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P443: Swallowing difficulty in oculopharyngodistal myopathy: electrophysiological evaluation
Abstracts of Poster Presentations / Clinical Neurophysiology 125, Supplement 1 (2014) S1–S339
S167
P442 The value of SSR recordings in the assessment of autonomic nervous dysfunction in patients with sacral-pudendal impairment
The aim of this study was to assess swallowing difficulty in patients with oculopharyngodistal myopathy via electrophysiological measurements to detect the primary site causing swallowing and/or respiratory difficulty, namely pharyngeal or laryngeal involvements. Methods: Patients diagnosed as having oculopharyngodistal myopathy in the department of neurology of Istanbul Faculty of Medicine underwent electrophysiological examination. For the clinical evaluation of swallowing, a previously described grading system is used. Electrophysiological measurements consisted piecemeal deglutition and dysphagia limit using 3, 5, 10, 15, and 20 ml bouts of water and sequential water swallowing of 100-ml water in 15 patients, swallowing assessment via needle electrodes placed on thyroarytenoid and cricopharyngeal sphincter muscles in 10 patients, percutaneous needle EMG evaluation on thyroarytenoid (TA) and cricopharyngeal (CP) sphincter muscles in 6 patients. Otolaryngologic examination evaluating the alignment and function of vocal cords, voice, articulation, and swallowing excluded non-neurologic swallowing and respiratory difficulties. Results: Dysphagia limit was abnormal in 12 patients with grade 2-3 dysphagia but normal in 3 patients with grade 1 dysphagia. Also sequential water swallowing was normal in 7 patients including all patients with grade 1 dysphagia and abnormal in 8. The recording of swallowing on cricopharyngeal sphincter and thyroarytenoid muscles muscles revealed normal swallowing patterns in all patients. Needle EMG of TA and CP sphincter muscles revealed myopathic pattern in thyroarytenoid muscles in 4 of them. Conclusion: Swallowing difficulty develop in a purely myopathic patern involving first laryngeal muscles in patients with oculopharyngodistal myopathy.
G. de Scisciolo, R. Caramelli, F. Del Corso, V. Schiavone, M.E. Bastianelli, C. Martinelli, E. Provvedi, A. Cassardo AOU Careggi, Spinal Unit, Florence, Italy
Poster session 26. Epilepsy 2
Methods: By screening Medline, existing knowledge and research about LEMG and laryngeal electrostimulation was identified. Additionally evidence-based recommendations for the performance and interpretation of LEMG and also for electrostimulation for functional evaluation were considered, as well as published reports based on expert opinion and single-institution retrospective case series. The working group discussed knowledge about LEMG and laryngeal electrostimulation and performed the techniques together. Consensus was achieved on: minimum technical equipment; best practical implementation; criteria for interpreting [1]. To disseminate the techniques, several modalities where used: Presentations using slides and videos, publications in scientific journals, handson-training on patients, animal models, and anatomic specimens, and a web-blog [2]. Results: Co-registration of the laryngeal EMG, voice, and breathing multiplies the scientific and didactic value of the LEMG-recordings. The advantages and disadvantages of the different teaching-modalities will be presented and discussed during this contribution. Conclusion: Successful learning LEMG benefits from multi-sensory teaching concept. Literature: [1] Volk GF, et al., Laryngeal electromyography: a proposal for guidelines of the European Laryngological Society. Eur Arch Otorhinolaryngol 2012 Oct;269(10):2227-45. [2] www.lemg.org
Question: Neural control of pelvic structures reveals a complex interaction between central and peripheral nervous pathways and a coordination of somatic and autonomic systems. While it is well know the usefulness of neurophysiological tests to investigate somatic nervous system of pelvic floor (needle electromyography, nerve conduction studies, evoked potentials, sacral reflexes), it is not yet recognized the real efficacy of sympathetic skin response (SSR) in patients with sacral-pudendal dysfunction. Methods: In the last six years we examined 1745 patients with different pelvic disorders (urinary, anorectal and sexual pathologies), age range 7-85 years. The SSR was recorded from the skin of the sacral area (dorsum of the penis or mons pubis, perineal area, perianal area, in function of clinic), applying random single square pulses at the wrist or supraorbital region. Also SSR and EMG -ENG of legs were performed in all patients. Latency and amplitude (peak to peak) of SSR were measured and the results were compared by mean age with those of control group. Results: We observed alterations in latency and amplitude in various diseases: spinal cord injury, sacral plexus pathology, sexual dysfunctions, etc. In some cases SSR changes were the only present abnormality (frequently in case of sexual dysfunction and syringomyelia). We have also observed it is important to differentiate anterior from posterior perineal area in SSR recording, mostly in pelvic disorders (traumatic or not). Conclusion: SSR test may be an useful tool to investigate autonomic sacral involvement, because until now it the only test to explore autonomic system, even if it explore only sympathetic system. In our experience SSR is always present, in absence of pathology, so it could become a standard investigation for clinical routine of patients with sacral disorders.
P443 Swallowing difficulty in oculopharyngodistal myopathy: electrophysiological evaluation M. Gokyigt (Celik) 1 , C. Ertekin 2 , I. Ercan 3 , N. Kuloglu Pazarci 1 , S. Seker 3 , H. Durmus 4 , P. Oflazer 4 1 Sisli Etfal Education and Research Hospital, Neurology, Istanbul, Turkey; 2 Ege University Medical Faculty, Neurology, Izmir, Turkey; 3 Sisli Etfal Education and Research Hospital, ENT, Istanbul, Turkey; 4 Medical Faculty of Istanbul, Neurology, Istanbul, Turkey Introduction: Patients with oculopharyngodistal myopathy (OPDM) present with progressive ocular, pharyngeal, and distal limb muscle involvement. Respiratory and swallowing difficulties occur at or soon after presentation.
P444 Anatomofunctional mapping of the opercular cortex by intra-cerebral electrical stimulations in epileptic patients explored by means of stereoelectroencephalography (SEEG) D.M. Maliia 1 , A. Barborica 2,3 , C. Donos 2 , J. Ciurea 4 , B. Balanescu 2,5 , A. Rasina 4 , I. Mindruta 6,1 1 University Emergency Hospital, Neurology-Sleep and Epilepsy Laboratory, Bucharest, Romania; 2 University of Bucharest, Physics, Bucharest, Romania; 3 FHC Inc, Bowdoin ME, Romania; 4 Bagdasar Arseni Hospital, Neurosurgery, Bucharest, Romania; 5 Fundeni Clinical Hospital, Cardiovascular surgery, Bucharest, Romania; 6 Carol Davila University of Medicine and Pharmacy, Neurology, Bucharest, Romania Background: Operculum is the part of cerebral cortex that covers the insula (Mazzola et al 2012). Although the literature addresses its functions in numerous studies, the vast majority of them consist of inferences made on activation patterns revealed by functional imaging. Few studies have been published using a direct electrical stimulation paradigm, and the ones that have done so, studied subjects with a pathological process involving this area. Objectives: Our aim is to perform a systematic mapping of this highly relevant cortical structure with different stimulation protocols. Methods: We elicited clinical responses at various stimulation parameters (mainly 1 Hz for 40 s, 50 Hz for 5s) in a lot comprised of 11 consecutive patients explored for drug resistant focal epilepsy during presurgical work up. Using the SEEG method, a number of depth electrodes ranging from 7-17 were implanted,to map the seizure onset zones (SOZ), propagation pathways and functional cortex that should be avoided during surgery. All the patients had SOZ located outside the opercular cortex and this areas had no particular pathology. Effects obtained on contacts touching the insular cortex, laying in the pericortical white matter, or producing any afterdischarges were discarded (Afif Afif & al 2010). The others were projected on a Maximum Intensity Projection (MIP) map with their relative position derived from the 3 D axial coordinates generated by the neuronavigation software. Results: We have applied about 350 stimulation trains, on 90 bipolar contact pairs situated on 30 different electrodes implanted orthogonally in the frontal, rolandic, parietal and temporal operculum. 18% of the 1 Hz and 50% of the 50 Hz stimulations produced a discernable clinical effect classified
S167
P442 The value of SSR recordings in the assessment of autonomic nervous dysfunction in patients with sacral-pudendal impairment
The aim of this study was to assess swallowing difficulty in patients with oculopharyngodistal myopathy via electrophysiological measurements to detect the primary site causing swallowing and/or respiratory difficulty, namely pharyngeal or laryngeal involvements. Methods: Patients diagnosed as having oculopharyngodistal myopathy in the department of neurology of Istanbul Faculty of Medicine underwent electrophysiological examination. For the clinical evaluation of swallowing, a previously described grading system is used. Electrophysiological measurements consisted piecemeal deglutition and dysphagia limit using 3, 5, 10, 15, and 20 ml bouts of water and sequential water swallowing of 100-ml water in 15 patients, swallowing assessment via needle electrodes placed on thyroarytenoid and cricopharyngeal sphincter muscles in 10 patients, percutaneous needle EMG evaluation on thyroarytenoid (TA) and cricopharyngeal (CP) sphincter muscles in 6 patients. Otolaryngologic examination evaluating the alignment and function of vocal cords, voice, articulation, and swallowing excluded non-neurologic swallowing and respiratory difficulties. Results: Dysphagia limit was abnormal in 12 patients with grade 2-3 dysphagia but normal in 3 patients with grade 1 dysphagia. Also sequential water swallowing was normal in 7 patients including all patients with grade 1 dysphagia and abnormal in 8. The recording of swallowing on cricopharyngeal sphincter and thyroarytenoid muscles muscles revealed normal swallowing patterns in all patients. Needle EMG of TA and CP sphincter muscles revealed myopathic pattern in thyroarytenoid muscles in 4 of them. Conclusion: Swallowing difficulty develop in a purely myopathic patern involving first laryngeal muscles in patients with oculopharyngodistal myopathy.
G. de Scisciolo, R. Caramelli, F. Del Corso, V. Schiavone, M.E. Bastianelli, C. Martinelli, E. Provvedi, A. Cassardo AOU Careggi, Spinal Unit, Florence, Italy
Poster session 26. Epilepsy 2
Methods: By screening Medline, existing knowledge and research about LEMG and laryngeal electrostimulation was identified. Additionally evidence-based recommendations for the performance and interpretation of LEMG and also for electrostimulation for functional evaluation were considered, as well as published reports based on expert opinion and single-institution retrospective case series. The working group discussed knowledge about LEMG and laryngeal electrostimulation and performed the techniques together. Consensus was achieved on: minimum technical equipment; best practical implementation; criteria for interpreting [1]. To disseminate the techniques, several modalities where used: Presentations using slides and videos, publications in scientific journals, handson-training on patients, animal models, and anatomic specimens, and a web-blog [2]. Results: Co-registration of the laryngeal EMG, voice, and breathing multiplies the scientific and didactic value of the LEMG-recordings. The advantages and disadvantages of the different teaching-modalities will be presented and discussed during this contribution. Conclusion: Successful learning LEMG benefits from multi-sensory teaching concept. Literature: [1] Volk GF, et al., Laryngeal electromyography: a proposal for guidelines of the European Laryngological Society. Eur Arch Otorhinolaryngol 2012 Oct;269(10):2227-45. [2] www.lemg.org
Question: Neural control of pelvic structures reveals a complex interaction between central and peripheral nervous pathways and a coordination of somatic and autonomic systems. While it is well know the usefulness of neurophysiological tests to investigate somatic nervous system of pelvic floor (needle electromyography, nerve conduction studies, evoked potentials, sacral reflexes), it is not yet recognized the real efficacy of sympathetic skin response (SSR) in patients with sacral-pudendal dysfunction. Methods: In the last six years we examined 1745 patients with different pelvic disorders (urinary, anorectal and sexual pathologies), age range 7-85 years. The SSR was recorded from the skin of the sacral area (dorsum of the penis or mons pubis, perineal area, perianal area, in function of clinic), applying random single square pulses at the wrist or supraorbital region. Also SSR and EMG -ENG of legs were performed in all patients. Latency and amplitude (peak to peak) of SSR were measured and the results were compared by mean age with those of control group. Results: We observed alterations in latency and amplitude in various diseases: spinal cord injury, sacral plexus pathology, sexual dysfunctions, etc. In some cases SSR changes were the only present abnormality (frequently in case of sexual dysfunction and syringomyelia). We have also observed it is important to differentiate anterior from posterior perineal area in SSR recording, mostly in pelvic disorders (traumatic or not). Conclusion: SSR test may be an useful tool to investigate autonomic sacral involvement, because until now it the only test to explore autonomic system, even if it explore only sympathetic system. In our experience SSR is always present, in absence of pathology, so it could become a standard investigation for clinical routine of patients with sacral disorders.
P443 Swallowing difficulty in oculopharyngodistal myopathy: electrophysiological evaluation M. Gokyigt (Celik) 1 , C. Ertekin 2 , I. Ercan 3 , N. Kuloglu Pazarci 1 , S. Seker 3 , H. Durmus 4 , P. Oflazer 4 1 Sisli Etfal Education and Research Hospital, Neurology, Istanbul, Turkey; 2 Ege University Medical Faculty, Neurology, Izmir, Turkey; 3 Sisli Etfal Education and Research Hospital, ENT, Istanbul, Turkey; 4 Medical Faculty of Istanbul, Neurology, Istanbul, Turkey Introduction: Patients with oculopharyngodistal myopathy (OPDM) present with progressive ocular, pharyngeal, and distal limb muscle involvement. Respiratory and swallowing difficulties occur at or soon after presentation.
P444 Anatomofunctional mapping of the opercular cortex by intra-cerebral electrical stimulations in epileptic patients explored by means of stereoelectroencephalography (SEEG) D.M. Maliia 1 , A. Barborica 2,3 , C. Donos 2 , J. Ciurea 4 , B. Balanescu 2,5 , A. Rasina 4 , I. Mindruta 6,1 1 University Emergency Hospital, Neurology-Sleep and Epilepsy Laboratory, Bucharest, Romania; 2 University of Bucharest, Physics, Bucharest, Romania; 3 FHC Inc, Bowdoin ME, Romania; 4 Bagdasar Arseni Hospital, Neurosurgery, Bucharest, Romania; 5 Fundeni Clinical Hospital, Cardiovascular surgery, Bucharest, Romania; 6 Carol Davila University of Medicine and Pharmacy, Neurology, Bucharest, Romania Background: Operculum is the part of cerebral cortex that covers the insula (Mazzola et al 2012). Although the literature addresses its functions in numerous studies, the vast majority of them consist of inferences made on activation patterns revealed by functional imaging. Few studies have been published using a direct electrical stimulation paradigm, and the ones that have done so, studied subjects with a pathological process involving this area. Objectives: Our aim is to perform a systematic mapping of this highly relevant cortical structure with different stimulation protocols. Methods: We elicited clinical responses at various stimulation parameters (mainly 1 Hz for 40 s, 50 Hz for 5s) in a lot comprised of 11 consecutive patients explored for drug resistant focal epilepsy during presurgical work up. Using the SEEG method, a number of depth electrodes ranging from 7-17 were implanted,to map the seizure onset zones (SOZ), propagation pathways and functional cortex that should be avoided during surgery. All the patients had SOZ located outside the opercular cortex and this areas had no particular pathology. Effects obtained on contacts touching the insular cortex, laying in the pericortical white matter, or producing any afterdischarges were discarded (Afif Afif & al 2010). The others were projected on a Maximum Intensity Projection (MIP) map with their relative position derived from the 3 D axial coordinates generated by the neuronavigation software. Results: We have applied about 350 stimulation trains, on 90 bipolar contact pairs situated on 30 different electrodes implanted orthogonally in the frontal, rolandic, parietal and temporal operculum. 18% of the 1 Hz and 50% of the 50 Hz stimulations produced a discernable clinical effect classified