Pharmacovigilance practice among pediatric neurologists from Poland and Germany.

OBJECTIVES: To compare the pediatric neurologists' knowledge, practice, and barriers to the pharmacovigilance (PV) process in Poland and Germany. METHODS: The research tool was an online anonymous questionnaire on Google Forms e-mailed to pediatric neurologists from Poland and Germany. RESULTS: The questionnaires were handed out to 830 pediatric neurologists and 371 expressed their consent to participate in the study. Most of the neurologists were familiar with the definition of PV and adverse drug reactions (ADRs). Only 34.10% of pediatric neurologists from Poland, and 38.88% from Germany believe that many ADRs are preventable and almost most of them believe it is necessary to report ADRs from children with epilepsy. Unfortunately, in opposite to this knowledge, only 37.79% of respondents from Poland and 40.32% from Germany felt co-responsible for reporting ADRs. The main reason for the neurologists not to report ADRs was a conviction that reporting ADRs would be an additional burden generating extra work. CONCLUSION: There is no big difference between the practice of PV by pediatric neurologists in Poland and Germany. System-regulated PV stabilization in the country translates into the practice of maintaining PV. Monitoring the safety of pharmacotherapy and knowledge of risks associated with ADRs should be included in the curricula of academic neurologics courses.

Epilepsy is a chronic disorder characterized by episodic, gratuitous seizures. Most children with epilepsy (CWE) rely on antiepileptic drugs causing adverse drug reactions (ADRs). Many ADRs are preventable if physicians actively participate in pharmacovigilance (PV), which its pivotal role is to ensure the safety of pharmacotherapy by e.g. permanent control of ADRs. The study aimed to compare the pediatric neurologists' (PN) knowledge, practice, and barriers to the PV process in Poland and Germany. The research tool was an online anonymous questionnaire on Google Forms e-mailed to PN from Poland and Germany. Only 34.10% of PN from Poland and 38.88% from Germany believe that many ADRs are preventable and almost most of them believe it is necessary to report ADRs from CWE. Unfortunately, in opposite to this knowledge, only 37.79% of respondents from Poland and 40.32% from Germany felt co-responsible for reporting ADRs. The main reason for the neurologists not to report ADRs was a conviction that reporting ADRs would be an additional burden generating extra work. There is no big difference between the practice of PV by PN in Poland and Germany. System-regulated PV stabilization in the country translates into the practice of maintaining PV.

Spontaneous continuous motor unit single discharges.

INTRODUCTION: Motor unit hyperexcitability (MUH) may become manifest in needle electromyography (EMG) recordings as fasciculation potentials, myokymic discharges, or neuromyotonic discharges. Here, we describe a further MUH phenomenon. METHODS: Needle EMG recordings of the Neurology Hospital of Halle (Saale) stored in a video mode as .wav data between 2000 and 2015 were screened for spontaneous continuous motor unit single discharges (SCMUSD). RESULTS: We identified 23 video needle EMG waveforms from 14 patients with SCMUSD. The corresponding motor units discharged at a rate of about 6 H Z (6.3 ± 4.0; range, 1.3-18.1). The coefficient of variation of the discharge rate was 3.5% ± 1.7%. Neurogenic disorders were diagnosed in 12 patients, limb girdle muscle dystrophy was diagnosed in one patient, and stiff-limb syndrome was diagnosed in one patient. DISCUSSION: Spontaneous continuous motor unit single discharge, as described here, widens the spectrum of MUH phenomena.

Nocturnal paroxysmal dystonia - case report.

Nocturnal paroxysmal dystonia describes a syndrome consisting of recurrent motor episodes of dystonic-dyskinetic features arising from non-rapid eye movement (NREM) sleep. In the article, the authors present female case of nocturnal paroxysmal dystonia. The patient has had attacks since her childhood and was eventually diagnosed at the age of 48. Therapy with carbamazepine considerably reduced the frequency and entent of seizures. The present case evidences that nocturnal paroxysmal dystonia still is a diagnostic challenge for clinicians. Especially, we emphasize the importance of polysomnography in the verification of the diagnosis.

Complex Repetitive Discharges: A Sign of Motor Axonal Reinnervation?

Complex repetitive discharges (CRDs) are poorly understood phenomena in needle electromyography (EMG) recordings. The data presented here suggest that CRDs may mainly be a sign of motor unit reinnervation. EMG "video" data of 108 CRDs from neurogenic (ND, n = 39) and myogenic (MD, n = 14) disorders were retrospectively analyzed for cycle duration, potential-free time intervals, spike components (SC), maximum amplitudes, blockade, and increased jitter. CRD-SC in ND disorders (9.3 ± 7.8) outnumbered those in MD disorders (6.3 ± 6.2). The CRD cycle duration was correlated with SC and silent periods (p each < 0.000001). Blockade was observed in 36% and increased jitter in 27% of the CRDs. A higher number of CRD-SC in ND vs. MD fits the known differences in motor unit dimensions. Blockade and increased jitter are known features of diseased neuromuscular junctions, such as during reinnervation. The SC patterns of single CRD cycles resemble reinnervation potentials. Thus, CRDs may result from myo-axonal re-excitation in sprouting motor units. The purpose of this investigation was to better understand the circumstances under which CRDs may occur and eventually to contribute to the understanding of their pathogenesis.

Motor unit potentials of the erector spinae muscle by concentric needle electromyography.

INTRODUCTION: The needle electromyography (EMG) serves to supply additional information in patients with suspected neuromuscular disorders. We aimed to provide motor unit potential (MUP) data by concentric needle EMG in the erector spinae (ES) in comparison with biceps brachii (BB) and lateral vastus (LV). METHODS: Electromyography MUP data (n) were obtained in ES (517), BB (539), and LV (627) in 32 healthy volunteers (16f). RESULTS: Motor unit potential data: amplitude (µV) 393 ± 174 (ES), 375 ± 162 (BB), and 577 ± 304 (LV); duration (ms) 10.4 ± 2.4 (ES), 10.1 ± 2.1 (BB), and 11.1 ± 2.3 (LV), area (µV × ms) 585 ± 327 (ES), 538 ± 267 (BB), and 881 ± 492 (LV); phase number 3.23 ± 0.94 (ES), 2.98 ± 0.76 (BB), and 3.19 ± 0.81 (LV); size index 0.60 ± 0.56 (ES), 0.51 ± 0.53 (BB), and 0.96 ± 0.55 (LV). LV displayed higher values (p at least <.001) for MUP amplitude, duration, area, and size index as compared to both, BB and ES. CONCLUSION: Concentric needle EMG investigations in healthy adult human subjects revealed similar MUP parameters in the ES and BB muscles, while in the LV muscle MUP amplitude, duration, area, and size index were significantly larger. Different neuromuscular disorders display a predominant involvement of proximally located muscles such as truncal muscles. The present results given here may facilitate the diagnosis of neuromuscular disorders.