Electrophysiological Testing Research Articles

Fibrin Glue Acutely Blocks Distal Muscle Contraction after Confirmed Polyethylene Glycol Nerve Fusion: An Animal Study.

Polyethylene glycol (PEG) is a synthetic, biodegradable, and hyperosmotic material promising in the treatment of acute peripheral nerve injuries. Our team set out to investigate the impact of fibrin glue upon PEG fusion in a rat model. Eighteen rats underwent sciatic nerve transection and PEG fusion. Electrophysiologic testing was performed to measure nerve function and distal muscle twitch. Fibrin glue was applied and testing repeated. Due to preliminary findings, fibrin glue was applied to an uncut nerve in five rodents and testing was conducted before and after glue application. Mann-Whitney U tests were used to compare median values between outcome measures. A Shapiro-Wilk test was used to determine normality of data for each comparison, significance set at a P value less than 0.05. PEG fusion was confirmed in 13 nerves with no significant change in amplitude (P = 0.054), latency (P = 0.114), or conduction velocity (P = 0.114). Stimulation of nerves following PEG fusion produced distal muscle contraction in 100% of nerves. Following application of fibrin glue, there was a significant reduction in latency (P = 0.023), amplitude (P < 0.001), and conduction velocity (P = 0.023). Stimulation of the nerve after application of fibrin glue did not produce distal muscle twitch. Five uncut nerves with fibrin glue application blocked distal muscle contraction following stimulation. Our data suggest that fibrin glue alters the nerve's function. The immediate confirmation of PEG fusion via distal muscle twitch is blocked with application fibrin glue in this experimental model. Survival and functional outcome studies are necessary to understand if this has implications on the long-term functional outcomes.

Multifocal electroretinogram in the diagnosis of Stargardt disease

Aims/Purpose: Electrophysiological vision tests have been a key method in the diagnosis and monitoring of inherited retinal dystrophies (IRD). With the development of mfERG, the clinical applications of these tests have expanded to cone dystrophies such as Stargardt disease, which is the most common cone‐IRD. This disease is typically associated with an autosomal recessive inheritance pattern, primarily, although not exclusively, due to mutations in the ABCA4 gene. The introduction of different diagnostic techniques such as Optical Coherence Tomography (OCT), OCT Angiography (OCTA), autofluorescence (FAF) and microperimetry has diminished the importance of fluorescein angiography (FA). These techniques have been joined by the diagnostic role of mfERG.The aim of this study is to evaluate functional changes in patients with Stargardt disease using mfERG.Methods: Eighteen eyes of patients with Stargardt disease were included. The disease was previously confirmed by genotyping with homozygous ABCA4 mutations or heterozygous PROM1 mutations. The patients were evaluated using mfERG, using the standardized international ISCEV guidelines. Electrical density and implicit time of each first‐order response (K1) in the five concentric rings centered on the fovea were measured and analysed.Results: Mean best‐corrected visual acuity (BCVA) was 0.5 (0.12–1.0). First‐order responses (K1) showed a decreased electrical density, predominantly in central rings, along with minimally prolonged latencies of the P1. FAF showed central atrophy in 6 patients, atrophy with flecks within or beyond the vascular branches in 8 and 4 patients respectively.Conclusions: Stargardt disease involves cones and is characterized by a significant decrease in foveal and central response in mfERG, even in early stages when BCVA remains relatively preserved and fundus findings are minimal. Flash ERG may show normal results or minimal abnormalities.

Stereotactic Awake Basal Ganglia Electrophysiological Recording and Stimulation (SABERS): A Novel Staged Procedure for Personalized Targeting of Deep Brain Stimulation in Pediatric Movement and Neuropsychiatric Disorders.

Selection of targets for deep brain stimulation (DBS) has been based on clinical experience, but inconsistent and unpredictable outcomes have limited its use in patients with heterogeneous or rare disorders. In this large case series, a novel staged procedure for neurophysiological assessment from 8 to 12 temporary depth electrodes is used to select targets for neuromodulation that are tailored to each patient's functional needs. Thirty children and young adults underwent deep brain stimulation target evaluation with the new procedure: Stereotactic Awake Basal ganglia Electrophysiological Recording and Stimulation (SABERS). Testing is performed in an inpatient neuromodulation monitoring unit over 5-7 days, and results guide the decision to proceed and the choice of targets for permanent deep brain stimulation implantation. Results were evaluated 3-6 months postoperatively with the Burke-Fahn-Marsden Dystonia Rating Scale and the Barry-Albright Dystonia Scale. Stereotactic Awake Basal ganglia Electrophysiological Recording and Stimulation testing allowed modulation to be tailored to specific neurologic deficits in a heterogeneous population, including subjects with primary dystonia, secondary dystonia, and Tourette syndrome. All but one subject were implanted with 4 permanent deep brain stimulation leads. Results showed significant improvement on both scales at postoperative follow-up. No significant adverse events occurred. Use of the Stereotactic Awake Basal ganglia Electrophysiological Recording and Stimulation protocol with evaluation in the neuromodulation monitoring unit is feasible and results in significant patient benefit compared with previously published results in these populations. This new technique supports a significant expansion of functional neurosurgery to predict effective stimulation targets in a wide range of disorders of brain function, including those for which the optimal target is not yet known.

Dock4 contributes to neuropathic pain by regulating spinal synaptic plasticity in mice.

Neuropathic pain (NP) conditions arising from injuries to the nervous system due to trauma, disease, or neurotoxins are chronic, severe, debilitating, and exceedingly difficult to treat. However, the mechanisms of NP are not yet clear. Here we explored the role of Dock4, an atypical Rac1 GEF, in the development of NP. Mechanical allodynia was assessed as paw withdrawal threshold by a dynamic plantar aesthesiometer. Immunofluorescence staining was conducted to investigate the expression and localization of Dock4, Rac1 and GluN2B. Quantitative analysis of Dock4, Rac1 and GluN2B were determined by qRT-PCR and Western blot assay. Spontaneous excitatory and inhibitory postsynaptic currents in spinal cord slices were examined using whole cell patch clam. Dendritic spine remodeling and synaptogenesis were detected in cultured dorsal spinal neurons. We found that SNL caused markedly mechanical allodynia accompanied by increase of Dock4, GTP-Rac1and GluN2B, which was prevented by knockdown of Dock4. Electrophysiological tests showed that SNL facilitated excitatory synaptic transmission, however, this was also inhibited by Dock RNAi-LV. Moreover, knockdown of Dock4 prevented dendritic growth and synaptogenesis. In summary, our data indicated that Dock4 facilitated excitatory synaptic transmission by promoting the expression of GluN2B at the synaptic site and synaptogenesis, leading to the occurrence of NP.

Inactivation of the NLRP3 inflammasome mediates exosome-based prevention of atrial fibrillation.

Rationale: Extracellular vesicles (EVs) from human explant-derived cells injected directly into the atria wall muscle at the time of open chest surgery reduce atrial fibrosis, atrial inflammation, and atrial fibrillation (AF) in a rat model of sterile pericarditis. Albeit a promising solution to prevent postoperative AF, the mechanism(s) underlying this effect are unknown and it is not clear if this benefit is dependent on EV dose. Methods: To determine the dose-efficacy relationship of EVs from human explant-derived cells in a rat model of sterile pericarditis. Increasing doses of EVs (106, 107, 108 or 109) or vehicle control were injected into the atria of middle-age male Sprague-Dawley rats at the time of talc application. A sham control group was included to demonstrate background inducibility. Three days after surgery, all rats underwent invasive electrophysiological testing prior to sacrifice. Results: Pericarditis increased the likelihood of inducing AF (p<0.05 vs. sham). All doses decreased the probability of inducing AF with maximal effects seen after treatment with the highest dose (109, p<0.05 vs. vehicle). Pericarditis increased atrial fibrosis while EV treatment limited the effect of pericarditis on atrial fibrosis with maximal effects seen after treatment with 108 or 109 EVs. Increasing EV dose was associated with progressive decreases in pro-inflammatory cytokine content, inflammatory cell infiltration, and oxidative stress. EVs decreased NLRP3 (NACHT, LRR, and PYD domains-containing protein-3) inflammasome activation though a direct effect on resident atrial fibroblasts and macrophages. This suppressive effect was exclusive to EVs produced by heart-derived cells as application of EVs from bone marrow or umbilical cords did not alter NLRP3 activity. Conclusions: Intramyocardial injection of incremental doses of EVs at the time of open chest surgery led to progressive reductions in atrial fibrosis and inflammatory markers. These effects combined to render atria resistant to the pro-arrhythmic effects of pericarditis which is mechanistically related to suppression of the NLRP3 inflammasome.

The role of electrophysiology tests and multimodal imaging in evaluation of nystagmus

Purpose This work aimed to highlight the role of electrophysiology testing and multimodal imaging in evaluation of nystagmus. Patients and methods This is a retrospective multi-centered analytic study that recruited patients with nystagmus from the outpatient clinics referred to the electrophysiology units of the hospitals of the University of Minia, Ain Shams, Banha, and Cairo, Egypt. The study included 60 (120 eyes) patients. The performed investigations included full-field electroretinography (ffERG), pattern electroretinography, flash visual evoked potential (FVEP), pattern visual evoked potential, optical coherence tomography (OCT), OCT-angiography (OCT-A), and fundus autofluorescence (FAF), whenever possible. Results There were no or minimal detectable fundus changes in 78 (65%) eyes, of which 40 (33.3%) eyes showed severe loss of photopic responses on ffERG, with Bull’s eye pattern observed on FAF and loss of foveal photoreceptors in OCT, while 18 (12.5%) eyes showed reduced photopic and scotopic responses in ffERG with loss of foveal photoreceptors and thinned parafovea in OCT, 14 (11.7%) eyes showed average photopic and scotopic responses with different stages of foveal hypoplasia in OCT and OCT-A, and six (5%) eyes showed normal results in tests assessing the function and structure of the macula and optic nerve, including ERG, FVEP, and OCT. On the other hand, macular or optic nerve abnormalities were observed in 42 (35%) eyes, of which 20 (16.7%) eyes showed significant loss of photopic responses in ffERG and abnormal fovea in OCT and FAF tests, 10 (8.3%) eyes showed loss of photopic and scotopic responses in ffERG and abnormal results in the OCT and FAF, four (3.3%) eyes had normal results in ERG and FVEP, but showed fovea hypoplasia in OCT, and eight (6.7%) eyes had normal photopic and scotopic responses by ffERG, but with abnormal visual evoked potential responses and thinning of the nerve fiber layer in the OCT. Conclusion Electrophysiological tests and multimodal imaging are crucial to diagnosing the underlying causes of nystagmus, especially in patients with normal fundus.

The role of mitochondrial dysfunction in the stabilization of the glaucomatous process

This study determines the extent of mitochondrial dysfunction in patients with primary open-angle glaucoma (POAG) and evaluates the potential for stabilizing the glaucomatous process by improving mitochondrial functional activity and energy production by therapy with Mexidol and Mexidol FORTE 250. The study included 80 patients with moderate POAG with compensated intraocular pressure and 20 healthy volunteers. The extent of mitochondrial dysfunction was assessed by measuring the activity levels of mitochondrial enzymes: succinate dehydrogenase (SDH) and α-glycerophosphate dehydrogenase (α-GPDH) in peripheral blood lymphocytes using cytochemical analysis and cytometric morphology and density analysis (cytomorphodensitometry). Patients in the main group received sequential therapy with Mexidol as follows: Mexidol solution for intravenous and intramuscular administration at 50 mg/ml, 300 mg daily intramuscularly for 14 days, followed by Mexidol FORTE 250 tablets, one tablet three times daily for 56 days. Stabilization of glaucomatous optic neuropathy during treatment was evaluated using a comprehensive set of perimetric, electrophysiological, and structural-topographical methods at 14, 56, and 90 days. Sequential therapy in the main group resulted in a significant increase in mitochondrial enzyme activity at 14 and 56 days compared to baseline, with a gradual regression by the end of the observation period (90 days). This was accompanied by an increase in the number of mitochondria and an increase in their optical density as measured by cytomorphodensitometry. The improvement in mitochondrial enzyme activity at 14 and 56 days was associated with positive changes in the structural and functional parameters of the retina, as evidenced by static perimetry, optical coherence tomography, and a series of electrophysiological tests. The obtained data can be used to optimize POAG therapy by reducing mitochondrial dysfunction and stabilizing glaucomatous optic neuropathy.

A simple method for preservation of embryonic rat brain tissue for primary neuron culture

Aims/Purpose: In this study we aim to investigate the effect of short‐term storage of embryonic rat brain tissue in Hibernate‐E medium to be used in the isolation of primary neurons for in vitro biocompatibility and electrophysiology tests of retinal and optic nerve prosthesis. Functionally viable neuron cells will be an alternative method for regular dissection from tissue for further experiments.Methods: Hippocampal tissues from E15‐E17 embryos of Wistar Albino rat were dissecting for primary neuron culture. Half of the tissues were isolated on the same day and used as a control group for further experiments. The test group was stored in a Hibernate‐E medium for 1 week and the same isolation method was performed for the test group. For tissue preservation, half medium replacement with fresh Hibernate‐E medium on the 4th day was enhanced cell viability. Morphology, characterization and synapse formation were determined with immunoflorescence staining.Results: Comparison of neuronal survival between cultures prepared from stored hippocampi and freshly isolated hippocampi resulted in similar neuronal survival rates in both preparations with cell viability assays. Cell viability rate of hippocampus tissue isolated from rat embryo brain was demonstrated as 96% and cell viability rate of preserved tissue in Hibernate‐E Medium for 7 days was revealed as 90.22% by trypan blue cell viability test (n = 4). Morphological and immunocytochemical characterizations of stored and fresh hippocampal cultures were similar by observing beta‐III Tubulin neuron cytoskeleton biomarker.Conclusions: As a conclusion, our method has the potential to increase repeatability of primary neuron cultures within 1 week and reduce the number of isolation steps in research. In addition, it will provide a great convenience in the experimental timelines of the researchers and contribute to the reduction of the workload despite any obstacles during primary neuron culture.

Ultrasound and magnetic resonance imaging neurography assessment of diagnostic criteria in patients with carpal tunnel syndrome using electrophysiological tests as gold standard: A prospective study.

Evaluating peripheral neuropathy mainly relies on physical examination, patient history, and electrophysiological studies. High-resolution ultrasound is a fast, noninvasive modality for dynamic nerve assessment that enables the length of the nerve to be examined. Magnetic resonance imaging is preferred for examining deeper nerves with a high contrast resolution; its use shows excellent benefit in patients with atypical presentation, equivocal diagnosis, suspected secondary causes, and postsurgical relapse. We aimed to assess the measurements and criteria for both ultrasound and magnetic resonance neurography for the diagnosis of carpal tunnel syndrome, based mainly on the three measurements assessed by Buchberger et al. This prospective study was conducted to test diagnostic accuracy. Thirty-two patients who presented clinically with, and were diagnosed by electrophysiological tests as having, carpal tunnel syndrome participated. Superficial ultrasound of the wrist joint was performed on all participants, followed by magnetic resonance imaging within 1 week of ultrasonography. The three main parameters of cross-sectional area measurement, distal nerve flattening, and flexor retinaculum bowing indices showed positive occurrences of 93.7%, 59.4%, and 59.4%, respectively; 90.6% of patients had decreased nerve echotexture. The diagnostic ability of magnetic resonance imaging was decreased when cross-sectional area measurements were used: positive results were achieved in 81.2% of patients, but the positive results showing the distal tunnel nerve increased flattening and bowed flexor retinaculum slightly decreased to 56.2% for each. A high T2 signal of the median nerve was observed in 90.6% of patients. In an agreement analysis, we found a statistically significant difference that supported the use of ultrasound as a primary diagnostic modality for carpal tunnel syndrome. However, magnetic resonance imaging improved tissue characterization and was a good diagnostic modality, with a statistically significant difference, for cases of secondary carpal tunnel syndrome, detection of the underlying entrapping cause, and early abnormality detection in the innervated muscle. Our results demonstrate that ultrasound examination can be used as the first imaging modality after physician evaluation, with results comparable to those of electrophysiological studies for evaluating carpal tunnel syndrome and determining its cause. Magnetic resonance neurography examination is the second step in detecting secondary causes in cases with suspected early muscle denervation changes that cannot be elicited by ultrasound or in cases with equivocal results.

Znaczenie elektrofizjologii siatkówki w chorioretinopatii typu Birdshot

Birdshot chorioretinopathy is a rare disease of probable autoimmune origin. Periodic retinal electrophysiological testing plays a significant role in the diagnosis and monitoring of this disease. This article reviews the literature available in pubmed from1988–2022 on Birdshot chorioretinopathy, with a focus on retinal electrophysiological studies.

Electrophysiology in Functional Movement Disorders: An Update.

Functional movement disorders (FMD) are a diagnostic and therapeutic challenge, both to the neurologist and psychiatrists. The phenomenology is varied and can present as tremors, dystonia, jerks/myoclonus, gait disorder, other abnormal movements or a combination. There has been an increase in the use of electrophysiological studies that are an important tool in the evaluation of FMDs. We searched the database platforms of MEDLINE, Google scholar, Web of Sciences, Scopus using the Medical Subject Heading terms (MeSH) for all the articles from 1st January 1970 till November 2022. A total of 658 articles were obtained by the search mechanism. A total of 79 relevant articles were reviewed thoroughly, of which 26 articles that had electrophysiological data were included in the present review. Variability, distractibility and entertainability can be demonstrated in functional tremors by using multichannel surface electromyography. Voluntary ballistic movements tend to decrease the tremor, while loading the tremulous limb with weight causes the tremor amplitude to increase in functional tremor. Presence of Bereitschaftspotential demonstrates the functional nature of palatal tremor and myoclonus. Co-contraction testing may be helpful in differentiating functional from organic dystonia. The R2 blink reflex recovery cycle has been found to be abnormally enhanced in organic blepharospasm, whereas it is normal in presumed functional blepharospasm. Plasticity is found to be abnormally high in organic dystonia and normal in functional dystonia, in addition to enhanced facilitation in patients with organic dystonia. Electrophysiological tests supplement clinical examination and helps in differentiating FMD from organic movement disorders.

Autoimmune Retinopathy in a Patient with Stiff-Person Syndrome: A Case Report.

To describe a patient who developed retinal degeneration associated with autoimmune retinopathy (AIR) and who was also found to have anti-glutamic acid decarboxylase (GAD65) autoantibodies and the diagnosis of stiff-person syndrome (SPS). Ophthalmologic workup consisted of clinical examination, multi-modality retinal imaging, and electrophysiologic testing. Further neurologic assessment including relevant serum and cerebrospinal fluid studies was also conducted. We highlight the case of a 45-year-old patient who developed subacute, sequential vision loss, along with bilateral lower extremity weakness. On initial presentation, optical coherence tomography (OCT) of the left eye was notable for diffuse attenuation of the outer retinal bands. Fundus autofluorescence demonstrated a ring of hyper-autofluorescence encircling the fovea of the left eye. At fifteen-month follow-up, the right eye also became similarly affected. He was found to have elevated serum and cerebrospinal anti-GAD65 autoantibodies and was diagnosed with both SPS and AIR. There is a potential association of anti-GAD65 autoantibodies with the development of AIR.

Comparison of ASSR and frequency specificity ABR induced by NB CE-Chirp for prediction of behavioral hearing thresholds in children with conductive hearing loss

BackgroundElectrophysiological tests are often used to evaluate hearing loss in infants and young children with conductive hearing loss, no matter to quantify or characterize. However, there are advantages and disadvantages associated with the various electrophysiological tests that are currently available. Therefore, there is no gold standard test. This study aimed to compare the value of narrow-band (NB) CE-Chirp-induced auditory steady-state response (ASSR) and auditory brainstem response (ABR) for assessing hearing thresholds in children with conductive hearing loss. We hope to identify an effective electrophysiological testing method to evaluate conductive hearing loss and provide a reference for clinical hearing assessment of infants with conductive hearing loss. Subjectsand Methods: We selected 27 children (41 ears) aged 3–6 years with otitis media with effusion (OME). Within 1 day, they underwent behavioral audiometry and NB CE-Chirp-induced ASSR and ABR tests in sequence. Pearson's correlation analysis was performed to compare behavioral audiometry thresholds and ASSR and ABR response thresholds at 500, 1000, 2000, and 4000 Hz. ResultsThe behavioral audiometry thresholds of all children were strongly correlated with the response thresholds of the two electrophysiological tests, with correlation coefficients of 0.659, 0.605, 0.723, and 0.857 for ASSR, and 0.587, 0.684, 0.753, and 0.802 for ABR. The proportion of children with a difference of ≤10 dB between ASSR and behavioral audiometry thresholds or between ABR and behavioral audiometry thresholds was not high, especially in the low frequencies. ABR results were superior to ASSR results in terms of predicting actual hearing levels. At 0.5, 1, 2, and 4 kHz, the average differences between the behavioral hearing thresholds and ASSR thresholds in the 41 ears were 5.6, 5.7, 2, and 5.6 dB, respectively. The average differences between behavioral hearing thresholds and ABR thresholds was −5.6, −1.4, −6.8, and 3.2 dB, respectively. The hearing loss configuration of the ASSR exhibited a peaked pattern, similar to behavioral audiometry, whereas the ABR exhibited an ascending pattern. The time to perform the single-ear ASSR test was 5.9 min, whereas the ABR test took 17.0 min. ConclusionASSR and ABR induced by the NB CE-Chirp correlated well with behavioral audiometry in children with conductive hearing loss. The NB CE-Chirp ASSR has advantages in terms of testing time and hearing configuration evaluation, whereas ABR has better reliability than ASSR. However, the stability of ASSR and ABR induced by the NB CE-Chirp is poor, and the thresholds obtained cannot replace behavioral audiometry in evaluating the true hearing of children with conductive hearing loss. However, ASSR and ABR can be used as auxiliary tests for cross-validation.

Ventromedial hypothalamus relays chronic stress inputs and exerts bidirectional regulation on anxiety state and related sympathetic activity.

Chronic stress can induce negative emotion states, including anxiety and depression, leading to sympathetic overactivation and disturbed physiological homeostasis in peripheral tissues. While anxiety-related neural circuitry integrates chronic stress information and modulates sympathetic nervous system (SNS) activity, the critical nodes linking anxiety and sympathetic activity still need to be clarified. In our previous study, we demonstrated that the ventromedial hypothalamus (VMH) is involved in integrating chronic stress inputs and exerting influence on sympathetic activity. However, the underlying synaptic and electrophysiological mechanisms remain elusive. In this study, we combined in vitro electrophysiological recordings, behavioral tests, optogenetic manipulations, and SNS activity analyses to explore the role of VMH in linking anxiety emotion and peripheral SNS activity. Results showed that the VMH played an important role in bidirectionally regulating anxiety-like behavior and peripheral sympathetic excitation. Chronic stress enhanced excitatory inputs into VMH neurons by strengthening the connection with the paraventricular hypothalamus (PVN), hence promoting anxiety and sympathetic tone outflow, an important factor contributing to the development of metabolic imbalance in peripheral tissues and cardiovascular diseases.

A female-specific odorant receptor mediates oviposition deterrence in the moth Helicoverpa armigera

Finding ideal oviposition sites is a task of vital importance for all female insects. To ensure optimal conditions for their progeny, females of herbivorous insects detect not only the odors of a relevant host plant but also chemicals released by eggs, named oviposition-deterring pheromones (ODPs). It is reported that such chemicals play critical roles in suppressing female oviposition behavior; however, the molecular mechanism underlying the detection of egg-derived ODPs remains elusive. Here, we have identified three specific fatty acid methyl esters from the surface of eggs of Helicoverpa armigera serving as ODPs-methyl oleate (C18:1ME), methyl palmitate (C16:0ME), and methyl stearate (C18:0ME). We demonstrated that these ODPs are detected by the receptor, HarmOR56, exclusively expressed in sensilla trichodea on female antennae. To assess the significance of this receptor, we disrupted HarmOR56 in H.armigera using CRISPR-Cas9 and found that mutant females did not respond to the ODPs, neither in behavioral nor in electrophysiological tests. We therefore conclude that HarmOR56 is indispensable for identifying the ODPs. This study explores, for the first time, how a female-specific odorant receptor detects chemicals from conspecific eggs. Our data elucidate the intriguing biological phenomenon of repulsion to conspecific eggs during oviposition and contribute new insight into a female-specific olfactory pathway linked to reproduction.

Task force of the Brazilian Society of Otology — evaluation and management of peripheral facial palsy

ObjectiveTo review key evidence-based recommendations for the diagnosis and treatment of peripheral facial palsy in children and adults. MethodsTask force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on peripheral facial palsy were eligible for inclusion. The American College of Physicians’ guideline grading system and the American Thyroid Association’s guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. ResultsThe topics were divided into 2 main parts: (1) Evaluation and diagnosis of facial palsy: electrophysiologic tests, idiopathic facial palsy, Ramsay Hunt syndrome, traumatic peripheral facial palsy, recurrent peripheral facial palsy, facial nerve tumors, and peripheral facial palsy in children; and (2) Rehabilitation procedures: surgical decompression of the facial nerve, facial nerve grafting, surgical treatment of long-term peripheral facial palsy, and non-surgical rehabilitation of the facial nerve. ConclusionsPeripheral facial palsy is a condition of diverse etiology. Treatment should be individualized according to the cause of facial nerve dysfunction, but the literature presents better evidence-based recommendations for systemic corticosteroid therapy.

Comparison of the Effects of Botulinum Toxin Doses on Nerve Regeneration in Rats with Experimentally Induced Sciatic Nerve Injury.

This study was designed to compare the effects of various doses of botulinum neurotoxin A (BoNT/A) on nerve regeneration. Sixty-five six-week-old rats with sciatic nerve injury were randomly allocated to three experimental groups, a control group, and a sham group. The experimental groups received a single session of intraneural BoNT/A (3.5, 7.0, or 14 U/kg) injection immediately after nerve-crushing injury. The control group received normal intraneural saline injections after sciatic nerve injury. At three, six, and nine weeks after nerve damage, immunofluorescence staining, an ELISA, and toluidine blue staining was used to evaluate the regenerated nerves. Serial sciatic functional index analyses and electrophysiological tests were performed every week for nine weeks. A higher expression of GFAP, S100β, GAP43, NF200, BDNF, and NGF was seen in the 3.5 U/kg and 7.0 U/kg BoNT/A groups. The average area and myelin thickness were significantly greater in the 3.5 U/kg and 7.0 U/kg BoNT/A groups. The sciatic functional index and compound muscle action potential amplitudes exhibited similar trends. These findings indicate that the 3.5 U/kg and 7.0 U/kg BoNT/A groups exhibited better nerve regeneration than the 14 U/kg BoNT/A and control group. As the 3.5 U/kg and the 7.0 U/kg BoNT/A groups exhibited no statistical difference, we recommend using 3.5 U/kg BoNT/A for its cost-effectiveness.

A retrospective study of accuracy and usefulness of electrophysiological exercise tests

ObjectivesThis study aimed to determine the usefulness of electrophysiological exercise tests. The significance of slightly abnormal exercise tests was also examined.MethodsWe identified all the patients who had undergone exercise testing between February 2007 to June 2022 in Tampere University Hospital, Finland. Their medical records after diagnostic workup and exercise test reports were reviewed. A binary logistic regression was performed to evaluate the association between positive test result in short exercise test, long exercise test, or short exercise test with cooling and genetically confirmed skeletal muscle channelopathy or myotonic disorder.ResultsWe identified 256 patients. 27 patients were diagnosed with nondystrophic myotonia, periodic paralysis, myotonic dystrophy type 1, myotonic dystrophy type 2, or other specified myopathy. 14 patients were suspected to have a skeletal muscle channelopathy, but pathogenic variants could not be identified. The remaining 215 patients were diagnosed with other conditions than skeletal muscle channelopathy or myotonic disorder. The combined sensitivity of exercise tests was 59.3% and specificity 99.1%. Abnormal exercise test result was associated with increased risk of skeletal muscle channelopathy or myotonic disorder (OR 164.3, 95% CI 28.3–954.6, p < 0.001).ConclusionsElectrophysiological exercise test is not optimal to exclude skeletal muscle channelopathy. It may be useful if a skeletal muscle channelopathy is suspected and genetic testing is negative or indeterminate and further evidence is required. Slightly abnormal exercise test results are possible in various conditions and result from different aetiologies. There is a demand for neurophysiological studies with higher sensitivity to detect skeletal muscle channelopathies.

Sensitivity of non-conventional electro-diagnostic techniques to detect neuropathy in patients with hepatitis “C” viral infection

BackgroundHepatitis “C” virus (HCV) infection has become a growing global health concern. Chronic HCV infection is usually accompanied by extra-hepatic manifestations. Peripheral neuropathies are the most common neurological complications. Electrophysiological tests play an important role in diagnosing polyneuropathy and identifying its pathophysiology. This study aimed at highlighting the sensitivity of non-conventional, quantitative, electrophysiological techniques in the detection of neuropathy in patients having HCV infection. Forty-five HCV patients were recruited for this study. Conventional motor and sensory conduction studies (NCS) were initially performed followed by interference pattern analysis (IPA). Two quantitative nerve conduction scores were also studied: motor amplitude score (MAS) and sensory amplitude score (SAS).ResultsIn the two patients’ subgroups (having numbness versus having no numbness), MAS showed a significant difference, while the SAS showed a non-significant difference. In the two subgroups (with normal NCS versus those with abnormal/suspected NCS), the MAS and the SAS mean values were significantly different. Also, there was a positive significant correlation found between the IPA mean amplitude and the SAS parameter. Finally, we presented the results of the diagnostic performance of the MAS and the SAS in detecting abnormal NCS.ConclusionsMAS and/or SAS scores could be used as good screening tools for the detection of neuropathy. We here recommend expanding the non-conventional NCSs to other etiologies of polyneuropathy.

Role of electrophysiological testing in the diagnosis of atypical retinitis pigmentosa

Retinitis pigmentosa (RP) is not a single entity but rather a disease spectrum. The classical triad of waxy disc pallor, bony spicules in the fundus, and arteriolar attenuation may not be found in all of the patients. In this series of seven atypical RP cases, we provide an overview of its varied clinical presentations and the role of electroretinogram in its diagnosis.