Proposal for Teaching Courses

90 minutes theory and 90 minutes hands-on session

Theoretical program

1. Introduction to neuromuscular ultrasound (15 minutes): Nens van Alfen
2. The complementary role of ultrasound in entrapment neuropathies (25 minutes): Josef Böhm
3. The complementary role of ultrasound in dysimmune neuropathies (20 minutes): Zsuzsanna Arányi
4. The complementary role of ultrasound in nerve trauma (20 minutes): Nens van Alfen
5. Ultrasound of nerve tumors (10 minutes): Zsuzsanna Arányi

The high resolution ultrasonography of peripheral nerves is a relatively new, but already firmly established and readily accessible tool in the assessment of peripheral nerve disorders. It has been shown that ultrasonography influences the diagnostic and therapeutic pathway in the majority of patients. The aim of this course is to demonstrate how ultrasonography complements electrophysiology with morphological information in common conditions, including entrapment syndromes, nerve trauma, dysimmune and hereditary neuropathies, and nerve tumors. The course has two parts. During the first half, experts in the field will give presentations in the above mentioned disease categories. The second half will be a hands-on session, where participants will be able to practice the ultrasonographic examination of normal and abnormal nerves.

An international taskforce endorsed by the IFCN developed a computer-based system for reporting EEGs. The features extracted by the electroencephalographer, are reported by choosing pre-defined terms from the SCORE-EEG software. This automatically generates a standardized report, and feeds the scored information into the database. In the first part of the teaching session, the participants will learn about the SCORE system. In the second, hands-on part, the participants, along with the lecturers, will review and score EEG recordings, including samples of standard recordings, sleep EEGs, long-term video-EEG recordings and neonatal EEGs.

Lecturers:
Dr. Sándor Beniczky. Danish Epilepsy Centre and Aarhus University, Denmark
Dr. Harald Aurlien. Haukeland University Hospital, Bergen, Norway
Dr. Jan Brøgger. Haukeland University Hospital, Bergen, Norway
Dr. Ronit Pressler, Great Ormond Street Hospital for Children, NHS Foundation Trust, London United Kingdom.

BACKGROUND

Intensive Care Unit (ICU)-acquired weakness is a major complication in intensive care, in particular prolonging mechanical ventilation and increasing mortality rate. This is a common complication correlated with hospitalization time and persistent disability. Neurophysiological assessment is mandatory in the differential diagnosis between myopathy and neuropathy as the main cause of ICU-acquired weakness, which is important for management. Moreover, diaphragmatic dysfunction causes difficult weaning or even failure to wean from mechanical ventilation in ICU patients. It has been described that weaning failure can affect approximately 25% of ventilated patients in ICU. Proper neurophysiological investigation is critical for the differential diagnosis between muscular fibre lesion, phrenic nerve damage or ventilator‐induced diaphragmatic dysfunction, which has been recently acknowledged. This information is essential to determine best intervention, for example the application of transvenous phrenic nerve stimulation. In this course we aim to detail the advanced techniques available to investigate ICU-acquired weakness, including respiratory muscles function.

COURSE STRUCTURE

1. Introduction – 15 minutes with discussion Quick overview of the neuromuscular complication in the intensive care setting. Mamede de Carvalho (Faculty Medicine, University of Lisbon and Department of Neurosciences, Centro Hospitalar Lisboa Norte)

2. Intensive Care Unit myopathy – 45 minutes with demo/video and discussion Hatice Kantisi and Kirsten Pugdhal (Department of Clinical Medicine - Department of Clinical Neurophysiology, Department of Clinical Medicine, Health, Aarhus University)

Needle EMG can be used to diagnose critical illness myopathy. The diagnostic background for using quantitative Individual MUP analysis, Interference Pattern Analysis i.e. Peak Ratio and multi fibre response to direct muscle stimulation, including Muscle Velocity Recovery Curve, will be discussed and demonstrated. By these methods information on random loss of muscle fibres and muscle fibre membrane excitability can be obtained.

2)– Intensive Care Unit neuropathy -- 45 minutes with demo/video and discussion Birger Johnsen (Department of Clinical Medicine - Department of Clinical Neurophysiology, Department of Clinical Medicine, Health, Aarhus University)

Motor nerve conduction studies (MNCS) or electromyography may often not differentiate between critical illness myopathy and critical illness polyneuropathy. In either case, MNCS may show decrease in CMAP amplitude with normal or near normal conduction velocity and EMG may show denervation activity without possibility for evaluation of motor unit potentials due to the patient’s incapability to perform a voluntary contraction. In this typical situation, reliable sensory nerve conductions studies (SNCS) are of paramount importance, as abnormal findings indicate the presence of a neuropathy. Traditional SNCS with surface electrodes may, however, show false positive results due to edema, electrical artifact or sweating. This is not the case for near-nerve studies, as the technique ensures placement of the recording needle electrode close to the nerve. Reliable SNCS are also important for the differential diagnosis to other sub-acute neuromuscular diseases, for example different types of Guillain-Barré’s syndrome, motor neuron disease and myasthenia. This session will include theoretical background and practical demonstration of SNCS with the near-nerve technique.

3)– Respiratory Evaluation -- 45 minutes with demo/video and discussion Mamede de Carvalho (Faculty Medicine, University of Lisbon and Department of Neurosciences, Centro Hospitalar Lisboa Norte)

Diaphragm weakness can primarily derive from phrenic nerve lesion (ICU neuropathy, Guillain-Barré’s syndrome), muscle fibre lesion (ICU myopathy, unknown previous myopathy), end-plate disorder, and ventilator-induced diaphragm inactivity or due to central drive dysfunction. For a complete assessment, phrenic nerve stimulation with needle and/or surface recordings, diaphragm needle sampling, and needle EMG of other respiratory muscle, like as internal intercostal muscles, is a critical evaluation set. We intend to detail these techniques and to show some of them in order to permit the participants to know their potentialities.

4)– Open Discussion – 30 minutes Mamede de Carvalho (Faculty Medicine, University of Lisbon and Department of Neurosciences, Centro Hospitalar Lisboa Norte) Anders Fuglsang-Frederiksen (Department of Clinical Medicine - Department of Clinical Neurophysiology, Department of Clinical Medicine, Health, Aarhus University)

We will encourage discussion. Short complex cases provided by the Faculty or participants will be discussed in order to approach how the diverse neurophysiological techniques could improve diagnostic accuracy.

The principles of needle EMG and of the changes in neurogenic and myogenic disorders (e.g. 30 min) will be presented at the beginning of the course, and a hands on pratice will follow.

Reinhard Dengler, Susanne Petri

We would discuss the El Escorial diagnostic Criteria and the role of clinical neurophysiology as described in the El Escorial Criteria. We would talk about the so called Awaji criteria which are more sensitive and equally specific as the El Escorial Criteria alone and would also refer to motor unit number estimation techniques (MUNE). We would discuss the impact of modern high resolution ultrasound on ALS diagnosis and would refer to the potential of clinical neurophysiology to detect upper motor neuron signs, e.g. paired pulse TMS and the triple stimulation techniques.
The target audience of this course would be neurologists and clinical neurophysiologists with some experience and a clinical interest in this field.

Epileptic spasms are a seizure type occuring in West syndrome, an electro-clinical syndrome of the first year of life with severe encephalopathy. Their most common clinical presentation is a sudden brief tonic contraction associated to the ictal electroencephalographic pattern of a slow wave transient. However epileptic spasms can occur at any age, and present many different electro-clinical patterns, possibly difficult to diagnose, especially when subtle.
As rapid control of this seizure type has been shown to be important for long-term outcome, in terms of seizures and cognition, especially in patients with unknown etiology, early recognition is crucial.
Therefore detailed knowledge of appropriate neurophysiological recording techniques and electroencephalographic patterns accompaying this seizure type is paramount to the correct diagnosis of the seizure type and the electro-clinical syndrome.
In the teaching course EEG-video recordings will illustrate the various electro-clinical aspects of epileptic spasms at the different age groups, in the neonatal period, early infancy and late infancy/ early childhood, and the numerous different etiologies as genetic diseases, inborn errors of metabolism, structural abnormalities as cortical malformations, or following hypoxic ischemic encephalopathy or herpetic encephalitis.

We would like to focus on the practical aspects of neuromonitoring in the neurosurgical field. The course will cover the most important monitoring techniques during the first part, with demonstration of our own experience. We would like to also focus on the complementary use of modern neuroimaging with neuromonitoring to achieve even better outcome, and less morbidity, for the benefit of the patients. In the second part we would like to broadcast live surgery from the operating room. The demonstration will start with the preoperative duties of the electrophysiologist until the end of the whole procedure.

The name of the presenters and the title of their lectures:

Loránd Erőss: An overwiev of intraoperative neuromonitoring from the neurosurgeons perspective

Dániel Fabó: Basic skills and equipment necessary to start a comprehensive neuromonitoring service

Dániel Fabó: Neuromonitoring of epilepsy surgical cases

László Entz : Intraoperative and extraoperative mapping with direct cortical stimulation in epilepsy surgical candidates, possible new ways to predict results using imaging techniques
Boglárka Hajnal: Neuromonitoring of intramedullary tumors

Balázs Markia: Electrophysiology and surgical technique during SDR operations in Budapest

Gábor Fekete : Monitoring of the cranial nerves during cerebello-pontine angle tumor removals

Dávid Kis: Complementary use of intraoperative neuromonitoring with the aid of diffusion tensor imaging during removal of brain tumors

Gertrúd Tamás : Electrophysiological guidance during DBS surgery, our own experience, pros and cons

László Halász : Probabilistic tractography may substitute intraoperative electrophysiology in DBS electrode placements, accuracy and results

Virág Bokodi : Evidences behind the regular use of neuromonitoring with special emphasis on the added benefit

We would like to focus on the practical aspects of neuromonitoring in the neurosurgical field. The course will cover the most important monitoring techniques during the first part, with demonstration of our own experience. We would like to also focus on the complementary use of modern neuroimaging with neuromonitoring to achieve even better outcome, and less morbidity, for the benefit of the patients. In the second part we would like to broadcast live surgery from the operating room. The demonstration will start with the preoperative duties of the electrophysiologist until the end of the whole procedure.

Live surgery broadcast from the operating room demonstrating intraoperativeneuromonitoring: Loránd Erőss, László Entz, Dániel Fabó

Names of the lecturers:

Walter Struhal General Hospital of the City of Linz, Linz, Austria

Ellen Merete Hagen Autonomic Unit National Hospital for Neurology and Neurosurgery

London, UK

Mario Habek, Referral Center for Autonomic Nervous System

Department of Neurology, University Hospital Center Zagreb

Proposed topics

1) How to test autonomic nervous system?

2) Functional disorders of the ANS

3) Structural disorders of the ANS

As almost every organ system receives regulatory information from the central nervous system (CNS) through the autonomic nervous system (ANS), and vice a versa, it is no wonder that the ANS plays a crucial role in homeostasis. Therefore, testing of the ANS is important in various medical specialties, resulting in numerous tests of autonomic function (more ANS tests exist than for any other neurological system). The anatomic location of the ANS renders it inaccessible to simple direct physiological testing, and this is one more reason why only few ANS tests are validated. Therefore, in the first part of this educational course a special emphasis will be given to fully quantitative and clinically validated protocol for testing of autonomic functions presented in the form of Composite Autonomic Severity Score.

The second part of the educational course will be clinically/patient orientated with interactive presentation of clinical vignettes. As for the best understanding of disorders of the ANS, dysautonomia can be divided either functional or structural. Functional disorders currently

have no consistently demonstrable pathologic basis, are primarily defined by symptomology and often constitute a syndrome. In this part of the educational course syncope, postural orthostatic tachycardia syndrome and complex regional pain syndrome will be discussed in details accompanied with patient presentations. A special emphasis will be given to differentiation between epileptic seizures, syncope and pseudosyncope. Structural disorders, on the other hand, are defined as having demonstrable pathologic abnormalities on ANS tests. They can be divided into central and peripheral dysautonomias, so diseases like myelopathy and spinal cord injury, multiple system atrophy, Parkinson’s disease, multiple sclerosis and autoimmune autonomic ganglionopathy, autonomic neuropathies, respectively.

We believe that this educational course would be of interest to wide range of participants of the European Conference of Clinical Neurophysiology. ANS disorders are far more frequent than reported, so with this educational course we aim to raise the recognition of this problem and to learn the participants how to perform and when to order testing of the ANS.

Names of the lecturers:

Eleftherios S. Papathanasiou The Cyprus Institute of Neurology & Genetics Nicosia, Cyprus

Leonel Luis, Translational Clinical Physiology Unit Institute of Molecular Medicine

Faculty of Medicine, University of Lisbon Lisbon, Portugal

Mario Habek Department of Neurology, University Hospital Center Zagreb, University of Zagreb, School of Medicine Zagreb, Croatia

Proposed topics

1) VEMPs methodology

2) VEMPs in peripheral vestibular disorders

3) VEMPs in central vestibular disorders

In recent years vestibular evoked myogenic potentials (VEMP) have gained a lot of popularity in assessing vestibular function and dysfunction. VEMPs are a clinical test of the otolith organs, sensors of linear acceleration and related reflex pathways. Cervical VEMP (cVEMP), universally regarded as representing predominantly saccular pathway function, is mediated by the vestibular nuclei and ipsilateral (uncrossed) medial vestibulospinal tract projecting to the spinal accessory nucleus. On the other hand, ocular VEMP (oVEMP) are regarded by most researchers to originate from the utricle, and reflects the functions of the vestibular nuclei and the vestibulo-ocular reflex pathways, which project to the contralateral oculomotor nucleus through the contralateral medial longitudinal fasciculus (MLF). Recently, guidelines for performing cVEMPs were published, however guidelines for oVEMPs are still missing. In the first part of this educational course a special emphasis will be given to different methodologies used to obtain responses, their advantages and disadvantages, differences in VEMPs recordings regarding the age of patients and/or diseases in question.

The second part of the educational course will be clinically/patient orientated with interactive presentation of clinical vignettes.

As VEMPs have been developed as a test for peripheral vestibular system, their clinical utility in peripheral vestibular disorders will be explored first. VEMPs have very well documented clinical importance for estimating the severity of peripheral vestibular damage due to different pathophysiologic processes such as Meniere’s disease, vestibular neuritis, benign paroxysmal positional vertigo and superior canal dehiscence syndrome.

On the other side, recent findings suggest that VEMPs reflect functions of the central otolithic pathways as well, indicating that they could also be applied to disorders of the central nervous system. Their usefulness for patients with vestibular migraine, multiple sclerosis, brainstem stroke and Parkinson’s disease/other neurodegenerative disorders will be explored. Furthermore, the new role of oVEMP as a diagnostic test for myasthenia gravis will be emphasized.

We believe that this educational course would be of interest to wide range of participants of the European Conference of Clinical Neurophysiology. As VEMPs have entered the mainstream of testing of vestibular disorders (both peripheral and central), the knowledge gained in this educational course will help the participant not only to understand the methodology, but also to refer the appropriate patient to VEMP testing and to appropriately interpret obtained findings.

During this presentation I will give brief history about the stereotactic neurosurgical procedure for movement disorders, components of deep brain stimulation (DBS), mechanism of DBS, preparation for stereotactic surgery starting from frame application, computer target determination, and general stereotactic technique. Then I will highlight the use of micro-electrode recording (MER) for anatomical target localization, and the purpose of stimulation during stereotactic procedures. During this presentation I will show videos of Parkinsonian patient with tremor and rigor intra-operatively as well videos of Parkinson patient before- and after deep brain stimulation. Video of parkinsonian patient showing the symptoms with medication ON-OFF and compare the symptom after DBS.

Name of presenters: Dr Laszlo Acsady, Dr Norbert Hajos, Dr Zoltan Nusser, Dr Balazs Rozsa

Short description:
The course will be held in the Institute of Experimental Medicine, Hungarian Academy of Sciences where the attendees will participate in state-of-the-art in vitro patch-clamp electrophysiological, in vitro and in vivo two-photon Ca2+ imaging and optogenetic experiments. The Institute is a basic neuroscience center where 15 independent research groups investigate different aspects of CNS functions, including molecular mechanisms of signal transduction, synaptic neurotransmission and integration; cellular mechanisms underlying dynamic network phenomena like network synchronization and oscillations; the role of distinct nerve cells, specific pathways and brain areas in behaviors. The research groups use multidisciplinary state-of-the-art approaches, including superresolution STORM imaging, electron microscopic tomography, in vivo and in vitro two-photon voltage- and Ca2+ imaging, optogenetics, connectomics and freely moving or head-restrained animal behavior in combination with electrophysiology, imaging and optogenetics.

Walter Paulus¹, Michael A Nitsche², Andrea Antal ¹, Ulrich Palm³

¹Department of Clinical Neurophysiology, University Medical Center, Göttingen, Germany

²Department of Psychology and Neurosciences, Leibniz Research Centre for Working Environment and Human Factors, Dortmund, Germany

³Department of Psychiatry, Ludwig-Maximilians University, Munich, Germany

Dynamic modifications of neuronal networks are an important substrate for learning and memory formation, both in healthy subjects and in patients. However, pathological neuroplasticity might be one of the foundations of numerous central nervous system diseases. A diversity of stimulation methods such as transcranial direct current (tDCS), alternating current (tACS) and random noise (tRNS) were developed non-invasive tools to induce and/or modify neuroplasticity in the human cerebral cortex. The most frequently used method is tDCS, both in research and in therapeutic applications such as in neurorehabilitation, chronic pain, depression and stroke. tDCS as a tool aims to induce prolonged neuronal excitability and activity alterations in the human brain via alterations of the neuronal membrane potential. Nevertheless, the precise physiological mechanisms by which stimulation influences brain function on long-term, are only partially understood. Quantification of plastic aftereffects can be realized by single pulse transcranial magnetic stimulation (TMS) at the human primary motor cortex (M1). Combining tES with concurrent functional magnetic resonance imaging (fMRI) and electroencephalogram (EEG) has recently opened new possibilities for quantifying altered neuronal activity both under the stimulating electrodes and in remote brain regions.

tACS is assumed to entrain endogenous neural oscillations possibly by modulating the power of oscillations or the phase-locking index between the driving and endogenous oscillations. tACS is used in the research and clinical practice to establish a causal role of different oscillations and functions of the central nervous system as well as to restore disturbed oscillations in neurological and psychiatric disorders. The effect of tACS is frequency, intensity and phase dependent. If applied long enough it may cause neuroplastic effects. tRNS probably interfere with ongoing network oscillations or with homeostatic mechanisms.

The educational course aims to give an overview about basic and clinical results, including clinical conditions, such as stroke, epilepsy, chronic pain, migraine, depression and schizophrenia. The duration of the talks will be 30 min each, followed by a 30 min general discussion.

Speakers:

Walter Paulus: Basics of tES

Michael A Nitsche: tDCS and stroke treatment

Andrea Antal: Clinical applications in chronic pain and migraine

Ulrich Palm: tES in psychiatry

The analysis of needle EMG remains a real challenge while performing EMG examination. Everyone basically knows how to differentiate a typical neuropathic pattern from a typical myopathic pattern, or how to differentiate myotonic discharges from pseudo-myotonic discharges. But there are some difficult cases where both myopathic and neuropathic characteristics can be mixed, or where one mimics the other, leaving the EMGist puzzled. The objective of this session is, after having reminded the pathophysiological bases of needle EMG interpretation, and using practical examples with EMG videos picked up among routine examinations, to show that ‘pseudo-myopathic’ and ‘pseudo-neuropathic’ patterns do exist and may represent pitfalls that one should avoid; that spontaneous activities may also be confusing. The objective is also to explain how this can occur and to provide to the participants the tools to get the better of the EMG exam.

The goal of this hands-on workshop is to demonstrate a combination of techniques that help increase the sensitivity over “standard” sensory distal latencies. Over the last two decades there has been more emphasis on comparing the median sensory response to that of the ulnar and radial responses, and to comparing the more distal portion of the median sensory response to that across the carpal ligament. The Robinson cumulative summary index (CSI) gives access to an easy protocol for a fast EDX diagnosis of carpal tunnel syndrome. Techniques:

• Median-radial sensory latency difference to the thumb
• Median-ulnar sensory latency difference to the ring finger
• Median-median mid-palmar sensory study
• Median-sensory short segment stimulation
• Robinson compound summary index
• Median-lumbrical to ulnar-interossei latency difference
• Median-thenar to ulnar-thenar latency difference

Video-EEG monitoring has become available at most large referral centers with an epilepsy program, and more recently in many smaller hospitals and on an outpatient basis. Evaluation is usually made by experienced epileptologists and/or neurophyisiologists. We can learn more about the epilepsy of our patient in few days of video-EEG in the EMU (Epilepsy Monitoring Unit) than during 10 years at the outpatient clinic. As a general rule, prolonged video-EEG monitoring should be obtained on any patient who continues to have frequent seizures despite antiepileptic medication. The purpose of video-EEG is to answer the following questions: 1.) Are the episodes epileptic seizures? 2.) If not, what are they? 3.) If they are epileptic seizures, what type of epilepsy is it? 4.) If the seizures are focal, from which cortical region are they arising (evaluation for epilepsy surgery). EEG-video is the criterion standard for the diagnosis of seizures or nonepileptic attacks.

"Hypermotor seizures": Complex partial seizures are often characterized by hypermotorbehaviors, and bizarre-looking episodes that may be mistaken for PNES(psychogenic nonepileptic seizures). Tonic posturing and head and eye deviation (version), usually contralateral to the side of the seizure focus, can occur in some patients. Vocalizations are also common. Most of hypermotor seizures are sleep-related.

The diagnosis of these attacks is generally challenging: we have to differentiate them not only from the PNES but from parasomnias as well, misdiagnosis of which have considerable impact on the patient’s life. In surgical cases we have to face the difficulties of the localization of the "epileptogenic zone" .

In most cases the seizures arise from hidden surfaces of the frontal cortex, from frontomedial or orbitofrontal region, besides, hypermotor seizures can start in extrafrontal regions as well, i.e. temporopolar/basal, insular or posterior cortex. It means that the ictal scalp EEG changes are often subtle , uncertain, misleading or not rarely absent.

We bring six cases from our practice which cover many aspects of the difficulties in differential diagnostics and/or in localization of the seizure onset with scalp and intracranial leads. The interactive structured analysis of these cases with the participants will be performed with discussion of a possible etiological and semiological classification of hypermotor seizures.

Lecturers: György Rásonyi MD, Anna Kelemen MD

Localization of focal epilepsies are responsible for special seizure semiologies in adults. However, children –especially infants and young children- may show variable semiology independently of seizure onset zone localization. Recent researches showed that brain maturation significantly influences the evolution of some important aspects (eg. motor seizures and lateralizing signs) of seizure semiology. Other seizure elements (such as aura, emotional and autonomic signs) seem to be independent of the maturation process. Our lecture will assess the most important semiological changes of childhood focal seizures supporting them with several video presentation.

The EEG of the newborn and premature infants follows a dynamic process during development that allows precise characterization of the gestational age of premature infants. The objective of this course is to be able to recognize the normal cerebral activity in the different stages of maturation by identifying and discriminating the specific neurobiomarkers of development (Theta Temporal activities, Delta brushs, “Encoches Frontales”) in parallel of the modifications of amplitude and synchronization between the hemispheres. We will also learn how to recognize the different stages of sleep at different ages. Finally, we will learn to identify and discriminate artefacts, regardless of their endogenous or exogenous origin.