nervous system (CNS) autoimmunity were observed in 10% of patients (n = 23: 19 children, 4 adults). The majority (n = 19, 83%) had pediatric disease onset. Focal motor seizures were the most common seizure semiology (16/23; 70%). Focal to bilateral tonic-clonic seizures were present in 12 patients (53%), and 3 patients (13%) developed status epilepticus. All patients had features of encephalitis at onset of seizures. Cerebral cortical encephalitis (CCE) was the most common radiological finding (10 unilateral and 5 bilateral cases). Eight of 23 patients (35%) had only CCE, six of 23 patients (26%) had only acute disseminated encephalomyelitis (ADEM), and seven of 23 patients (30%) had features of both. Fifteen patients (65%) had leptomeningeal enhancement. Three patients (13%) had coexistence of N-methyl-d-aspartate receptor (NMDAR) IgG. Only 3 of 23 patients (13%) developed drug-resistant epilepsy. Although the majority had MOGAD relapses (14/23, 60%) had only 5 of 23 patients had recurrence of episodes of encephalitis with associated seizures.Twenty-one of 23 patients (91%) had seizure freedom at last follow-up.Significance: MOG-IgG evaluation should be considered in patients who present with encephalitis and focal motor and/or focal to bilateral tonic-clonic seizures, especially pediatric patients with magnetic resonance imaging (MRI) brain findings consistent with CCE, ADEM, or other MOGAD presentations. The majority of these seizures are self-limited and do not require maintenance/chronic

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