Introduction Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by degeneration of the upper- (UMN) and lower motor neurons (LMN). The disease diagnosis is clinical and is based on signs of the involvement within these groups of neurons. The problem arises when during the early course of the disease some patients lack overt clinical signs of the UMN lesion or when, in the latter stages, severe LMN involvement may mask the UMN signs. Laboratory confirmation of the UMN degeneration would therefore be appropriate, but currently there is no sensitive method for this. Among the potential EEG means for studying UMN and other cortical motor functions there are movement-related cortical potentials (MRCPs) and the so called event-related desynchronization/synchronization (ERD/ERS). Their capability in studying ALS patients has not been properly explored yet. They measure different aspects of sensory-motor cortical processes during the preparation and execution of volitional movements. ALS usually progresses to involve virtually all skeletal muscle groups in the body: limb, trunk, bulbar, and respiratory. The latter is the main cause of death. We therefore decided to study the above mentioned EEG phenomena related to the inspiratory (sniffing), expiratory and right index finger flexion motor tasks. Aim and hypotheses Our aim was to investigate changes of sniffing-, expiratory- and finger flexion-related MRCPs and ERD/ERS in ALS patients in correlation with motor impairment, in particular with the upper motor neuron dysfunction. We also wanted to study alterations of the cortical control of voluntary movements in these patients. The following hypotheses were tested: 1. ALS patients have smaller MRCP amplitudes compared to controls; 2. MRCP amplitudes are smaller in patients who perform worse on hand and respiratory function tests; 3. ALS patients with higher UMN burden scores have smaller MRCPs compared to those with lower; 4. Patients have smaller resting and movement-related electroencephalographic alpha and beta spectral power densities compared to controls; 5. ALS patients have lower ERD and higher ERS compared to controls; 6. Patients with lower ERD or higher ERS will also do worse on hand and respiratory function tests; 7. Changes in MRCP amplitudes do not correlate with changes of ERD/ERS. Patients and methods Subjects (21 ALS patients, 19 controls for the sniffing and finger flexion task; 13 ALS patients and 12 controls for the expiratory task) were assessed for their hand dexterity and strength, respiratory function, speech capacity, spasticity, electromyographic parameters and functional rating scales. EEGs were recorded when patients and controls performed three self-paced motor tasks. The first task required flexions of the right index finger (FF), the second brisk nasal inspirations (SN) and the third brisk expirations (EX). A follow-up study was performed for the FF task on 5 ALS subjects that underwent a second EEG recording. In evaluating results ALS patients were divided into two subgroups, one with lower (LUB) and the other with higher UMN burden scores (HUB). Data analysis was divided into the evaluation of sniffing-, expiratory and finger flexion-related MRCPs and EEG time-frequency analysis. In the first part we investigated the Bereitschaftspotential 1, Bereitschaftspotential 2 and motor potential (MP) components of MRCPs at the relevant electrode positions for the FF, SN and EX tasks. In the second part alpha and beta spectral power densities and ERD/ERS analyses were performed for the FF and SN tasks. Clinical EEG data and movement execution parameters were compared by using a two-tailed Student's t-test with Holm-Bonferroni correction within the group of tests. A one-way ANOVA was used to compare our final MRCP data between the groups. Correlations were quantified by the Pearson’s r coefficient. Differences were considered significant at p ( 0.05. Results Patients generated large
B.06 Other
COBISS.SI-ID: 266048768Introduction The role of cerebral cortex in the control of breathing in health and disease is poorly understood. Therefore we wanted to answer some open questions. With electroencephalography (EEG) we wanted to (A) establish the pattern of cortical activation in relation to a volitional respiratory manoeuvre – a sniff. (B) We wanted to find out if there is an activation of motor cortex during automatic breathing in patients with amyotrophic lateral sclerosis (ALS) with symptoms and signs of respiratory insufficiency. And (C) we wanted to understand how the pattern of motor neuron degeneration correlates with breathing frequency and patients' ability to volitionally change it, how it affects the recruitment of accessory respiratory muscles, and the arterial blood gases. We wanted to (D) explore the impact of focusing on breathing-related sensations, on event-related desynchronization (ERD) and synchronization (ERS) of alpha and beta brain rhythms. Participants and methods A: In seventeen healthy people participated we recorded a 32-channel EEG while they performed two tasks: volitional sniffing and volitional index finger flexion. We measured the amplitudes of Bereitshaftspotential (BP), mapped them with the current source analysis and determined the location and orientation of the BP sources with the principal component analysis. B: Twenty ALS patients with at least one symptom or sign of respiratory insufficiency and 17 healthy participants were included. We assessed their degree of functional impairment and tested the myotatic reflexes to determine the extent of upper motor neuron degeneration. We measured the amplitudes of the compound muscle action potentials (CMAP) of both diaphragms, registered the symptoms of respiratory insufficiency, measured the respiratory function and respiratory muscle strength, and analysed the arterial blood gases. In wakefulness and sleep we continuously recorded EEG, breathing and sternocleidomastoid EMG. Based on the respiratory signal we analysed the EEG segments with the methods: BP and ERD/ERS in alpha and beta spectra. C: We estimated the degeneration of upper and lower respiratory motor neurons, the respiratory frequency in wakefulness and sleep and the extent of volitional control over it, the activity of the accessory respiratory muscles and measured the pO2 and pCO2 in the arterial blood. D: In seventeen meditators and 14 participants with no meditative experience we recorded the 128-channel EEG and simultaneously measured breathing with the nasal pressure sensors. Participants performed the following tasks: (i) active listening to the sounds and (ii) neutral thinking about themselves for eliciting automatic breathing, and the meditation-like tasks for eliciting attention to breathing: (iii) focusing on the breathing-related sensations and (iv) focusing on all bodily sensations. After the tasks, the participants filled in the questionnaires about their subjective perceptions during the tasks. EEG was analysed with the method of ERD/ERS in the alpha and beta spectra. Results A: Sniffing-related amplitudes of BP1 and BP2 were similar as finger flexion-related ones, whereas MP amplitudes were significantly different. The orientation of cortical sources in sniffing was similar as in finger-flexions during BP1, BP2 and MP, while the exact locations of the sources were different, especially during MP. B: Sniffing-related BP was present in all healthy participants and in 17 out of 19 patients. During automatic breathing in wakefulness, the negative potentials were noticed only in slightly more than one third of patients and approximately one quarter of healthy participants. The occurrence of these potentials in sitting and supine positions in wakefulness and in sleep was inconsistent. During automatic breathing in wakefulness, an ERD beta appeared in the central derivations. It did not differ between the groups. C: Half of the patients had predominantly lower respiratory motor neuron i
B.06 Other
COBISS.SI-ID: 274458112In patients with neuromuscular (NM) disorders, the failure of the lower motoneuron, neuromuscular junction or muscles causes muscle weakness, including respiratory muscle weakness. The aim of our work was to define breathing disorders in patients with myotonic dystrophy type 1 (DM1) and type 2 (DM2) and patients with amyotrophic lateral sclerosis (ALS) by all-night polysomnography (PSG) and diaphragm weakness by phrenic conduction studies and quantitative diaphragm MUP analyses. The following hypotheses were tested: 1. DM2 patients have milder sleep related breathing disorders than DM1 patients. 2. In DM2 patients there is no correlation between the tetra-nucleotide CCTG repeats in the affected gene and respiratory abnormalities. 3. Diaphragm and tongue MUPs are myopathic in DM patients and neurogenic in ALS patients. 4. Quantitative MUP analysis is a sensitive method for distinguishing myopathy (in DM patients) and neuropathy (in ALS patients) also in the diaphragm and tongue. 5. Patients with smaller phrenic CMAP amplitudes and more extensive changes of diaphragm MUPs have greater abnormalities in PSG, arterial gas analyses (AGA) and and poorer results of pulmonary function tests. 6. In DM and ALS patients despite the absence of daytime symptoms of respiratory insufficiency, signs of breathing disorders can be discovered. 7. There is no correlation between the number of obstructive apneas and tongue MUP abnormalities. 16 healthy subjects, 25 DM1, 16 DM2 and 52 ALS patients were included. Respiratory volumes and pressures were measured. Standard PSG and phrenic conduction studies were performed Quantitative MUP analyses were performed in diaphragm, tongue and biceps muscles. Clinical and electrophysiological data were analysed using parametric and nonparametric statistical tests. The results of respiratory function tests, average SaO2, pO2 and Hb/HbO2 in AGA were significantly smaller and EtCO2 and TCM-CO2 higher in patients with DM and ALS than in healthy subjects. DM1 patients were more affected than those with DM2. Night hypoventilation was present in 12% of DM1 and in none of the DM2 patients. Night hypoventilation was also present in 18% of ALS patients. Phrenic CMAP amplitudes were significantly lower in DM1 than in DM2 patients. In DM1 patients, signs of respiratory insufficiency correlated significantly with greater trinucleotides CTG repeats. No correlation was found between genotype and respiratory abnormalities in DM2 patients. Quantitative diaphragm MUP analyses could not be performed in 21% of DM1 patients, because their MUPs were too small and the EMG system did not recognise them. From those MUPs, which could be analysed, the average MUP parameters were neurogenic and thus greater than in the healthy subjects. Only one DM patient had neurogenic and myogenic MUP outliers, another seven DM patients had only neurogenic outliers. 18% of ALS patients had no MUP in the diaphragm and in those, only signs of denervation were present. Average MUP parameters were neuropathic with regard to the healthy subjects. 92% percent of ALS patients had neuropathic outliers, and 4% miopathic. A single ALS patient had normal MUPs. In DM patients, the greatest sensitivity of diaphragm MUP parameters for myopathy was only 9%, while in biceps muscle in the same patients it was 67%. In ALS patients, the sensitivity of MUP parameters was for neuropathy 88%. In the tongue, the greatest sensitivity of quantitative MUP analyses was for myopathy in DM patients 45%. In six DM patients, tongue MUPs were myopathic and neuropathic, 22 had only neuropathic and 3 myopathic MUPs. 28 ALS patients (97%) had neuropathic tongue MUP outliers and one myopathic. The sensitivity of tongue MUP parameters was for neuropathy in ALS patients 93%. Smaller phrenic CMAP amplitudes correlated in DM and ALS patients statistically significantly with smaller respiratory volumes and pressures and a smaller amount of REM sleep. In DM patients, it correlated also with
B.06 Other
COBISS.SI-ID: 267727360The 2nd Alpine Sleep Summer School (the first took place in summer 2011 in Lugano, Switzerland) was organised under the auspices and with the financial & organisational help of the European Sleep Research Society. Its aim is to offer Central (“Alpine”) European students, MDs, PhDs, technicians and other health and scientific professionals involved or interested in sleep medicine a thorough introduction to sleep medicine. The course lasted five days, each of which covers one of the main topics (the basics of sleep on Monday, sleep and psychiatry on Tuesday, sleep and respiratory medicine on Wednesday, sleep and neurology on Thursday and sleep and paediatrics on Friday). Four special lectures on the “hot topics” concerning sleep medicine and sleep research, 60 lectures, 12 case-study discussions, and many video sessions completed the programme of the week. There 83 participants from different countries. Proceedings with the programme is available at http://www2.kclj.si/ikn/DEJA/FAGA/Zborniki/Zbornik2013.pdf
F.17 Transfer of existing technologies, know-how, methods and procedures into practice
COBISS.SI-ID: 268303872