Postparalytic Facial Syndrome Research Articles

Continual rehabilitation motivation of patients with postparalytic facial nerve syndrome

PurposeTo evaluate the continued rehabilitation motivation in patients with postparalytic facial synkinesis (PFS).MethodsIn this single-center cross-sectional survey, the multidimensional patient questionnaire for assessment of rehabilitation motivation (PAREMO-20) was used to assess the rehabilitation motivation. Associations Sunnybrook and Stennert index grading, Facial Clinimetric Evaluation (FaCE) survey, general quality of life (SF-36), Liebowitz Social Anxiety Scale (LSAS), Patient Health Questionnaire (PHQ)-9, technology commitment and affinity, and interest in further therapy were analyzed.Results69 adults with PFS (73% women; median age: 54 years) answered the survey. In comparison to prior treatment forms, there was a significant higher future interest in computer-based home facial training (p < 0.0001). For PAREMO Psychological burden subscore, SF36 Emotional role was the highest negative correlative factor (p < 0.0001). For PAREMO Physical burden subscore, SF-36 General health was the highest negative correlative factor (p = 0.018). Working (p = 0.033) and permanent relationship (p = 0.029) were the only independent factors correlated to PAREMO Social Support Subscore. Higher positive impacts of technology affinity was inversely correlated to PAREMO Knowledge subscore (p = 0.017). Lower SF-36 Role physical subscore p = 0.045) and a lower SF-36 General health (p = 0.013) were correlated to a higher PAREMO Skepticism subscore.ConclusionsPatients with PFS seem to have a high facial motor and non-motor psychosocial impairment even after several facial therapies. Rehabilitation-related motivation increases with both, higher facial motor and non-motor dysfunction. Social and emotional dysfunction are drivers to be interested in innovative digital therapy forms.

Role of Body Dysmorphic Disorder in Patients With Postparalytic Facial Synkinesis.

To evaluate the role of body dysmorphic disorder (BDD) in patients with postparalytic facial nerve syndrome with synkinesis (PFS). A single-center retrospective cohort study. A total of 221 adults (74% women; median age: 44 years; median duration since onset of facial palsy: 1.6 years) were included. To diagnose BDD, the BDD Munich Module was used. Associations with House-Brackmann grading, Stennert index grading, Facial Clinimetric Evaluation (FaCE) survey, Facial Disability Index (FDI), general quality of life (SF-36), Beck Depression Inventory (BDI), and the Liebowitz Social Anxiety Scale (LSAS) was analyzed. A total of 59 patients (27%) were classified as patients with BDD. Significant associations were found between the diagnosis of BDD and female gender and lower FDI, FaCE, and SF-36 scores and higher BDI and LSAS scores. Multivariate analysis revealed BDI, FaCE total score, and FaCE social function subscore as independent factors associated with BDD. BDD was a relevant diagnosis in patients with PFS. A higher BDD level was associated with general and facial-specific quality of life and more psychosocial disabilities. Optimal treatment of PFS has to include these nonmotor dysfunctions. 3 Laryngoscope, 131:E2518-E2524, 2021.

Evaluation of Societal Health Utility of Facial Palsy and Facial Reanimation

The severity of a health state may be quantified using health utility measures. The utility of flaccid unilateral facial paralysis and unilateral moderate to severe postparalytic facial nerve syndrome with synkinesis may be challenging to discern from photographs alone. To determine the societal health utility of flaccid unilateral facial paralysis, unilateral moderate to severe postparalytic facial nerve syndrome, and post-facial reanimation using standard video. This survey study was conducted at the Massachusetts Eye and Ear and the Harvard Decision Science Laboratory from June 14, 2017, to August 3, 2017. Healthy adult naïve observers were recruited through advertising in the Cambridge, Massachusetts, area. Participants (n = 298) completed the web-based, interactive survey in person. The survey comprised clinical vignettes consisting of symptom summaries, videos, and pictures depicting 5 health states. Adult naïve observers ranked the utility of 5 randomized health states (flaccid unilateral facial paralysis, unilateral moderate to severe postparalytic facial nerve syndrome, post-facial reanimation, monocular blindness, and binocular blindness) according to the visual analog scale (VAS), standard gamble (SG), and time trade-off (TTO) measures. Standard videos of patients' facial function were used. In total, 377 naïve observers were recruited and completed the survey in its entirety. Of the 377 participants, 298 (79.0%) were included for analysis. Among the 298 participants, 151 (50.7%) were female, 146 (49.0%) were male, with a mean (SD) age of 33.0 (15.1) years. No differences in health utility scores (SD) were observed between flaccid unilateral facial paralysis and unilateral moderate to severe postparalytic facial nerve syndrome (VAS: 0.598 [0.213] vs 0.629 [0.207]; SG: 0.714 [0.245] vs 0.748 [0.237]; TTO: 0.716 [0.248] vs 0.741 [0.247]). Both health states rated substantially worse than monocular blindness (VAS: 0.691 [0.212]; SG: 0.817 [0.204]; TTO: 0.826 [0.196]) and post-facial reanimation (VAS: 0.742 [0.189]; SG: 0.833 [0.206]; TTO: 0.838 [0.19]). Health utility scores for flaccid unilateral facial paralysis and unilateral moderate to severe postparalytic facial nerve syndrome appeared to be equivalent and worse than that for monocular blindness, whereas scores for post-facial reanimation were substantially higher than the scores for the 2 facial movement disorders. These findings may provide insights into the societal advantages of facial reanimation surgery. NA.

Bilateral Facial Spasm Following Guillain–Barré Syndrome

<strong>Background:</strong> We report a patient who developed lower facial muscle spasm at rest and bilateral facial synkinesis several months after treatment of Guillain–Barré syndrome (GBS); this finding, to our knowledge, is hitherto unreported. <strong>Phenomenology Shown:</strong> Bilateral synkinesis, facial muscles spasm at rest, bilateral postparalytic facial syndrome. <strong>Educational Value:</strong> Aberrant regeneration of nerve fibers post GBS, resulting in facial muscles spasm at rest, bilateral synkinesis.

Reorganization of sensory input at brainstem in hemifacial spasm and postparalytic facial syndrome.

We hypothesized the filtering of sensory input from face and hand at brainstem may reorganize in hemifacial spasm (HFS) and postparalytic facial syndrome (PFS). Thus, we examined the prepulse inhibition of blink reflex (BR-PPI) in HFS and PFS. We included 12 healthy subjects, 13 patients with HFS, and 11 patients with PFS. Baseline BR, BR recovery at interstimulus interval (ISI) of 300ms and BR-PPI at ISI of 100ms were performed on the right sides of healthy subjects and on both sides of patients. Within-subject analysis showed baseline BR and BR-PPI were similar between asymptomatic and symptomatic sides of patients with HFS whereas BR recovery was higher on the symptomatic side. In the PFS group, latency of R2 during baseline BR recording was longer (p=0.022) and R2 amplitude (p=0.046) was reduced on the symptomatic side compared to asymptomatic side. Reduction of R2 area in BR-PPI recordings was also the lowest in HFS compared to other two groups (p=0.000); however, it was also lower in patients with PFS compared to healthy subjects (p=0.018). BR-PPI was decreased on both sides of patients. The mean R2 recovery was higher on both sides of patients with HFS and PFS (p=0.007). Filtering of facial sensory input is decreased probably to monitor and to correct the sequence of facial movements in these disorders.

Role of the “other Babinski sign” in hyperkinetic facial disorders

BackgroundThe “other Babinski sign” consists in the co-contraction of the orbicularis and frontalis muscles, causing an eyebrow elevation during ipsilateral eye closure. It cannot be voluntarily reproduced. Aims of the studyTo determine the utility of this sign in the differential diagnosis of hyperkinetic facial disorders. MethodsThe presence of the sign was assessed in consecutive patients with blepharospasm, primary hemifacial spasm or post-paralytic facial syndrome treated in a botulinum toxin outpatient clinic. ResultsOf the 99 patients identified, 86 were included, 41 with blepharospasm (32 female, mean age 71±11years), 28 with hemifacial spasm (16 female, mean age 65±12years) and 17 with post-paralytic facial syndrome (14 female, mean age 50±17years). The sign was detected in 67.9% of the patients with hemifacial spasm, in 23.5% of the post-paralytic facial syndrome group and in none of the patients with blepharospasm, exhibiting a sensitivity of 51% and a specificity of 100% for the diagnosis of hemifacial spasm/post-paralytic facial syndrome and a specificity of 76% for hemifacial spasm, compared to post-paralytic facial syndrome. ConclusionsThis sign is highly specific for the diagnosis of peripherally induced hyperkinetic facial disorders. Its assessment should integrate the routine examination of patients with abnormal facial movements.

An excitatory reflex between R1 and R2 responses of the blink reflex to supraorbital nerve stimuli

Background The typical pattern of the blink reflex to ipsilateral electrical stimuli to the supraorbital nerve (SN) is composed by an early, short duration, well synchronized, R1 response and a late, long duration, polyphasic R2 response. We examined whether the period between R1 and R2 results from lack of excitatory inputs or active inhibition. Methods Tests were performed in 5 healthy subjects and 10 patients of various disorders affecting blink reflex excitability, including postparalytic facial syndrome and hemifacial spasm. All subjects wore surface electrodes attached to the orbicularis oculi and stimuli were applied to the SN. After obtaining the conventional blink reflex, healthy subjects were requested to close their eyes at various levels of force while applying again the same stimulus. Results In healthy subjects closing the eyes with force, SN stimulation induced a response of smaller amplitude and shorter latency than the R2 (25.2 ± 1.3 ms). A short and incomplete silent period emerged after R1 and after R2. Patients with postparalytic facial syndrome and essential hemifacial spasm showed activity interfering with the silence between R1 and R2, with often expanding between the two responses, repeating in successive trials. Conclusions Our results show that there can be reflex responses between R1 and R2 in healthy subjects during contraction and in patients with abnormal reflex excitability. This suggests that the absence of activity between the two responses in normal conditions is the consequence of an active inhibition that reaches the facial motoneurons at that point.

Blink reflex studies in postparalytic facial syndrome and blepharospasm: trigeminal and extratrigeminal somatosensory stimulation.

The somatosensory-evoked blink reflex (SBR) is one of the release phenomena of blink reflex, possibly resulting from increased excitability of brainstem reticular formation. The authors investigated trigeminal blink responses and SBR in 26 patients with postparalytic facial syndrome (PFS) with synkinesia, 18 patients with essential blepharospasm, and 36 healthy volunteers (control participants). Trigeminal blink reflex responses were elicited in all participants, whereas SBRs were elicited in 44.4% of control participants, 38.9% of patients with essential blepharospasm, and 65.4% of patients with PFS. The mean R2 amplitude and duration and the mean amplitude and duration of SBR were highest in patients with essential blepharospasm. The mean latency of SBR was shorter on the symptomatic side of patients with PFS when compared with the asymptomatic side. The mean R2 duration on the symptomatic side of the patients with PFS was longer than the control participants. These results showed that somatosensory stimulation could be used as an alternative method to demonstrate increased excitability in facial motor neuron in patients with PFS and essential blepharospasm. Disease states relating to different peripheral and/or suprasegmental structures could also influence blink reflex and change its basal excitability and manner in which the reflex responds to modulatory factors.

Effect of Endoscopic Brow Lift on Contractures and Synkinesis of the Facial Muscles in Patients with a Regenerated Postparalytic Facial Nerve Syndrome

Delayed recovery after facial palsy results in aberrant nerve regeneration with symptomatic movement disorders, summarized as the postparalytic facial nerve syndrome. The authors present an alternative surgical approach for improvement of periocular movement disorders in patients with postparalytic facial nerve syndrome. The authors proposed that endoscopic brow lift leads to an improvement of periocular movement disorders by reducing pathologically raised levels of afferent input. Eleven patients (seven women and four men) with a mean age of 54 years (range, 33 to 85 years) and with postparalytic facial nerve syndrome underwent endoscopic brow lift under general anesthesia. Patients' preoperative condition was compared with their postoperative condition using a retrospective questionnaire. Subjects were also asked to compare the therapeutic effectiveness of endoscopic brow lift and botulinum toxin type A. Mean follow-up was 52 months (range, 22 to 83 months). No intraoperative or postoperative complications occurred. During follow-up, patients and physicians observed an improvement of periorbital contractures and oculofacial synkinesis. Scores on quality of life improved significantly after endoscopic brow lift. Best results were obtained when botulinum toxin type A was adjoined after the endoscopic brow lift. Patients described a cumulative therapeutic effect. These findings suggest endoscopic brow lift as a promising additional treatment modality for the treatment of periocular postparalytic facial nerve syndrome-related symptoms, leading to an improved quality of life. Even though further prospective investigation is needed, a combination of endoscopic brow lift and postsurgical botulinum toxin type A administration could become a new therapeutic standard.

Blink reflex in patients with postparalytic facial syndrome and blepharospasm: Trigeminal and auditory stimulation

ObjectiveThe enhancement of blink reflex (BR) excitability was shown in patients with postparalytic facial syndrome (PFS) and essential blepharospasm (EB). We prospectively investigated patients with PFS and EB whether BR alterations demonstrated by trigeminal stimulation will similarly be observed upon auditory stimulation. MethodsFifteen patients with PFS, 15 patients with EB, and 30 healthy volunteers were involved. Electrically stimulated trigeminal BR and auditory BR were studied bilaterally. ResultsThe mean R2 amplitude and duration values were highest in EB patients, being significantly higher than PFS patients (p<0.05) and control group (p<0.01). The mean R2 duration in PFS patients were also significantly longer in compared to control group (p=0.025). EB patients showed a higher mean R (auditory) amplitude and duration than PFS patients (p<0.05) and controls (p<0.04). The mean R (auditory) duration was longer on symptomatic side of PFS patients in compared to controls (p=0.05). ConclusionsWe observed that there is an enhanced excitability of BR circuit in postparalytic facial syndrome and essential blepharospasm, which could be evoked by auditory stimulation in addition to trigeminal stimulation. SignificanceThe enhanced excitability in patients with EB and PFS probably originates from the final common pathway of BR circuit, namely facial motor or premotor neurons.

Auditory evoked blink reflex and posterior auricular muscle response: Observations in patients with HFS and PFS

Our goal was to investigate the characteristics of the auditory brainstem reflexes in patients with hemifacial spasm (HFS) and postparalytic facial syndrome (PFS). The spasm activities and responses by supraorbital and auditory stimuli were recorded from the orbicularis oculi, the posterior auricular and the mentalis muscles in 27 HFS and 21 PFS patients. The results were compared with those of 20 controls. Blink reflex (BR) was obtained by supraorbital stimulation in normal controls and on both sides of HFS and PFS patients whereas sound evoked bilateral auditory blink reflex (ABR) in 96.3%, 90.5% and 100%, respectively. Both BR and ABR showed synkinetic spread on symptomatic sides in all patients. The posterior auricular muscle response (PAMR) was observed bilaterally in 59.3%, 42.9% and 75.0% of groups, respectively. However, there was no synkinetic spread of PAMR. Since PAMR does not show synkinetic spread even in the presence of synkinetic spread of ABR and BR, we may suggest that a distal origin may be responsible of the synkinetic spread, or PAM is probably governed by a smaller nucleus in the brainstem. Thus it may be speculated that its excitability is insufficient to stimulate the ABR nucleus, whereas the reverse process is possible.

Somatosensory eye blink reflex in peripheral facial palsy

To investigate the association between somatosensory blink reflex (SBR) and peripheral facial palsy (PFP) severity and trigeminal blink reflex (BR) changes in cases with PFP and subsequent postparalytic facial syndrome development (PFS). One hundred and twenty subjects with peripheral facial palsy and post-facial syndrome and 44 age and gender matched healthy volunteers were enrolled to this study. Blink reflexes and somatosensory blink reflex were studied in all. The association between R1 and R2 responses of the BR and SBR positivity was investigated. SBR was elicited in 36.3% of normal subjects, in 18.3% of PFP and in 65.3% of PFS patients. In the paralytic side, the frequency of SBR positivity was significantly lower in PFP group compared to controls and SBR was most frequently observed in patients with PFS. Compared to PFP and control groups, SBR positivity on the non-paralytic side significantly revealed a higher rate in PFS patients. SBR positivity of patients in whom R1 or R2 were absent, was significantly lower than those subjects with prolonged or normal R1 or R2 responses. PFP and successive PFS are good models for the sensory motor gate mechanisms and/or excitability enhancement of brainstem neurons responsible for SBR.

P01. Auditory and electrically evoked brain stem reflexes in hemifacial spasm and postparalytic facial syndrome

P01. Auditory and electrically evoked brain stem reflexes in hemifacial spasm and postparalytic facial syndrome

Electrodiagnostic studies of the facial nerve in peripheral facial palsy and hemifacial spasm

Electrodiagnostic (EDX) assessment is one of the most important aspects in the evaluation of the two most common disorders of the facial nerve: facial palsy and hemifacial spasm. Facial palsy is usually an acute disorder that resolves in a few weeks but, in a number of cases, leads to a postparalytic facial syndrome featuring muscle synkinesis, myokymia, and involuntary mass contractions of muscles on the affected side. Hemifacial spasm is usually a chronic disorder characterized by paroxysms of involuntary, clonic, and synchronous twitching of all facial muscles on the affected side. EDX studies provide information on lesion location and severity, pathophysiology underlying the two disorders, and differential diagnosis between syndromes presenting with abnormal facial muscle activity. This monograph is intended to describe the most relevant EDX findings in the two disorders and the most appropriate timing for the examinations in order to provide useful information for prognosis and therapeutic decision-making.

Movement disorders in patients with peripheral facial palsy.

Acute unilateral facial paralysis is usually a benign neurological condition that resolves in a few weeks. However, it can also be the source of a transient or long-lasting severe motor dysfunction, featuring disorders of automatic and voluntary movement. This review is organized according to the two most easily recognizable phases in the evolution of facial paralysis: (1). Just after presentation of facial palsy, patients may exhibit an increase in their spontaneous blinking rate as well as a sustained low-level contraction of the muscles of the nonparalyzed side, occasionally leading to blepharospasm-like muscle activity. This finding may be due to an increase in the excitability of facial motoneurons and brainstem interneurons mediating trigeminofacial reflexes. (2). If axonal damage has occurred, axonal regeneration beginning at approximately 3 months after the lesion leads inevitably to clinically evident or subclinical hyperactivity of the previously paralyzed hemifacial muscles. The full-blown postparalytic facial syndrome consists of synkinesis, myokymia, and unwanted hemifacial mass contractions accompanying normal facial movements. The syndrome has probably multiple pathophysiological mechanisms, including abnormal axonal branching after aberrant axonal regeneration and enhanced facial motoneuronal excitability. Although the syndrome is relieved with local injections of botulinum toxin, fear of such uncomfortable contractions may lead the patients to avoid certain facial movements, with the implications that this behavior might have on their emotional expressions.

Facial palsy, postparalytic facial syndrome, and hemifacial spasm.

Keywords: facial nerve; facial palsy; postparalytic facial syndrome; essential hemifacial spasm; blink reflex; brainstem