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
Our aim was to investigate changes in movement-related cortical potentials (MRCPs) in ALS patients with different degrees of upper motor neuron (UMN) involvement. Since respiratory failure is the main cause of death in ALS, changes in inspiratory-related (sniffing) potentials were studied in addition to finger-flexion-related potentials. Subjects (21 ALS, 19 controls) performed two self-paced motor tasks while their EEGs were recorded. The first task required flexions of the right index finger and the second, brisk nasal inspirations. The early (BP1), late (BP2) and motor potential (MP) components of MRCPs were evaluated. Results showed that patients generated higher MRCPs than controls. However, this effect was most significant in the subgroup of patients with low UMN burden (LUB). The high UMN burden (HUB) subgroup did not differ from controls, but had significantly lower MP amplitudes than the LUB subgroup. Progressive UMN deterioration was associated with an initial increase, followed by a later decrease, in MP amplitudes in ALS. In conclusion, the increased MRCPs in LUB compared to HUB patients indicate different processes of ALS pathophysiology that force opposing changes in MRCP amplitudes.
COBISS.SI-ID: 1001132
Purpose The objective was to determine the separate and combined effects of hypoxia and inactivity/unloading on sleep architecture during a 10-day period of confinement. Methods T en subjects participated in three 10-day trials in random order: hypoxic ambulatory (HAMB), hypoxic bedrest (HBR), and normoxic bedrest (NBR). During the HAMB and HBR trials, subjects were confined to a hypoxic facility. The hypoxia profile was: simulated altitude of 2,990 m on day 1, 3,380 m on day 2, and 3,881 m on day 3. In the NBR and HBR trials, subjects maintained a horizontal position throughout the confinement period. During each trial, sleep polysomnography was conducted one night prior to (baseline; altitude of facility is 940 m) and on the first (NT1, altitude 2,990 m) and tenth (NT10, altitude 3,881 m) night of the 10-day intervention. Results Average time in sleep stage 1 decreased from NT1 to NT10 irrespective of trial. Overall incidence and time spent in periodic breathing increased from NT1 to NT10 in both HAMB and HBR. During NT1, both HAMB and HBR reduced slow-wave sleep and increased light sleep, whereas NBR and HBR increased the number of awakenings/night. There were fewer awakenings during HAMB than NBR. Conclusions Acute exposure to both hypoxia and bedrest (HBR) results in greater sleep fragmentation due to more awakenings attributed to bedrest, and lighter sleep as a result of reduced slow wave sleep caused by the hypoxic environment.
COBISS.SI-ID: 1690284
Introduction: The most common etiology of hypercapnic respiratory failure is chronic obstructive pulmonary disease (COPD). However, the differential diagnosis also includes neuromuscular disorders. We studied the specificity ofreduced amplitude phrenic nerve compound motor action potential (CMAP) to diagnose neuromuscular disorders. Methods: A group of patients with advanced COPD were recruited prospectively and compared with controls. Phrenic nerve CMAPs were measured bilaterally using supraclavicular surface stimulation and bipolar recording (G1: 5 cm above the xiphoid; G2: 16 cm from G1). Results: A group of 20 patients (15 men) and a group of 29 controls (15 men) were included. Phrenic nerve CMAPs of patients with COPD had significantly longer latency and higher amplitude. Conclusion: Our study demonstrates that patientswith hypercapnic respiratory failure and reduced phrenic nerve CMAP amplitude most probably have a neuromuscular disorder affecting the diaphragm and not COPD or another lung disorder.
COBISS.SI-ID: 715948
This study estimated the whole-scalp topography and possible generators of thecortical potential associated with volitional self-paced inspirations (sniffs). In 17 healthy subjects we recorded a 32-channel electroencephalogram(EEG) during sniffing, for comparison during finger flexions. We averaged the EEG with respect to movement onset, and performed current source density and principal component analysis on the grand averaged data. We identified an early negative sniffing-related cortical potential starting Ž1.5s before movement at the vertex, which, in its time-course and dipole orientation, closely resembled Bereitshaftspotential preceding finger flexions. Around the movement onset, its topography became unique with three negative current sources: one at the vertex, and two bilaterally over the fronto-temporal derivations. We conclude that sequential cortical activation in preparation for sniffing is similar to other volitional movements. The current sources at sniff onset at the vertex likely reflect somatotopic motor representation of the diaphragm, neck and intercostal muscles, whereas currentsources over fronto-temporal derivations likely reflect the somatotopicrepresentation of the orofacial muscles.
COBISS.SI-ID: 9459284