Autoimmune Neuromuscular Disorders: Emerging Insights and Future Frontiers

: In recent years, our knowledge rapidly increased with respect to the immunology and immunological aspects of neuromuscular disorders [...]

Cathodal Occipital tDCS is unable to modulate The Sound Induced Flash Illusion in migraine

Migraine is a highly disabling disease characterized by recurrent pain.Despite an intensive effort, mechanisms of migraine pathophysiology, still represent an unsolved issue. Evidences from both animals and humans studies suggest that migraine is characterized by hyperresponsivity or hyperexcitability of sensory cortices, especially the visual cortex. This phenomenon, in turn, may affect multisensory processing. Indeed, migraineurs present with an abnormal, reduced, perception of the Sound-induced Flash Illusion (SiFI), a crossmodal illusion that relies on optimal integration of visual and auditory stimuli by the occipital visual cortex. Decreasing visual cortical excitability with transcranial direct current stimulation (tDCS) can increase the SiFI in healthy subjects. Moving from these issues , we applied cathodal tDCS over the visual cortex of migraineurs, with and without aura, in order to decrease cortical excitability and thus physiologically restoring the perception of a reliable SiFI. Differently from our expectations tDCS was unable to reliably modulate SiFI in migraine. The chronic, relatively excessive, visual cortex hyperexcitability, , featuring the migraineur brain, may render tDCS ineffective for restoring multisensory processing in this disease

A pilot study on non-invasive treatment of migraine: The self-myofascial release

The aims of this paper was to determine the effect of self-myofascial release (SMFR) on postural stability and to analyze if it can influence migraine condition. Twenty-five subjects (age 49.7±12.5) affected by migraine were enrolled. Assessments included a stabilometric analysis in order to evaluate balance and plantar support, with eyes open (OE) and closed (CE); cervical ROM measurement; evaluation of upper limb strength through handgrip. All the analysis were carried out before and after the administration of a single SMFR protocol, using medium density small balls laid in the three most painful trigger points in migraine patients: trapezius, sternocleidomastoids and suboccipital muscles. Performing a T test for paired samples, there was a significant increase in two ranges of the stabilometric analysis: ellipse surface, both with open and closed eyes (p value EO = 0.05; p value EC = 0.04) and length of the sway path, but just with closed eyes (p value = 0.05). SMFR might have a positive impact on postural stability in subjects with migraine. Further investigation should be conducted to confirm the hypothesi

Visuospatial attention lateralization in volleyball players and in rowers.

In the present study, differences in visuospatial attention lateralization were evaluated in athletes engaged in open-compared to closed-skill sports and sedentary nonathletes. 23 volleyball players (open skill; Italian national level and regional level), 10 rowers (closed skill, Italian national level), and 23 sedentary participants responded to a computerized line-length judgment task. Five lines, differing in the length of their right and left segments, were randomly presented; the respondent made a forced-choice decision about the respective length of the two segments. Volleyball players responded significantly faster; those at the higher competitive level were also more accurate, making a statistically significantly lower number of leftward errors as compared with rowers and controls. If such responses are due to training rather than self-selection of ability, then the results may suggest the possibility of changing the distribution of visuospatial attention by training in open-skill sports

Telecoaching: a potential new training model for Charcot-Marie-Tooth patients: a systematic review

Introduction: Charcot-Marie-Tooth disease (CMT) is an inherited neuropathy that affects the sensory and motor nerves. It can be considered the most common neuromuscular disease, with a prevalence of 1/2500. Methods: Considering the absence of a specific medical treatment and the benefits shown by physical activity in this population, a systematic review was completed using several search engines (Scopus, PubMed, and Web of Science) to analyze the use, effectiveness, and safety of a training program performed in telecoaching (TC). TC is a new training mode that uses mobile devices and digital technology to ensure remote access to training. Results: Of the 382 studies identified, only 7 met the inclusion criteria. The effects of a TC training program included improvements in strength, cardiovascular ability, and functional abilities, as well as gait and fatigue. However, the quality of the studies was moderate, the size of the participants in each study was small, and the outcome measured was partial. Discussion: Although many studies have identified statistically significant changes following the administration of the TC training protocol, further studies are needed, with appropriate study power, better quality, and a higher sample size

Efgartigimod beyond myasthenia gravis: the role of FcRn-targeting therapies in stiff-person syndrome

Stiff-person syndrome (SPS) is a rare autoimmune neurological disorder characterized by high titers of antibodies against glutamic acid decarboxylase (GAD) causing impaired GABAergic inhibitory neurotransmission. To date, there is not a defined therapy for such condition, but immunomodulating therapies, such as plasma exchange, intravenous immunoglobulins, and rituximab, have been widely used in clinical practice. However, the efficacy and tolerability of these treatments is not well established. Efgartigimod, a new neonatal Fc receptor (FcRn) blocker, is a human IgG1 antibody Fc fragment engineered with increased affinity for FcRn binding, leading to a reduction in IgGs levels, including pathogenic IgG autoantibody showing promising results in neurological autoimmune disorders and has been approved for the treatment of AChR-seropositive generalized myasthenia gravis (MG). In this study, we report and describe the first data on treatment with efgartigimod in three patients affected by both AChR-seropositive generalized MG and anti-GAD-seropositive SPS. Patients were followed since the start of efgartigimod and for the whole treatment period (12 weeks). MG symptoms were assessed with the "MG activity of daily living score" and the Quantitative Myasthenia Gravis score, while SPS ones were assessed with the "SPS activity of daily living score"; muscle strength was assessed with the Medical Research Council Sum score; the overall disability from MG and SPS was assessed by the modified Rankin Scale. All patients showed an improvement in symptoms of both SPS and MG after 2 cycles of treatment. Our data suggest that efgartigimod may be considered as a candidate drug for SPS and other autoantibody-mediated neurological disorders

The effects of estradiol levels on crossmodal perception: a study on the sound induced flash illusion in healthy and menstrually related migraine individuals

ObjectiveThe sound-induced flash illusion (SIFI) is a valid paradigm to study multisensorial perception. In the "fission" SIFI, multiple flashes are perceived when observing a single flash paired with two or more beeps. SIFI is largely dependent on visual and acoustic cortex excitability; in migraine, dysfunctional cortical excitability affects SIFI perception. Since estrogen peak occurring during ovulation can increase neuronal excitability, the present study aims to verify whether cortical excitability shifts linked to the menstrual cycle could influence SIFI.MethodsIn a comparative prospective study, we tested the effect of estrogens on crossmodal perception using the SIFI. We recruited 27 females in reproductive age, including 16 healthy and 11 menstrually related migraine females, testing their proneness to SIFI on day 14 (high estradiol) and day 27 (low estradiol) of menstrual cycle.ResultsWomen on day 14 reported less flashes than on day 27 (p = 0.02) in the fission illusion, suggesting a pro-excitatory effect of estradiol on visual cortex excitability during ovulation. Moreover, we confirmed that migraine women perceived less flashes (p = 0.001) than controls, independently from cycle phase. Non-migraineurs women significantly reported more flashes on day 27 than on day 14 (p = 0.04).ConclusionsThis study suggests that estradiol may influence the multisensory perception due to changes of visual cortex excitability, with high estradiol peak leading to increased visual cortical sensitivity during ovulation in non-migraineurs. Visual cortex hyperresponsiveness, here reflected by reduced SIFI, is not influenced by estradiol fluctuations in migraine women, as shown by reduced fission effects on day 14 and 27

Diagnostic and therapeutic aspects of hemiplegic migraine

Hemiplegic migraine (HM) is a clinically and genetically heterogeneous condition with attacks of headache and motor weakness which may be associated with impaired consciousness, cerebellar ataxia and intellectual disability. Motor symptoms usually last <72 hours and are associated with visual or sensory manifestations, speech impairment or brainstem aura. HM can occur as a sporadic HM or familiar HM with an autosomal dominant mode of inheritance. Mutations in CACNA1A, ATP1A2 and SCN1A encoding proteins involved in ion transport are implicated. The pathophysiology of HM is close to the process of typical migraine with aura, but appearing with a lower threshold and more severity. We reviewed epidemiology, clinical presentation, diagnostic assessment, differential diagnosis and treatment of HM to offer the best evidence of this rare condition. The differential diagnosis of HM is broad, including other types of migraine and any condition that can cause transitory neurological signs and symptoms. Neuroimaging, cerebrospinal fluid analysis and electroencephalography are useful, but the diagnosis is clinical with a genetic confirmation. The management relies on the control of triggering factors and even hospitalisation in case of long-lasting auras. As HM is a rare condition, there are no randomised controlled trials, but the evidence for the treatment comes from small studies