Contribution of the Synkinesis Assessment Questionnaire and the Sunnybrook Facial Grading System to the evaluation of synkinesis after peripheral facial palsy: A STROBE observational study.

OBJECTIVES: The main aim of the study was to determine whether the perception of synkinesis by patients with peripheral facial palsy (PFP) matched their clinician's severity assessment. Secondary objectives comprised: (1) to determine whether objective measurement of synkinesis matched the patient's perception; and (2) is to identify factors influencing patients' perceptions. METHODS: This retrospective study took place from January to May 2020. Forty patients (8 per PFP grade, I-V/VI; 20 women, 20 men) filled out the Synkinesis Assessment Questionnaire (SAQ) and were assessed on the Sunnybrook Facial Grading System (SFGS). Photographs were analyzed on MEEI-Facegram software. RESULTS: Perceived synkinesis (total SAQ) matched objective grades (SFGS) (Z=2.89; P=0.004), especially for smiling (Z=3.84; P<0.001) and lip protrusion (Z=3.79; P<0.001). Synkinesis on lip protrusion was a more sensitive indicator of perceived synkinesis than synkinesis on smiling (Z=2.96; P=0.003). Duration (ρ=0.5137; P<0.001) and grade of PFP (Chi2=13.82; P=0.008) heightened the perception of synkinesis. CONCLUSION: Patient-reported outcome measures (PROMs) such as the SAQ are relevant for clinical evaluation.

Exploratory Study of the Brain Response in Facial Synkinesis after Bell Palsy with Systematic Review and Meta-analysis of the Literature.

BACKGROUND: Facial synkinesis, characterized by unintentional facial movements paired with intentional movements, is a debilitating sequela of Bell palsy. PURPOSE: Our aim was to determine whether persistent peripheral nerve changes arising from Bell palsy result in persistent altered brain function in motor pathways in synkinesis. DATA SOURCES: A literature search using terms related to facial paralysis, Bell palsy, synkinesis, and fMRI through May 2021 was conducted in MEDLINE and EMBASE. Additionally, an fMRI study examined lip and eyeblink movements in 2 groups: individuals who fully recovered following Bell palsy and individuals who developed synkinesis. STUDY SELECTION: Task-based data of the whole brain that required lip movements in healthy controls were extracted from 7 publications. Three studies contributed similar whole-brain analyses in acute Bell palsy. DATA ANALYSIS: The meta-analysis of fMRI in healthy control and Bell palsy groups determined common clusters of activation within each group using activation likelihood estimates. A separate fMRI study used multivariate general linear modeling to identify changes associated with synkinesis in smiling and blinking tasks. DATA SYNTHESIS: A region of the precentral gyrus contralateral to the paretic side of the face was hypoactive in synkinesis during lip movements compared with controls. This region was centered in a cluster of activation identified in the meta-analysis of the healthy controls but absent from individuals with Bell palsy. LIMITATIONS: The meta-analysis relied on a small set of studies. The small sample of subjects with synkinesis limited the power of the fMRI analysis. CONCLUSIONS: Premotor pathways show persistent functional changes in synkinesis first identifiable in acute Bell palsy.

Parálisis facial periférica recidivante como manifestación inicial del síndrome de Melkersson-Rosenthal / Recurrent peripheral facial palsy as an initial manifestation of Melkersson-Rosenthal syndrome

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Laryngeal Synkinesis: A Viable Condition for Laryngeal Pacing.

Laryngeal synkinesis as a form of defective healing is the rule rather than the exception in persistent vocal fold paralysis. It typically occurs 4-6 months after the onset of the recurrent laryngeal nerve paralysis. The incidence is up to 85%. Not all laryngeal muscles need to be equally affected. Reliable evidence can only be provided by a laryngeal electromyography. Physiological co-activation of the laryngeal muscles during antagonistic maneuvers must be considered. Although synkinesis undeniably worsens the prognosis for a motion recovery, it protects the muscle fibers from degeneration. A differentiation is required between favorable synkinesis (type I according to Crumley), which does not always require further therapy in the case of unilateral paralysis, and unfavorable forms of synkinesis (type II-IV) according to Crumley, which are associated with a functionally relevant malposition of the vocal fold(s) or with vocal fold jerks. Particularly when bilateral vocal fold motion does not return, type I synkinesis can be a good prerequisite for new dynamic therapy approaches, such as laryngeal pacing. The rarely occurring type II-IV synkinesis should, whenever possible, be transformed into a more favorable type I synkinesis by selective or non-selective reinnervation at an early stage of the disease. The latter applies to expected muscle atrophy with insufficient regrowth of nerve fibers.

Characteristics of a nationwide cohort of patients presenting with isolated hypogonadotropic hypogonadism (IHH).

OBJECTIVE: Isolated hypogonadotropic hypogonadism (IHH) is a rare disorder with pubertal delay, normal (normoosmic-IHH, nIHH) or defective sense of smell (Kallmann syndrome, KS). Other reproductive and non-reproductive anomalies might be present although information on their frequency are scanty, particularly according to the age of presentation. DESIGN: Observational cohort study carried out between January 2008 and June 2016 within a national network of academic or general hospitals. METHODS: We performed a detailed phenotyping of 503 IHH patients with: (1) manifestations of hypogonadism with low sex steroid hormone and low/normal gonadotropins; (2) absence of expansive hypothalamic/pituitary lesions or multiple pituitary hormone defects. Cohort was divided on IHH onset (PPO, pre-pubertal onset or AO, adult onset) and olfactory function: PPO-nIHH (n = 275), KS (n = 184), AO-nIHH (n = 36) and AO-doIHH (AO-IHH with defective olfaction, n = 8). RESULTS: 90% of patients were classified as PPO and 10% as AO. Typical midline and olfactory defects, bimanual synkinesis and familiarity for pubertal delay were also found among the AO-IHH. Mean age at diagnosis was significantly earlier and more frequently associated with congenital hypogonadism stigmata in patients with Kallmann's syndrome (KS). Synkinesis, renal and male genital tract anomalies were enriched in KS. Overweight/obesity are significantly associated with AO-IHH rather than PPO-IHH. CONCLUSIONS: Patients with KS are more prone to develop a severe and complex phenotype than nIHH. The presence of typical extra-gonadal defects and familiarity for PPO-IHH among the AO-IHH patients indicates a common predisposition with variable clinical expression. Overall, these findings improve the understanding of IHH and may have a positive impact on the management of patients and their families.

Relation between unimanual capacities and bimanual performance in hemiplegic cerebral-palsied children: impact of synkinesis.

OBJECTIVE: Analyze the link between unimanual capacities and bimanual performance in cerebral-palsied (CP) hemiplegic children, aged between 5 and 18 years old, studying specifically the impact of synkinesis. MATERIAL AND METHODS: 71 CP hemiplegic children (35 boys and 36 girls - with average age of 8 years and 6 months; MACS levels from I to III; GMFCS from I to IV) took part in a transversal study, assessed - Melbourne Test (MUUL) for unimanual capacities, and Assisting Hand Assessment (AHA) for bimanual performance - with a specific scale to analyze synkinesis during Box and Block test for affected and healthy hands, collecting synkinesis type, duration and intensity. RESULTS: There is a strong correlation between unimanual capacities (MUUL) and bimanual performance (AHA) (r = 0.871). Neither age nor gender contribute to bimanual performance (AHA). Multiple linear regression shows that MUUL contributes to bimanual performance variance (AHA) by 70%. Synkinesis is partly correlated to capacities (MUUL) and accounts for 10% of the variance of the gap between capacities and bimanual performance. CONCLUSION: A high relationship between unimanual capacities and bimanual performance is confirmed by this study; some authors demonstrated impact of sensory troubles, we demonstrate that synkinesis influences the use of unimanual capacities in bimanual performance.

Idiopathic oculostapedial synkinesis without history of facial nerve palsy.

OBJECTIVE: To report a case of idiopathic oculostapedial synkinesis without facial nerve disorder. PATIENT: A 30-year-old woman with tinnitus synchronous with eye closure is presented. The patient had no history of facial nerve disorder. RESULT: An impedance audiometer, in the absence of an auditory stimulus, was used to record tympanic membrane compliance without sound stimulation, revealing decreased compliance in the concomitant with eye blinking. Her symptoms disappeared spontaneously, so no intervention was undertaken. CONCLUSION: Although oculostapedial synkinesis is often observed as one of the sequelae of facial nerve palsy, idiopathic oculostapedial synkinesis is very rare. The use of an impedance audiometer in the absence of an auditory stimulus is very useful for demonstrating objective changes in the compliance of the tympanic membrane. It is assumed that the cause of the synkinesis in our case was abnormal transmission of signals for orbicularis oculi muscle to the stapedial and orbicularis oris muscles rather than misdirected regenerating fibers. Resection of the stapedial muscle tendon should be considered if her symptoms recur.

Stapedial synkinesis causing change in hearing threshold with facial motion.

OBJECTIVE: Synkinetic facial movement after facial nerve regeneration is a well-documented phenomenon. Rarely, patients recovering from facial nerve injury report feelings of auditory ringing, fullness, and a sensation of ear tightness as a result of stapedial muscle involvement. It is exceedingly rare for such synkinesis to produce perceivable changes in hearing threshold. We report a unique case of stapedial synkinesis causing pure-tone changes in hearing threshold with activation of the facial musculature. PATIENT: A single patient is presented who developed stapedial synkinesis after suboccipital resection of a unilateral acoustic neuroma. RESULTS: Despite facial nerve sparing, surgery resulted in House-Brackmann grade V/VI right facial nerve paralysis that improved to Grade III/VI after 7 months. Synkinesis developed that caused eye closure with puckering of the lips. Puckering of the lips likewise caused decreased hearing in the right ear, corresponding to a measured decrease of 10 dB in the PTA. Over the next several months, facial motion continued to improve, and hearing changes became less bothersome, so no intervention was undertaken. CONCLUSION: The changes presented in the hearing threshold fit within the classically described 15-dB attenuation provided by the stapedial reflex. Although no intervention was undertaken in this particular case, some patients with unremitting stapedial synkinesis might benefit from sectioning of the stapedial muscle. Thus, consideration should be made for audiometric evaluation with and without facial muscle contraction in the evaluation of individuals with synkinetic facial movement.

Bilateral Marcus Gunn jaw winking synkinesis with monocular elevation deficiency: a case report and literature review.

Marcus Gunn jaw winking synkinesis (MGJWS) occurs due to an aberrant innervation of the levator palpebrae superioris muscle by a branch of the motor division of the trigeminal nerve that supplies the muscles of mastication. MGJWS is mostly unilateral occurring in isolation and is less frequently associated with ocular or systemic abnormalities. Although MGJWS is mostly unilateral, few bilateral cases have been reported. Here we describe a rare case of bilateral MGJWS in an 18 year-old male patient with asymmetric bilateral ptosis and monocular elevation deficiency in the right eye.

Síndrome de Marin-Amat y Marcus-Gunn invertido. Presentación de 2 casos de sincinesia facial / Marin-Amat and inverted Marcus-Gunn syndrome. Two case report

El síndrome de Marin-Amat es una sincinesia trigémino-facial generalmente adquirida, debida a una regeneración aberrante del nervio facial que produce una contracción simultánea de orbicular de los párpados y los pterigoideos externos. Clínicamente, se manifiesta con blefarospasmo al abrir la boca o sonreír. Existen casos congénitos conocidos como fenómeno de Marcus-Gunn invertido por tener clínica inversa, ptosis palpebral con la apertura bucal, pero con la misma sincinesia (III-VII). Presentamos 2 casos clínicos, uno adquirido secundario a cirugía de una adenitis cervical tuberculosa y otro congénito, de forma gráfica y haciendo referencia a la forma de presentación, evolución y el tratamiento. Concluimos que es una entidad infrecuente en pediatría, con pocos casos descritos, aunque probablemente infradiagnosticada por su desconocimiento. El diagnóstico es esencialmente clínico y no precisa pruebas complementarias para su confirmación, si bien, la electromiografía puede ser de gran utilidad para demostrar la sincinesia (AU)

Marin-Amat’s syndrome is a rare associated movement, wherein contraction of or bicularisoculi is brought about by opening of the jaw in association with aberrant regeneration of facial nerve. This is the opposite of the Marcus-Gunn phenomenon; hence it has also been called Inverted Marcus-Gunn Phenomenon. Moreover in this case the opened the mouth or moved the jaw, the eye would close. This phenomenon is congenital and the closure of the eye is due to inhibition of the levator palpebrae superior is. We present two cases, one acquired after the surgery of tuberculosus cervical adenitis and other congenital with reference to the presentation, course and treatment. This entity is rare in children, with few reported cases, although probably will be found more frequently if looked for carefully. The diagnosis is clinical and does not require additional tests, although EMG may be useful to demonstrate the synkinesis (AU)

The novel concurrence of Noonan syndrome and bilateral Duane-like synkinesis.

A 9-year-old boy with Noonan syndrome was referred for evaluation of abnormal eye movements. Ocular motility examination revealed bilateral globe retraction with narrowing of the palpebral fissure in adduction and mild restriction of abduction consistent with a bilateral Duane-like synkinesis. This novel association expands the ocular phenotype of Noonan syndrome and may suggest a commonality in the pathogenesis of these two congenital malformation complexes.

A case with a rare type of trigemino-oculomotor synkinesis: clinical and blink reflex study.

Several abnormal synkinetic eye movements during jaw movements may often be seen after trauma or congenitally in the Marcus Gunn jaw winking phenomenon. The most frequent type consists of unilateral ptosis and retraction of the ptotic lid upon moving the jaw. The authors describe a case with isolated simultaneous adduction of the left eye upon jaw movement. This paper presents a rare case of Marcus Gunn jaw winking with trigemino-oculomotor synkinesis.

Unilateral true vocal fold synkinesis presenting with airway obstruction.

OBJECTIVES: We present a case series of 10 patients with unilateral true vocal fold paralysis who presented with airway obstruction. METHODS: A retrospective review of the authors' patients at 2 institutions with unilateral true vocal fold motion impairment was carried out over a 10-year period. Of these, 10 patients were identified who presented with stridor and dyspnea as a result of synkinesis. Six cases were a result of thyroidectomy, 1 case resulted from recurrent laryngeal nerve section for spasmodic dysphonia, 1 case occurred after anterior cervical diskectomy and fusion, and in 2 cases no cause was identified. RESULTS: Three patients underwent tracheotomy. Two patients underwent partial arytenoidectomy. Seven patients underwent botulinum toxin injection; 2 were treated with breathing therapy, and in 1 case breathing therapy was recommended. Seven patients underwent treatment with more than 1 method. CONCLUSIONS: Unilateral vocal fold paralysis may present with airway obstruction as a result of synkinesis. Treatment should be incremental and starts with breathing therapy and botulinum toxin injection. Partial arytenoidectomy or tracheotomy may be necessary for refractory cases.

Correction of congenital blepharoptosis in oculomotor-abducens synkinesis.

A 4-year-old boy was referred for blepharoptosis. Examination revealed the presence of oculomotor-abducens synkinesis (right upper eyelid elevation with right gaze). Reports of congenital oculomotor-abducens synkinesis syndromes are rare. Two surgeries required more levator resection than anticipated to obtain the first, to our knowledge, reported successful result in this condition.

Congenital oculomotor nerve synkinesis associated with fetal retinoid syndrome.

INTRODUCTION: Isotretinoin (RA), used for the treatment of cystic acne, is a powerful teratogen, causing craniofacial dysmorphisms and neural tube defects. We present two patients with RA embryopathy and oculomotor nerve synkinesis. METHODS: Retrospective review of patient records. RESULTS: Two patients presented with third nerve synkinesis and fetal RA exposure. Both had marked elevation of the upper eyelids on adduction such that the lid fissures alternately opened and closed on gaze from side to side. Both patients showed typical dysmorphisms of RA embryopathy. The first patient had complete agenesis of the cerebellar vermix and died at 2 years. The second patient had restricted extraocular muscles in one eye and was exotropic and hypotropic. DISCUSSION: Both patients demonstrated simultaneous innervation of the medial rectus and levator palpebrae muscles causing coincident lid elevation in adduction. This evidence of oculomotor nerve synkinesis is consistent with animal studies showing abnormalities in the formation of cranial nerve ganglia following fetal RA exposure. CONCLUSION: RA is a powerful teratogen. These patients provide additional clinical evidence of its influence on neural migration during early development.