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Myelination of axons provides the structural basis for rapid saltatory impulse propagation along vertebrate fiber tracts, a well-established neurophysiological concept. However, myelinating oligodendrocytes and Schwann cells serve additional functions in neuronal energy metabolism that are remarkably similar to those of axon-ensheathing glial cells in unmyelinated invertebrates. Here we discuss myelin evolution and physiological glial functions, beginning with the role of ensheathing glia in preventing ephaptic coupling, axoglial metabolic support, and eliminating oxidative radicals. In both vertebrates and invertebrates, axoglial interactions are bidirectional, serving to regulate cell fate, nerve conduction, and behavioral performance. One key step in the evolution of compact myelin in the vertebrate lineage was the emergence of the open reading frame for myelin basic protein within another gene. Several other proteins were neofunctionalized as myelin constituents and help maintain a healthy nervous system. Myelination in vertebrates became a major prerequisite of inhabiting new ecological niches.
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Axones , Vaina de Mielina , Animales , Neuroglía , Neuronas , OligodendroglíaRESUMEN
At times electrodiagnostic medical consultants (EMCs) are asked to perform studies in both a neutral position, and then again after the patient is in a provocative position that exacerbates symptoms, to assess for measurable electrophysiologic changes. While this approach might seem initially appealing, particularly when standard studies are not effective at diagnosis, empiric studies in several conditions have been unimpressive. Studies in median neuropathy at the wrist, thoracic outlet syndrome, piriformis syndrome, and radial tunnel syndrome have failed to demonstrate reproducible changes in nerve conduction studies in positions that exacerbate symptoms. Furthermore, there is lack of a plausible pathophysiologic mechanism for producing both measurable and rapidly reversible electrophysiologic changes after just a few minutes, or less, of compression. Axon loss and demyelination would not be rapidly reversible, and positional changes of 2 min or less (the durations generally studied) would be insufficient to produce measurable nerve ischemia. Last, we have gained a greater appreciation for how much nerves move within limbs with changes in joint position; this movement can lead to misleading changes in nerve conduction studies. It is thus appropriate to conclude that testing nerve conduction in provocative or symptomatic positions adds no value to electrodiagnostic testing.
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Síndrome del Túnel Carpiano , Neuropatía Mediana , Síndrome del Desfiladero Torácico , Humanos , Síndrome del Desfiladero Torácico/diagnóstico , Conducción Nerviosa/fisiología , Articulación de la Muñeca , Extremidad Superior , Nervio MedianoRESUMEN
INTRODUCTION/AIMS: Electrodiagnostic examinations, such as nerve conduction studies (NCS) and needle electromyography (EMG), are perceived as painful by children and their parents/guardians. Methods to reduce peri-procedural pain improve compliance and have neurocognitive and neuropsychiatric benefits. This study aimed to assess the efficacy of combined oral and topical analgesics (COTA), oral analgesics (OA), and placebo in reducing pain during NCS/EMG in children. METHODS: We performed a double-blind, randomized, placebo-controlled trial on children presenting to our neurophysiology lab. Patients were stratified into two age groups (6M-6Y and 7Y-18Y) and randomized into three arms: COTA, OA, and placebo. Pain scores post-NCS/EMG were assessed using the Modified Behavioral Pain Scale (MBPS) and Faces Pain Scale-Revised (FPS-R). RESULTS: One hundred thirteen participants were enrolled. A comparison of participants from both age groups combined revealed no significant differences in guardian FPS-R scores across all arms for NCS and EMG. A significant difference in the distribution of post-NCS FPS-R score severities in children aged 7Y-18Y was noted between OA and placebo (p = .007). EMG was more painful than NCS across all arms (p < .05). In children aged 6M-6Y undergoing at least 10 muscle samplings during EMG, those receiving COTA had significantly lower pain scores (p = .014). DISCUSSION: This study reveals the complexity of pediatric pain perception during NCS/EMG and highlights that other methods to reduce experienced pain are required. Our findings suggest that procedural characteristics, such as number of muscles sampled, may influence the effectiveness of analgesia and serve as a foundation for future research aimed at optimizing pain management strategies.
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Administración Tópica , Electromiografía , Dimensión del Dolor , Humanos , Niño , Masculino , Femenino , Adolescente , Método Doble Ciego , Administración Oral , Preescolar , Dimensión del Dolor/métodos , Analgésicos/administración & dosificación , Analgesia/métodos , Electrodiagnóstico/métodos , Conducción Nerviosa/efectos de los fármacos , Conducción Nerviosa/fisiología , Dolor/tratamiento farmacológico , Dolor/diagnósticoRESUMEN
INTRODUCTION/AIMS: Not all patients with chronic inflammatory demyelinating polyneuropathy (CIDP) have evidence of demyelination on nerve conduction studies (NCS). Patients with "supportive" evidence of CIDP on cerebrospinal fluid (CSF), magnetic resonance imaging (MRI), ultrasound (US), or nerve biopsy but not on NCS, often receive immunomodulating therapy. We evaluated the treatment response of patients with clinical and supportive features of CIDP lacking NCS evidence of demyelination. METHODS: Retrospective chart review was conducted on 232 patients who met CIDP clinical criteria and were treated with disease-modifying therapy. Patients included did not have NCS criteria of demyelination, but did have supportive CSF, MRI, or US findings consistent with CIDP. A positive treatment response was defined as at least a one-point improvement in the modified Rankin scale (mRS), or a four-point increase in the Medical Research Council sum score (MRCSS). RESULTS: Twenty patients met criteria: 17 of the 18 (94%) patients with CSF protein >45 mg/dL, 6 of the 14 (43%) with MRI lumbosacral root or plexus enhancement, and 4 of the 6 (67%) with enlarged proximal nerves on US. Eighteen patients received intravenous immunoglobulin, 10 corticosteroids, one plasma exchange, and six other immunomodulatory therapies. Twelve patients had a positive treatment response on the MRCSS or mRS. The presence of MRI lumbosacral root or plexus enhancement was associated with a positive treatment response. DISCUSSION: A trial of immunomodulating treatment should be considered for patients with clinical features of CIDP in the absence of NCS evidence of demyelination, particularly when there is MRI lumbosacral root or plexus enhancement.
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INTRODUCTION/AIMS: Electrodiagnostic (EDX) studies play a crucial role in the evaluation of patients with peripheral nervous system disorders. Accurate and succinct communication of test results is critical to patient safety and clinical decision-making. The objective of this study was to explore EDX reporting preferences of referring physicians to improve quality of communication and patient care. METHODS: An online survey was developed, and a purposive sampling strategy was used to recruit physicians in the authors' professional networks. Quantitative and qualitative survey data underwent frequency and thematic analyses, respectively. RESULTS: There were 40 respondents, including: 21 non-surgical specialists, 12 surgical specialists, and 7 family physicians. Sections rated as most critical were diagnostic impression (97%) and summary/interpretation (72%). Only 18% reported numeric data as critical to their needs, preferring this data to be formatted as bullet points or tables without nerve conduction study waveforms. Regarding the format of the data summary and diagnostic impression sections, the majority of respondents preferred bullet points rather than paragraphs. DISCUSSION: The results of this exploratory survey suggest that physicians who refer patients for EDX studies prefer reports that emphasize the interpretation of EDX data and a clear diagnostic impression, particularly in bullet point format. This project highlights important preferences and how they compare to recommended reporting guidelines, which may help improve communication and ultimately patient care. Future efforts should explore larger sample sizes with all key stakeholders in the EDX process to better understand reporting styles and preferences with greater nuance and context.
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Enfermedades del Sistema Nervioso Periférico , Médicos , Humanos , Encuestas y Cuestionarios , Enfermedades del Sistema Nervioso Periférico/diagnóstico , Comunicación , Actitud del Personal de SaludRESUMEN
INTRODUCTION/AIMS: It is unclear whether the revised European Academy of Neurology/Peripheral Nerve Society diagnostic criteria (EAN/PNS 2021 criteria) improved the diagnostic yield for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) compared with the previous version. Therefore, this study aimed to compare the sensitivity and specificity of the EAN/PNS 2021 criteria and the European Federation of Neurological Societies/Peripheral Nerve Society 2010 diagnostic criteria (EFNS/PNS 2010 criteria), with a specific focus on the electrodiagnostic criteria. METHODS: Data of patients with clinically suspected CIDP who exhibited objective treatment response, and of those with chronic axonal neuropathies, obtained between 2009 and 2021, were extracted retrospectively from our database. Patients who underwent nerve conduction studies in at least unilateral upper and lower extremities were enrolled. We compared the sensitivity and specificity of the EAN/PNS 2021 and EFNS/PNS 2010 criteria. RESULTS: In total, 55 patients with clinically suspected CIDP and 36 patients with chronic axonal neuropathies were enrolled. When considering the "possible CIDP" category, the EAN/PNS 2021 criteria showed lower sensitivity than the EFNS/PNS 2010 criteria (78% vs. 93%, p < .05), whereas its specificity was higher (78% vs. 61%, p < .05). The lower sensitivity was caused mainly by the failure to fulfill the sensory nerve conduction criteria. The revised definition of abnormal temporal dispersion of the tibial nerve contributed markedly to the improved specificity. DISCUSSION: To improve the sensitivity of the EAN/PNS 2021 criteria, increasing the number of tested sensory nerves may be necessary.
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Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante , Humanos , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/terapia , Estudios Retrospectivos , Nervios Periféricos , Conducción Nerviosa/fisiología , Nervio TibialRESUMEN
Phrenic nerve conduction studies (NCSs) and needle electromyography (EMG) can provide important information on the underlying pathophysiology in patients presenting with unexplained shortness of breath, failure to wean from the ventilator, or consideration of phrenic nerve pacemaker implantation. However, these techniques are often technically challenging, require experience, can lack sensitivity and specificity, and, in the case of diaphragm EMG, involve some degree of risk. Diagnostic high-resolution ultrasound has been introduced in recent years as an adjuvant technique readily available at the bedside that can increase the overall sensitivity and specificity of the neurophysiologic evaluation of respiratory symptoms. Two-dimensional ultrasound in the zone of apposition can identify atrophy and evaluate contractility of the diaphragm, in addition to localizing a safe zone for needle EMG. M-mode ultrasound can identify decreased excursion or paradoxical motion of the diaphragm and can increase the reliability of phrenic NCSs. When used in combination, ultrasound, phrenic NCSs and EMG of the diaphragm can differentiate neuropathic, myopathic, and central disorders, and can offer aid in prognosis that is difficult to arrive at solely from clinical examination. This article will review techniques to successfully perform phrenic NCSs, needle EMG of the diaphragm, and ultrasound of the diaphragm. The discussion will include technical pitfalls and clinical pearls as well as future directions and clinical indications.
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Disnea , Enfermedades del Sistema Nervioso Periférico , Humanos , Reproducibilidad de los Resultados , Electromiografía/métodos , Diafragma/inervación , Nervio Frénico/diagnóstico por imagenRESUMEN
INTRODUCTION/AIMS: Accurately diagnosing Guillain-Barré syndrome (GBS) in its early stages and distinguishing it from mimics poses challenges. This study aimed to evaluate the utility of an existing electrodiagnostic criterion in very early GBS (VEGBS) for discerning mimics. Additionally, we explored specific electrophysiological abnormalities in VEGBS to design a new diagnostic criterion for more accurate VEGBS diagnosis. METHODS: We retrospectively identified all patients with flaccid quadriparesis initially suspected of GBS who underwent nerve conduction studies (NCS) ≤4 days from symptom onset. We then retrieved their NCS data and applied an existing electrodiagnostic criterion for sensitivity and specificity analyses based on the final discharge diagnosis. Furthermore, we designed a new criterion based on the observed electrophysiological abnormalities that have maximum specificity and at least 50% sensitivity. RESULTS: Among 70 patients suspected of VEGBS, 44 (63%) received a final diagnosis of GBS, while in 26 (37%), the GBS diagnosis was later refuted. Umapathi's definite criterion exhibited a sensitivity of 61.36% and a specificity of 92.31%. The probable and possible groups showed very high sensitivity (90.91% and 100%, respectively); however, specificity was low (57.69% and 30.77%, respectively) in the very early stage. Our proposed criterion demonstrated a sensitivity of 88.64% (CI: 75.44%-96.21%) and a specificity of 96.15% (CI: 80.36%-99.90%). DISCUSSION: The criterion based on presumed electrophysiological correlates of specific early GBS pathophysiology proved more effective than the existing electrodiagnostic criterion in differentiating VEGBS from mimics.
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INTRODUCTION/AIMS: Nerve conduction studies (NCSs) are widely used to support the clinical diagnosis of neuromuscular disorders. The aims of this study were to obtain reference values for peroneal, tibial, and sural NCSs and to examine the associations with demographic and anthropometric factors. METHODS: In 5099 participants (aged 40-79 years) without type 2 diabetes of The Maastricht Study, NCSs of peroneal, tibial, and sural nerves were performed. Values for compound muscle action potential (CMAP) and sensory nerve action potential amplitude, nerve conduction velocity (NCV), and distal latency were acquired. The association of age, sex, body mass index (BMI), and height with NCS values was determined using uni- and multivariate linear regression analyses. RESULTS: Detailed reference values are reported per decade for men and women. Significantly lower NCVs and longer distal latencies were observed in all nerves in older and taller individuals as well as in men. In these groups, amplitudes of the tibial and sural nerves were significantly lower, whereas a lower peroneal nerve CMAP was only significantly associated with age. BMI showed a multidirectional association. After correction for anthropometric factors in the multivariate analysis, the association between sex and NCS values was less straightforward. DISCUSSION: These values from a population-based dataset could be used as a reference for generating normative values. Our findings show the association of NCS values with anthropometric factors. In clinical practice, these factors can be considered when interpreting NCS values.
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Diabetes Mellitus Tipo 2 , Nervio Sural , Masculino , Humanos , Femenino , Anciano , Nervio Tibial/fisiología , Estudios de Conducción Nerviosa , Conducción Nerviosa/fisiología , Valores de Referencia , Nervio Peroneo/fisiología , DemografíaRESUMEN
BACKGROUND AND PURPOSE: Various electrodiagnostic criteria have been developed in Guillain-Barré syndrome (GBS). Their performance in a broad representation of GBS patients has not been evaluated. Motor conduction data from the International GBS Outcome Study (IGOS) cohort were used to compare two widely used criterion sets and relate these to diagnostic amyotrophic lateral sclerosis criteria. METHODS: From the first 1500 patients in IGOS, nerve conduction studies from 1137 (75.8%) were available for the current study. These patients were classified according to nerve conduction studies criteria proposed by Hadden and Rajabally. RESULTS: Of the 1137 studies, 68.3% (N = 777) were classified identically according to criteria by Hadden and Rajabally: 111 (9.8%) axonal, 366 (32.2%) demyelinating, 195 (17.2%) equivocal, 35 (3.1%) inexcitable and 70 (6.2%) normal. Thus, 360 studies (31.7%) were classified differently. The areas of differences were as follows: 155 studies (13.6%) classified as demyelinating by Hadden and axonal by Rajabally; 122 studies (10.7%) classified as demyelinating by Hadden and equivocal by Rajabally; and 75 studies (6.6%) classified as equivocal by Hadden and axonal by Rajabally. Due to more strictly defined cutoffs fewer patients fulfilled demyelinating criteria by Rajabally than by Hadden, making more patients eligible for axonal or equivocal classification by Rajabally. In 234 (68.6%) axonal studies by Rajabally the revised El Escorial (amyotrophic lateral sclerosis) criteria were fulfilled; in axonal cases by Hadden this was 1.8%. CONCLUSIONS AND DISCUSSION: This study shows that electrodiagnosis in GBS is dependent on the criterion set utilized, both of which are based on expert opinion. Reappraisal of electrodiagnostic subtyping in GBS is warranted.
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Electrodiagnóstico , Síndrome de Guillain-Barré , Conducción Nerviosa , Humanos , Síndrome de Guillain-Barré/diagnóstico , Síndrome de Guillain-Barré/clasificación , Síndrome de Guillain-Barré/fisiopatología , Conducción Nerviosa/fisiología , Electrodiagnóstico/métodos , Masculino , Femenino , Persona de Mediana Edad , Adulto , Esclerosis Amiotrófica Lateral/diagnóstico , Esclerosis Amiotrófica Lateral/clasificación , Esclerosis Amiotrófica Lateral/fisiopatología , Anciano , Estudios de CohortesRESUMEN
BACKGROUND AND PURPOSE: The role of high-resolution nerve ultrasound (HRUS) and corneal confocal microscopy (CCM) in the early detection of taxane-induced polyneuropathy (TIPN) is unclear. The present prospective longitudinal controlled observational pilot study estimates the role of HRUS and CCM in the early diagnosis of TIPN in breast cancer patients. METHODS: Fifteen breast cancer patients receiving paclitaxel and 15 healthy age matched controls were included. Visits before and 3 weeks, 8 weeks and 6 months after treatment included clinical examination, the total neuropathy score, nerve conduction studies (NCS), monocular CCM including corneal nerve fibre length, density and branching and HRUS of bilateral median, ulnar, radial, tibial, peroneal and sural nerves. Patients were compared between different visits and to healthy controls. RESULTS: Total neuropathy score increased from 2.2 at baseline to 5.8 (p < 0.001) at week 8. NCS showed a decreased sensory amplitude in the sural, radial, ulnar and median nerve after 6 months (p < 0.001). HRUS revealed a significant increase of cross-sectional area in the sural nerve (p = 0.004), the median nerve (p = 0.003) at the carpal tunnel and the ulnar nerve in the forearm (p = 0.006) after 6 months. CCM showed no changes at different visits. CONCLUSIONS: Corneal confocal microscopy and HRUS do not detect early signs of TIPN during the paclitaxel treatment period. HRUS and NCS might detect congruent signs of an axonal, predominantly sensory polyneuropathy after 6 months. The clinical examination remains the most sensitive tool in the early detection of TIPN in breast cancer patients.
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Neoplasias de la Mama , Neuropatías Diabéticas , Enfermedades del Sistema Nervioso Periférico , Polineuropatías , Femenino , Humanos , Neoplasias de la Mama/tratamiento farmacológico , Neuropatías Diabéticas/diagnóstico , Microscopía Confocal , Conducción Nerviosa/fisiología , Paclitaxel , Enfermedades del Sistema Nervioso Periférico/inducido químicamente , Enfermedades del Sistema Nervioso Periférico/diagnóstico por imagen , Polineuropatías/inducido químicamente , Polineuropatías/diagnóstico por imagen , Estudios Prospectivos , Taxoides/efectos adversos , Proyectos PilotoRESUMEN
BACKGROUND AND AIMS: Leprosy is a chronic infectious disease caused by Mycobacterium leprae (M. leprae), an intracellular bacillus that systematically invades the peripheral nerves. Diagnosing leprosy neuropathy is still a defying skill, and late diagnosis and treatment are still a reality. Based on the biological characteristics of M. leprae, particularly its preference for invading the Schwann cells localized at the coldest areas of human body, we hypothesized that these areas have focal demyelination that may escape detection through standard nerve conduction studies (NCSs) protocols. METHODS: Twenty-five patients with confirmed multibacillary leprosy and 14 controls were accessed. A multisegmented NCS protocol (MP) was performed, targeting short segments through the coldest areas, to identify focal areas of slowed conduction velocity. The effectiveness of this multisegmented protocol was compared to the standard protocol (SP) to detect abnormalities. RESULTS: All leprosy patients presented an abnormal study with the MP, contrasting to 19 with the SP. The most frequent NCS pattern was an asymmetric neuropathy with focal slowing of conduction velocity, found in 23 out of 25 leprosy patients. Significant differences favoring the proposed method were observed when comparing the MP with the SP. Notably, the MP increased the sensitivity to detect abnormalities by 122%, 133%, and 257% for the median, peroneal, and tibial nerves, respectively. MP also increases sensitivity to detect focal abnormalities in the ulnar nerve. INTERPRETATION: The MP protocol significantly increases the sensitivity of NCSs to detect neurophysiological abnormalities in leprosy neuropathy.
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BACKGROUND: Decades following the introduction of vincristine as treatment for haematological malignancies, vincristine-induced peripheral neuropathy (VIPN) remains a pervasive, untreatable side-effect. However there remains a gap in understanding the characteristics of VIPN in adults. This study presents a comprehensive phenotyping of VIPN. METHODS: Adult patients (n = 57; age = 59.8 ± 14.6) were assessed cross-sectionally following completion of vincristine (months post treatment = 16.3 ± 15.6, cumulative dose = 7.6 ± 4.4), with a subset of 20 patients assessed prospectively during treatment. Patient reported measures (EORTC-QLQ-CIPN20, R-ODS) were used to profile symptoms and disability. Neurological assessment was undertaken using the Total Neuropathy Score and nerve conduction studies. Sensory threshold and fine motor tasks were also undertaken. Comparisons of data between timepoints were calculated using paired-sample t tests or Wilcoxon matched-pairs signed-rank test. Comparisons between outcome measures were calculated with independent sample t tests or Mann-Whitney U tests for non-parametric data. RESULTS: The majority of patients developed VIPN by mid-treatment (77.8%, 7.0 ± 3.3 weeks post baseline) with the prevalence remaining stable by end-of-treatment (75%, 8.1 ± 1.7 weeks post mid-treatment). By 3 months post-completion, 50% of patients still reported VIPN although there were significant improvements on neurological grading and functional assessment (P < 0.05). VIPN presented with sensorimotor involvement in upper and lower limbs and was associated with decreased sensory and motor nerve amplitudes, reduced fine-motor function and increased disability. CONCLUSION: VIPN in adults presents as a sensorimotor, upper- and lower-limb neuropathy that significantly impacts disability and function. Neuropathy recovery occurs in a proportion of patients; however, VIPN symptoms may persist and continue to affect long-term quality of life.
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Neoplasias Hematológicas , Enfermedades del Sistema Nervioso Periférico , Adulto , Humanos , Persona de Mediana Edad , Anciano , Calidad de Vida , Vincristina/efectos adversos , Enfermedades del Sistema Nervioso Periférico/inducido químicamente , Extremidad InferiorRESUMEN
MRI is the most appropriate imaging method for visual evaluation of lumbosacral plexopathy (LSP) and a reference for comparing with nerve conduction study (NCS). Eight patients with clinical, electrophysiological, and lumbosacral plexus MRI findings suggestive of LSP were prospectively recruited. Saphenous nerve abnormalities were present in seven patients (88%), compared to three for the superficial fibular (38%), and three for the sural nerve (38%). MRI showed tumor, hematoma, abscess, contrast enhancement, or hyperintense signals on the T2-weighted sequences. The SN has the highest yield in MRI positive LSP and may be a vital adjunct for electrophysiological evaluation of LSP.
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Neoplasias , Estudios de Conducción Nerviosa , Humanos , Conducción NerviosaRESUMEN
BACKGROUND: To determine whether sensory nerve conduction stimulus threshold measurements of the infraorbital nerve are able to differentiate horses with idiopathic trigeminal-mediated headshaking (i-TMHS) from healthy horses and from horses with secondary trigeminal-mediated headshaking (s-TMHS). In a prospective trial, headshaking horses were examined using a standardized diagnostic protocol, including advanced diagnostics such as computed tomography and 3-Tesla-magnetic resonance imaging (MRI), to differentiate s-TMHS from i-TMHS. Clinically healthy horses served as controls. Within this process, patients underwent general anesthesia, and the minimal sensory nerve conduction stimulus threshold (SNCT) of the infraorbital nerve was measured using a bipolar concentric needle electrode. Sensory nerve action potentials (SNAP) were assessed in 2.5-5 mA intervals. Minimal SNCT as well as additional measurements were calculated. RESULTS: In 60 horses, SNAP could be recorded, of which 43 horses had i-TMHS, six had suspected s-TMHS, three horses had non-facial headshaking, and eight healthy horses served as controls. Controls had a minimal SNCT ≥ 15 mA, whereas 14/43 horses with i-TMHS and 2/6 horses with s-TMHS showed a minimal SNCT ≤ 10 mA. Minimal SNCT ≤ 10 mA showed 100% specificity to distinguish TMHS from controls, but the sensitivity was only 41%. CONCLUSION: A minimal SNCT of the infraorbital nerve ≤ 10 mA was able to differentiate healthy horses from horses with TMHS. Nevertheless, a higher minimal SNCT did not exclude i-TMHS or s-TMHS and minimal SNCT does not distinguish s-TMHS from i-TMHS.
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Enfermedades de los Caballos , Conducción Nerviosa , Animales , Caballos , Enfermedades de los Caballos/diagnóstico , Femenino , Masculino , Conducción Nerviosa/fisiología , Cabeza , Estudios Prospectivos , Nervio Trigémino/fisiologíaRESUMEN
INTRODUCTION: In the arms of patients with Amyotrophic lateral sclerosis (ALS) two peculiar patterns of dissociated muscular atrophy have been described: the split-hand sign (with predominant atrophy of the lateral aspect of the hand, compared to hypothenar eminence) and the split-hand-plus sign (SHPS), a predominant abductor pollicis brevis (ABP) atrophy with sparing of flexor pollicis longus (FPL). AIMS: In this case-control study, we evaluated the diagnostic utility of a neurophysiological indicator of SHPS and assessed its association with clinical features. METHODS: We prospectively studied 59 incident ALS patients, 61 patients with ALS-mimic disorders (OND) and 61 non-neurological controls (NNCs). ABP and FPL compound muscle action potentials (CMAP) amplitudes were obtained by supramaximal stimulation of median nerve at elbow. Split-hand plus index (SHPI) was calculated according to the formula: APB-CMAP/FPL-CMAP. RESULTS: SHPI was significantly lower in ALS compared to OND patients and NNCs (p < 0.0001). SHPI value < 1 was observed in 2% of NNCs and 9% of OND patients and demonstrated an accuracy of 71% in differentiating ALS from OND and an accuracy of 74% in differentiating ALS from NNC. SHPI was associated with higher LMN score, and higher disease severity as quantified by the ALSFRS-r. CONCLUSION: Our results indicate that SHPI is a reliable indicator to distinguish ALS patients from ONDs and NNCs. SHPI was significantly associated to the degree of lower motor neuron impairment but showed no association with upper motoneuron impairment.
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OBJECTIVE: To evaluate the use of a computer-based biodex balance exercise system (BBS) on balance, neuropathic pain, clinical presentation and nerve function in patients with diabetic peripheral neuropathy (DPN). METHODS: A total of 32 participants with DPN were randomly assigned in a 1:1 ratio to an intervention group (IG) or control group (CG). The IG performed exercises using the BBS twice weekly for 8 weeks, while CG were informed regarding diabetes self-management. At baseline and after study completion, participants underwent balance (postural stability and fall risk) and neuropathic pain assessment (DN4 questionnaire) and were screened using the Michigan Neuropathy Screening Instrument and nerve conduction test. RESULTS: Among the baseline participants, 14 in the IG and 13 in the CG completed the study. Balance training improved postural stability (overall, p<0.001), fall risk (p<0.001), neuropathic pain (p=0.01) and symptoms (p<0.001), and clinical presentation (p=0.02), but not nerve function, within the IG. At follow-up, IG displayed significantly improved stability (p<0.001) and fall risk (p=0.02) and decreased neuropathic symptoms (p=0.01) compared to the CG. CONCLUSION: Computer-based balance exercises improve balance, pain, and clinical presentation of DPN, but not nerve function, in patients with DPN. CLINICALTRIALS: gov ID: NCT05255497.
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Neuropatías Diabéticas , Terapia por Ejercicio , Equilibrio Postural , Humanos , Neuropatías Diabéticas/fisiopatología , Neuropatías Diabéticas/terapia , Equilibrio Postural/fisiología , Masculino , Femenino , Persona de Mediana Edad , Terapia por Ejercicio/métodos , Anciano , Neuralgia/terapia , Neuralgia/fisiopatología , Neuralgia/rehabilitaciónRESUMEN
OBJECTIVES: Carpal tunnel syndrome (CTS) is the most common entrapment neuropathy in the body and impacts approximately 5% of the U.S. population costing nearly $5 billion/year. Electrodiagnostic (EDX) testing is considered the gold standard for CTS diagnosis. Classification systems exist that categorize CTS severity based on EDX findings. This investigation evaluated EDX findings across consecutive CTS severity categories within existing classification systems and consolidated classifications. METHODS: This retrospective study analyzed 665 hands from 468 patients undergoing EDX testing for suspected CTS. Complete classification systems and consolidated classifications were evaluated for discrimination capability across consecutive CTS severity categories based on EDX findings. Additional analysis evaluated the relationship of sex and age factors and CTS severity. RESULTS: Consolidated classifications demonstrated superior discrimination capability between consecutive CTS severity categories regardless of classification system used. Demographic factors significantly influenced EDX findings and categorization of CTS severity. CONCLUSIONS: This study underscores the value of consolidated classifications for enhancing discrimination between consecutive CTS severity categories based on EDX findings. Demographic factors should be considered when interpreting EDX findings for the purpose of categorizing CTS severity. Future research should refine existing classification systems and explore additional factors influencing CTS severity used to inform medical management.
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Síndrome del Túnel Carpiano , Electrodiagnóstico , Índice de Severidad de la Enfermedad , Humanos , Síndrome del Túnel Carpiano/diagnóstico , Síndrome del Túnel Carpiano/clasificación , Masculino , Femenino , Persona de Mediana Edad , Estudios Retrospectivos , Electrodiagnóstico/métodos , Electrodiagnóstico/normas , Adulto , AncianoRESUMEN
OBJECTIVE: To use the ulnar compound muscle action potential (CMAP) to abductor digiti minimi (ADM) to identify the proportion of individuals with cervical spinal cord injury (SCI) who have lower motor neuron (LMN) abnormalities involving the C8-T1 spinal nerve roots, within 3-6 months, and thus may influence the response to nerve transfer surgery. DESIGN: Retrospective analysis of prospectively collected data. Data were analyzed from European Multicenter Study About SCI database. SETTING: Multi-center, academic hospitals. PARTICIPANTS: We included 79 subjects (age=41.4±17.7, range:16-75; 59 men; N=79), who were classified as cervical level injuries 2 weeks after injury and who had manual muscle strength examinations that would warrant consideration for nerve transfer (C5≥4, C8<3). INTERVENTIONS: None. MAIN OUTCOME MEASURES: The ulnar nerve CMAP amplitude to ADM was used as a proxy measure for C8-T1 spinal segment health. CMAP amplitude was stratified into very abnormal (<1.0 mV), sub-normal (1.0-5.9 mV), and normal (>6.0 mV). Analysis took place at 3 (n=148 limbs) and 6 months (n=145 limbs). RESULTS: At 3- and 6-month post-injury, 33.1% and 28.3% of limbs had very abnormal CMAP amplitudes, respectively, while in 54.1% and 51.7%, CMAPs were sub-normal. Median change in amplitude from 3 to 6 months was 0.0 mV for very abnormal and 1.0 mV for subnormal groups. A 3-month ulnar CMAP <1 mV had a positive predictive value of 0.73 (95% CI 0.69-0.76) and 0.78 (95% CI 0.75-0.80) for C8 and T1 muscle strength of 0 vs 1 or 2. CONCLUSION: A high proportion of individuals have ulnar CMAPs below the lower limit of normal 3- and 6-month post cervical SCI and may also have intercurrent LMN injury. Failure to identify individuals with LMN denervation could result in a lost opportunity to improve hand function through timely nerve transfer surgeries.
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Médula Cervical , Transferencia de Nervios , Traumatismos de la Médula Espinal , Masculino , Humanos , Adulto Joven , Adulto , Persona de Mediana Edad , Estudios Retrospectivos , Nervio CubitalRESUMEN
PURPOSE: This study aimed to establish an in-vitro alternative to existing in-vivo systems to analyze nerve dysfunction using continuous neuromonitoring (C-IONM). METHODS: Three hundred sixty-three recurrent laryngeal nerves (RLN) (N(pigs) = 304, N(cattle) = 59) from food industry cadavers were exposed by microsurgical dissection following euthanasia. After rinsing with Ringer's lactate, they were tempered at 22 °C. Signal evaluation using C-IONM was performed for 10 min at 2 min intervals, and traction forces of up to 2N were applied for a median time of 60 s. Based on their post-traumatic electrophysiological response, RLNs were classified into four groups: Group A: Amplitude ≥ 100%, Group B: loss of function (LOS) 0-25%, Group C: ≥ 25-50%, and Group D: > 50%. RESULTS: A viable in-vitro neuromonitoring system was established. The median post-traumatic amplitudes were 112%, 88%, 59%, and 9% in groups A, B, C, and D, respectively. A time-dependent further dynamic LOS was observed during the 10 min after cessation of strain. Surprisingly, following initial post-traumatic hyperconductivity, complete LOS occurred in up to 20% of the nerves in group A. The critical threshold for triggering LOS was 2N in all four groups, resulting in immediate paralysis of up to 51.4% of the nerves studied. CONCLUSION: Consistent with in-vivo studies, RLN exhibit significant intrinsic electrophysiological variability in response to tensile forces. Moreover, nerve damage progresses even after the complete cessation of strain. Up to 20% of nerves with transiently increased post-traumatic amplitudes above 100% developed complete LOS, which we termed the "weepy cry." This time-delayed response must be considered during the interpretation of C-IONM signals.