Clinical Neurophysiology Practice

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OBJECTIVE: We searched for long-term peripheral nerve complications 10-15 years after Roux-en-Y gastric bypass surgery (RYGB), using a comprehensive nerve conduction study (NCS) protocol. METHODS: Patients (n = 175, mean age 52.0, BMI 35.2) and 86 community-controls (mean age 56.8, BMI 27.2) had NCS of one upper and lower limb. New abnormality scores from 27 polyneuropathy-relevant (PNP27s) and four carpal tunnel syndrome-relevant NCS-measures (CTS4s) were compared between groups with non-parametric statistics...

OBJECTIVE: To establish if induced current direction across the motor cortex alters the sensitivity of transcranial magnetic stimulation (TMS)-evoked short-interval intracortical inhibition (SICI) as an ALS biomarker. METHODS: Threshold tracking-TMS was undertaken in 35 people with ALS and 39 controls. Using a coil orientation which induces posterior-anterior (PA)-directed current across the motor cortex, SICI (1 ms and 3 ms interstimulus intervals) and intracortical facilitation (ICF, 10 ms interstimulus interval) were recorded...

OBJECTIVE: To determine if compression sites of the facial nerve correlate with immediate postoperative outcomes in patients with hemifacial spasm (HFS), and if changes in the waveform of abnormal muscle response (AMR) during microvascular decompression (MVD) for HFS can predict the postoperative course. METHODS: In this retrospective review, we evaluated 50 patients with HFS who underwent AMR monitoring during MVD. The ratios of amplitude and duration of AMR waveforms were computed by comparing baseline with final examinations...

OBJECTIVE: Epilepsy patients with mesial temporal sclerosis (MTS) on imaging who are drug-resistant usually undergo epilepsy surgery without previous invasive evaluation. However, up to one-third of patients are not seizure-free after surgery. Prior studies have identified risk factors for surgical failure, but it is unclear if they are associated with bilateral or discordant seizure onset. METHODS: In this retrospective case series, we identified 17 epilepsy patients who had MRI-confirmed MTS but received invasive stereo-EEG (SEEG) evaluation before definitive intervention...

INTRODUCTION: Pathogenic variants of the MTOR gene result in the Smith-Kingsmore syndrome, whose phenotypical spectrum includes facial dysmorphisms and neurological features. Expressivity is variable, patients exhibit a combination of intellectual disability, macrocephaly and epilepsy. The diagnosis can be missed, failing to detect the causative pathogenic mutation in patients with somatic mosaicism or even skipping to analyze MTOR when the phenotype is not completely expressed. CASE STUDY: Herein, we report two children harboring the same MTOR recurring mutation (c...

OBJECTIVE: Vincristine, a widely used anticancer chemotherapy drug, may cause polyneuropathy (PNP), potentially resulting in permanent functional impairment. We characterized the occurrence and development of vincristine-induced neuropathy (VIPN) in early treatment of childhood leukemia. METHODS: This prospective study of 35 pediatric acute lymphoblastic leukemia (ALL) patients comprised systematic clinical and electrophysiological studies at both the time of diagnosis and at least one time point during the first months of treatment...

OBJECTIVE: The present study aimed to explore first the impact of perinatal risk factors on flash-VEP waves and morphology in a group of preterm infants studied at term equivalent age (TEA). Second, to correlate VEP morphology with neurological outcome at 2 years corrected age (CA). METHODS: Infants with a gestational age (GA) at birth <32 weeks, without major brain injury, were enrolled. Multivariate regression analyses were performed, and the models were run separately for each dependent variable N2, P2, N3 latencies and P2 amplitude...

OBJECTIVE: To assess impact of ultrasound guidance (USG) on patient's perception of nerve conduction studies (NCS). METHODS: In this single-center, randomized, sham-controlled, parallel, single-blind trial, we evaluated ultrasound (US) in identifying NCS stimulation site. Consecutive adults (18-80 old) without neuropathy referred for NCS were electronically randomized 1:1 to USG or Sham US. The primary outcome was sensory supramaximal intensity (SSMI) for each site/nerve; motor supramaximal intensity (MSMI), amplitudes, number of non-routine muscle punctured, Visual Analogue Scale (VAS), satisfaction were secondary outcomes...

Functional Motor Disorders are common and disabling. Clinical diagnosis has moved from one of exclusion of other causes for symptoms to one where positive clinical features on history and examination are used to make a "rule in" diagnosis wherever possible. Clinical neurophysiological assessments have developed increasing importance in assisting with this positive diagnosis, not being used simply to demonstrate normal sensory-motor pathways, but instead to demonstrate specific abnormalities that help to positively diagnose these disorders...

OBJECTIVE: Nerve conduction studies (NCS) in children remain technically challenging and depend on the cooperation of the child. Motor NCS are not compromised by analgosedation but data for sensory NCS are lacking. Here, we ask whether sensory NCS is influenced by analgosedation. We also compare the present data with NCS studies from the 1990s regarding anthropometric acceleration of the contemporary paediatric population. METHODS: Sensory NCS of the median nerve and sural nerve were performed in 182 healthy subjects aged 1 to 18 years during general anaesthesia and in 47 of them without analgosedation...

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Parasomnias are due to a transient unstable state dissociation during entry into sleep, within sleep, or during arousal from sleep, and manifest with abnormal sleep related behaviors, perceptions, emotions, dreams, and autonomic nervous system activity. Rapid eye movement (REM) parasomnias include REM sleep behavior disorder (RBD), isolated recurrent sleep paralysis and nightmare disorder. Neurophysiology is key for diagnosing these disorders and provides insights into their pathophysiology. RBD is very well characterized from a neurophysiological point of view, also thank to the fact that polysomnography is needed for the diagnosis...

Though a common symptom, fatigue is difficult to define and investigate, occurs in a wide variety of neurological and systemic disorders, with differing pathological causes. It is also often accompanied by a psychological component. As a symptom of long-term COVID-19 it has gained more attention. In this review, we begin by differentiating fatigue, a perception, from fatigability, quantifiable through biomarkers. Central and peripheral nervous system and muscle disorders associated with these are summarised...

BACKGROUND: Creutzfeldt-Jakob disease (CJD) is a devastating degenerative brain disorder caused by an abnormal isoform of a cellular glycoprotein which is known as the prion protein. A diagnosis of CJD is usually based on specific clinical signs, EEG and MRI findings, as well as the presence of the 14-3-3 protein in the cerebrospinal fluid. Although end-stage CJD usually has a typical clinical presentation, early symptoms may be variable. CASE PRESENTATION: We present an uncommon case of CJD which manifested with primary progressive aphasia, leading to an incorrect diagnosis of frontotemporal dementia...

Accurate and rapid diagnosis of amyotrophic lateral sclerosis (ALS) is essential in order to provide accurate information for patient and family, to avoid time-consuming investigations and to permit an appropriate management plan. ALS is variable regarding presentation, disease progression, genetic profile and patient reaction to the diagnosis. It is obviously important to exclude treatable conditions but, in most patients, for experienced neurologists the diagnosis is clear-cut, depending on the presence of progressive upper and lower motor neuron signs...

OBJECTIVE: Insufficient sleep is linked to several health problems. Previous studies on the effects of sleep deprivation on cortical excitability using conventional transcranial magnetic stimulation (TMS) included a limited number of modalities, and few inter-stimulus intervals (ISIs) and showed conflicting results. This study aimed to investigate the effects of sleep deprivation on cortical excitability through threshold-tracking TMS, using a wide range of protocols at multiple ISIs...

Amyotrophic lateral sclerosis (ALS) is a rapidly progressive neurodegenerative disorder of the human motor system, first described in the 19th Century. The etiology of ALS appears to be multifactorial, with a complex interaction of genetic, epigenetic, and environmental factors underlying the onset of disease. Importantly, there are no known naturally occurring animal models, and transgenic mouse models fail to faithfully reproduce ALS as it manifests in patients. Debate as to the site of onset of ALS remain, with three competing theories proposed, including (i) the dying-forward hypothesis , whereby motor neuron degeneration is mediated by hyperexcitable corticomotoneurons via an anterograde transsynaptic excitotoxic mechanism, (ii) dying-back hypothesis, proposing the ALS begins in the peripheral nervous system with a toxic factor(s) retrogradely transported into the central nervous system and mediating upper motor neuron dysfunction, and (iii) independent hypothesis , suggesting that upper and lower motor neuron degenerated independently...

OBJECTIVE: We aimed to evaluate differences in ultrasonographic nerve enlargement sites among typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), distal CIDP, multifocal CIDP and multifocal motor neuropathy (MMN) in a Japanese population. METHODS: We retrospectively reviewed medical records and selected 39 patients (14 with typical CIDP, 7 with multifocal CIDP, 4 with distal CIDP, and 14 with MMN) who underwent ultrasonography. Median and ulnar nerve cross-sectional areas (CSAs) were measured at the wrist, forearm, elbow, and upper arm...

No abstract text is available yet for this article.