Cranial nerve involvement, visual complications and headache syndromes in Lyme disease.

PURPOSE OF REVIEW: To provide a summary of the visual manifestations and cranial neuropathies seen in Lyme disease. RECENT FINDINGS: Lyme facial palsy remains the most common manifestation of Lyme neuroborreliosis. Recent investigations show likely evidence of vagal involvement in Lyme disease. SUMMARY: The literature on Lyme neuroborreliosis continues to evolve. Lyme disease can affect nearly any cranial nerve in addition to causing various headache syndromes. The most common manifestation is Lyme disease facial palsy, occurring in up to 5-10% of patients with documented Lyme disease. Headache syndromes are common in the context of facial palsy but can occur in isolation, and more specific headache syndromes including trigeminal and geniculate neuralgias can occur rarely. Signs and symptoms indicative of vestibulocochlear nerve involvement are relatively common, although it could be that these represent other vestibular involvement rather than a specific cranial neuropathy. Optic neuritis is a controversial entity within Lyme disease and is likely overdiagnosed, but convincing cases do exist. Physicians who see any cranial neuropathy, including optic neuritis, in an endemic area can consider Lyme disease as a possible cause.

Comparison of the Reliability of the House- Brackmann, Facial Nerve Grading System 2.0, and Sunnybrook Facial Grading System for the Evaluation of Patients with Peripheral Facial Paralysis.

BACKGROUND: To compare the reliability of the House-Brackmann (HB), Facial Nerve Grading System 2.0 (FNGS 2.0), and Sunnybrook Facial Grading System (SB) which are widely used in the evaluation of peripheral facial paralysis (PFP) patients. METHODS: Thirty-five video-recorded adult PFP patients were included in the study. The evaluators comprised 6 physicians. Evaluations were conducted twice independently, utilizing video recordings. Simultaneously, the evaluators were asked to keep time during the evaluation. For the analysis of reliability, Fleiss' kappa coefficient was used for the HB, and the intraclass correlation coefficient (ICC) was used for the FNGS 2.0 and SB. RESULTS: The mean evaluation time of 1 patient was found to be 1.06 ± 0.24, 1.47 ± 0.23, and 2.32 ± 0.41 minutes for the HB, FNGS 2.0, and SB, respectively. For interrater reliability, Fleiss' kappa for the HB was 0.495 and 0.403; ICC for the FNGS 2.0 was 0.966 and 0.958; ICC for the SB was 0.960 and 0.967 for the first and second measurements, respectively. For intrarater reliability, Fleiss' kappa for the HB was 0.391, 0.446, 0.564, 0.502, 0.626, and 0.455; ICC for the FNGS 2.0 was 0.87, 0.982, 0.966, 0.929, 0.933, and 0.948; ICC for the SB was 0.935, 0.96, 0.895, 0.941, 0.96, and 0.94 for the 6 raters, respectively. CONCLUSION: In the present study, statistically high intra- and interrater correlations were found for the FNGS 2.0 and SB, while a moderate correlation was found for the HB. Although the HB seems to be more practical, it has been concluded that the FNGS 2.0 and SB are more reliable.

Parálisis facial, la importancia de realizar una buena historia clínica / Facial palsy, the importance of taking a good medical history

La parálisis facial plantea un diagnóstico diferencial amplio en Pediatría, sobre todo cuando se acompaña de sintomatología que orienta en contra de una parálisis de Bell, por lo que resulta imprescindible realizar una correcta anamnesis. La enfermedad de Lyme es una de las posibles causas de parálisis facial, habiendo sido poco descrita en niños en España. Presentamos el caso de un varón de 11 años con diagnóstico de parálisis facial asociada a infección por B. burgdorferi con evolución favorable tras tratamiento con doxiciclina. (AU)

Facial palsy poses a wide differential diagnosis in pediatrics, especially when it is accompanied by symptoms that make it doubtful whether Bell's palsy is present. It is essential to perform a correct anamnesis to rule out diagnoses that require early detection and treatment to improve the prognosis. Lyme disease (EL) is one of the possible causes of facial palsy, having been seldom described in children in Spain. We present the case of an 11-years-old male with diagnosis of facial palsy associated with B. burgdorferi infection with favorable evolution after treatment with doxycycline. (AU)

Toward an application of automatic evaluation system for central facial palsy using two simple evaluation indices in emergency medicine.

A stroke is a medical emergency and thus requires immediate treatment. Paramedics should accurately assess suspected stroke patients and promptly transport them to a hospital with stroke care facilities; however, current assessment procedures rely on subjective visual assessment. We aim to develop an automatic evaluation system for central facial palsy (CFP) that uses RGB cameras installed in an ambulance. This paper presents two evaluation indices, namely the symmetry of mouth movement and the difference in mouth shape, respectively, extracted from video frames. These evaluation indices allow us to quantitatively evaluate the degree of facial palsy. A classification model based on these indices can discriminate patients with CFP. The results of experiments using our dataset show that the values of the two evaluation indices are significantly different between healthy subjects and CFP patients. Furthermore, our classification model achieved an area under the curve of 0.847. This study demonstrates that the proposed automatic evaluation system has great potential for quantitatively assessing CFP patients based on two evaluation indices.

Predictive value of facial motor-evoked potential and electromyography for facial motor function in vestibular schwannoma surgery.

PURPOSE: Intraoperative neuromonitoring (IONM) aims to preserve facial nerve (FN) function during vestibular schwannoma (VS) surgery. However, current techniques such as facial nerve motor evoked potentials (FNMEP) or electromyography (fEMG) alone are limited in predicting postoperative facial palsy (FP). The objective of this study was to analyze a compound fEMG/FNMEP approach. METHODS: Intraoperative FNMEP amplitude and the occurrence of fEMG-based A-trains were prospectively determined for the orbicularis oris (ORI) and oculi (OCU) muscle in 322 VS patients. Sensitivity and specificity of techniques to predict postoperative FN function were calculated. Confounding factors as tumor size, volume of intracranial air, or IONM duration were analyzed. RESULTS: A relevant immediate postoperative FP was captured in 105/322 patients with a significant higher risk in large VS. While fEMG demonstrated a high sensitivity (77% and 86% immediately and 15 month postoperative, respectively) for identifying relevant FP, specificity was low. In contrast, FNMEP have a significantly higher specificity of 80.8% for predicting postoperative FP, whereas the sensitivity is low. A retrospective combination of techniques demonstrated still an incorrect prediction of FP in ~ 1/3 of patients. CONCLUSIONS: FNMEP and fEMG differ in sensitivity and specificity to predict postoperative FP. Although a combination of IONM techniques during VS surgery may improve prediction of FN function, current techniques are still inaccurate. Further development is necessary to improve IONM approaches for FP prediction.

Complete Hemifacial Paralysis Post-facelift: Making Sense of a Plastic Surgeon's Worst Nightmare.

BACKGROUND: Postrhytidectomy hemifacial paralysis is a frightening clinical condition affecting the proximal facial nerve and most often associated with Bell's palsy. Associated symptoms are common and include auditory, salivary, vestibular, and gustatory complaints. OBJECTIVES: The aim of the study was to provide increased awareness of postrhytidectomy hemifacial paralysis secondary to Bell's palsy in the plastic surgery community. METHODS: Following a roundtable discussion with the senior author's (J.C.G.) plastic surgery colleagues located all over the world, 8 surgeons reported having had firsthand experience with hemifacial paralysis in patients following facelift. Descriptions of their cases, including preoperative, intraoperative, and postoperative courses were collected and reported. RESULTS: A total of 10 cases of postrhytidectomy hemifacial paralysis were analyzed based on results of a clinical questionnaire. Eight of the 10 cases involved all facial nerve branches, with 2 cases sparing the marginal mandibular branch. The vast majority of cases were referred to a neurologist and steroids initiated. Two patients were returned to the operating room for exploration. Associated symptoms reported included pain in the ear, hearing loss, ocular symptoms such as tearing or dryness, vestibular symptoms such as vertigo, changes in taste, and in 1 patient an electric-shock type sensation to the face. CONCLUSIONS: Hemifacial paralysis associated with Bell's palsy following rhytidectomy is a rare but known clinical entity that should be included in the preoperative informed consent process before facelift. Current management trends are neurology referral and steroid initiation.

Infiltrative fibrous lesion of the facial nerve mimicking a facial nerve tumor.

PURPOSE: To report three cases of facial nerve lesions that were clinically expected to be facial nerve tumors but showed fibrotic infiltration without any apparent signs of a specific tumor on histopathological findings. We also aimed to investigate the clinical characteristics of these cases. METHODS: Medical records of patients who underwent surgery for facial nerve lesions were reviewed. RESULTS: All three cases initially had House-Brackmann (HB) grade IV-V facial nerve palsy. On radiological imaging, schwannoma or glomus tumor originating from the facial nerve was suspected. All patients underwent complete surgical removal of the neoplasm followed by facial nerve reconstruction using the sural nerve. The lesions were histologically confirmed as infiltrative fibrous lesions without tumor cells. In two cases, facial nerve palsy improved to HB grade III by nine months post-surgery, and there were no signs of recurrence on follow-up MRI. The other case, after 1 year of follow-up, showed persistence of HB grade V facial nerve palsy without any evidence of recurrence. CONCLUSION: Fibrotic lesions of the facial nerve could mimic primary facial nerve tumors. Clinicians should consider this condition even when a facial nerve tumor is suspected.

Facial surface electromyography: A systematic review on the state of the art and current perspectives.

PURPOSE: Peripheral facial nerve palsy is a severely disabling condition. In current clinical practice, the commonest tools to assess facial palsy are grading scales, digital face image analyses or facial muscle electrophysiology. However, these techniques suffer from subjectivity or invasiveness and cannot be applied as part of a routine clinical assessment. Therefore, novel non-invasive office-based tools are needed. Surface electromyography (sEMG) may potentially fulfill the requirements of objectivity, low examiner-dependence, and minimal invasiveness. The aim of this systematic review is to define the state of the art on the use of sEMG for facial nerve functional assessment. MATERIALS AND METHODS: Pubmed, Scopus and Web of Science databases were systematically searched. The study protocol was registered on PROSPERO in January 2023. The review was conducted according to the PRISMA guidelines. RESULTS: After the application of inclusion-exclusion criteria, 15 manuscripts with adequate relevance to this topic were included in the review. CONCLUSIONS: Facial sEMG represents a potentially useful tool to implement objective quantification of facial nerve function in clinical practice. Given the heterogeneity of methods and analysis in the available studies, sEMG results are hardly comparable. The introduction of methodological guidelines, followed by large prospective studies on well-defined subsets of patients with facial nerve impairment, is advocated.

Continuous intraoperative neuromonitoring of the facial nerve predicts postoperative facial palsy in parotid surgery: a prospective study.

PURPOSE: Facial palsy (FP) is the most significant complication of parotidectomy. Currently, the use of intermittent intraoperative neuromonitoring (iIONM) in parotid surgery facilitates nerve detection, which is paramount to nerve protection. Continuous IONM (cIONM), as applied in thyroid surgery, enables real-time information on electrophysiological nerve status through continuous nerve stimulation, thereby allowing consequent amplitude analysis. To date, the application of cIONM in parotid surgery has not been noted in literature. METHODS: We performed parotidectomies with anterograde facial nerve visualization using cIONM in 32 consecutive patients in a prospective study (German Register of clinical studies-DRKS 00011051) during the period October 2016 to January 2020. After the facial trunk had been exposed, an atraumatic stimulation electrode was placed and the nerve was stimulated at 3 Hz, at a low threshold (0.62 ± 0.06 mA), for the entire duration of the preparation. Selected electrophysiological parameters were collected and compared to postoperative facial nerve function, measured by the House-Brackmann grading system. RESULTS: In the post hoc analysis, a significant correlation between a drop in amplitude (< 50% of the "baseline" amplitude) and postoperative FP was recorded (p = 0.001). True positive prediction of FP was noted in 14 out of 16 patients and true negative in 10 out of 16. The sensitivity was 87.5% (AUC 0.75), with a high negative predictive value of 83.3%. CONCLUSION: cIONM has significant value in predicting postoperative FP in parotidectomy. Future development of an acoustic/optic warning system in IONM devices could prevent nerve injury in real time.

Clinical features, evaluation, and management of ophthalmic complications of facial paralysis: A review.

The purpose of this review was to study the evaluation, diagnosis, and management of ophthalmic complications associated with facial nerve palsy and to discuss the current and future interventions. The ophthalmic complications of facial paralysis include lagophthalmos, ectropion, exposure keratopathy, ocular synkinesis, and crocodile tears. Evaluation by an ophthalmologist skilled in recognizing and managing complications of facial paralysis shortly after its initial diagnosis can help identify and prevent long-term complications. Several types of grading scales are used to evaluate, measure the severity, and track surgical and patient-reported treatment outcomes. Lagophthalmos or ectropion are managed using temporary measures aimed at lubricating and covering the eye, including scleral lenses; however, these measures can be expensive and challenging to acquire and maintain. Temporary surgical interventions include lateral tarsorrhaphy, weighted eyelid implants, lateral canthoplasty, and other procedures that tighten or lift the eyelid or surrounding tissues. Management of flaccid facial paralysis due to iatrogenic injury or neoplasm requires neurorrhaphy or graft repair. The most common techniques for dynamic reconstruction in chronic facial paralysis are regional and free muscle flap transfer. Future directions for the management of ophthalmic complications aim to induce blinking and eye closure by developing systems that can detect blinking in the normal eye and transmit the signal to the paralyzed eye using mechanisms that would stimulate the muscles to induce eyelid closure. Blink detection technology has been developed, and a study demonstrated that blinking can be stimulated using electrodes on the zygomatic branch of the facial nerve. Further studies are needed to develop a system that will automate blinking and synchronize it with that of the normal eye.

Transmastoid Facial Nerve Decompression for Craniometaphyseal Dysplasia.

OBJECTIVE: We document the first successful transmastoid surgical treatment of facial nerve palsy for a patient with craniometaphyseal dysplasia (CMD), a rare genetic disease. PATIENT: A 9-month-old girl with bilateral facial nerve palsies and conductive hearing loss. Genetic testing made a diagnosis of CMD, and imaging showed narrowing of the facial nerve canals and ossicular fixation. INTERVENTION: Right transmastoid facial nerve decompression and ossicular chain reconstruction. MAIN OUTCOME MEASURE: Facial nerve function (House-Brackmann grade). RESULTS: Facial nerve function initially worsened, then improved within 12 months from House-Brackmann grade IV-V to grade III. CONCLUSION: Surgical cranial nerve decompression of and ossicular chain reconstruction may be effective treatments for patients with CMD.

Rationale for the Development of a Novel Clinical Grading Scale for Postoperative Facial Nerve Function: Results of a Multidisciplinary International Working Group.

OBJECTIVE: The objective of the current study was to present the results of an international working group survey identifying perceived limitations of existing facial nerve grading scales to inform the development of a novel grading scale for assessing early postoperative facial paralysis that incorporates regional scoring and is anchored in recovery prognosis and risk of associated complications. STUDY DESIGN: Survey. SETTING: A working group of 48 multidisciplinary clinicians with expertise in skull base, cerebellopontine angle, temporal bone, or parotid gland surgery. RESULTS: House-Brackmann grade is the most widely used system to assess facial nerve function among working group members (81%), although more than half (54%) agreed that the system they currently use does not adequately estimate the risk of associated complications, such as corneal injury, and confidence in interrater and intrarater reliability is generally low. Simplicity was ranked as the most important attribute of a novel postoperative facial nerve grading system to increase the likelihood of adoption, followed by reliability and accuracy. There was widespread consensus (91%) that the eye is the most critical facial region to focus on in the early postoperative setting. CONCLUSIONS: Members were invited to submit proposed grading systems in alignment with the objectives of the working group for subsequent validation. From these data, we plan to develop a simple, clinically anchored, and reproducible staging system with regional scoring for assessing early postoperative facial nerve function after surgery of the skull base, cerebellopontine angle, temporal bone, or parotid gland.

Acquired bilateral facial palsy: a systematic review on aetiologies and management.

OBJECTIVE: To systematically review the published cases of bilateral facial palsy (BFP) to gather evidence on the clinical assessment and management of this pathology. METHODS: Following PRISMA statement recommendations, 338 abstracts were screened independently by two authors. Inclusion criteria were research articles of human patients affected by BFP, either central or peripheral; English, Italian, French or Spanish language; availability of the abstract, while exclusion criteria were topics unrelated to FP, and mention of unilateral or congenital FP. Only full-text articles reporting the diagnostic work-up, the management, and the prognosis of the BFP considered for further specific data analysis. RESULTS: A total of 143 articles were included, resulting a total of 326 patients with a mean age of 36 years. The most common type of the paralysis was peripheral (91.7%), and the autoimmune disease was the most frequent aetiology (31.3%). The mean time of onset after first symptoms was 12 days and most patients presented with a grade higher than III. Associated symptoms in idiopathic BFP were mostly non-specific. The most frequently positive laboratory exams were cerebrospinal fluid analysis, autoimmune screening and peripheral blood smear, and the most performed imaging was MRI. Most patients (74%) underwent exclusive medical treatment, while a minority were selected for a surgical or combined approach. Finally, in more than half of cases a complete bilateral recovery (60.3%) was achieved. CONCLUSIONS: BFP is a disabling condition. If a correct diagnosis is formulated, possibilities to recover are elevated and directly correlated to the administration of an adequate treatment.

Pontine ischaemic stroke syndromes.

Classic Raymond syndrome is a rare neurological presentation comprising ipsilateral abducens palsy, contralateral facial paresis and contralateral hemiparesis. We present a man in his late 60s who presented with diplopia, dysarthria and right-sided limb weakness. This syndrome is one of a group of 'crossed paralyses' of the caudal pons.

[Facial palsy: diagnostic traps]. / Paralysie faciale : pièges diagnostiques.

Peripheral facial palsy is a common, often idiopathic and self-limiting mononeuropathy. However, secondary facial palsies require specific management: they are most often of infectious, vascular or dysimmune causes. The presence of red flags in the history, clinical examination or medical follow-up should alert clinicians. Because of the high incidence of Lyme disease in our region, this etiology deserves special attention. The management is based on general measures (eye protection, rehabilitation) and corticosteroid therapy; antivirals may provide additional benefit.

La paralysie faciale périphérique (PFP) est une mononeuropathie fréquente, souvent idiopathique et autorésolutive. Cependant, les paralysies faciales secondaires nécessitent une prise en charge spécifique : elles sont le plus souvent de causes infectieuses, vasculaires ou dysimmunes. La présence de drapeaux rouges à l'anamnèse, à l'examen clinique ou lors du suivi médical doivent alerter les cliniciens. En raison de l'incidence élevée de la borréliose dans nos régions, cette étiologie mérite une attention particulière. La prise en charge repose sur des mesures générales (protection oculaire, rééducation) et sur la corticothérapie ; l'ajout d'antiviraux pourrait apporter un bénéfice supplémentaire.