Objective Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disorder characterized by degeneration of the upper and lower motor neurons. Each voluntary movement, including inspiration, is preceded by movement-related cortical potential (MRCP) that can be recorded from the scalp. MRCPs of ALS patients with severe upper motor neuron involvement are smaller. Our aim was to explore whether the inspiratory-(sniffing)-related cortical potentials (SRCPs) and index-finger-flexion MRCPs (FFRCPs) can be used as markers of cortical involvement in ALS. Methods Thirteen ALS patients and 15 healthy volunteers were assessed for their hand dexterity and strength, respiratory function, speech capacity, spasticity, electromyographic parameters and functional rating scales. EEG was recorded during self-paced sniffing and the right index finger flexion. The MRCP amplitudes were assessed at the relevant electrode positions. Results No statistically significant difference was found between the MRCP amplitudes of the ALS patients and the control subjects. However, patients with more severely affected upper limb functions generated smaller FFRCPs and those with more affected respiratory functions generated smaller SRCPs. Excessively high FFRCPs were associated with better while excessively low FFRCPs with worse scores on some of the clinical measures of the upper limb function. Conclusion Our preliminary results demonstrate that it is feasible to record SRCP in ALS patients, which combinedwith FFRCP, may be useful to determine the spectrum of motor control changes in this population.
COBISS.SI-ID: 176812
Background: Various breathing abnormalities (Neurology 2009; 73: 1218) have been proposed as indicators for the introduction of non-invasive positive-pressure ventilation (NIV) in patients with amyotrophic lateral sclerosis (ALS). We were interested in the usefulness of symptoms of respiratory insufficiency and abnormal results of daytime arterial gas analyses (AGA) as predictors of survival and the effect of NIV on respiratory volumes and pressures. Methods: Reported symptoms, respiratory subscore of the ALS Functional Rating Scale (ALSFRS-r), Norris scale (Norris-r), and AGA were retrospectively analyzed in 189 ALS patients. Longitudinal follow-up of forced vital capacity (FVC), maximal inspiratory and expiratory pressure (MIP, MEP), and sniff nasal pressure (SNP) were analyzed with regard to the introduction of NIV. Results: Respiratory symptoms were a bad prognostic sign (P = 0.007). Abnormalities in Norris-r, ALSFRS-r, pO2, pCO2, and oxygen saturation tended to be associated with a shorter survival, although they were not statistically significant. NIV prolonged survival and reduced the decline in FVC (P = 0.007), MIP, MEP, and SNP (the last three were not statistically significant). Symptoms, abnormal FVC, and AGA do not always coincide, and they can appear in a different sequence. Conclusions: Any respiratory abnormality should prompt the clinician to start discussing NIV with the patient. NIV prolongs survival and improves respiratory function.
COBISS.SI-ID: 1663081
The present paper describes standardized procedures within clinical sleep medicine. As such, it is a continuation of the previously published European guidelines for the accreditation of sleep medicine centres and European guidelines for the certification of professionals in sleep medicine, aimed at creating standards of practice in European sleep medicine. It is also part of a broader action plan of the European Sleep Research Society, including the process of accreditation of sleep medicine centres and certification of sleep medicine experts, as well as publishing the Catalogue of Knowledge and Skills for sleep medicine experts (physicians, non-medical health care providers, nurses and technologists), which will be a basis for the development of relevant educational curricula. In the current paper, the standard operationalprocedures sleep medicine centres regarding the diagnostic and therapeutic management of patients evaluated at sleep medicine centres, accredited according to the European Guidelines, are based primarily on prevailing evidence-based medicine principles. In addition, parts of the standard operational procedures are based on a formalized consensus procedure applied by a group of Sleep Medicine Experts from the European National Sleep Societies. The final recommendations for standard operational procedures are categorized either as 'standard practice', 'procedure that could be useful', 'procedure that is not useful' or 'procedure with insufficient information available'. Standard operational procedures described here include both subjective and objective testing, as well as recommendations for follow-up visits and for ensuring patients' safety in sleep medicine. The overall goal of the actual standard operational procedures is to further develop excellencein the practice and quality assurance of sleep medicine in Europe.
COBISS.SI-ID: 502444