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Normal data on axonal excitability in Koreans
Lee, Ju Young,Yu, Jin Hyeok,Pyun, So Young,Ryu, Sanghyo,Bae, Jong Seok The Korean Society of Clinical Neurophysiology 2017 Annals of Clinical Neurophysiology Vol.19 No.1
Background: Automated nerve excitability testing is used to assess various peripheral neuropathies and motor neuron diseases. Comparing these excitability parameters with normal data provides information regarding the axonal excitability properties and ion biophysics in diseased axons. This study measured and compared normal values of axonal excitability parameters in both the distal motor and sensory axons of normal Koreans. Methods: The axonal excitability properties of 50 distal median motor axons and 30 distal median sensory axons were measured. An automated nerve excitability test was performed using the QTRACW threshold-tracking software (Institute of Neurology, University College London, London, UK) with the TRONDF multiple excitability recording protocol. Each parameter of stimulus-response curves, threshold electrotonus, current-voltage relationship, and recovery cycle was measured and calculated. Results: Our Korean normal data on axonal excitability showed ranges of values and characteristics similar to previous reports from other countries. We also reaffirmed that there exist characteristic differences in excitability properties between motor and sensory axons: compared to motor axons, sensory axons showed an increased strength-duration time constant, more prominent changes in threshold to hyperpolarizing threshold electrotonus (TE) and less prominent changes in threshold to depolarizing TE, and more prominent refractoriness and less prominent subexcitability and superexcitability. Conclusions: We report normal data on axonal excitability in Koreans. These data can be used to compare various pathological conditions in peripheral nerve axons such as peripheral neuropathies and motor neuron disease.
How predictive are temporal lobe changes of underlying TDP-43 pathology in the ALS-FTD continuum?
Bueno, Ana Paula Arantes,Bertoux, Maxime,de Souza, Leonardo Cruz,Hornberger, Michael The Korean Society of Clinical Neurophysiology 2017 Annals of Clinical Neurophysiology Vol.19 No.2
Detection of underling proteinopathies is becoming increasingly important across neurodegenerative conditions due to upcoming disease intervention trials. In this review, we explored how temporal lobe changes in amyotrophic lateral sclerosis (ALS) and frontotemporal dementia (FTD) can potentially predict underlying TDP-43 pathology subtypes in FTD. To date, emphasis has been given to frontal lobe changes in the study of the cognitive and behavioural impairments in both syndromes but an increasing number of pathological, imaging and neuropsychological studies suggest how temporal lobe changes could critically affect the cognition and behaviour of these conditions. In this current article, we reviewed pathological, imaging as well as clinical/neuropsychological findings of temporal involvement in the ALS-FTD continuum, how they relate to temporal lobe changes and the underlying TDP-43 pathology in FTD. Findings across studies show that TDP-43 pathology occurs and coincides in many structures in ALS and FTD, but especially in the temporal lobes. In particular, anterior and medial temporal lobes atrophy is consistently found in ALS and FTD. In addition, memory and language impairment as well as emotional and Theory of Mind processing deficits that are characteristics of the two diseases are highly correlated to temporal lobe dysfunction. We conclude by showing that temporal lobe changes due to TDP-43 type B might be particular predictive of TDP-43 type B pathology in behavioural variant FTD, which clearly needs to be investigated further in the future.
Diagnosis of complex regional pain syndrome
Kim, Young-Do The Korean Society of Clinical Neurophysiology 2022 Annals of Clinical Neurophysiology Vol.24 No.2
Complex regional pain syndrome (CRPS) is a chronic regional pain disorder that most frequently affects the limbs. It is characterized by hyperalgesia, allodynia, edema, motor disturbance, and vasomotor instability, and typically occurs following surgery or trauma. In type-I CRPS there is no confirmed nerve injury, while peripheral nerve injury is present in type-II CRPS. The multifactorial pathophysiological etiology of CRPS includes inflammation, autoimmune responses, abnormal cytokine production, autonomic dysfunction, altered blood flow, psychological factors, and central cortical reorganization. There are no specific laboratory diagnostic tools for CRPS, and so it is diagnosed clinically. The Budapest criteria are currently the most-accepted diagnostic criteria.
Basic concepts of needle electromyography
Kim, Jee-Eun,Seok, Jin Myoung,Ahn, Suk-Won,Yoon, Byung-Nam,Lim, Young-Min,Kim, Kwang-Kuk,Kwon, Ki-Han,Park, Kee Duk,Suh, Bum Chun,Korean Society of Clinical Neurophysiology Education Committee Korean Society of Clinical Neurophysiology 2019 Annals of Clinical Neurophysiology Vol.21 No.1
Clinical evaluations, nerve conduction studies, and electromyography play major complementary roles in electrophysiologic diagnoses. Electromyography can be used to assess pathologic changes and localize lesions occurring in locations ranging from motor units to anterior-horn cells. Successfully performing electromyography requires knowledge of the anatomy, physiology, and pathology of the peripheral nervous system as well as sufficient skill and interpretation ability. Electromyography techniques include acquiring data from visual/auditory signals and performing needle positioning, semiquantitation, and interpretation. Here we introduce the basic concepts of electromyography to guide clinicians in performing electromyography appropriately.
Electroencephalography for the diagnosis of brain death
Lee, Seo-Young,Kim, Won-Joo,Kim, Jae Moon,Kim, Juhan,Park, Soochul,Korean Society of Clinical Neurophysiology Education Committee The Korean Society for Clinical Neurophysiology 2017 Annals of Clinical Neurophysiology Vol.19 No.2
Electroencephalography (EEG) is frequently used to assist the diagnosis of brain death. However, to date there have been no guidelines in terms of EEG criteria for determining brain death in Korea, despite EEG being mandatory. The purpose of this review is to provide an update on the evidence and controversies with regarding to the utilization of EEG for determining brain death and to serve as a cornerstone for the development of future guidelines. To determine brain death, electrocerebral inactivity (ECI) should be demonstrated on EEG at a sensitivity of $2{\mu}V/mm$ using double-distance electrodes spaced 10 centimeters or more apart from each other for at least 30 minutes, with intense somatosensory or audiovisual stimuli. ECI should be also verified by checking the integrity of the system. Additional monitoring is needed if extracerebral potentials cannot be eliminated. Interpreting EEG at high sensitivities, which is required for the diagnosis of brain death, can pose a diagnostic challenge. Furthermore, EEG is affected by physiologic variables and drugs. However, no consensus exists as to the minimal requirements for blood pressure, oxygen saturation, and body temperature during the EEG recording itself, the minimal time for observation after the brain injury or rewarming from hypothermia, and how to determine brain death when the findings of ECI is equivocal. Therefore, there is a strong need to establish detailed guidelines for performing EEG to determine brain death.
Fundamental requirements for performing electroencephalography
Koo, Dae Lim,Kim, Won-Joo,Lee, Sang-Ahm,Kim, Jae Moon,Kim, Juhan,Park, Soochul,Korean Society of Clinical Neurophysiology Education Committee The Korean Society for Clinical Neurophysiology 2017 Annals of Clinical Neurophysiology Vol.19 No.2
The performance of electroencephalogram (EEG) recordings is affected by electrode type, electronic parameters such as filtering, amplification, signal conversion, data storage; and environmental conditions. However, no single method has been identified for optimal EEG recording quality in all situations. Therefore, we aimed to provide general principles for EEG electrode selection as well as electronic noise reduction, and to present comprehensive information regarding the acquisition of satisfactory EEG signals. The standards provided in this document may be regarded as Korean guidelines for the clinical recording of EEG data. The equipment, types and nomenclature of electrodes, and the details for EEG recording are discussed.
Uncommon coexistence of myasthenia gravis and amyotrophic lateral sclerosis
Cho, Eun Bin,Yang, Tae-won,Jeong, Heejeong,Yoon, Changhyo,Jung, Seunguk,Park, Ki-Jong The Korean Society of Clinical Neurophysiology 2019 Annals of Clinical Neurophysiology Vol.21 No.2
Myasthenia gravis (MG) and amyotrophic lateral sclerosis (ALS) are distinct disorders. ALS affects motor neurons that control muscle movement, while MG controls communication between neurons and muscles, which occurs at neuromuscular junctions. However, on rare occasions, ALS develops after MG and vice versa. The coexistence of the two diseases represents a diagnostic challenge and requires thoughtful interpretation of clinical features. We present the case of a 53-year-old Korean male who developed ALS after MG, confirmed by clinical and electrophysiological follow-up.
Evoked potential: basic requirements and guidelines for writing reports
Lee, Eun-Mi,Seok, Hung Youl,Park, Kee Duk,Seo, Dae-Won,Korean Society of Clinical Neurophysiology Education Committee The Korean Society for Clinical Neurophysiology 2018 Annals of Clinical Neurophysiology Vol.20 No.1
Evoked potentials (EPs) measures the electrophysiologic responses of the nervous system to variety of stimuli. In clinical practice, only a few are used on a routine basis. Because of the small amplitude of EPs recorded by noninvasive methods, computer summation or averaging generally is necessary to resolve them from background noise. Therefore, waveform acquisition under good condition according to standard method is important. We aimed to provide the standards for clinical EP equipment, technical consideration and minimal requirements for obtaining good clinical EP waveforms, and general criteria for writing EP reports in practice as Korean guidelines.
A Korean family with AGel amyloidosis presenting with progressive facial and bulbar palsies
Kang, Minsung,Shin, Jin-Hong,Kim, Dae-Seong The Korean Society of Clinical Neurophysiology 2022 Annals of Clinical Neurophysiology Vol.24 No.2
AGel amyloidosis is an autosomal dominantly inherited disease caused by a GSN mutation, and affected patients typically present with the clinical triad of corneal lattice dystrophy, progressive cranial neuropathy, and cutis laxa. We report a Korean family with AGel amyloidosis with predominant manifestations of facial and bulbar muscle weakness. Whole-exome sequencing revealed a common missense mutation (p.Asp214Tyr) in GSN. This case strongly suggests that AGel amyloidosis should be considered when a patient presents with progressive facial and bulbar palsies.