Development of the Alberta facial clinical evaluation scale: A patient-centred outcomes instrument for facial nerve paralysis.

OBJECTIVES: Facial nerve paralysis has functional, psychological and social consequences for patients. Traditionally, outcome measurements for facial nerve injuries have been clinician derived. Recent literature has shown that patient perspective is valuable and necessary in outcomes research. This study aimed to identify patient domains of concern and subsequently, develop a point-of-care questionnaire for clinical use. DESIGN: This mixed-methods prospective study was completed in three phases. In Phase I, 15 facial nerve injury patients were interviewed individually. Interviews were digitally recorded, transcribed and coded with NVivo software. Analysis led to a conceptual framework detailing the most important quality of life outcomes. During Phase II, a focus group was held with five new patients in order to prioritise the outcome themes to a top six list. A second focus group was held with Otolaryngology-Head and Neck staff surgeons to create a 25-item questionnaire based on these six themes. In Phase III, the questionnaire was administered to 10 new patients to test for comprehension. SETTING: University of Alberta Hospital, Edmonton, Alberta, Canada. PARTICIPANTS: A total of 30 patients with facial nerve injury were included in the various phases in the study. In addition, 5 staff Otolaryngology-Head and Neck surgeons participated as focus group contributors. MAIN OUTCOME MEASURES: Domains of concern and quality of life outcomes as reported by facial nerve injury patients. RESULTS: Patients identified a total of 16 themes encompassing both functional and psychological deficits related to their facial nerve injury. From these findings, a 25-item Likert-type scale, the A-FaCE scale, was developed for clinical use. CONCLUSIONS: Patients with facial nerve paralysis experience functional and psychological deficits. This study led to the creation of the first patient-reported instrument for this population that addresses functional impairment, social function, psychological well-being and self-perception of appearance.

DEVELOPMENT OF GANGLIONOPATHY AND TABETIC VISCERAL CRISES ON THE BACKGROUND OF POLYRADICULONEUROPATHY ASSOCIATED WITH MONOCLONAL GAMMOPATHY (CASE REPORT).

The article presents an analysis of the clinical occurrence of development of chronic polyradiculoneuropathy associated with monoclonal IgG/k (kappa) gammopathy of the undetermined significance. The peculiarity of this occurrence is the uniqueness of the development of the symptoms which are characteristic of tabes dorsalis in this pathology with episodic severe visceral crises and also with ganglionopathy. The example describes the clinical polymorphism of the course of visceral crises, the problems of their diagnosis and as a consequence of inadequate treatment with the development of severe social maladaptation. The importance of timely diagnosis and treatment of such conditions is discussed.

Neuro-ophthalmology in the Horse.

This article provides a brief, clinically relevant review of neurologic disorders of the eye. A description of the neuro-ophthalmic examination is provided. Stepwise descriptions of the most common neuro-ophthalmic abnormalities are provided along with common rule outs.

IgG4-related disease causing facial nerve and optic nerve palsies: Case report and literature review.

IgG4-related disease (IgG4-RD) is increasingly being recognized as an entity effecting the head and neck region. Although most commonly seen with salivary gland or paranasal sinus involvement, IgG4-RD may also involve the temporal bone and skull base. We report a rare care of a 61-year-old female with IgG4-RD presenting as synchronous lesions of the middle ear and middle cranial fossa with polyneuropathy of cranial nerves II, VI, and VII. Initial histopathological evaluation of her resected ear mass suggested a benign inflammatory process but no specific diagnosis. Her symptoms progressed over 10months prompting re-evaluation of the specimen and consideration of the IgG4-RD diagnosis. Key pathologic features included prominent lymphoplasmacytic population, storiform fibrosis, obliterative phlebitis, and IgG4 specific staining. The patient was treated with high-dose intravenous and oral steroids but was transitioned to azathioprine secondary to steroid-induced myopathy. Radiographic studies before and after treatment reveal marked improvement of the intracranial and extracranial disease. Correspondingly, her cranial neuropathies resolved. A high degree of clinical suspicion is necessary to diagnosis IgG4-RD. The diagnosis can be supported by elevated serum IgG, elevated IgG index, and pathognomonic histopathological findings. Primary treatment is with corticosteroids. However, immunotherapy using azathioprine or rituximab can be utilized in recurrent disease or patients with steroid intolerance.

Basilar skull fractures: the petrous bone.

Basilar skullfractures: the petrous bone. OBJECTIVES: to provide suggestions for the management of three of the most dangerous or important lesions (internal carotid artery lesions, cerebrospinal fluid leaks and facial nerve paralysis) associated with the petrous part of basilar skull fractures, thereby trying to assess categories of evidence and determine strengths of recommendation. METHODOLOGY: A PubMed-based literature review was carried out, as well as a consultation of online sources as encountered in the literature review. Also, a non-systematic search of chapters of well-known books dealing with the subject of temporal bone traumata was conducted. RESULTS: Specific levels of evidence and/or strength of recommendation can be retrieved from the literature, but only with respect to the prophylactic use of antibiotics, the prescription of antithrombotic medications and the indications for angiography. CONCLUSION: The ample amount of available literature allows for sound management decisions, with reference made to algorithms when available in the literature. Nevertheless, for most of the management/search questions, categories of evidence and strength of recommendation are low or lacking.

The Therapeutic Effect of Stellate Ganglion Block on Facial Nerve Palsy in Patients with Type 2 Diabetes Mellitus.

BACKGROUND/AIMS: The conventional systemic corticosteroid treatment for acute peripheral facial nerve palsy in patients with type 2 diabetes mellitus can induce hyperglycemia, and an alternative local therapy may be necessary. Our purpose in this study is to evaluate therapeutic effects of stellate ganglion block (SGB) on facial nerve palsy in patients with type 2 diabetes mellitus. METHODS: A total of 361 cases of acute peripheral, chronic peripheral, acute central and chronic central facial nerve palsy treated with SGB or conventional therapy were included in this retrospective study. The facial nerve function score (Sunnybrook Facial Grading System) obtained at before and after treatment in non-SGB and SGB groups was used to assess the outcome. Furthermore, the blood glucose level in acute peripheral facial nerve palsy was measured. RESULTS: The facial nerve function score in the SGB group was higher than that in the non-SGB group after treatment in peripheral facial nerve palsy, while the blood glucose level in the non-SGB group increased and was higher than that in the SGB group during the treatment in acute peripheral facial nerve palsy. CONCLUSIONS: Our findings suggest that SGB has better therapeutic effect than conventional treatment on acute and chronic peripheral facial nerve palsy in patients with type 2 diabetes mellitus.

Facial nerve stimulation associated with cochlear implant use following temporal bone fractures.

OBJECTIVE: To describe the incidence and management of patients with facial nerve stimulation (FNS) associated with cochlear implant (CI) use in the setting of a prior temporal bone fracture. PATIENTS: One adult CI recipient is reported who experienced implant associated FNS with a history of a temporal bone fracture. Additionally, a literature search was performed to identify similar patients from previous descriptions of CI related FNS. MAIN OUTCOME MEASURES: Presence of FNS after implantation and ability to modify implant programming to avoid FNS. RESULTS: The patient in the present report experienced FNS for middle and basal electrodes during intraoperative neural response telemetry (NRT) in the absence of any surgical exposure or manipulation of the facial nerve. FNS was absent during device activation, but it recurred during follow-up programming sessions. However, additional programming has prevented further FNS during regular implant use. Four other patients with FNS after temporal bone fracture were identified from the literature, and the present case represents the one of two cases in which reprogramming allowed for implant use without FNS. CONCLUSIONS: CI associated FNS is uncommon in patients with a history of a temporal bone fracture, but it is likely that fracture lines provide a lower impedance pathway to the adjacent facial nerve and thus reduce the threshold for FNS. The present report suggests that, in the setting of a prior temporal bone fracture, NRT is not always a reliable predictor of FNS during implant use, and programming changes can help to mitigate FNS when it occurs.

Eight and a half syndrome with hemiparesis and hemihypesthesia: the nine syndrome?

"Eight-and-a-half" syndrome is "one-and-a-half" syndrome (conjugated horizontal gaze palsy and internuclear ophthalmoplegia) plus ipsilateral fascicular cranial nerve seventh palsy. This rare condition, particularly when isolated, is caused by circumscribed lesions of the pontine tegmentum involving the abducens nucleus, the ipsilateral medial longitudinal fasciculus, and the adjacent facial colliculus. Its recognition is therefore of considerable diagnostic value. We report a 71-year-old man who presented with eight and a half syndrome associated with contralateral hemiparesis and hemihypesthesia, in which brain magnetic resonance imaging scans revealed a lacunar pontine infarction also involving the corticospinal tract and medial lemniscus. These features could widen the spectrum of pontine infarctions, configuring a possible "nine" syndrome.

Progress in treatment of facial neuritis by acupuncture combined with medicine from the perspective of modern medicine: A review.

Facial neuritis is a common clinical disease with high incidence, also known as Bell palsy or idiopathic facial nerve paralysis, which is an acute onset of peripheral facial neuropathy. In modern medicine, there have been obstacles to the effective treatment of facial neuritis. At present, the clinical use of Western medicine treatment is also a summary of clinical experience, the reason is that the cause of facial neuritis is unknown. Facial neuritis belongs to the category of "facial paralysis" in traditional Chinese medicine. For thousands of years, Chinese medicine has accumulated a lot of relevant treatment experience in the process of diagnosis and treatment. At the same time, traditional Chinese medicine, acupuncture and the combination of acupuncture and medicine play an important role in the treatment of facial neuritis. This article discusses the treatment of facial neuritis with acupuncture combined with Chinese medicine, based on the research progress of modern medicine. In this review, we provide an overview of the effectiveness of acupuncture and medication combinations and facial neuritis with current studies investigating acupuncture and medication combinations in the treatment of facial neuritis.

Amphiphysin-IgG autoimmune sciatic neuropathy and facial neuropathy related to primary central nervous system lymphoma: A case report.

We reported a 61-year-old man presented with 10-month progressing left sciatic neuropathy and 10-day right facial neuropathy. Serum amphiphysin-IgG was positive. 18F-FDG PET/CT of the whole body showed no signs of malignancy. Treatment with plasma exchange and oral prednisone relieved the symptoms. Nine months later, right hemiparesis and seizure of right limbs developed. 18F-FDG and 18F-PBR06 (18 kDa translocator protein, TSPO) radioligand PET/MRI of the whole body revealed intense uptake in the intracranial lesions. Intracranial lymphoma was diagnosed by stereotactic needle brain biopsy. Mononeuropathies could be paraneoplastic syndromes. TSPO shows high uptake in intracranial lymphoma on 18F-PBR06 PET images.

Eye exercises for treatment of idiopathic cranial nerve VII paresis: pilot study.

BACKGROUND: To determine if fine-motor eye exercises can be used for treatment of unilateral, idiopathic cranial nerve VII paresis to improve rate of recovery. METHODS: In this prospective, randomized controlled trial, eligible patients were randomized to perform fine-motor eye exercises (n=18) or to do no exercise (n=9) for a period of four weeks. Orbicularis oculi muscle strength was measured in paretic and unaffected eyes at baseline, two weeks and four weeks using an Orbicularis Oculi Pressure Sensor. RESULTS: The average initial strength of the paretic orbicularis oculi muscle was 34±10 mm Hg compared to the unaffected muscle which was 103±17 mm Hg at baseline (n=27). By four weeks, patients who performed eye exercises improved more than those who did not (74.4 versus 47.4 mm Hg, p=0.029). While there was some loss to follow-up, 63.8% of patients performing exercises (7/11) achieved functional recovery at four weeks compared to 12.5% (1/8) of those who did not (p=0.059). Steroids and antivirals were found to have independent positive effects on improving functional outcome. CONCLUSIONS: Eye exercises have a potential role in the treatment of idiopathic cranial nerve VII paresis and warrant consideration in the management of these patients.

Effectiveness of acupuncture in treatment of facial spasm: a meta-analysis.

CONTEXT: Facial spasm is one of the common facial diseases, especially in the aged. It is mostly characterized by initially progressive, involuntary, irregular, recurrent, clonic, or tonic movements of muscles innervated by the facial nerve on one side. Acupuncture is a low-risk treatment with purported claims of effectiveness for facial spasm. OBJECTIVE: To assess the efficacy of acupuncture in facial spasm comprehensively. DESIGN: The research team conducted a systematic review and meta-analysis of all randomized clinical trials (RCTs) that examined the effectiveness of acupuncture for facial spasm. OUTCOME MEASURE(S): The research team categorized results from each of the reviewed studies in two ways: (1) the number of participants who showed a positive response to therapy (total effectiveness rate) and (2) the number of participants who made a full recovery (clinical cure rate). RESULTS: The research team reviewed a total of 13 studies involving 1262 participants with facial spasm. Researchers in China had conducted all studies, and most studies were poor in methodological quality. All studies reported that acupuncture was superior to other treatments, including carbamazepine, mecobalamin, and massage, and the meta-analysis on these low-quality studies yielded similar results. CONCLUSION: Present trials evaluating the efficacy of acupuncture in treatment of facial spasm are mostly poor in methodological quality. These studies showed that acupuncture was superior to other treatments for facial spasm; however, in its meta-analysis, the research team could not draw an affirmative conclusion as to the benefits of acupuncture due to the poor methodological quality and localized population of the included trials. The field needs large international, well-conducted RCTs.

Psychometric Validation of the FACE-Q Craniofacial Module for Facial Nerve Paralysis.

Background: Systematic reviews have identified the need for a patient-reported outcome measure for facial nerve paralysis (FNP). The aim of this study was to determine the psychometric properties of FACE-Q Craniofacial module scales when used in a combined sample of children and older adults with FNP. Methods: Data were collected between December 2016 and December 2019. We conducted qualitative interviews with children and adults with FNP. FACE-Q data were collected from patients aged 8 years and older with FNP. Rasch measurement theory analysis was used to examine the reliability and validity of the relevant scales in the FNP sample. Results: Twenty-five patients provided 2052 qualitative codes related to appearance, physical, psychological, and social function. Many patient concerns were common across age. The field-test sample included 235 patients aged 8-81 years. Of the 13 scales examined, all 122 items had ordered thresholds and good item fit to the Rasch model. For 12 scales, person separation index values were ≥0.79 and Cronbach's alpha values were ≥0.82. The 13th scale's reliability values were ≥0.71. Conclusion: The FACE-Q Craniofacial module scales described in this study can be used to collect and compare evidence-based outcome data from children and adults with FNP.

Gradenigo's syndrome--surgical management in a child.

Otits media is a common problem. Some of its complications that were seen frequently in the preantibiotic era are rare today. We report a case of an 8 year boy who presented with earache, retro-orbital pain and diplopia secondary to a sixth nerve palsy--Gradenigo's syndrome. In this syndrome infection from the middle ear spreads medially to the petrous apex of the temporal bone. Work-up includes CT scan of the temporal bones. Timely management with intravenous antibiotics (+ surgery) is needed to prevent intra-cranial complications.

Combination of in-situ collagen injection and rehabilitative treatment in long-lasting facial nerve palsy: a pilot randomized controlled trial.

BACKGROUND: Many rehabilitative attempts have been made to prevent or reduce residual deficits in patients with established and long-term facial palsy (FP). In many clinical settings in-situ injection of collagen-based medical devices have been demonstrated to provide nutritional support for tissues. AIM: To test the effectiveness of a collagen-based treatment for patients complaining of long standing FP, who are following a proprioceptive neuromuscular facilitation protocol (Kabat method) (group A), compared to a FP group only undergoing the Kabat method (group B). DESIGN: Randomised controlled trial. SETTING: Tertiary referral outpatient center and University Hospital. POPULATION: Forty-one patients with a medical diagnosis of long-term unilateral peripheral FP. METHODS: Twenty-one Group A patients were compared, after randomization, to nineteen matched group B patients after 8 weeks of treatment. The outcomes were electromyographic findings, validated questionnaires (Facial Disability Index, FDI and General health-related quality of life assessment, QOL) and clinical grading (House-Brackmann, HB, and synkinesis grading scale). A correlation analysis was performed between pre-/post-treatment differences (Δ) in outcome and clinical-demographic measures. RESULTS: A significant within-subjects improvement, both in electrophysiological and questionnaire scores, was found in both groups. When compared with group B, group A patients exhibited a significant reduction of post-treatment polyphasic potentials of voluntary activity of orbicularis oculi (P=0.017) and oris (P=0.015) and a significant increase in post-treatment duration of voluntary activity of orbicularis oris (P=0.018). Group A subjects demonstrated a significant improvement in questionnaire subscales regarding overall disease perception. Although positive correlations between the ∆FDI and ∆percentage of polyphasic potentials of voluntary activity were found in both groups, negative correlations in group A were found between disease duration and ∆duration of voluntary activity of orbicularis oculi and oris. CONCLUSIONS: The combination of physical rehabilitative procedures with in-situ collagen injections, possibly acting in redirecting the phenomena of reinnervation/reorganization, demonstrated encouraging results in patients affected by long term FP. CLNICAL REHABILITATION IMPACT: In-situ collagen injection could be a safe option enlarging the 'window of opportunity' to improve the voluntary muscle contraction pattern and general and specific disability referred by patients affected by long standing FP.

Tissue-engineered nerve guides with mesenchymal stem cells in the facial nerve regeneration.

Nerve guides with mesenchymal stem cells have been investigated in the rat facial nerve defect model to promote peripheral nerve regeneration and shorten recovery time to improve patients' quality of life. A 7-mm facial nerve gap experimental rat model is frequently employed in facial nerve regeneration studies. Facial nerve regeneration with nerve guides is evaluated by (1) assessing myelinated fiber counts using toluidine blue staining, (2) immunohistological analysis, (3) determining the g-ratio (axon diameter/total outer diameter) of regenerated nerve on transmission electron microscopic images, (4) retrograde nerve tracing in the facial nucleus, (5) electrophysiological evaluations using compound muscle action potential, and (6) functional evaluations using rat facial palsy scores. Dental pulp and adipose-derived stem cells, easily harvested using a minimally invasive procedure, possess characteristics of mesenchymal tissue lineages and can differentiate into Schwann-like cells. Cultured dental pulp-derived cells can produce neurotrophic factors, including nerve growth factor, brain-derived neurotrophic factor, and glial cell line-derived neurotrophic factor. These neurotrophic factors promote peripheral nerve regeneration and afford protection against facial motor neuron death. Moreover, artificial nerve guides can maneuver axonal regrowth, and dental pulp-derived cells and adipose-derived Schwann cells may supply neurotrophic factors, promoting axonal regeneration. In the present review, the authors discuss facial nerve regeneration using nerve guides with mesenchymal stem cells.

Facial nerve palsy: etiology and approach to diagnosis and treatment.

Facial nerve palsy has a broad differential diagnosis and possible psychological and anatomical consequences. A thorough investigation must be performed to determine the cause of the palsy and to direct treatment. If no cause can be found, therapy with prednisone with or without an antiviral medication can be considered and begun as early as possible after onset of symptoms. Resolution and time to recovery vary with etiology, but overall prognosis is good.

Deletion of CD38 and supplementation of NAD+ attenuate axon degeneration in a mouse facial nerve axotomy model.

Following facial nerve axotomy, nerve function is not fully restored even after reconstruction. This may be attributed to axon degeneration/neuronal death and sustained neuroinflammation. CD38 is an enzyme that catalyses the hydrolysis of nicotinamide adenine dinucleotide (NAD+) and is a candidate molecule for regulating neurodegeneration and neuroinflammation. In this study, we analyzed the effect of CD38 deletion and NAD+ supplementation on neuronal death and glial activation in the facial nucleus in the brain stem, and on axon degeneration and immune cell infiltration in the distal portion of the facial nerve after axotomy in mice. Compared with wild-type mice, CD38 knockout (KO) mice showed reduced microglial activation in the facial nucleus, whereas the levels of neuronal death were not significantly different. In contrast, the axon degeneration and demyelination were delayed, and macrophage accumulation was reduced in the facial nerve of CD38 KO mice after axotomy. Supplementation of NAD+ with nicotinamide riboside slowed the axon degeneration and demyelination, although it did not alter the level of macrophage infiltration after axotomy. These results suggest that CD38 deletion and supplementation of NAD+ may protect transected axon cell-autonomously after facial nerve axotomy.

Eight-and-a-half syndrome: video evidence and updated literature review.

The eight-and-a-half syndrome (EHS)-defined by the combination of a seventh cranial nerve palsy and an ipsilateral one-and-a-half syndrome-is a rare brainstem syndrome, which localises to the caudal tegmental region of the pons. We present a case of the EHS secondary to an inflammatory lesion on a previously healthy 26-year-old woman, with a literature review emphasising the relevance of aetiological assessment.

Critical literature review on the management of intraparotid facial nerve schwannoma and proposed decision-making algorithm.

Management of intraparotid facial nerve schwannomas (IFNS) is very challenging because the diagnosis is often made intra-operatively and in most cases, resection could lead to severe facial nerve (FN) paralysis, with important aesthetic consequences. Articles in the English language focused on the management of FN schwannoma have been selected and critically reviewed. A decision-making algorithm is proposed. In the case of type A or B neoplasms, or in case of a pre-operative FN House-Brackmann (HB) grade IV or worse, the authors would favor a resection of the IFNS and (where necessary) a reconstruction of the nerve. In the case of pre-operative HB grade III or better and type C or D neoplasms, patients would undergo an intra-operative biopsy to rule out malignancy, and a possible conservative management could be adopted. Localization and adherences of IFNS, as well as pre-operative FN function are important factors that must be considered in the decision-making process for IFNS to optimize the functional outcomes.