An electrophysiological study of the sequential water swallowing

WOS: 000318905600014PubMed ID: 23466271Background: Sequential water swallowing (SWS) was mostly investigated by the videofluoroscopic and endoscopic methods. However some physiological features of SWS was rarely evaluated by neurophysiological techniques. Our aim was to investigate some neural and muscular changes on sequences of SWS using electromyography (EMG) methods. Methods: Fifty-eight normal adults were investigated. SWS was initiated voluntarily with 50 ml and 100 ml water volumes from a cup. Submental EMG, respiratory signals, heart rate, and sympathetic skin responses (SSR) were measured during SWS. Key results: All parameters were increased significantly during the 100 ml SWS. During swallowing apnea period, compensatory respiration cycles occurred in 24% and 48% of participants in the 50 ml and 100 ml SWS, respectively. Heart rate increased during swallowing apnea. SSR were evoked just before and just after the SWS in more than halves of participants. A foreburst EMG in SM muscles at the initiation of SWS was recorded in 86% of normal participants. Older age was associated with a prolonged duration of the apnea period. Conclusions: All parameters of the SWS could be recorded numerically and objectively using electrphysiological methods. These are similar to those obtained by videofluoroscopic and similar methods. The foreburst activity of the initiation of SWS may represents preparatory activity from the activation of the fast cortical descending motor pathway. Increasing heart rate and the prolonged apnea urged that older people and patients could be carefully tested for respiratory and cardiac rhythm disorders. (C) 2012 Elsevier Ltd. All rights reserved

Diagnostic value of lumbar root stimulation at the early stage of Guillain-Barré syndrome

PubMed ID: 22925837Objective: The aim of this study is to investigate diagnostic value of electrical lumbar root stimulation (RS) at the laminar level in the early stage of Guillain-Barré syndrome (GBS). Methods: Fifteen patients (30 sides) and nine controls (17 sides) were included in the study. Conventional nerve conduction studies, needle electromyography, F responses and electrical lumbar RS were obtained from both groups. The needle electrical stimulation was performed at the L2-3 intervertebral level. Vastus lateralis, tibialis anterior and soleus muscles were investigated bilaterally and simultaneously in the first and fourth weeks. Results: In all patients, the amplitudes elicited by lumbar RS were significantly attenuated while the conventional electrophysiological findings were normal and/or not diagnostic in 6 of 15 patients (40%) within the first week. Motor latencies by the lumbar RS were prolonged in the patients, compared to the controls, but the results were not statistically significant. Conclusions: M-responses elicited by lumbar RS appear to be helpful in disclosing proximal conduction abnormalities of GBS early in the course. Significance: Lumbar RS seems to be a useful method in making the diagnosis of GBS early and there is no considerable side effect of this particular method. © 2012 International Federation of Clinical Neurophysiology

Sympathetic skin responses in adult humans during sequential swallowing

WOS: 000313863200002PubMed ID: 23290172Study aims.-Autonomic changes, especially those of sympathetic skin responses (SSR), during sequential water swallowing (SWS) have not been systematically investigated. This study aims to electrophysiologically examine these autonomic changes (SSR and heart rate) that occur during 50 ml sequential water swallowing from a cup. Materials and methods.-Fifty-eight normal healthy adults were included in the study. Their submental muscle activity, respiratory activity, heart rate changes, and sympathetic skin responses were recorded during 50 ml water swallowing. In addition, we requested subjects to imagine drinking water as they did just before. The same recordings were performed during this imagination period. Results.-SSR appeared at the beginning and at the end of SWS in 52% of subjects. A first sympathetic skin response was evoked at the onset of SWS, and a second one appeared 8.6 +/- 1.7 seconds after the first one and at the end of swallowing. Similar double SSRs were also obtained during imagination in most investigated subjects (33 out of 35 of selected subjects in a total group of 58 subjects). Swallowing tachycardia was observed during the SWS-associated apnea period, but not during the imagination period. Heart rate significantly increased during the SWS-associated apnea period. Conclusion.-The first SSR that appeared at the onset of swallowing is likely related to arousal. The appearance of a second response is a novel finding, which is probably related to the activity of subtil corticosubcortical networks. While discrete/single swallows can be used to evoke SSRs, SWS is unlikely to be clinically useful in its current form. In contrast, swallowing tachycardia could be a useful tool to examine dysphagic patients. (C) 2012 Elsevier Masson SAS. All rights reserved

A database for screening and registering late onset Pompe disease in Turkey

The aim of this study was to search for the frequency of late onset Pompe disease (LOPD) among patients who had a myopathy with unknown diagnosis registered in the pre-diagnostic part of a novel registry for LOPD within a collaborative study of neurologists working throughout Turkey. Included in the study were 350 patients older than 18 years who have a myopathic syndrome without a proven diagnosis by serum creatine kinase (CK) levels, electrodiagnostic studies, and/or muscle pathology, and/or genetic tests for myopathies other than LOPD. Acid alpha glucosidase (GAA) in dried blood spot was measured in each patient at two different university laboratories. LOPD was confirmed by mutation analysis in patients with decreased GAA levels from either both or one of the laboratories. Pre-diagnostic data, recorded by 45 investigators from 32 centers on 350 patients revealed low GAA levels in a total of 21 patients; from both laboratories in 6 and from either one of the laboratories in 15. Among them, genetic testing proved LOPD in 3 of 6 patients and 1 of 15 patients with decreased GAA levels from both or one of the laboratories respectively. Registry was transferred to Turkish Neurological Association after completion of the study for possible future use and development. Our collaborative study enabled collection of a considerable amount of data on the registry in a short time. GAA levels by dried blood spot even from two different laboratories in the same patient may not prove LOPD. LOPD seemed to be rarer in Turkey than in Europe. © 2017 Elsevier B.V

A database for screening and registering late onset Pompe disease in Turkey

The aim of this study was to search for the frequency of late onset Pompe disease (LOPD) among patients who had a myopathy with unknown diagnosis registered in the pre-diagnostic part of a novel registry for LOPD within a collaborative study of neurologists working throughout Turkey. Included in the study were 350 patients older than 18 years who have a myopathic syndrome without a proven diagnosis by serum creatine kinase (CK) levels, electrodiagnostic studies, and/or muscle pathology, and/or genetic tests for myopathies other than LOPD. Acid alpha glucosidase (GAA) in dried blood spot was measured in each patient at two different university laboratories. LOPD was confirmed by mutation analysis in patients with decreased GAA levels from either both or one of the laboratories. Pre-diagnostic data, recorded by 45 investigators from 32 centers on 350 patients revealed low GAA levels in a total of 21 patients; from both laboratories in 6 and from either one of the laboratories in 15. Among them, genetic testing proved LOPD in 3 of 6 patients and 1 of 15 patients with decreased GAA levels from both or one of the laboratories respectively. Registry was transferred to Turkish Neurological Association after completion of the study for possible future use and development. Our collaborative study enabled collection of a considerable amount of data on the registry in a short time. GAA levels by dried blood spot even from two different laboratories in the same patient may not prove LOPD. LOPD seemed to be rarer in Turkey than in Europe. © 2017 Elsevier B.V