Perioral Muscles Research Articles

Clinical and x-ray oral evaluation in patients with congenital Zika Virus.

Objective:The aim of this study was to investigate possible malformations in the soft, bone and/or dental tissues in patients with congenital Zika Virus (ZIKV) by clinical and x-ray evaluation.Methodology:Thirty children born with ZIKV and 30 children born without ZIKV (control group) were included in the study. Patients were evaluated over 24 consecutive months according to the variables: sex, age, cleft palates, soft tissue lesions, alveolar ridge hyperplasia, short labial and lingual frenums, inadequate posture of the lingual and perioral muscles at rest, micrognathia, narrow palatine vaults, changes in the teeth shape and/or number, sequence eruption, spasms, seizures and eruption delay were evaluated. Chi-square test, Student's t-test and nominal logistic regression were used (p<0.05).Results:Among the 30 babies examined, the mean age of the first dental eruption was 10.8±3.8 with almost two-thirds of the children (n=18, 60%) experiencing eruptions of their first tooth after 9 months of age, nine children (30%) had inadequate lingual posture at rest, more than half of the children (n=18, 60%) had short labial or lingual frenums. ZIKV babies showed a high prevalence of clef palate (p<0.001), inadequate lingual posture at rest (p=0.004), micrognathia (p=0.002), changes in the shape and/or number of teeth (p=0.006), alteration in sequence of dental eruption (p<0.001) and muscles spasms (p=0.002). The delay eruption was associated with inadequate lingual posture at rest (p=0.047), micrognathia (p=0.002) and changes in the shape and/or number of teeth (p=0.021). The delayed eruption (p=0.006) and narrow palatine vaults (p=0.008) were independently associated with ZIKV. Moreover, female patients showed the most narrow palatine vaults (p=0.010).Conclusions:The children with ZIKV showed a greater tendency to have delayed eruption of the first deciduous tooth, inadequate lingual posture and short labial and lingual frenums.

A case report of oral rehabilitation after partial glossectomy for tongue cancer using removable prosthesis matching with perioral muscles

A case report of oral rehabilitation after partial glossectomy for tongue cancer using removable prosthesis matching with perioral muscles

Effects of gum chewing training on oral function in normal adults: Part 1 investigation of perioral muscle pressure

Background/purposeThe strength of the intraoral and extraoral muscles that assist the function of tooth and jaw movement during mastication is important for performing oral function. The aim of this study was to investigate the usefulness of gum chewing training to improve the swallowing and feeding function. Materials and methodsIn experiment 1, the differences in maximum tongue pressure (TP) and cheek pressure (CP) at the measurement time point for both groups with and without training were examined. We instructed subjects to perform gum chewing training 3 times daily for 3 months. TP and CP were measured before training and at 1, 2, and 3 months after starting training. In experiment 2, the changes of TP and CP based on the sex and duration of training were examined. The effect of the training was evaluated before training, at 2 weeks and 1, 2, and 3 months after starting training, and at 1 and 3 months after cessation of training. ResultsExperiment 1 showed TP and CP increased with the progress of continuous training. In experiment 2, TP and CP were higher in men than in women and markedly increased at 2 weeks and 1 month in both sexes. After cessation of training, TP and CP tended to decrease, but there was no significant difference between 3 months after starting training, and also significantly higher than before training. ConclusionThis study suggested that gum chewing training is a useful to improve the swallowing and feeding function.

The effect of facial expression on facial symmetry in surgically managed unilateral cleft lip and palate patients (UCLP)

To evaluate the symmetry of facial expression in surgically managed UCLP patients. The study was conducted on 13 four-year-old children. Facial images were captured at rest and at maximum smile using stereophotogrammetry. A generic mesh, which is a mathematical facial mask consisting of a fixed number of indexed vertices, was utilised for the assessment of facial asymmetry. This was quantified by measuring the disparity between the left- and right-hand sides of the face after superimposing the original 3D images on their mirror copies. Residual asymmetries at rest were identified at the vermillion of the upper lip and at the nares with a deviation of the philtrum towards the scar tissue. Vertical and anteroposterior asymmetries were identified on the cleft side. At maximum smile, the asymmetry increased noticeably at the vermillion of the upper lip and at the alar base. In the mediolateral direction, the philtrum deviated towards the cleft side with a significant increase of the asymmetry scores. Asymmetry of the upper lip has significantly increased at maximum smile as a result of the upward forces of all perioral lifting muscles, which affected the lip directly. The innovation of this study is the measurement of facial asymmetry for the objective outcome measure of the surgical repair of UCLP. The philtrum was the main site of residual asymmetry, which indicates the need for refining the primary repair of the cleft lip. Further corrective surgery may be required.

Morphological and functional peri-oral changes during early treatment of Class II division 1 malocclusions using EF Line ® functional education devices

The aim of this article was to assess the impact of a flexible functional education splint for interceptive treatment of Class II division 1 dysmorphoses. An observational, prospective, multi-center non-comparative EGIDYS study was conducted in 81 children with a Class II division 1 malocclusion presenting one or several functional disorders. Patients wore a flexible splint in the EF Line® range for a minimum of two hours per day and all night over 12 months in combination with educational exercises depending on the case. Patients were followed at one month, four months and twelve months of treatment. Ventilation, lingual position, labial peri-oral muscle tension, arch forms and dentoskeletal cephalometric measurements were assessed at initiation and completion of the functional education. Functional examination on inclusion showed that the majority of children displayed a low tongue position at rest, oral or mixed ventilation, abnormal labial and peri-oral muscle tone and, in many cases, a narrow maxilla. Initial cephalometric examination recorded the following parameters: overjet, deep bite, and inclination of the lower incisors relative to the A-Pog line, and the SNA, SNB and ANB angles. After twelve months of functional education with a flexible EF Line® splint, improvement was observed in the orofacial functions as shown by correction of the tongue position at rest as well as the lingual index indicating the position of the tongue against the palate, reduced tension of both the lower lip and the buccinator muscles at rest and in function, increased nasal breathing ability and a more rounded maxillary arch form. In addition, a major reduction in overjet and deep bite were visible as well as a significant reduction of the ANB angle due to its increased SNB component. This first stage in the orthodontic treatment plan resulted in a 73% improvement of Class II division 1 malformations. Correction of the malocclusion and functional disorders correlates significantly with compliant wearing of the EF Line® splints. This study demonstrates that the design of EF Line® flexible functional education devices effectively corrects functional disorders linked to poor tongue position and triggers positive dentoskeletal changes, as compared with no treatment. From the clinical viewpoint, flexible EF Line® splints can contribute to orthopedic and orthodontic treatment of Class II division 1 malformations.

Speech-like movements emerge from simulated peri-oral muscle activation space without neural control

Muscle synergies have been proposed as a solution to the degrees of freedom problem inherent in the biomechanics of speech production [Gick & Stavness, 2013, Front. Psych, 4, 977]. However, the majority of experiments involving the extraction of muscle synergies prove theoretically insufficient to determine whether such synergies are of neural origin or simply reflect the lower dimensionality of an under-sampled biomechanical/neural task space [Kutch & Valero-Cuevas, 2012, PLoS computational biology, 8(5), e1002434]. Therefore, to what extent biomechanics of the human vocal tract may constrain the “ecological state space” of a speaker during locution remains uninvestigated. As a proof of concept, we created a simplified version of the peri-oral region using FEM modeling in a physics-based simulator (ArtiSynth [Lloyd et al., 2012, Soft tissue biomech…surgery, pp. 355]). Systematic simulations enabled us to model the full kinematic/biomechanical space. Visualization of the resulting biomechanical state space using t-SNE [van der Maaten & Hinton, 2008, J.MLR., 9, 2579] illustrates that speech-like movements (e.g., lip rounding or spreading) emerge as self-organizing structures (muscle synergies) critically without a direct neural controller. These results are discussed in the context of current and future explorations of motor control of speech. [Funded by NSERC.]Muscle synergies have been proposed as a solution to the degrees of freedom problem inherent in the biomechanics of speech production [Gick & Stavness, 2013, Front. Psych, 4, 977]. However, the majority of experiments involving the extraction of muscle synergies prove theoretically insufficient to determine whether such synergies are of neural origin or simply reflect the lower dimensionality of an under-sampled biomechanical/neural task space [Kutch & Valero-Cuevas, 2012, PLoS computational biology, 8(5), e1002434]. Therefore, to what extent biomechanics of the human vocal tract may constrain the “ecological state space” of a speaker during locution remains uninvestigated. As a proof of concept, we created a simplified version of the peri-oral region using FEM modeling in a physics-based simulator (ArtiSynth [Lloyd et al., 2012, Soft tissue biomech…surgery, pp. 355]). Systematic simulations enabled us to model the full kinematic/biomechanical space. Visualization of the resulting biomechanical state spac...

Relationship between tongue pressure and maxillofacial morphology in Japanese children based on skeletal classification.

During childhood, perioral muscle function is closely associated with malocclusion. To clarify the effects of tongue function on maxillofacial morphology in children, tongue pressure and maximum lip-closing force (LCF) were measured and the relationship between perioral muscle function and maxillofacial morphology was evaluated according to the skeletal classification. Maximum tongue pressure (MTP) and swallowing tongue pressure (STP) were measured on the anterior palatine rugae in 100 children (Hellman's dental stages IIIA-IIIC) using a balloon-type tongue pressure measurement device. Lip-closing force was measured using an LCF measurement device. Lateral cephalograms were examined to classify subjects into skeletal (S)-I, S-II and S-III groups. Correlations of skeletal classification with tongue pressure and LCF were examined. Correlations of lateral cephalometric measurements with palatal volume (PV), measured using a three-dimensional optical scanner, were evaluated. Maximum tongue pressure was significantly lower in the S-II group than in other groups. Swallowing tongue pressure was significantly lower in the S-II group than in the S-III group. Lip-closing force was significantly higher in the S-III group than in other groups. Swallowing tongue pressure was positively correlated with MTP and PV. Correlations between tongue pressure and anteroposterior skeletal classification indicated the importance of quantitative tongue function assessment.

The impact of oronasal breathing on perioral musculature

ABSTRACT Purpose: to compare the behavior of perioral muscles in nasal, oral and oronasal respirators. Methods: a sample consisting of three distinct groups, equally subdivided into Nasal, Oral and Oronasal Respirators. The behavior of the orbicular muscle of the mouth (upper part) and mentual one was measured by surface electromyography at rest, swallowing and labial isometry. Results: in all situations investigated, the orbicular muscle of the mouth (upper part) and mental muscle showed no significant difference in relation to Root Means Square, that is, average electrical activity between Oral and Oronasal Respirators. The data showed a significant difference in In Nasal Respirators, as compared to the other groups. Conclusion: similarity was seen in the comparison of perioral muscles behavior between oral and oronasal respirators, however, a significant difference in relation to nasal respirators.

Evaluation of biomechanical effects of interocclusal surfaces on the mandible

Introduction : Only few studies in the literature employ a mathematical model in the evaluation of the stress which follows the application of loads and constraints onto the mandible. Therefore, new approaches are needed so that the study of this phenomenoncan rely less on the clinical hypothesis and experience of the operator, while taking advantage of the many benefits that virtual representations and mathematical calculations present. Aim of the study is to determine, by means of the finite element method (FEM), the stress produced onto the mandible as a result of the application of a force on itself, in association or not to the perioralmusculature and according to the dental support given by the positioning of an interocclusal surface at three different levels: mesial, intermediate and distal. Aim: The aim is to allow a more objective evaluation of this phenomenon, its absolute repeatability, as well as to acquire importantclinical informations concerning the role of orthodontic and gnathologic appliances. Materials and methods: Starting from a 1:1 scale model of the mandible (human adult male), a virtual three - dimensional (3D) representation was first obtained thanks to a dedicate software; it was then imported into a second software in order to permit the discretization into finite elements of the virtual model and the attribution of its mechanical properties. Finally, thanks to a specificsoftware, it was possible to simulate the presence of load and constraints and to evaluate the stress status by using pseudo - colors. Results: The stress generated following the application of a force onto the mandible, undergoes significant variations in relation to thedental support and the presence or absence of the perioral musculature. Conclusions: Following the results of our research, we consider FEM as a valid and interesting method for this purpose, however additional FEM conducted studies are necessary in order to assess this phenomenon in more detail and determine the role of the perioral musculature as well as the possible clinical implications.

Effects of mouth rehabilitation with removable complete dentures on stimulus perception and the electromyographic activity of the orbicularis oris muscle

Statement of problemMany elderly individuals are rehabilitated with removable complete dentures, which require an initial adaptation period for both oral perception and the perioral muscles. Studies assessing the changes in stimulus perception and the electromyographic (EMG) activity of the orbicularis oris muscle shortly after conventional complete denture insertion are lacking. PurposeThe purpose of this clinical study was to evaluate the effect of mouth rehabilitation with removable complete dentures on stimulus perception and the EMG activity of the orbicularis oris muscle. Material and methodsThis study was approved by the Human Research Ethics Committee of the Araçatuba Dental School (São Paulo State University). Fifteen participants who had worn their removable complete dentures for at least 5 years and needed rehabilitation with new prostheses were enrolled in the study. A perception questionnaire was applied, and surface EMG examinations of the orbicularis oris muscle during rest, suction of water with a straw, and pronunciation of the syllables /bah/, /mah/, /pah/, and the word ‘Mississippi’ were performed before (T0) and 30 (T1) and 100 (T2) days after insertion of the new prostheses. The data were analyzed with the Cochran Q test, McNemar test, 2-way repeated measures ANOVA, and honestly significant difference (HSD) Tukey test (α=.05). ResultsSignificant improvement was reported in the perception questionnaire in terms of the oral discomfort sensation in the T2 period. EMG activity decreased during rest and suction after insertion of the new prostheses. A statistical difference between the upper and lower fascicles of the orbicularis oris muscle was detected, with a decrease of EMG activity between the T0 and T1 periods on the lower fascicle, except for when pronouncing the /pah/ syllable. ConclusionsMouth rehabilitation with removable complete dentures decreased oral discomfort and, depending on the oral function, decreased or increased EMG activity of the orbicularis oris muscle. In addition, the lower fascicle was more active than the upper fascicle during rest and most functional activities.

Comparison of age-related morphological changes in the masseter muscles of senescence-accelerated mouse (SAM)

ObjectiveAge-related morphological changes in the perioral muscles are still garnering attention today. We planned an experiment using a senescence-accelerated mouse (SAM) to compare age-related morphological changes in the masseter muscle in young and old mice. The masseter muscle in elderly mice (40 -week- old) of SAM-prone 8 (SAMP8) mice has been found to demonstrate greater muscle fiber atrophy than that in a control group of SAM-resistant 1(SAMR1) mice. In the present study, in order to assess age-related morphological changes in the masseter muscles of SAMP8 mice, we compared three groups of mice: young mice (12 -week- old), elderly mice (40 -week- old), and later-stage elderly mice (55 -week- old). Materials and methodsThe body weight, the masseter muscle wet weight and the serum albumin levels of each stages in SAMP8 mice were measured. Also, SAMR1 mice were measured as control. Frozen sections of sampled muscles were prepared and dyed with Hematoxylin-eosin (H-E) and immunohistochemical staining. ResultsThe mean cross-sectional area of muscle fibers decreased from 12- week- old to 40- week-old and from 12- week- old to 55- week- old. Immunohistochemical staining showed that the percentage(%) of MyHC-IIa increased from 12- week- old to 55- week- old, whereas that of MyHC-IIb decreased from 12- week- old to 55- week- old and 40- week- old to 55- week- old. ConclusionsIn the present study, it showed that atrophy of the masseter muscle had occurred by 40- week- old and that there were additional subsequent changes in contraction characteristics.

Development and application of a perioral force measurement system for infants with cleft lip and palate

Muscles converge or intertweave around the perioral area, and this can be treated with sequential therapy in infants with cleft lip and palate (CLP). The force of perioral muscles has a great influence on maxillary development and morphology. Perioral force in infants with CLP has not been well studied, and accurate and reliable measurement of perioral force in infants remains a challenge. This study aimed to investigate a new way to accurately and reliably measure perioral force in infants with unilateral CLP (UCLP) and explore the change before and after cheiloplasty. A perioral force measurement system was developed and applied to measure perioral force at labial frenum area and the commissures on both the normal and the cleft sides of four infants with UCLP before and after cheiloplasty. The results were analyzed using the SPSS 19.0 software. The perioral force measurement system appears to produce valid results in infants with UCLP. Before cheiloplasty, the perioral force of labial frenum area was 1.79±0.94g/cm2 and that of commissure on the normal and cleft sides was 5.41±1.01g/cm2 and 3.12±1.55g/cm2, respectively (P<0.05). After cheiloplasty, perioral force of labial frenum area was 12.73±3.51g/cm2 and that of commissure on the normal and cleft sides was 7.64±1.64g/cm2 and 7.27±1.89g/cm2, respectively (P>0.05).

Cataplexy and Its Mimics: Clinical Recognition and Management.

This review describes the diagnosis and management of cataplexy: attacks of bilateral loss of muscle tone, triggered by emotions and with preserved consciousness. Although cataplexy is rare, its recognition is important as in most cases, it leads to a diagnosis of narcolepsy, a disorder that still takes a median of 9years to be diagnosed. The expression of cataplexy varies widely, from partial episodes affecting only the neck muscles to generalized attacks leading to falls. Moreover, childhood cataplexy differs from the presentation in adults, with a prominent facial involvement, already evident without clear emotional triggers ('cataplectic facies') and 'active' motor phenomena especially of the tongue and perioral muscles. Next to narcolepsy, cataplexy can sometimes be caused by other diseases, such as Niemann-Pick type C, Prader Willi Syndrome, or lesions in the hypothalamic or pontomedullary region. Cataplexy mimics include syncope, epilepsy, hyperekplexia, drop attacks and pseudocataplexy. They can be differentiated from cataplexy using thorough history taking, supplemented with (home)video recordings whenever possible. Childhood narcolepsy, with its profound facial hypotonia, can be confused with neuromuscular disorders, and the active motor phenomenona resemble those found in childhood movement disorders such as Sydenham's chorea. Currently, the diagnosis of cataplexy is made almost solely on clinical grounds, based on history taking and (home) videos. Cataplexy shows remarkable differences in childhood compared to adults, with profound facial hypotonia and complex active motor phenomena. Over time, these severe symptoms evolve to the milder adult phenotype, and this pattern is crucial to recognize when assessing the outcome of uncontrolled case series with potential treatments such as immunomodulation. Symptomatic treatment is possible with antidepressants and sodium oxybate. Importantly, management also needs to involve sleep hygiene advice, safety measures whenever applicable and guidance with regard to the social sequelae of cataplexy.

Facial twitches in ADCY5-associated disease - Myokymia or myoclonus? An electromyography study

ObjectiveA clinical feature in patients with ADCY5 gene mutations are perioral muscle twitches initially described as facial myokymia. MethodsFive patients with ADCY5-associated disease with facial twitches and truncal jerks underwent electrophysiological investigations of the orbicularis oris and trapezius muscles to delineate neurophysiological characteristics of these phenomena. ResultsElectromyography (EMG) recordings showed a complex electrophysiological pattern with brief bursts of less than 100 ms and longer bursts with a duration of 100–300 ms up to several seconds in keeping with myoclonus and chorea, respectively, as key findings. None of the patients had EMG patterns of myokymia. ConclusionsIn this series of five ADCY5 mutation carriers, perioral twitches and truncal jerks do not represent myokymia. In view of characteristic clinical signs and electrophysiological patterns with a combination of myoclonus and chorea it might be preferable to refer to these phenomena as myoclonus-chorea.

Polar-phase indices of perioral muscle reciprocity during syllable production in Parkinson’s disease

Purpose: This research characterised perioral muscle reciprocity and amplitude ratio in lower lip during bilabial syllable production [pa] at three rates to understand the neuromotor dynamics and scaling of motor speech patterns in individuals with Parkinson’s disease (PD).Method: Electromyographic (EMG) signals of the orbicularis oris superior [OOS], orbicularis oris inferior [OOI] and depressor labii inferioris [DLI] were recorded during syllable production and expressed as polar-phase notations.Result: PD participants exhibited the general features of reciprocity between OOS, OOI and DLI muscles as reflected in the EMG during syllable production. The control group showed significantly higher integrated EMG amplitude ratio in the DLI:OOS muscle pairs than PD participants. No speech rate effects were found in EMG muscle reciprocity and amplitude magnitude across all muscle pairs.Conclusion: Similar patterns of muscle reciprocity in PD and controls suggest that corticomotoneuronal output to the facial nucleus and respective perioral muscles is relatively well-preserved in our cohort of mild idiopathic PD participants. Reduction of EMG amplitude ratio among PD participants is consistent with the putative reduction in the thalamocortical activation characteristic of this disease which limits motor cortex drive from generating appropriate commands which contributes to bradykinesia and hypokinesia of the orofacial mechanism.

관악기 연주가 유발할 수 있는 구강 안면 질환

Wind instruments are different from other musical instruments because they play with mouth. Perioral muscles, cranial bones, teeth, lips, and tongue participate in the performance. In addition, breathing from the lungs is transmitted to the musical instrument, and both hands are responsible for the fingertip, so the blowing can be defined as an active process of the neuromuscular system to accomplish breathing, embouchure and fingering.&#x0D; The purpose of this study is to describe the playing-related disorders among instrumental musicians by reviewing the papers on the disease published in between 1960 and 2016. We reviewed the disease reports of the wind instrument players and classified them into maloccusion, tooth problem, temporomandibular joint, orofacial muscle and skin. The review of these musculoskeletal disorders suggests the categories of diseases that can occur and makes it possible to utilize them as basic data for the study of performing art medicine. Based on this review, musicians should understand each disease and will be able to cope with the treatment.

Orofacial problems from the wind instrument

관악기는 앙부슈어를 취해 연주하므로 다른 악기들과 구분이 된다. 입 주위의 근육과 골격, 치아, 입술, 혀가 연주에 능동적으로 참여하는 것이다. 더불어 폐로부터의 호흡이 악기에 전달되고 두 손이 운지를 담당하므로 취주는 호흡과 앙부슈어, 운지를 완수하기 위한 근신경계의 능동적인 프로세스라고 정의할 수 있다. 악기 연습과 공연 연주가 이 복잡한 프로세스를 집중하므로 과하게 반복된 동작은 앙부슈어 관련 신체구조에 질환과 외상을 유발하기도 한다. 이에 본 연구는 국내외 취주악기 연주자 질환 보고에 대한 리뷰(review)를 통해 관악 연주가 유발할 수 있는 치과질환을 살펴보는 것을 목적으로 하였다. 심층적인 연구를 위해 1960년대부터 2016년 11월까지 50여 년 동안 동료검증(peer-review) 하에 이루어진 전문연주인들의 직업병 보고를 조사하였으며 부정교합, 치아질환, 측두하악장애, 근육질환, 피부질환으로 구분하고 검토하였다. 이러한 연주자 질환의 제(齊) 검토를 통해 발생 가능한 질환의 범주를 제시하고 연주자에게 지침을 제공하고자 한다. 이를 통해 공연연주의학 연구를 위한 기초자료로 활용할 수 있도록 하는 데에 본 종설의 의의가 있으며 나아가 치과의가 악기 연주가 유발할 수 있는 질환을 인식하고 능동적인 치료를 담당할 수 있을 것이다.Wind instruments are different from other musical instruments because they play with mouth. Perioral muscles, cranial bones, teeth, lips, and tongue participate in the performance. In addition, breathing from the lungs is transmitted to the musical instrument, and both hands are responsible for the fingertip, so the blowing can be defined as an active process of the neuromuscular system to accomplish breathing, embouchure and fingering. The purpose of this study is to describe the playing-related disorders among instrumental musicians by reviewing the papers on the disease published in between 1960 and 2016. We reviewed the disease reports of the wind instrument players and classified them into maloccusion, tooth problem, temporomandibular joint, orofacial muscle and skin. The review of these musculoskeletal disorders suggests the categories of diseases that can occur and makes it possible to utilize them as basic data for the study of performing art medicine. Based on this review, musicians should understand each disease and will be able to cope with the treatment.

The training effect of routine hard gummy chewing on perioral muscles

The training effect of routine hard gummy chewing on perioral muscles

Changes in resting-state connectivity in musicians with embouchure dystonia.

Embouchure dystonia is a highly disabling task-specific dystonia in professional brass musicians leading to spasms of perioral muscles while playing the instrument. As they are asymptomatic at rest, resting-state functional magnetic resonance imaging in these patients can reveal changes in functional connectivity within and between brain networks independent from dystonic symptoms. We therefore compared embouchure dystonia patients to healthy musicians with resting-state functional magnetic resonance imaging in combination with independent component analyses. Patients showed increased functional connectivity of the bilateral sensorimotor mouth area and right secondary somatosensory cortex, but reduced functional connectivity of the bilateral sensorimotor hand representation, left inferior parietal cortex, and mesial premotor cortex within the lateral motor function network. Within the auditory function network, the functional connectivity of bilateral secondary auditory cortices, right posterior parietal cortex and left sensorimotor hand area was increased, the functional connectivity of right primary auditory cortex, right secondary somatosensory cortex, right sensorimotor mouth representation, bilateral thalamus, and anterior cingulate cortex was reduced. Negative functional connectivity between the cerebellar and lateral motor function network and positive functional connectivity between the cerebellar and primary visual network were reduced. Abnormal resting-state functional connectivity of sensorimotor representations of affected and unaffected body parts suggests a pathophysiological predisposition for abnormal sensorimotor and audiomotor integration in embouchure dystonia. Altered connectivity to the cerebellar network highlights the important role of the cerebellum in this disease. © 2016 International Parkinson and Movement Disorder Society.

Weakness and Progressive Muscle Tightness Diagnosed as Kennedy Disease

Kennedy disease is a rare X-linked neurodegenerative disorder that affects patients in 30-50 years of age. It is caused by CAG-repeat in androgen receptor gen. There is no known effective treatment for Kennedy disease. We report a 60-year-old man who had fasciculations and proximal and distal muscle weakness. Physical examination showed involvement of the bulbar musculature accompanied by tongue atrophy and perioral muscle weakness. Furthermore, he had bilateral gynecomastia. Laboratory and imaging findings were normal, except electromyography that showed chronic proximal and distal denervation. Finally, the patient diagnosed with Kennedy disease according to clinical presentation and EMG abnormality that confirmed with genetic study.