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An approximately 2-day-old neonate was brought with a fleshy intraoral mass and an inability to suckle adequately, without a preceding history of trauma. Contrast-enhanced CT revealed a fat density mass-like lesion occupying and partially filling the oral cavity. Following detection of a defect in the buccinator muscle with associated herniation of the buccal pad of fat into the mouth, a surgical approach was adopted to tackle the condition. Intraoperatively, the defects in the buccinator muscle and buccal mucosa were repaired after excision of the prolapsed fat pad. Age-appropriate feeding practices were initiated following surgery without residual anatomical or functional limitation. Early radiological diagnosis directing surgical intervention was helpful in management of a benign but functionally impeding lesion., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Muscular dysplasia is the key factor in influencing surgical outcomes in patients with cleft lip/palate. In this research, we attempted to evaluate a new acellular dermal matrix (ADM) as a substitute for reconstruction of the orbicularis oris muscle with growth factors such as Insulin-Like Growth Factor I (IGF-I), vascular endothelial growth factor (VEGF) in a rabbit model. 30 male New Zealand Rabbits (2-3 m, 1700-2000 g) were divided into four groups as follows; a group in which the orbicularis oris muscle of the upper lip was implanted with ADM, a group in which the orbicularis oris muscle of the upper lip was implanted with ADM + IGF-I + VEGF, a group in which the upper lip was operated without implantation of an ADM scaffold, and a normal upper lip for comparison. Macroscopic observation, histological evaluation, and immunohistochemistry were employed to evaluate levels of the muscle regeneration, vascularization, and inflammation at 1, 2, 4, 6, and 12 weeks after the operation. All wounds healed well without infection, immune rejection and so on. Histological evaluation showed that ADM was totally degraded and replaced by connective tissue. The area in which the ADM scaffold was coated with growth factors show a significant increase in the formation of new myofibers after injury, and the vascularization improved compared to the control group and the normal group. In regard to the degrees of inflammation, there were no notable differences among the groups. In conclusion, Our study indicated that ADM grafts combined with IGF-I and VEGF have potential advantages in alleviating muscular dysplasia in cleft lip treatment., (© 2022 John Wiley & Sons Ltd.)

The Aim of the Study: Was the increasing the effectiveness of treatment and prevention of postoperative complications in the patients with benign tumors of the parotid salivary gland through the combined use of medications, acupuncture, hirudotherapy and a complex of physiotherapy., Materials and Methods: The study comprised 94 patients with postoperative complications after surgical treatment of benign tumors of the parotid salivary gland. All patients suffering from paresis of facial muscles were prescribed a physiotherapy complex developed during the study consisting of two series of exercises with alternating execution of the series every other day. The exercises begin with the upper third of the face, gradually descending to the lower third. A series of exercises is performed every hour and a half, the session duration is twenty minutes, the course is 14 days. The exercises are performed by the patient in front of the mirror, gently and at a slow pace., Results: The duration of the recovery period of motor function of the facial muscles on the affected side was 31.2±19.6 days, with the amplitude of the M-response 1.45±0.28 mV, the duration of the M-response 8.04±0.27 ms and the latent time during EMG 3.18±0.86 ms. Conclusion. The combined treatment, which included surgical and conservative treatment complex with methods of acupuncture, hirudotherapy and physiotherapy, was significantly more effective ( p <0.05) in terms of the severity of paresis of facial muscles than in the control group.

Bell's palsy is the most common cause of unilateral facial paralysis and is defined as an idiopathic and acute inability to control movements of the facial muscles on the affected side. While the pathogenesis remains unknown, previous studies have implicated post-viral inflammation and resulting compression of the facial nerve. Reported heritability estimates of 4-14% suggest a genetic component in the etiology and an autosomal dominant inheritance has been proposed. Here, we report findings from a meta-analysis of genome-wide association studies uncovering the first unequivocal association with Bell's palsy (rs9357446-A; P = 6.79 × 10 -23 , OR = 1.23; N cases  = 4714, N controls  = 1,011,520). The variant also confers risk of intervertebral disc disorders (P = 2.99 × 10 -11 , OR = 1.04) suggesting a common pathogenesis in part or a true pleiotropy.

There are limited MRI studies of craniofacial and velopharyngeal features in children with 22q11.2 deletion syndrome (22q11.2DS) and to date, none have explored the potential relationship between these features and the speech phenotype. The purpose of this study was to examine the relationship between craniofacial and related velopharyngeal structures in children with 22q11.2DS and to assess their correlation to resonance features using an unsedated MRI protocol. Fifteen children with 22q11.2DS and 15 age- and sex-matched controls with normal velopharyngeal anatomy (ages 4-12 years) successfully completed the study. Analysis of covariance was used to compare differences between the experimental (22q11.2DS) and control (children with normal anatomy) groups. Correlation analyses and regression models were also utilized. The 22q11.2DS group demonstrated significantly shorter nasion-to-sella, sella-to-basion, and basion-to-opisthion distances. The anterior cranial base angle was significantly more obtuse. The levator veli palatini (levator) muscle was significantly thinner and shorter, with an obtuse angle of origin in the 22q11.2DS group. Levator length was significantly correlated with the sella-to-basion measure and hypernasality was correlated with levator origin-to-origin distance. Preliminary results from this study indicate a significant association between hypernasality and levator origin-to-origin distance. Findings from the present study, provide an insight into the pathophysiology of velopharyngeal dysfunction related to this clinically complex population., Competing Interests: Declaration of Competing Interest None., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Tarsal ectropion and involutional entropion are two frequent age-related lower eyelid malpositions with a mirrored clinical presentation. The recent anatomical confirmation of two layers of lower eyelid retractors makes it possible to conceive of the role of each of these layers in the stability of the two palpebral lamellae and their involvement in the pathogenesis of these two malpositions. This study proposes a theory of common pathogenesis involving an involutional change in only the lower lid retractors, leading to the description of two new clinical-anatomical entities., (Copyright © 2020 Elsevier Masson SAS. All rights reserved.)

The aim of this study was to demonstrate the extent of preseptal orbicularis oculi muscle (OOM) override onto the pretarsal OOM in the lower eyelids. In this experimental microscopic study, 22 exenterated specimens from 22 Japanese cadavers were prepared as full-thickness sagittal sections. All exenterated specimens were devoid of lower eyelid entropion. The tarsal dimension and distance from the lower tarsal edge to the tip of overriding OOM were microscopically measured. The rotation axis of the tarsus was presumed to pass through the tarsal centroid and the distance from an estimated tarsal centroid to the lower tarsal edge was calculated. Consequently, 2 eyelids did not exhibit any overriding of the OOM. In the remaining 20 eyelids with OOM override, the average distance of the overriding OOM was found at a level covering 48.0% of the whole tarsal height. In 15 out of the 20 eyelids, the tip of the overriding OOM was located lower than the centroid. These microscopic findings suggest that overriding of the preseptal OOM does not always produce an inward rotational force on the tarsus.

Background: Paramyotonia congenita (PC; OMIM 168300) is a non-dystrophic myotonia caused by mutations in the SCN4A gene. Transient muscle stiffness, usually induced by exposure to cold and aggravated by exercise, is the predominant clinical symptom, and interictal persistent weakness is uncommon., Case Report: We report a family with a history of PC accompanied by persistent hand muscle weakness with masticatory muscle involvement. Persistent weakness was exacerbated with age, and MR analysis showed marked atrophy of temporal, masseter, and finger flexor muscles with fatty replacement. The PC causative mutation T1313M in the SCN4A gene was prevalent in the family. Administration of acetazolamide chloride improved clinical symptoms and the results of cold and short exercise tests. Phenotypic variation within the family was remarkable, as the two younger affected patients did not present with persistent weakness or muscle atrophy., Conclusions: PC associated with the T1313M mutation is a possible cause of persistent distal hand weakness.

A 23 year female presented with bilateral recurrent swelling of eyelids along with ptosis and proptosis for last 3 years. She also had swellings over the right cheek, parotid gland, and retro auricular area along with regional lymphadenopathy. Systemic laboratory workup revealed raised serum IgE and a high peripheral eosinophil count. Computed tomography and magnetic resonance imaging showed bilateral enlargement of extraocular muscles, lacrimal glands, and ipsilateral parotid gland. Excision biopsy of the retro-auricular lymph node was suggestive of Kimura's disease (KD). The patient responded well to systemic corticosteroid. KD rarely affects orbit, but it should be included in the differential diagnosis of inflammatory diseases of the orbit. To our knowledge, this is the first reported case of KD from India involving the orbit, lacrimal gland, extraocular muscles, parotid gland and buccal area., Competing Interests: None

Neurosarcoidosis when encountered by neurologists most commonly presents as cranial neuropathy, peripheral mononeuropathy,polyneuropathy, myopathy, meningitis or myelopathy. There are limited reports in the current literature on the cases of neurosarcoidosis patients presenting with ischaemic stroke. We discuss a 52-year-old patient with a known previous history of cutaneous sarcoidosis presenting with an acute third nerve palsy, facial weakness and ataxia. His magnetic resonance imaging (MRI) brain demonstrated focal signal changes in the midbrain consistent with an acute ischaemic event in the region of his third nucleus, suggesting a partial Claude syndrome presentation. Cerebrospinal fluid (CSF) examination demonstrated an elevated angiotensin-converting enzyme (ACE) level. We discuss the difficulties associated with confirming a diagnosis for his presentation and consider distinctions in stroke in neurosarcoid and its management in comparison to more common causes., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2019. No commercial re-use. See rights and permissions. Published by BMJ.)

Introduction: Lash ptosis is often an overlooked sign that may coexist with congenital and acquired blepharoptosis. This is a report of case series of patients presented in an oculoplastic clinic with visual field loss associated with lash ptosis. On examination, the primary pathology was attributed to lash ptosis dehiscence., Methods: All patients underwent anterior lamellar repositioning and were followed for an average of 15 (10-24) months., Results: All patients had resolution of visual field loss and heaviness of eyelids., Conclusions: Lash ptosis is associated with abnormalities such as floppy eyelid syndrome. However it may be a primary condition, with no background eyelid pathology and no external explanation for the eyelash ptosis. The condition might result from anatomical changes in the orbicularis oculi, Riolan's muscle, and tarsal plate. Patients in this series complained of upper lid visual field restriction. Anterior lamellar repositioning resulted in complete resolution of complaints. Additional studies are needed to learn about the pathophysiology of this entity.

Purpose: We morphometrically analyzed human facial muscles, and evaluated the Yanagihara facial nerve grading system using our data., Methods: We used 15 types of human facial muscle, 2 types of masticatory muscle and 2 types of skeletal muscle. The materials were obtained from 11 Japanese male cadavers aged 43-86 years. We counted the muscle fibers and measured the transverse area of the muscle fibers (TAMF), and then calculated the number of muscle fibers (NMF) per mm 2 and the average TAMF., Results: We found a significant correlation between average TAMF and NMF (r = - 0.70; p < 0.01). We classified facial muscles into three types based on the correlational results. Type A had a low average TAMF and high NMF. Type C had a high average TAMF and low NMF. Masticatory and skeletal muscles were characterized as Type C. Type B was intermediate between Types A and C., Conclusions: Pathological changes in the facial muscles in facial nerve palsy seem to vary according to the type of facial muscle, because each facial muscle has a unique fiber-type composition. As the nine discrete facial expressive states evaluated in the Yanagihara system involve all three facial muscle types of our classification, the Yanagihara system is an outstanding system for grading facial nerve palsy in terms of the facial muscle morphology.

Background: Extranodal natural killer/T-cell lymphoma (ENKTL), nasal type, is an aggressive entity within the World Health Organization classification of lymphoid tumors. The International Prognostic Index is reported to be prognostically meaningful for ENKTL, but lacks discriminatory power for stage I/II ENKTL with extensive local invasion. This study aimed to evaluate the prognostic effects of local invasion by site and tissue type in patients with ENKTL., Methods: We retrospectively analyzed data of 86 patients who were diagnosed with ENKTL by the Department of Pathology of Beijing Tongren Hospital from June 2002 to April 2016, and ascertained tumor infiltration of adjacent structures (AS), bone, and soft tissue for each patient, using physical findings and imaging scans. We used univariate and multivariate analysis to assess the association of each involved tissue or site with patients' overall survival (OS)., Results: Of the 86 patients, 71 (82.6%) experienced invasion of AS, 22 (25.6%) of soft tissue, and 26 (30.2%) had bone involvement. Overall, patients with AS involvement did not show significantly shorter survival than those without AS involvement (Log rank χ = 1.177, P = 0.278); however, patients who had involved eyeballs or brains showed significantly lower 2-year OS rates than those without eyeball involvement (Log rank χ = 4.105, P = 0.043) or brain involvement (Log rank χ = 7.126, P = 0.008). Patients with involved local soft tissue or bones, respectively, showed lower 2-year OS rates than those without involved local soft tissue (Log rank χ = 10.390, P = 0.001) or bones (Log rank χ = 8.993, P = 0.003). Multivariate analysis showed that involvement of the cheek or facial muscles (hazard ratio, HR = 5.471, 95% confidence interval [CI]: 1.466-20.416, P = 0.011) and the maxilla bone (HR = 6.120, 95% CI: 1.517-24.694, P = 0.011) were significantly independent predictors of lower 2-year OS rates., Conclusions: Imaging can accurately detect ENKTL invasion of AS, soft tissue, and bone. Involvement of local soft tissue or bone was significantly associated with lower 2-year OS rates. Involvements of the cheek or facial muscle, as well as maxilla bone, are independent predictors of lower 2-year OS rates in ENKTL patients.

The authors describe a sculpture from Daumier, called "Le Hargneux" (The peevish one), whose physiognomic study evokes hitherto unrecognized cranial-cervical dystonia. It is probably the first representation of dystonia in sculpture, before its scientific identification by Horatio Wood, in 1887., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background: The "post-paralytic syndrome" after facial nerve reconstruction has been attributed to (i) malfunctioning axonal guidance at the fascicular (branches) level, (ii) collateral branching of the transected axons at the lesion site, and (iii) intensive intramuscular terminal sprouting of regenerating axons which causes poly-innervation of the neuromuscular junctions (NMJ)., Objective: The first two reasons were approached by an innovative technique which should provide the re-growing axons optimal conditions to elongate and selectively re-innervate their original muscle groups., Methods: The transected facial nerve trunk was inserted into a 3-way-conduit (from isogeneic rat abdominal aorta) which should "guide" the re-growing facial axons to the three main branches of the facial nerve (zygomatic, buccal and marginal mandibular). The effect of this method was tested also on hypoglossal axons after hypoglossal-facial anastomosis (HFA). Coaptational (classic) FFA (facial-facial anastomosis) and HFA served as controls., Results: When compared to their coaptation (classic) alternatives, both types of 3-way-conduit operations (FFA and HFA) promoted a trend for reduction in the collateral axonal branching (the proportion of double- or triple-labelled perikarya after retrograde tracing was slightly reduced). In contrast, poly-innervation of NMJ in the levator labii superioris muscle was increased and vibrissal (whisking) function was worsened., Conclusions: The use of 3-way-conduit provides no advantages to classic coaptation. Should the latter be impossible (too large interstump defects requiring too long interpositional nerve grafts), this type of reconstruction may be applied. (230 words).

Background: Port-wine macrocheilia (PWM) involves a congenital, progressive capillary malformation that results in soft-tissue hypertrophy in the lips. Its aetiology has not yet been fully elucidated., Objectives: To investigate frequencies of GNAQ mutation in different tissues from patients with PWM, including skin, mucosa, gland and muscle, using samples obtained during cheiloplasty., Methods: Targeted next-generation sequencing of GNAQ was designed and performed to assess DNA extracted from 80 different affected tissues from 20 patients with PWM., Results: The GNAQ R183Q mutation was not detected in gland samples but was found in 90%, 95% and 95% of the skin, mucosal and muscle samples, respectively. The lowest levels of mutations were found in the glands (P < 0·001 vs. skin, mucosa and muscle), and levels were second lowest in the skin (P = 0·023 vs. mucosa; P = 0·012 vs. muscle). The mutation frequencies in mucosa and muscle were the highest and showed no statistically significant difference (P = 0·92)., Conclusions: In patients with PWM, GNAQ was mutated in all tissues except for glands. PWM is congenital, and all tissue layers exhibit primary hypertrophy rather than acquired or partially related hypertrophy. Given the advantages of mucosal biopsy, including practicality, lack of scarring and rapid healing, GNAQ mutation in the lip mucosa may be a useful predictor for early-stage PWM in patients with port-wine stains affecting the lips., (© 2018 British Association of Dermatologists.)

Purpose: To compare needle and microcannula injection techniques in regards to the microanatomical location of hyaluronic acid (HA) gel injected in the upper lip vermillion border of cadaver specimens., Methods: The upper lip vermillion border was injected transcutaneously with HA gel in 8 fresh hemifaces of 4 female human cadavers. Each hemiface was injected by a single experienced injector, the right side using a 27-gauge microcannula and the left side using a 30-gauge needle. A 2-cm region of each lip was excised lateral to a point 1-cm lateral to the philtrum. Specimens were fixed in 95% alcohol, embedded in paraffin, and stained with hematoxylin-eosin for histologic examination., Results: Most HA injected with either a needle or a microcannula was located within the orbicularis oris muscle, and the remaining HA resided within the subcutaneous fat. In 3/4 right (microcannula) hemifaces, 100% of the HA was located within the muscle. Only 2/4 left (needle) hemifaces had at least 95% of the HA located within the muscle. Overall, in right (microcannula) hemifaces, 93% of the filler was located within the muscle, and in left (needle) hemifaces, 79% of the filler was located within the muscle (p =0.14)., Conclusions: Most HA filler injected into the vermillion border after either microcannula or needle injection resides within the orbicularis oris muscle rather than in a subcutaneous/submucosal location. Injection with a microcannula shows a trend for more uniform intramuscular location compared with needle injection.

Primary hemifacial spasm (pHFS) is due to a benign compression of the facial motor nerve by an offending vessel, leading to increased nerve excitability. Facial nerve hyperexcitability presents two different aspects. First, there is a spontaneous and ectopic generation of action potentials on the incriminated nerve and then this ectopic impulse can propagate and spread "laterally" from one facial nerve branch to another. This results in spontaneous and synkinetic spasms affecting one hemiface. Although the increase in excitability certainly concerns the nucleus of the facial motor nerve in the brainstem, it seems unlikely that the primary origin of this hyperexcitability and the associated phenomenon of lateral spreading strictly originate at the nuclear level. In fact, the mechanisms causing facial nerve hyperexcitability per se remain unknown. The leading implication of a structural nerve lesion, such as segmental demyelination, induced by vessel compression, is also unconvincing. In contrast, a functional mechanical factor increasing nerve excitability is extremely probable, that it is either due to compression or stretch resulting from the neurovascular conflict. Axonal ion channel changes are obviously associated with this mechanism. Then the lateral spreading of nerve fibre hyperexcitability probably results from an ephaptic process, the "cross-talk" between axons being located in the region of the conflict or in the transition zone between central and peripheral myelin, at the end of the facial nerve root exit zone. In any event, pHFS is due to a functional increase in facial nerve excitability triggered by an offending vessel and this clearly explains the remarkable and rapid efficacy of surgical microvascular decompression., (Copyright © 2018 Elsevier Masson SAS. All rights reserved.)

Background and Objectives: Early persistent facial paralysis is characterized by intact muscles of facial expression through maintained perfusion but lacking nerve supply. In facial reanimation procedures aiming at restoration of facial tone and dynamics, neurotization through a donor nerve is performed. Critical for reanimating target muscles is axonal capacity of both donor and recipient nerves. In cases of complete paralysis, the proximal stump of the extratemporal facial nerve trunk may be selected as a recipient site for coaptation. To further clarify the histological basis of this facial reanimation procedure we conducted a human cadaver study examining macro and micro anatomical features of the facial nerve trunk including its axonal capacity in human cadavers. Axonal loads, morphology and morbidity of different donor nerves are discussed reviewing literature in context of nerve transfers., Methods: From 6/2015 to 9/2016 in a group of 53 fresh frozen cadavers a total of 106 facial halves were dissected. Biopsies of the extratemporal facial nerve trunk (FN) were obtained at 1 cm distal to the stylomastoid foramen. After histological processing and digitalization of 99 specimens available, 97 were selected eligible for fascicle counts and 87 fulfilled quality criteria for a semi-automated computer-based axon quantification software using ImageJ/Fiji., Results: An average of 3.82 fascicles (range, 1 to 9) were noted (n = 97). 6684±1884 axons (range, 2655- 12457) were counted for the entire group (n = 87). Right facial halves showed 6364±1904 axons (n = 43). Left facial halves demonstrated 6996±1833 axons (n = 44) with no significant difference (p = 0.73). Female cadavers featured 6247±2230 (n = 22), male showed 6769±1809 axons (n = 40). No statistical difference was seen between genders (p = 0.59). A comparison with different studies in literature is made. The nerve diameter in 82 of our specimens could be measured at 1933±424 μm (range, 975 to 3012)., Conclusions: No donor nerve has been described to match axonal load or fascicle number of the extratemporal facial nerve main trunk. However, the masseteric nerve may be coapted for neurotization of facial muscles with a low complication rate and good clinical outcomes. Nerve transfer is indicated from 6 months after onset of facial paralysis if no recovery of facial nerve function is seen.

Our aim was to find out first whether the extrinsic muscles of the tongue are histologically identifiable, and secondly to what degree the use of the new criteria in the 8th editions of the American Joint Committee on Cancer(AJCC)/Union for International Cancer Control (UICC) manuals (which have recognised the importance of depth of invasion of tumour, rather than invasion of the extrinsic muscles of the tongue and extranodal extension), will alter staging of lingual squamous cell carcinoma (SCC). The histological sections from 165 patients who had had primary resection of lingual SCC were reviewed, and one or more extrinsic muscles of the tongue was identified in 100 patients (61%), with the genioglossus seen the most often (in 96). By contrast, the hyoglossus was identified in only eight patients, the styloglossus in two, and the palatoglossus in none. Identification was straightforward only in extensive resections. Applying the criteria from the 8th edition increased the number of pT3 SCC with a simultaneous reduction in pT4a tumours. The number of pN2b SCC was also reduced, but the new category of pN3b meant that overall 53% of tumours were upstaged. The kappa scores for agreement between the two sets of criteria were 0.221 (weighted 0.410) for the pT values, 0.508 (0.713) for pN values (but 0.227, weighted 0.386, if the pN0 values were removed before calculation), and 0.243 (0.514) for overall stage, indicating poor to fair agreement. We conclude that the removal of invasion of extrinsic muscles of the tongue as a criterion for a pT4a SCC is justified, and that many SCC of the tongue will be upstaged as a result of implementation of the 8th editions., (Copyright © 2017 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.)

Objective: To determine the efficacy of levetiracetam in oromandibular or cranial dystonia., Methods: We recruited seven subjects with oromandibular or cranial dystonia. Five completed the study, median age was 71 years (range 42-79 years), median disease duration was 12 years (range 2-30 years). Participants were randomized to receive levetiracetam or placebo and were then crossed over. They titrated up to a total daily dose of 4000 mg or the maximum tolerated dose over 3 weeks and maintained that dose for another 3 weeks. The primary endpoint was the percent change of the eyes, mouth, speech, and swallowing Burke-Fahn-Marsden (BFM) subscores from baseline to weeks 6 and 14. Additional endpoints included the BFM subscore at weeks 3 and 11, and the global dystonia severity (GDS) subscore at weeks 3, 6, 11, and 14, as well as all adverse side effects., Results: The mean percent increase in the BFM subscore (placebo: 31.25%, levetiracetam: 12.16%) was not significantly different between the two arms according to the Friedman analysis. The Wilcoxon signed-rank test showed that these percent changes were not significant, indicating that there was no statistical clinical worsening in either arm. The mean percent change of the BFM subscore at weeks 3 and 11 and the mean percent change of the GDS subscore at weeks 3, 6, 11, and 14 were not significantly different between the two arms, and the Wilcoxon signed-rank test did not show statistical significance., Conclusion: Levetiracetam does not appear to be efficacious in patients with oromandibular or cranial dystonia., (© 2016 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)

This case is an addition to scarce literature available for a rare condition, chewing-induced task-specific dystonia. The patient was a 63-year-old woman who presented with a 4-year history of progressive difficulty in eating food only during chewing associated with abnormal facial grimaces without any difficulty in drinking, swallowing, speaking or singing. Examination revealed dystonia of facial muscles every time she chewed but absent during drinking and speaking. As movements were consistent and reproducible with the specific task, other differential diagnosis like motor tics, psychogenic disorder, tardive dystonia and parkinsonism syndrome were excluded leading to a diagnosis of task-specific facial dystonia triggered by chewing. Treatment was started with trihexyphenidyl and later on tetrabenazine was also added but she got only mild relief of symptoms. As she did not agreed for botulinum toxin therapy, so we continued with the same treatment without much improvement., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)

Objective: To provide biomechnical basis for orthodontics of centronuclear myopathy (CNM) patients, we studied the oral and maxillofacial clinical features and MRI image manifestations to explore application of MRI to objective evaluation the affected facial muscles. Methods: The study consisted of 8 patients who were diagnosed as CNM (CNM group) and 20 healthy volunteers (control group). Their medical information were gathered and then we examined the ptosis situation and the facial index calculation of them. To measure the maximal hight of palate and the width of palate, patients and volunteers were made impressions. We also checked their maximum bite force with occlusion pressure tester. And they took lateral cephalometric radiographs to measure mandibular plane-Frankfort horizontal plane angle (MP-FH). At last, they were taken oral and maxillofacial region MRI to observe the affected situation of masseter muscle, medial pterygoid muscle and lateral pterygoid muscle. Results: Six patients were ptosis; 6 patients had inverse V-shaped mouth; 3 patients were difficulty in swallowing; 4 patients were anterior open bites; 4 patients were mouth breathing; 7 patients liked to eat soft foods. Morphological facial index ([91.3±0.5]%), MP-FH (34.9°±2.0°) of CNM group were greater than the control group, male maximal hight of palate ([19.0±0.2] mm), female maximal hight of palate ([18.0±0.6] mm) of CNM group were greater than the control group ( P< 0.05). Male width of palate ([34.5±0.8] mm), female width of palate ([33.4±1.0] mm), male maximum bite force ([464.3±78.2] N), female maximum bite force ([320.7±13.8] N), maximal opening of mouth ([3.4±0.3] cm) of CNM group were less than the control group ( P< 0.05). And these had significant difference compared with the control group ( P< 0.05). In MRI examination, there were 7 patients ' masseter muscles, 4 patients ' medial pterygoid muscles and 6 patients ' lateral pterygoid muscles to atrophy asymmetrically. These three pieces of muscular fatty infiltration were inordinately, focused on Grade 0 to 4 and the both sides were similar. Conclusions: CNM patients with long and thin face, high palatine arches and low bite force together were the biomechanical basis of the maxillofacial deformities. MRI can clearly show the affected masseter muscle, medial pterygoid muscle, lateral pterygoid muscle, and can serve as an objective examination method for the evaluation of facial muscles. It can be worth of clinical popularization and application.

Background: Individualized neuromodulator injections of the forehead are increasingly important to maximize patient safety and satisfaction and to yield the best aesthetic outcome. To assess the location of maximal muscle contraction, it would beneficial to relate the shape and location of the forehead lines to the morphology of the underlying frontalis muscle.

Materials and Methods: We investigated the shape of forehead lines and their relationship to the morphology of the underlying frontalis muscle using anatomic dissection in a sample of 31 human body donors, with a mean age at death of 77.2 ± 13.2 years (range, 47-93) of which n=29 (93.5%) were of Caucasian and n=2 (6.5%) of African-American ethnicity and of which n=16 (51.6%) were females (n=15 males; 48.4%).

Results: Horizontal forehead lines were classified into straight n=13 (41.9%) and wavy n=18 (58.1%) lines. The presence of wavy forehead lines (as compared to straight lines) was significantly related to the presence of a midline aponeurosis (r = 0.69; P less than 0.001) and to a greater muscle fascicle angle (12.67 ± 2.60° vs 10.18 ± 2.12°) (r = 0.47; P less than 0.001), but was not statistical significantly influenced by age, gender, or ethnicity (all P greater than 0.05).

Conclusion: According to the shape of the forehead lines, physicians can appreciate the morphology of the underlying frontalis muscle. This understanding will facilitate more accurate neuromodulator injections and a better aesthetic outcome. It may also result in decreased dosages and increased injection intervals for maximal patient safety., Level of Evidence: IV

J Drugs Dermatol. 2017;16(5):471-477.

Objective: Evaluation of histopathological changes in lower eyelid involutional entropion., Design: Case-control, comparative study at a single institution., Participants: A total of 20 consecutive patients with previously untreated involutional lower eyelid entropion and 20 matching patients with lateral lower eyelid basal cell carcinoma (BCC)., Methods: Patients with involutional entropion were operated using our modified surgical method, and patients with BCC underwent full-thickness pentagonal excision with 3-mm surgical margins. Histopathological analysis of the full-thickness eyelid specimens of both groups included measurements of tarsal thickness and height, thickness of the pretarsal orbicularis oculi muscle, diameter of muscle fibres, and qualitative changes in lower eyelid retractor attachment., Results: The tarsus was significantly thicker in the entropion group (p = 0.006). The mean tarsal thickness was 1.40 ± 0.32 mm, whereas in the BCC group it was 1.16 ± 0.19 mm. There was no statistically significant difference in the tarsal height and the thickness of the pretarsal orbicularis oculi muscle between the 2 groups. In the entropion group, 60% of the lids had total and 35% partial dehiscence of the retractor, whereas in the BCC group, dehiscence was found in only 45% of the lids. The difference was statistically significant (p = 0.002)., Conclusions: To the best of our knowledge, this is the first histopathological study documenting thickening of the tarsus in involutional lower eyelid entropion. Moreover, dehiscence of the lower eyelid retractor was proven histopathologically in 95% of the entropic lids. With this in mind, correction of vertical instability should be mandatory in involutional lower eyelid entropion repair., (Copyright © 2016 Canadian Ophthalmological Society. Published by Elsevier Inc. All rights reserved.)

Objective: Motor control of the muscles of the face, lips, and tongue of horn players has traditionally been described from externally observed phenomena. Developments in real-time, high-speed magnetic resonance imaging (MRI) extend the scope of study to include descriptive and quantitative information from within the mouth. We employed these developments to compare oral movement strategies between 12 elite horn players and 5 horn players with embouchure dystonia (ED)., Methods: Serial images with an acquisition time of 33.3 ms were obtained from each subject as they performed 6 exercises on an MRI-compatible horn: 1) a slurred ascending harmonic sequence, 2) a slurred descending harmonic sequence, 3-6) sustained high and low notes, each performed softly and loudly. Temporal changes in pixel luminescence along a series of lines positioned within the oral cavity were calculated using a customized MATLAB toolkit. This allowed the extraction of temporal line profiles for comparative analyses between elite and dystonic horn players., Results: Differing motor strategies of controlling the tongue and jaw were observed during ascending and descending exercises. In ascending exercises, the elite players employed a strategy of elevation and anterior tongue displacement and elevation of the jaw, whereas dystonic players exhibited more variability in their responses. With descending exercises, both groups exhibited a lowering of the tongue and jaw, though this was more pronounced and consistent in the elite horn players. Sustained note exercises also elicited differences between groups. We suggest that elite strategies are more efficient and that the less-efficient patterns of dystonic players may exacerbate muscular tension with ED.

After peripheral nerve injury, recovery of motor performance negatively correlates with the poly-innervation of neuromuscular junctions (NMJ) due to excessive sprouting of the terminal Schwann cells. Denervated muscles produce short-range diffusible sprouting stimuli, of which some are neurotrophic factors. Based on recent data that vibrissal whisking is restored perfectly during facial nerve regeneration in blind rats from the Sprague Dawley (SD)/RCS strain, we compared the expression of brain derived neurotrophic factor (BDNF), fibroblast growth factor-2 (FGF2), insulin growth factors 1 and 2 (IGF1, IGF2) and nerve growth factor (NGF) between SD/RCS and SD-rats with normal vision but poor recovery of whisking function after facial nerve injury. To establish which trophic factors might be responsible for proper NMJ-reinnervation, the transected facial nerve was surgically repaired (facial-facial anastomosis, FFA) for subsequent analysis of mRNA and proteins expressed in the levator labii superioris muscle. A complicated time course of expression included (1) a late rise in BDNF protein that followed earlier elevated gene expression, (2) an early increase in FGF2 and IGF2 protein after 2 days with sustained gene expression, (3) reduced IGF1 protein at 28 days coincident with decline of raised mRNA levels to baseline, and (4) reduced NGF protein between 2 and 14 days with maintained gene expression found in blind rats but not the rats with normal vision. These findings suggest that recovery of motor function after peripheral nerve injury is due, at least in part, to a complex regulation of lesion-associated neurotrophic factors and cytokines in denervated muscles. The increase of FGF-2 protein and concomittant decrease of NGF (with no significant changes in BDNF or IGF levels) during the first week following FFA in SD/RCS blind rats possibly prevents the distal branching of regenerating axons resulting in reduced poly-innervation of motor endplates., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Myopathic changes are frequent a electrophysiological finding in patients with muscle specific tyrosine kinase (MuSK) positive myasthenia gravis (MG). The aim of this study was to explore the importance of quantitative electromyography (EMG) in the detection of myopathic changes in MuSK MG patients. Classical and quantitative EMG were performed in 31 MuSK and 28 acetylcholine receptor (AChR) positive MG patients, matched by sex, age, disease duration and severity. Classical EMG revealed the presence of myopathic changes more frequently in MuSK MG compared to AChR MG patients, especially in the facial muscles. Quantitative EMG registered myopathic lesions more frequently than classical EMG, but the frequency was similar between MuSK and AChR MG patients. Quantitative EMG revealed myopathic changes in the majority of both MuSK and AChR positive MG patients. This examination is sensitive, but it cannot be used to differentiate between MG patients belonging to the different disease groups. It should not be used in isolation. Rather, it should complement classical EMG in the detection of myopathic changes., (Copyright © 2015 Elsevier Ltd. All rights reserved.)

Electroneurography (ENoG) is one of the most objective tests in grading the damage and prediction of prognosis in peripheral facial palsy (PFP). We aimed to determine temporal changes of ENoG recorded over occipitalis muscle in acute idiopathic PFP. Consecutive 21 patients with unilateral acute idiopathic PFP and age- and sex-matched 15 healthy volunteers were included in the study. Nasal and occipital ENoG values were recorded once in the control group and the same procedure was repeated daily between the second and eight days of the disorder in the PFP group. Occipital ENoG value began to increase on the third day while nasal ENoG value was still within the normal range (27.04 vs 7.69 %, p = 0.0001). In the fourth, fifth and sixth days, occipital ENoG value was significantly high compared to nasal ENoG value (p = 0.0001 for each day) whereas nasal and occipital ENoG values were very similar in the seventh and eighth days (p = 0.181 and p = 0.584, respectively). Our study presents further support for technical possibility of occipital ENoG which may reflect the degree of fiber degeneration earlier than the nasalis muscle in PFP.

Intramuscular haemangiomas (IMHs) are rare benign vascular neoplasms that account for approximately 0.8% of all haemangiomas. The histology of IMHs can reveal cavernous dilated spaces. We report an interesting case of haemangioma in the deep skeletal muscle of the right labial mucosa in a young man involving the orbicularis oris muscle which showed additional features of sinusoidal arrangement with a secondary Masson's phenomenon., (2016 BMJ Publishing Group Ltd.)

Purpose: Many children present with excessive blinking. Categorization, associated conditions, and prognosis are controversial., Methods: All children with excessive blinking were reviewed, excluding those with known uveitis, glaucoma, or obvious eyelid abnormalities. Parents were telephoned for follow-up., Results: No ocular pathology was identified in 31 of 34 children with excessive blinking (91%). Parents were able to report a specific cause of blinking in 7 (21%). In 24 of 34 (71%), parents reported complete resolution of excessive blinking. No new ophthalmologic diagnoses were uncovered on follow-up., Conclusions: Episodes of excessive blinking rarely indicate neurologic disorders and frequently resolve spontaneously., (Copyright 2016, SLACK Incorporated.)

Background: Over the last decade surgical treatments for migraine involving proposed trigger sites have been described and popularized by plastic surgeons in particular. Various related techniques aim to free up "trigger sites" by removal of small facial muscles or "decompressing" small facial nerves., Discussion: The basis for migraine trigger site surgery is without merit. There is one positive placebo controlled study with many limitations. Natural history and placebo mechanisms explain the outcomes from migraine surgery. The American Headache Society recommends that the migraine surgery not be performed outside of a clinical trial., Conclusion: Migraine trigger site surgery should not be performed., (© 2015 American Headache Society.)

The protection of sensitive structures (e.g., nerves) from iatrogenic damage is of major importance when performing laser surgical procedures. Especially in the head and neck area both function and esthetics can be affected to a great extent. Despite its many benefits, the surgical utilization of a laser is therefore still limited to superficial tissue ablation. A remote feedback system which guides the laser in a tissue-specific way would provide a remedy. In this context, it has been shown that nerval structures can be specifically recognized by their optical diffuse reflectance spectra both before and after laser ablation. However, for a translation of these findings to the actual laser ablation process, a nerve protection within the laser pulse is of utmost significance. Thus, it was the aim of the study to evaluate, if the process of Er:YAG laser surgery--which comes with spray water cooling, angulation of the probe (60°) and optical process emissions--interferes with optical tissue differentiation. For the first time, no stable conditions but the ongoing process of laser tissue ablation was examined. Therefore, six different tissue types (nerve, skin, muscle, fat, cortical and cancellous bone) were acquired from 15 pig heads. Measurements were performed during Er:YAG laser ablation. Diffuse reflectance spectra (4500, wavelength range: 350-650 nm) where acquired. Principal component analysis (PCA) and quadratic discriminant analysis (QDA) were calculated for classification purposes. The clinical highly relevant differentiation between nerve and bone was performed correctly with an AUC of 95.3% (cortial bone) respectively 92.4% (cancellous bone). The identification of nerve tissue against the biological very similar fat tissue yielded good results with an AUC value of 83.4% (sensitivity: 72.3%, specificity: of 82.3%). This clearly demonstrates that nerve identification by diffuse reflectance spectroscopy works reliably in the ongoing process of laser ablation in spite of the laser beam, spray water cooling and the tissue alterations entailed by tissue laser ablation. This is an essential step towards a clinical utilization.

Background: If a child cries with an asymmetrical mouth it can be a sign of a nerve compression or a developmental defect in a facial muscle. In the latter case, a 22q11 deletion can be the cause, and multiple organs might be involved., Case Description: A 2-month-old infant was referred to the paediatric outpatient clinic because he had an asymmetrical mouth when crying or laughing. There were no further symptoms. Genetic investigations and ultrasounds of the heart and kidneys were performed to exclude an underlying syndrome caused by a 22q11 deletion. These revealed no significant findings. It appeared to be a case of isolated 'asymmetric crying facies' as a result of hypoplasia of the depressor anguli oris muscle., Conclusion: An asymmetrical mouth in an infant can be a part of a genetic syndrome, in which there can be anomalies in multiple organ systems. For this reason, additional investigations are essential in cases of 'asymmetric crying facies'.

Aim of the Study: Assessment of the usefulness of intravital lesions in the proximal attachment of the sternocleidomastoid muscle and the mastoid process of the temporal bone in medico-legal evaluation of death by hanging., Material and Methods: The study material was obtained from the bodies of 35 people who died by hanging. The control group comprised specimens collected from 30 people who died of non-traumatic causes. The structures under study were examined macro- and microscopically. The basic change which could be recognized as a marker of intravitality of hanging was the presence of a macroscopically extensive blotchy area of abundant ecchymosis in the proximal muscle attachment, similar to that found in the distal attachment, and the presence of abundant diffuse intraosseous ecchymoses in the mastoid process., Results: None of the cases revealed any ecchymoses in the proximal attachment of the muscle that would be similar to those present in the distal attachment. Discolourations within the mastoid processes, macroscopically suggestive of extensive intraosseous effusions arising from the mechanism of stretching, were not confirmed by microscopic evaluation and occurred at the same frequency as in the control group. Limitations of the study were related to the method which involved sample collection by means of bone chisels, decalcification and preparation of specimens, which had an effect, for example, on the measurable evaluation of the degree of congestion., Conclusions: The study has failed to provide convincing and unambiguous data on the usefulness of examining mastoid processes and proximal attachments of the sternocleidomastoid muscles during autopsy to determine the presence of intravitality features of hanging. A description of research methodology and its associated difficulties, e.g. with the interpretation of results, can also be useful for the planning of similar studies by other researchers.

Temporomandibular disorders (TMD) affecting the articular disc and/or the facial muscles are common among the population, recording a higher incidence in women age 20-40 years. The aim of this study was to investigate the correlation between facial types and muscle TMD in women. This study comprised 56 women age 18 to 49 years, seeking treatment for TMD at the School of Medicine, Federal University of São Paulo. All of the study individuals were diagnosed with muscle TMD, based on the Research Diagnostic Criteria (RDC). Facial type was determined using the Facial Brugsch Index and classified as euryprosopic (short and/or broad), mesoprosopic (average width) and leptoprosopic (long and/or narrow). The data were submitted to the Chi-square test and ANOVA-Tukey's test to conduct the statistical analysis. The faces of 27 individuals were classified as euryprosopic (48%), 18 as mesoprosopic (32%), and 11 as leptoprosopic (20%). A statistically significant difference (Chi-square, p = 0.032) was found among the facial types, in that leptoprosopic facial types showed the lowest values for muscle TMD. A greater number (p = 0.0007) of cases of muscle TMD were observed in the 20 to 39 year-old subjects than in the subjects of other age segments. In conclusion, women with euryprosopic facial types could be more susceptible to muscle TMD. Further studies are needed to investigate this hypothesis.

Background: A quantitative numerical method for assessing the extent and duration of the inhibitory action of botulinum neurotoxin type A on mimetic muscles would potentially enable more detailed evaluation of the overall efficacy of this aesthetic treatment., Aim: To evaluate skin displacement analysis (SDA) as a tool for assessment of the extent and duration of effect of incobotulinumtoxinA on mimetic muscles in the glabellar region in routine daily practice., Methods: A total dose of 30 U incobotulinumtoxinA was injected into the fronto-glabellar region of 16 subjects. SDA was performed at baseline and during onset and recovery from incobotulinumtoxinA treatment at various timepoints up to 24 weeks after treatment. The reference point for SDA was located at the upper margin of the eyebrow, 30 mm lateral to the median line. To correlate and validate SDA results, semi-quantitative wrinkle score assessments were performed in parallel., Results: At baseline, the mean horizontal skin displacement was 5.4 mm (standard deviation: ± 1.4 mm). During onset of action after treatment, skin displacement values decreased from baseline (100%) by 46%, 85%, and 90% at day 2, week 1, and week 2, respectively. During recovery from incobotulinumtoxinA action, skin displacement values increased to 33%, 50%, and 93% of the baseline value at 6, 12, and 24 weeks after treatment, respectively. The inhibitory effect of incobotulinumtoxinA was highly variable among subjects, ranging from 25% to 68% of the baseline value 12 weeks after treatment. Overall agreement between SDA values and wrinkle scores was good., Conclusion: SDA represents a novel, objective method for the quantitative evaluation of the effect of incobotulinumtoxinA on mimetic muscles underlying the fronto-glabellar region, and showed good correlation with wrinkle score assessments. Both SDA and wrinkle score indicated the persistence of treatment effects 24 weeks after injection.

We report a case of cephalic tetanus which initially presented with acute lower motor neurone facial weakness. Tetanus is a rare diagnosis in the developed world but sporadic cases do occur. People born before 1960 in New Zealand are less likely to be immune. Judicious use of human tetanus immunoglobulin (TIG) and immunisation prevents the development of tetanus following injury and should always be considered in the elderly who are less likely to have immunity.

Background: For Asians, prominent zygomatic region is a sign of masculinity; therefore, reduction malarplasty is becoming more and more popular in Asian women., Objective: The purpose of this study is to analyze the biomechanical changes of zygomaticus and masseter, and to explore the underlying causes of relevant complications using the 3 popular techniques of reduction malarplasty., Methods: From May 2000 to August 2009, 259 female patients underwent traditional osteotomy through coronary incision, malomaxillary suture with L-shaped osteotomy through intraoral approach, and the modified X.M.'s technique. The mechanical and anatomical causes of the relevant complications such as facial asymmetry caused by detachment of zygomaticus and masseter, facial sagging, and downward movement of malar point in 3 surgical procedures were analyzed and compared postoperatively among the 3 procedures., Results: In the traditional osteotomy through coronary incision, the inside cheek fat pad moved interoinferiorly because the greater and lesser zygomatic muscles were stripped from the attachment points, whereas masseter was relatively less stripped from its attachment point, which affects mechanical direction and contraction strength less, which possibly leads to the downward movement of the fractured extremity of zygomatic bone. In the L-shaped osteotomy, the zygomatic arch slided interoinferiorly because of the operative design, and masseter's strong extroinferior traction often caused fixation loosening between the former and latter bone margin. As a result, the fractured zygomatic bone moved downward along with zygomaticus, masseter, and fat pad. In the modified X.M.'s technique, complications were relatively fewer, which may be related to less injury to the fixation system of zygomatic bone and less biomechanical changes in masseter and zygomatic muscles., Conclusions: Surgeons should base their choice of reduction malarplasty not only on thoroughness of the operation but also on biomechanical changes in the zygomatic region and patients' individual conditions.

Alveolar soft part sarcoma (ASPS) is a rare, histologically distinct, soft tissue malignancy with nonspecific clinical features usually described as a nonulcerated, painless, expanding mass. It has a pseudoalveolar appearance with clustered polygonal cells lacking central cohesion. It accounts for approximately 0.5 to 1% of all soft tissue sarcomas. It has a strong predilection for adolescents and young adults 15 to 35 years old, with a female predominance. In general, ASPS grows slowly, with a predilection for the trunk and extremities and rarely in the head and neck region. A literature review found only 11 cases of cheek ASPS that have been reported since 1952. This report describes the case of an unusually rapidly growing mass in the cheek of a 36-year-old woman. The superficial location of the mass led to early detection and treatment., (Copyright © 2014 American Association of Oral and Maxillofacial Surgeons. Published by Elsevier Inc. All rights reserved.)

Purpose: The objectives of this study were to evaluate the factors that contribute to aging in the lower eyelids and periorbital changes in Asians and to analyze the contribution of different anatomic factors before surgery., Methods: We analyzed photographs and examination findings of 108 patients older than 80 years who had consultations for lower eyelid blepharoplasty. Structural changes were categorized to 7 anatomical categories, and each category was scored from 0 to 3 based on severity. The total point for each category for all patients was divided by the 456 total possible points and was considered a cumulative contribution score for that category. "A uniqueness score" for each patient was calculated as the maximum score in 1 category divided by the patient's total score to reflect the percentage contribution of the patient's most severe anatomic problem., Results: A total of 108 cases (51 male and 57 female patients) were evaluated. The mean age was 57 ± 11 years (age range, 30-75 years). The cumulative contribution score for each category was as follows: orbital fat prolapse 33.8 (medial 15.3, central 11.7, lateral 6.8), skin laxity 22.3%, periorbital hollow 20.8 (tear trough 15.5, lateral orbital rim 5.3), septal confluence 9.5, orbicularis oculi muscle hypertrophy 3.6, triangular malar mound 3.4, and eyelid fluid 0.5. The average uniqueness score was 40.1%, and skin laxity and orbital fat prolapse had the highest average uniqueness score, but no single category played a dominant role in most patients. Medial orbital fat prolapse and skin laxity had the highest proportion of grade 3 scores of 9.5% and 6.7%, respectively. When the patients were grouped according to age, either younger than 50 years or older than 50 years, there was a prominent difference in orbital fat prolapse and skin laxity between the 2 groups., Conclusions: Various anatomic factors were related to periorbital changes in Asian patients. Understanding of the complex factors that contribute to periorbital changes during the aging process may allow for adequate and customized surgery for each patient and help in prevention of postoperative complications.

Ageing is a biological process that results from changes at a cellular level, particularly modification of mRNA. The face is affected by the same physiological process and results in skeletal, muscular, and cutaneous ageing; ligamentous attenuation, descent of fat, and ageing of the appendages. I describe these changes on a structural and clinical basis and summarise possible solutions for a rejuvenation surgeon., (Crown Copyright © 2013. Published by Elsevier Ltd. All rights reserved.)