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Study design: We performed transcranial magnetic stimulation (TMS) in children with sequelae of acute transverse myelitis. Single-pulse TMS protocol was implemented. Twenty controls and 24 patients with myelitis were enrolled. TMS was performed on 12-24 day after the onset of the first symptoms, average on 16th day. Objectives: The objective of our study was to evaluate motor pathways in children with viral myelitis using the TMS technique. Setting: All investigations were performed in Scientific Research Institute for Children's Infections, Russia. Results: There were statistically significant differences between the groups on central motor conduction time and motor evoked potential (MEPs) amplitudes. MEP thresholds were elevated, and MEP shape was abnormal in 96% of patients with myelitis (n = 23). Three neurophysiologic patterns were observed: the presence of both cortical and spinal MEP, the absence of cortical MEP with the spinal MEP present and the total absence of both cortical and spinal MEP. Last finding was associated with paraplegia, resistant to any sort of treatment. Conclusion: Thus, myelitis in 96% of the cases causes neurophysiologic changes, which may be detected by TMS; the method may be used as a predicting tool. Spinal Cord (2016) 54, 226-228; doi:10.1038/sc.2015.129; published online 4 August 2015 INTRODUCTIONAcute transverse myelitis is the infrequent, severe and potentially devastating condition that has been estimated to occur with an annual worldwide incidence of 4 cases per 100 000 individuals. 1 In pediatric population, it often results in motor, sensory and autonomic dysfunction, with growing demand for reliable diagnostic and prognostic tools. 2 Clinical examination in children is sometimes unreliable, and standard neurophysiologic tests could be insufficient in setting precise diagnosis; thus, transcranial magnetic stimulation (TMS) is considered as one of the valuable diagnostic methods. 3,4 TMS is a method for focal noninvasive brain stimulation that is based on the principles of electromagnetic induction. 5 TMS is an effective diagnostic and a therapeutic technique with applications in neurology, psychiatry and rehabilitation medicine, which can provide insight into developmental neurology and neurophysiology in children. 6 TMS can detect corticospinal tract abnormalities not identified by neuroimaging. 3,4,6 In the patients with compressive myelopathies, TMS was found to be helpful in determining the functional significance of neuroimaging findings. TMS can supplement clinical examination and neuroimaging findings also in the assessment of the spinal cord injury level. 3,4 However, reports of TMS use in pediatric acute myelitis are relatively rare, and mostly such works are case reports or small series consisting of 2-3 patients. 7 We have performed the investigation of corticospinal pathway characteristics in children with acute myelitis, with special attention toward clinical significance of TMS and its prognostic value in this case.

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Primary immune deficiencies are usually attributed to genetic defects and, therefore, frequently referred to as inborn errors of immunity (IEI). We subjected the genomic DNA of 333 patients with clinical signs of IEI to next generation sequencing (NGS) analysis of 344 immunity‐related genes and, in some instances, additional genetic techniques. Genetic causes of the disease were identified in 69/333 (21%) of subjects, including 11/18 (61%) of children with syndrome‐associated IEIs, 45/202 (22%) of nonsyndromic patients with Jeffrey Modell Foundation (JMF) warning signs, 9/56 (16%) of subjects with periodic fever, 3/30 (10%) of cases of autoimmune cytopenia, 1/21 (5%) of patients with unusually severe infections and 0/6 (0%) of individuals with isolated elevation of IgE level. There were unusual clinical observations: twins with severe immunodeficiency carried a de novo CHARGE syndrome‐associated SEMA3E c.2108C>T (p.S703L) allele; however, they lacked clinical features of CHARGE syndrome. Additionally, there were genetically proven instances of Netherton syndrome, Х‐linked agammaglobulinemia, severe combined immune deficiency (SCID), IPEX and APECED syndromes, among others. Some patients carried recurrent pathogenic alleles, such as AIRE c.769C>T (p.R257*), NBN c.657del5, DCLRE1C c.103C>G (p.H35D), NLRP12 c.1054C>T (p.R352C) and c.910C>T (p.H304Y). NGS is a powerful tool for high‐throughput examination of patients with malfunction of immunity.

Background. Unlike parameters of phrenic nerve conduction in healthy adults characterized by stability, in children variations with age are observed. The objective is to investigate the M-wave latency and amplitude in electroneurographic (ENG) examination of the phrenic nerve in healthy children of different ages for development of a normative database of neurophysiological data.Materials and methods. 48 healthy children (28 girls and 20 boys) were examined. Mean age was 9.19 ± 5.43 years (1–18 years). ENG examination of the phrenic nerve was performed with the modified standard procedure of stimulation at the level of the outer margin of the lower third of the sternocleidomastoid muscle with registration of M-wave from standard diaphragm point and parallel registration of the muscle activity from m.deltoideus and m.serratus anterior.Results. Mean values of the M-response latency were 5.64 ± 1.25 ms, amplitude – 0.66 ± 0.34 mV. For age-differentiated subgroups 1–2 years (n = 7), 3–5 years (n = 9), 6–12 years (n = 15), and 13–18 years (n = 17), the latency was 4.96 ± 1.94; 5.01 ± 1.13; 5.42 ± 0.84, and 6.44 ± 1.43 ms, respectively; the amplitude was 1.01 ± 0.37; 0.87 ± 0.31; 0.61 ± 0.24, and 0.45 ± 0.21 mV, respectively. The M-response amplitude values in children aged 1–2 years significantly differed from the values in children aged 6–12 and 13–18 years.Conclusion. ENG examination of the phrenic nerve is a technically uncomplicated procedure, and the obtained data is easy to interpret. During phrenic nerve ENG in children, it is necessary to take age variability of the M-wave latency and amplitude into account. The M-wave amplitude in healthy toddlers (1–2 years old) was significantly lower than in children aged 6–18 years.

Background: Gadolinium is known to produce side effects and has recently been shown to accumulate in the brain parenchyma especially in the basal ganglia. Since RRMS patients do undergo several MR examinations, these potential risks have to be considered in this group of patients.Although guidelines advocate the use of gadolinium enhanced imaging in the follow-up of RRMS patients, the benefit of the use of gadolinium enhancement in the subgroup treated with natalizumab could be questionable. Methods and materials: After IRB approval 201 brain scans and 24 spine exams were reviewed in consensus by 3 readers in a series of 27 patients treated with natalizumab (PS, EC, PwdG). These 27 patients were followed during a time span of 6 to 84 months (mean 49 months). Evolution of lesion load and contrast enhancement were scored. Particular interest was given to the diagnosis of PML. The results were compared with the clinical status and the treatment as reported in the patient charts. Results: Clinical evaluation resulted in 12 episodes of clinical relapse in patients treated with natalizumab, 5 patients demonstrated respectively 1, 2 or 3 relapses.None of the MR scans in the asymptomatic patients nor in the symptomatic patient group demonstrated contrast enhancement. No sign of PML were detected. Conclusion: The benefit of T1 MR sequences post contrast as put forward in many guidelines could be questionable in the subgroup of RRMS patients treated with natalizumab.

Bacterial purulent meningitis is a life-threatening disease characterized by high mortality and severe consequences in survivors. Despite the modern possibilities of medicine, the disease continues to be a heavy burden on health care, the economy and society everywhere.Aim. To draw the attention of doctors to the problems associated with modern features of epidemiology, the consequences and possibilities of preventing bacterial purulent meningitis, especially in children, who constitute the main risk group for the development of this pathology.Literature review of Russian and foreign publications on the problem under consideration presented.Vaccination is recognized as one of the main tools for reducing morbidity and mortality from meningitis. Prophylactic vaccinations against N. meningitidis, Str.pneumoniae, H. influenzae, along with strict adherence to anti-epidemic measures in hospitals providing care to newborns, can help reduce the incidence of purulent meningitis in children and improve outcomes if they develop.

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