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S21.4 Sleep studies in facioscapulohumeral muscular distrophy
14th ECCN / 4th ICTMS/DCS Our objective is to evaluate the efficacy of Rivastigmine treatment in PD patients with RBD in whom traditional treatment failed. Methods: We enrolled 12 PD (11 males) patients (middle: 64.5 years) with RBD confirmed by polisomnography; 4 had severe RBD (>5 episodes/ week) 8 mild RBD. A double blind, cross-over design was applied. Each patient received Exelon® 4.5 mg/day or placebo for three weeks, then shifted to the alternative treatment. Patients and their bed partners filled in a diary of RBD episodes as long as the study. Polisomnography was performed after each treatment in three patients. Patients underwent neuropsychological assessment before starting the treatment. Results: 2 patients dropped-out of the study for hypotension. RBD episodes frequency was reduced over the 50% in all patients with severe RBD and in three patient with mild RBD who referred refreshing sleep. In the last three the RBD frequency remained unchanged. The efficacy of rivastigmine was not related to the cognitive impairment of patients. Conclusions: Rivastigmine seems an effective secondary option for RBD treatment in a cohort of patients refractory to conventional therapies.
S49 hypothalamus with the objective to treat impulsivity and aggressive behavior refractory to politherapy. Results: Stimulation with pulse amplitude of 0.5 1 V (bilateral bipolar montage), frequency of 180 Hz and a pulse width of 90 ms, led to the emergence of hypersomnolence, hyperphagia and hypersexuality (masturbatory behavior and pornography vision) during nine days. The amplitude reduction to 0.8 V wiped out sintomatology but worsened the control of aggressive behavior and impulsivity. Currently, the patient has deep brain stimulation with pulse width of 2.1 right and 2.0 left. There is a good control of psychiatric condition, with reduced medication and no recurrence of the symptoms described above. Conclusions: The triad of hypersomnia, hyperphagia and hypersexuality is characteristic of Kleine Levin syndrome. This is usually presented in an episodic-recurrent course. The etiology and pathogenesis are unknown but it is postulated a hypothalamic origin. The presentation of this triad in our patient after deep brain stimulation to a specific range in posteroventral hypothalamus provides a new evidence on the origin of this syndrome.
S21.4 Sleep studies in facioscapulohumeral muscular distrophy R. Frusciante1 , S. Dittoni2 , F. Madia1 , B. Merico1 , C. Vollono3 , A. Losurdo2 , E. Testani2 , E. Scarano4 , S. Colicchio2 , G. Rizzotto1 , G. Mennuni1 , P.A. Tonali5 , E. Ricci2 , G. Della Marca2 1 Neurophysiopathology, Complesso Integrato Columbus, Rome, Italy, 2 Dept. of Neurosciences, Catholic University, Rome, Italy, 3 Ospedale Pediatrico “Bambino Ges` u” IRCCS, Rome, Italy, 4 Institute of Otorhinolaryngology, Catholic University, Rome, Italy, 5 Fondazione Don Carlo Gnocchi, Rome, Italy Introduction: Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant disease with an estimated prevalence of 1:20000, corresponding to the third most frequent form of Muscular Dystrophy. Muscular Dystrophies are associated with a high prevalence of sleep disorders. Objectives: The aim of the present study was to evaluate sleep in a group of FSHD patients. Methods: We enrolled 51 consecutive adult patients affected by FSHD, 23 women and 28 men, with mean age of 45.7±13.0 years (range: 26 72). Patients underwent: (1) a subjective evaluation of sleep (including Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index, Berlin questionnaire); (2) a full-night, laboratory based polysomnography (for the evaluation of the sleep structure, the respiratory pattern and the nocturnal motor activity) and (3) an anatomical evaluation of upper airways (clinical evaluation, cephalometry and sleep endoscopy). Results: FSHD group showed: (1) a poor perceived sleep quality; (2) high prevalence of sleep disordered breathing (SDB) (20% of the sample); (3) reduced nocturnal motility; (4) normal anatomy of the upper airways; (5) increased collapsibility of upper airway, at the oro-pharyngeal, hypopharyngeal and laryngeal level. Conclusions: FSHD patients have a poor sleep quality. This is consequent to a high prevalence of sleep related respiratory disorders, and reduced motility during sleep. No clinical finding can predict the occurrence of SDB in FSHD patients. Sleep evaluation should be included in the routinary clinical evaluation of FSHD patients. S21.5 Evidence for the hypothalamic origin of Kleine Levin syndrome: hypersomnia, hypersexuality and hyperphagia after psychosurgery with hypothalamic deep brain stimulation erez-Díaz1 , J.J. Rodríguez Uranga2 , O. Prian M. Aguilar-Andujar1 , H. P´ alez3 , R.F. Camacho2 , Serrano2 , G.M. Castro3 , M.A. Rodríguez Gonz´ nal2 J.A. Narros Gago2 , J.M. Montero Elena2 , F. Trujillo Madro˜ 1 Multidisciplinary Unit of Wakefulness and Sleep Disorders. Institute of Neurological Specialties (IENs). USP Clinic (Sevilla), Seville, Spain, 2 Unit of Functional Neurosurgery. Institute of Neurological Specialties (IENs). USP Clinic (Sevilla), Seville, Spain, 3 Multidisciplinary Unit of Wakefulness and Sleep Disorders. Institute of Neurological Specialties (IENs). USP Clinic (Sevilla), Seville, Spain Objectives: To provide evidences about the probable hypothalamic origin of Kleine Levin syndrome. Material and Methods: 37 years patient with a personal history of rubella encephalopathy with severe psychomotor retardation. The patient is subjected to deep brain stimulation, with target in ventral posterolateral
Workshop 15. tDCS and rTMS W15.1 TMS and tDCS F. Hummel1 , L.G. Cohen2 Department of Neurology, University Medical Center HamburgEppendorf, Hamburg, Germany, 2 Human Cortical Physiology and Stroke Neurorehabilitation Section, NINDS, NIH, Bethesda, MD 20892, USA
1
Introduction: Non-invasive brain stimulation through transcranial direct current stimulation (tDCS) influences cognitive functions in healthy volunteers and may facilitate motor performance and learning in patients with chronic stroke. tDCS can be applied continuously and safely, close to the typical duration of a session of rehabilitative treatment, and can be administered in synchrony with motor training protocols. This abstract discusses the evaluation of perceived sensations, discomfort, ratings of attention and fatigue, and the ability to retrospectively identify sham and tDCS interventions. Additionally, it discusses advantages and disadvantages of the use of each TMS and tDCS in relation to particular experimental protocols. Objectives: To evaluate the ability of investigators and healthy subjects to identify tDCS and SHAM sessions applied over the primary motor cortex. To determine the quality and effectiveness of this technique for double-blind sham-controlled experimental designs. Attention, fatigue, and discomfort were self rated by study participants using visual analog scales. Duration of perceived sensations and the ability to distinguish tDCS from Sham sessions were determined. Investigators questioning the patients were blind to the intervention type. Methods: Data was pooled from several studies performed over a 3 year period and 170 sessions at the Human Cortical Physiology Section, NINDS. Results: tDCS and SHAM elicited comparably minimal discomfort and duration of sensations in the absence of differences in attention or fatigue, and overall could not be distinguished from SHAM by study participants nor investigators. Conclusions: Successful blinding of subjects and investigators and ease of application simultaneously with training protocols supports the use of tDCS in double-blind, sham-controlled randomized trials. tDCS is evolving into a useful tool, in addition to TMS, to modulate cortical activity in systems neuroscience. W15.2 Effects of bilateral simultaneous transcranial direct current stimulation (tDCS) of human motor cortex an Pascual2 , A. Oliviero1 L. Mordillo Mateos1 , L. Turpin Fenoll2 , J. Mill´ Grupo FENNSI, Hospital Nacional de Parapl´ ejicos de Toledo, Toledo, azar de Spain, 2 Servicio de Neurología, Hospital La Mancha Centro, Alc´ San Juan, Spain
1
Introduction: Transcranial direct current stimulation (tDCS), is a non invasive technique that has been investigated for different neurological disorders. Neuronal excitability can be modified by application of direct current (DC) in a polarity-specific manner: anodal tDCS increases excitability whereas cathodal tDCS decreases it. Research has shown
S49 hypothalamus with the objective to treat impulsivity and aggressive behavior refractory to politherapy. Results: Stimulation with pulse amplitude of 0.5 1 V (bilateral bipolar montage), frequency of 180 Hz and a pulse width of 90 ms, led to the emergence of hypersomnolence, hyperphagia and hypersexuality (masturbatory behavior and pornography vision) during nine days. The amplitude reduction to 0.8 V wiped out sintomatology but worsened the control of aggressive behavior and impulsivity. Currently, the patient has deep brain stimulation with pulse width of 2.1 right and 2.0 left. There is a good control of psychiatric condition, with reduced medication and no recurrence of the symptoms described above. Conclusions: The triad of hypersomnia, hyperphagia and hypersexuality is characteristic of Kleine Levin syndrome. This is usually presented in an episodic-recurrent course. The etiology and pathogenesis are unknown but it is postulated a hypothalamic origin. The presentation of this triad in our patient after deep brain stimulation to a specific range in posteroventral hypothalamus provides a new evidence on the origin of this syndrome.
S21.4 Sleep studies in facioscapulohumeral muscular distrophy R. Frusciante1 , S. Dittoni2 , F. Madia1 , B. Merico1 , C. Vollono3 , A. Losurdo2 , E. Testani2 , E. Scarano4 , S. Colicchio2 , G. Rizzotto1 , G. Mennuni1 , P.A. Tonali5 , E. Ricci2 , G. Della Marca2 1 Neurophysiopathology, Complesso Integrato Columbus, Rome, Italy, 2 Dept. of Neurosciences, Catholic University, Rome, Italy, 3 Ospedale Pediatrico “Bambino Ges` u” IRCCS, Rome, Italy, 4 Institute of Otorhinolaryngology, Catholic University, Rome, Italy, 5 Fondazione Don Carlo Gnocchi, Rome, Italy Introduction: Facioscapulohumeral muscular dystrophy (FSHD) is an autosomal dominant disease with an estimated prevalence of 1:20000, corresponding to the third most frequent form of Muscular Dystrophy. Muscular Dystrophies are associated with a high prevalence of sleep disorders. Objectives: The aim of the present study was to evaluate sleep in a group of FSHD patients. Methods: We enrolled 51 consecutive adult patients affected by FSHD, 23 women and 28 men, with mean age of 45.7±13.0 years (range: 26 72). Patients underwent: (1) a subjective evaluation of sleep (including Epworth Sleepiness Scale, Pittsburgh Sleep Quality Index, Berlin questionnaire); (2) a full-night, laboratory based polysomnography (for the evaluation of the sleep structure, the respiratory pattern and the nocturnal motor activity) and (3) an anatomical evaluation of upper airways (clinical evaluation, cephalometry and sleep endoscopy). Results: FSHD group showed: (1) a poor perceived sleep quality; (2) high prevalence of sleep disordered breathing (SDB) (20% of the sample); (3) reduced nocturnal motility; (4) normal anatomy of the upper airways; (5) increased collapsibility of upper airway, at the oro-pharyngeal, hypopharyngeal and laryngeal level. Conclusions: FSHD patients have a poor sleep quality. This is consequent to a high prevalence of sleep related respiratory disorders, and reduced motility during sleep. No clinical finding can predict the occurrence of SDB in FSHD patients. Sleep evaluation should be included in the routinary clinical evaluation of FSHD patients. S21.5 Evidence for the hypothalamic origin of Kleine Levin syndrome: hypersomnia, hypersexuality and hyperphagia after psychosurgery with hypothalamic deep brain stimulation erez-Díaz1 , J.J. Rodríguez Uranga2 , O. Prian M. Aguilar-Andujar1 , H. P´ alez3 , R.F. Camacho2 , Serrano2 , G.M. Castro3 , M.A. Rodríguez Gonz´ nal2 J.A. Narros Gago2 , J.M. Montero Elena2 , F. Trujillo Madro˜ 1 Multidisciplinary Unit of Wakefulness and Sleep Disorders. Institute of Neurological Specialties (IENs). USP Clinic (Sevilla), Seville, Spain, 2 Unit of Functional Neurosurgery. Institute of Neurological Specialties (IENs). USP Clinic (Sevilla), Seville, Spain, 3 Multidisciplinary Unit of Wakefulness and Sleep Disorders. Institute of Neurological Specialties (IENs). USP Clinic (Sevilla), Seville, Spain Objectives: To provide evidences about the probable hypothalamic origin of Kleine Levin syndrome. Material and Methods: 37 years patient with a personal history of rubella encephalopathy with severe psychomotor retardation. The patient is subjected to deep brain stimulation, with target in ventral posterolateral
Workshop 15. tDCS and rTMS W15.1 TMS and tDCS F. Hummel1 , L.G. Cohen2 Department of Neurology, University Medical Center HamburgEppendorf, Hamburg, Germany, 2 Human Cortical Physiology and Stroke Neurorehabilitation Section, NINDS, NIH, Bethesda, MD 20892, USA
1
Introduction: Non-invasive brain stimulation through transcranial direct current stimulation (tDCS) influences cognitive functions in healthy volunteers and may facilitate motor performance and learning in patients with chronic stroke. tDCS can be applied continuously and safely, close to the typical duration of a session of rehabilitative treatment, and can be administered in synchrony with motor training protocols. This abstract discusses the evaluation of perceived sensations, discomfort, ratings of attention and fatigue, and the ability to retrospectively identify sham and tDCS interventions. Additionally, it discusses advantages and disadvantages of the use of each TMS and tDCS in relation to particular experimental protocols. Objectives: To evaluate the ability of investigators and healthy subjects to identify tDCS and SHAM sessions applied over the primary motor cortex. To determine the quality and effectiveness of this technique for double-blind sham-controlled experimental designs. Attention, fatigue, and discomfort were self rated by study participants using visual analog scales. Duration of perceived sensations and the ability to distinguish tDCS from Sham sessions were determined. Investigators questioning the patients were blind to the intervention type. Methods: Data was pooled from several studies performed over a 3 year period and 170 sessions at the Human Cortical Physiology Section, NINDS. Results: tDCS and SHAM elicited comparably minimal discomfort and duration of sensations in the absence of differences in attention or fatigue, and overall could not be distinguished from SHAM by study participants nor investigators. Conclusions: Successful blinding of subjects and investigators and ease of application simultaneously with training protocols supports the use of tDCS in double-blind, sham-controlled randomized trials. tDCS is evolving into a useful tool, in addition to TMS, to modulate cortical activity in systems neuroscience. W15.2 Effects of bilateral simultaneous transcranial direct current stimulation (tDCS) of human motor cortex an Pascual2 , A. Oliviero1 L. Mordillo Mateos1 , L. Turpin Fenoll2 , J. Mill´ Grupo FENNSI, Hospital Nacional de Parapl´ ejicos de Toledo, Toledo, azar de Spain, 2 Servicio de Neurología, Hospital La Mancha Centro, Alc´ San Juan, Spain
1
Introduction: Transcranial direct current stimulation (tDCS), is a non invasive technique that has been investigated for different neurological disorders. Neuronal excitability can be modified by application of direct current (DC) in a polarity-specific manner: anodal tDCS increases excitability whereas cathodal tDCS decreases it. Research has shown