Single simple question in axonal polyneuropathy.

INTRODUCTION/AIMS: The single simple question (SSQ), "What percentage of normal (0%-100%) do you feel regarding your disease?" has proven feasible and valid in assessing myasthenia gravis and a heterogeneous spectrum of neuropathies. This study explores the utility of the SSQ in axonal polyneuropathies (PNPs), encompassing diabetic neuropathy, and evaluates its responsiveness to scale changes. METHODS: A retrospective chart review of 150 patients with axonal PNP responding to the SSQ was performed. Patients underwent clinical and electrophysiological evaluations, and were evaluated by clinical and disability scales, including the Medical Research Council sum score, modified Toronto Clinical Neuropathy score (mTCNS), Overall Neuropathy Limitation Scale, and Rasch-built Overall Disability Scale (RODS). RESULTS: The SSQ total scores correlated moderately with both the RODS score (r = .59, p < .001) and the mTCNS symptom score (r = -.43, p < .001), maintaining significance after adjustment for multiple comparisons. Longitudinally, after adjusting for multiple comparisons, the change in mTCNS symptom score retained statistical significance (adjusted p = .048). The SSQ did not show any association with electrophysiological parameters or sensory symptoms, other than a lower score in those with pain (100% with SSQ <40%, 85% with SSQ 40%-70%, and 34% with SSQ >70%). DISCUSSION: The SSQ is a feasible, valid scale that may be utilized to assess and follow patients with length-dependent axonal PNPs. Given that the SSQ is not strongly associated with clinical and disability scales or electrophysiological findings, additional investigations are required for a comprehensive assessment of PNP.

Using jitter analysis with concentric needle electrodes to assess disease status and treatment responses in myasthenia gravis.

Objective: This study assesses the utility of jitter analysis with concentric needles to evaluate disease severity in myasthenia gravis (MG), correlate changes in jitter with clinical status as well as identify reasons for any discordance. Methods: We performed a retrospective chart review of 82 MG patients and extracted data on demographics, MG subtype, antibody status, clinical scales, electrophysiology, and interventions at baseline and follow-up. Results: Baseline MGII scores correlated with jitter (r = 0.25, p = 0.024) and abnormal pairs (r = 0.24, p = 0.03). After 28 months, MGII scores correlated with jitter (r = 0.31, p = 0.006), abnormal pairs (r = 0.29, p = 0.009), and pairs with blocks (r = 0.35, p = 0.001). Changes in MGII scores correlated with changes in jitter (r = 0.35, p = 0.002), abnormal pairs (r = 0.27, p = 0.014), and pairs with blocks (r = 0.36, p = 0.001). Conclusions: Concentric needle jitter analysis may have the potential to evaluate baseline and sequential disease severity in MG. Significance: This study highlights the potential for improved MG patient care through precise assessment and management using concentric needle jitter analysis to improve the accuracy of MG diagnosis and monitoring of disease activity.

FcRN receptor antagonists in the management of myasthenia gravis.

Myasthenia gravis (MG) is an autoimmune disorder characterized by autoantibodies specifically directed against proteins located within the postsynaptic membrane of the neuromuscular junction. These pathogenic autoantibodies can be reduced by therapies such as plasma exchange, IVIG infusions and other immunosuppressive agents. However, there are significant side effects associated with most of these therapies. Since there is a better understanding of the molecular structure and the biological properties of the neonatal Fc receptors (FcRn), it possesses an attractive profile in treating myasthenia gravis. FcRn receptors prevent the catabolism of IgG by impeding their lysosomal degradation and facilitating their extracellular release at physiological pH, consequently extending the IgG half-life. Thus, the catabolism of IgG can be enhanced by blocking the FcRn, leading to outcomes similar to those achieved through plasma exchange with no significant safety concerns. The available studies suggest that FcRn holds promise as a versatile therapeutic intervention, capable of delivering beneficial outcomes in patients with distinct characteristics and varying degrees of MG severity. Efgartigimod is already approved for the treatment of generalized MG, rozanolixizumab is under review by health authorities, and phase 3 trials of nipocalimab and batoclimab are underway. Here, we will review the available data on FcRn therapeutic agents in the management of MG.

Disease Transmission and Diagnosis of Zika Virus.

An arbovirus belonging to the Flaviviridae family and the Flavivirus genus, the Zika virus (ZIKV), has profoundly transformed global health perception. Historically, ZIKV infections were considered infrequent, with generally mild manifestations. However, this perception changed dramatically when the virus quickly spread from Asia to the Americas, impacting many nations. It was alarming that there was a connection between ZIKV infection in pregnant women and the beginning of microcephaly in their offspring. ZIKV control and treatment are further complicated because Aedes mosquitoes, which primarily bite during the day, are the primary vectors of the virus. ZIKV diagnostic processes are complex since the virus shares symptoms with other illnesses like dengue and chikungunya. Despite the effectiveness of current diagnostic methods like real-time reverse transcription-polymerase chain reaction (RT-PCR) and enzyme-linked immunosorbent assay (ELISA), there is a clear need for more accurate antibody tests. This is especially true given that many people undergo testing while asymptomatic or after the ideal detection window. The capacity of ZIKV to infect human-derived neural progenitor cells raises worrying possibilities for severe neurological effects. With all these characteristics and their connection to birth abnormalities, research efforts into the virus's efficient treatment and prevention have increased. Overall, the emergence of ZIKV has demonstrated the necessity of a comprehensive and team-based strategy to address its myriad problems. This entails comprehending its transmission dynamics, enhancing diagnostic accuracy, and creating efficient therapies and preventive measures, all crucial to lessening the threat that ZIKV poses to the world's health.