Progressive spastic weakness of the lower limbs is a major feature of hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS). The motor band sign (MBS) is characterized by a band-like hypointensity along the precentral gyrus in susceptibility-weighted image of brain magnetic resonance imaging. This sign has been reported in amyotrophic lateral sclerosis and is recognized as an indirect marker of upper motor neuron degeneration. The presence of MBS could serve as a marker for PLS but not for HSP.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic autoimmune disease of the peripheral nervous system, primarily treated with corticosteroids and intravenous immunoglobulin as first-line therapies. Early treatment yields better outcome before nerve damage caused by the immune response. Once axonal damage has progressed, immunotherapy becomes ineffective, making early intervention crucial. Additionally, as treatment responses vary among patients, it is essential to assess treatment efficacy objectively and tailor therapy accordingly. Since there are currently no biomarkers that accurately reflect disease status, regular physical examinations are necessary to evaluate treatment effectiveness and adjust maintenance therapy. This review outlines the current clinical guidelines for the treatment of CIDP and explores emerging therapeutic options, including neonatal Fc receptor inhibitors and complement pathway inhibitors.

Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.

Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.

Progressive spastic weakness of the lower limbs is a major feature of hereditary spastic paraplegia (HSP) and primary lateral sclerosis (PLS). The motor band sign (MBS) is characterized by a band-like hypointensity along the precentral gyrus in susceptibility-weighted image of brain magnetic resonance imaging. This sign has been reported in amyotrophic lateral sclerosis and is recognized as an indirect marker of upper motor neuron degeneration. The presence of MBS could serve as a marker for PLS but not for HSP.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic autoimmune disease of the peripheral nervous system, primarily treated with corticosteroids and intravenous immunoglobulin as first-line therapies. Early treatment yields better outcome before nerve damage caused by the immune response. Once axonal damage has progressed, immunotherapy becomes ineffective, making early intervention crucial. Additionally, as treatment responses vary among patients, it is essential to assess treatment efficacy objectively and tailor therapy accordingly. Since there are currently no biomarkers that accurately reflect disease status, regular physical examinations are necessary to evaluate treatment effectiveness and adjust maintenance therapy. This review outlines the current clinical guidelines for the treatment of CIDP and explores emerging therapeutic options, including neonatal Fc receptor inhibitors and complement pathway inhibitors.

Background: and Purpose Treatments for neuromyelitis optica spectrum disorder (NMOSD) such as eculizumab, ravulizumab, satralizumab, and inebilizumab have significantly advanced relapse prevention, but they remain expensive. Rituximab is an off-label yet popular alternative that offers a cost-effective solution, but its real-world efficacy needs better quantification for guiding the application of newer approved NMOSD treatments (ANTs). This study aimed to determine real-world rituximab failure rates to anticipate the demand for ANTs and aid in resource allocation. Methods: We conducted a nationwide retrospective study involving 605 aquaporin-4-antibody-positive NMOSD patients from 22 centers in South Korea that assessed the efficacy and safety of rituximab over a median follow-up of 47 months. Results: The 605 patients treated with rituximab included 525 (87%) who received continuous therapy throughout the follow-up period (median=47 months, interquartile range=15–87 months). During this period, 117 patients (19%) experienced at least 1 relapse. Notably, 68 of these patients (11% of the total cohort) experienced multiple relapses or at least 1 severe relapse.Additionally, 2% of the patients discontinued rituximab due to adverse events, which included severe infusion reactions, neutropenia, and infections. Conclusions This study has confirmed the efficacy of rituximab in treating NMOSD, as evidenced by an 87% continuation rate among patients over a 4-year follow-up period. Nevertheless, the occurrence of at least one relapse in 19% of the cohort, including 11% who experienced multiple or severe relapses, and a 2% discontinuation rate due to adverse events highlight the urgent need for alternative therapeutic options.