Characteristics and outcome of facial nerve palsy from Lyme neuroborreliosis in the United States.

OBJECTIVES: Facial palsy is the most common manifestation of Lyme neuroborreliosis (LNB) in the United States. This study aimed to describe features of patients with early LNB presenting with facial palsy and to determine if corticosteroids in addition to antibiotic therapy was associated with unfavorable outcome. METHODS: Retrospective analysis of participants enrolled in clinical studies investigating Lyme disease (N = 486) identified 44 patients who had facial palsy from LNB. The House-Brackmann scale was used to quantify the facial nerve dysfunction. RESULTS: Most patients presented in the summer months. Erythema migrans, frequently associated with systemic symptoms, occurred in 29 patients. Thirteen patients presented with bilateral facial palsy, usually with sequential involvement. Fourteen patients had painful radiculopathy. Of the 38 patients treated with antibiotics before the resolution of the palsy who had complete follow-up, 24 received both antibiotics and corticosteroids. Of these 38 patients, 34 recovered completely, 3 had nearly complete recovery, and 1 had moderate dysfunction. There were no differences between the treatment groups in achieving complete resolution of the palsy at 12 months or in time to complete recovery. INTERPRETATION: A history of rash compatible with erythema migrans or febrile illness in the weeks preceding the palsy are helpful clues pointing toward LNB and should be actively sought when evaluating patients with acute-onset peripheral facial palsy, particularly bilateral facial palsy. Treatment with antibiotic therapy is highly effective and most patients will fully recover facial nerve function. Adjunctive corticosteroid therapy appears to not affect the speed of recovery or overall outcome in this retrospective observational study.

[A case of facial-onset sensory and motor neuronopathy (FOSMN) with cerebellar ataxia and abnormal decrement in repetitive nerve stimulation test].

A 59-year-old woman presented with a 7-year history of facial numbness on the left side, and gradual worsening of symptoms. Over several years, facial muscle weakness, dysarthria, tongue atrophy and fasciculation had progressed. Then, she developed cerebellar ataxia affecting the left extremities, in addition to earlier symptoms. Brain MRI revealed cerebellar atrophy, and 99mTc-SPECT depicted cerebellar hypoperfusion. A repetitive nerve stimulation test (RNS) indicated abnormal decrement in the nasalis and trapezius muscles on the left side. Facial-onset sensory and motor neuronopathy (FOSMN) was diagnosed. Administration of intravenous immunoglobulin resulted in improvement of some symptoms. Although cerebellar ataxia is not a common symptom of FOSMN, a case showing TDP-43-positive glial cytoplasmic inclusions in cerebellar white matter has been reported. Therefore, it is possible that FOSMN may cause cerebellum impairment in some patients. Furthermore, RNS positive rate in the trapezius muscle is known to be high in amyotrophic lateral sclerosis (ALS) patients. It is speculated that RNS of the affected muscles in FOSMN may show abnormal decrement by the same mechanisms as ALS.

Severe invasive Listeria monocytogenes rhombencephalitis mimicking facial neuritis in a healthy middle-aged man: a case report and literature review.

Neurolisteriosis is a foodborne infection of the central nervous system that is easily misdiagnosed, especially in healthy adults with atypical symptoms. A 50-year-old man presented with a 3-day history of distortion of the oral commissure. Facial neuritis was diagnosed and treated with intravenous dexamethasone. His condition deteriorated rapidly, and he presented with a slow pharyngeal reflex, stiff neck, and signs of peripheral facial paralysis. Brain magnetic resonance imaging revealed multiple ring-enhanced foci in the brainstem. Routine and biochemical cerebrospinal fluid (CSF) analyses showed increased white blood cells and microproteins. Blood culture and high-throughput genome sequencing revealed Listeria monocytogenes DNA in the CSF. Ampicillin, amikacin, and meropenem were administered, and the patient was transferred from the intensive care unit to a standard medical ward after 2 months. The patient could walk and eat normally; however, he required intermittent mechanical ventilation at 11 months after discharge. Although L. monocytogenes meningitis is rare in healthy immunocompetent adults, it must be considered as a differential diagnosis, especially in adults whose conditions do not improve with cephalosporin antibiotic administration. L. monocytogenes rhombencephalitis mimics facial neuritis and develops quickly. Prompt diagnosis is essential for rapid initiation of antibiotic therapy to achieve the best outcome.

Etiology and therapy of delayed facial paralysis after middle ear surgery.

PURPOSE: This review focuses on the etiology, incidence and therapy of delayed paralysis of the facial nerve (DFP) after different types of middle ear surgery. METHODS: Retrospective review of studies published in English from 1970 until 2019 reporting DFP after tympanoplasty, tympanomastoid surgery, stapedotomy and stapedectomy. The search used the databases of PubMed, Scopus and Cochrane Library. Studies reporting from adult patients and DFP onset after 48 h after surgery were included. Studies dealing with iatrogenic or preexisting facial palsy and case reports were excluded. The initial literature search resulted in 52 studies. The relevance of the publications was verified using title, abstract and full-text analysis. Data were analyzed with descriptive statistics using median, simple sum and statistical significance. RESULTS: Ten studies having 12,161 patients could be included in this review. The incidence of DFP after the middle ear surgeries varies between 0.2 and 1.9%. The surgical stress of the middle ear surgeries is the main trigger for the development of DFP and leads to a virus reactivation and/or neuronal edema. Patients with a dehiscence of the facial canal have a significantly higher probability for a DFP. The recommended therapy of DFP based on the data of the therapy of Bell's palsy, consists of the administration of a steroid. For patients having a case history of previous viral infections, an antiviral prophylaxis is recommended. CONCLUSION: Overall, DFP has a very good prognosis, with mostly complete healing with appropriate therapy. Viral reactivation is the most favored genesis of DFP. Immunization or antiviral prophylaxis is recommended to those patients being at risk for a viral reactivation.

A Rare Case of Adenoid Cystic Carcinoma Isolated in the Mastoid.

Adenoid cystic carcinoma (ACC) isolated in the mastoid is very rare. Its diagnosis, especially in the early stage, is often challenging as during that stage, the signs and symptoms may be nonspecific. Our paper describes a case of a patient with an isolated mass in the left mastoid with persistent peripheral facial paralysis, and this was initially diagnosed as facial neuritis and granulation. However, histological examination later revealed an ACC exhibiting tubular and cribriform patterns. Our paper discusses the diagnostic basis, treatment, and outcomes for this case to improve the understanding of ACC isolated in the mastoid.

A Review of Periocular Botulinum Neurotoxin on the Tear Film Homeostasis and the Ocular Surface Change.

Clinical usage of botulinum neurotoxin (BoNT) in ophthalmology has dramatically increased since the 1980s and has become one of the most widely used agents for treating facial movement disorders, autonomic dysfunction and aesthetic wrinkles. Despite its high efficacy, there are some complications with periocular BoNT injections due to its chemodenervation effect. Among these, there is still controversy over the BoNT effect on tear film homeostasis and the ocular surface. A periocular BoNT injection could dry the eye by reducing tear production of the lacrimal gland and increase tear evaporation due to potential eyelid malposition and abnormal blinks. On the contrary, the injection of BoNT in the medial eyelids could treat dry eye disease by impairing lacrimal drainage. Regarding the ocular surface change, corneal astigmatism and high-order aberrations may decrease due to less eyelid tension. In conclusion, the entire awareness of the effect of BoNT and the patients' ocular condition is crucial for successful and safe results.

A clinically relevant decrease in abiraterone exposure associated with carbamazepine use in a patient with castration-resistant metastatic prostate cancer.

ADVERSE EVENT: Decreased abiraterone exposure after introducing carbamazepine. DRUGS IMPLICATED: Abiraterone acetate and carbamazepine. THE PATIENT: A 65-year-old man with metastatic castration resistant prostate cancer, was treated with abiraterone acetate and prednisolone, and received concomitant carbamazepine for treatment of facial neuropathy. EVIDENCE THAT LINKS THE DRUG TO THE EVENT: The interaction was confirmed by a decrease in abiraterone exposure >2-fold (area-under-the-curve and trough levels). After discontinuation of carbamazepine therapy, the abiraterone exposure normalized. No alternative causes were found that explain the decrease in abiraterone exposure. MECHANISM: Induction of CYP3A and potentially phase I metabolism (SULT2A1) by carbamazepine. IMPLICATIONS FOR THERAPY: Clinicians and pharmacists should be aware of this clinically relevant interaction. The national drug-drug interaction checker does not warn for this interaction, whereas both the Lexicomp® and Micromedex® advice to avoid if possible or to increase the abiraterone dose frequency to twice daily. Carbamazepine should not be combined with abiraterone to avoid underexposure and suboptimal therapy. Therapeutic drug monitoring of abiraterone is useful to guide therapy when drug-drug interactions cannot be avoided.

Factors affecting the effect of physical rehabilitation therapy for synkinesis as a sequela to facial nerve palsy.

OBJECTIVE: To investigate factors affecting the effect of physical rehabilitation therapy for synkinesis as a sequela to facial nerve palsy. METHODS: A total of 37 patients with peripheral facial nerve palsy in Teine-Keijinkai Hospital were enrolled in this study. All patients showed synkinesis at 6 months after the onset of facial nerve palsy and were instructed in physical rehabilitation by expert staff from their first visit. The degree of synkinesis was evaluated at 6, 9 and 12 months after the onset of facial nerve palsy based on Sunnybrook facial grading system score and asymmetry in eye opening width. The patients were divided into two groups by age, gender, cause of palsy, electroneurography (ENoG) value, onset of synkinesis, initial treatment and timing of the start of physical rehabilitation. RESULTS: Female patients and younger patients did not show any deterioration in synkinesis. Patients in the lower ENoG group and the later onset of synkinesis group showed significant deterioration in synkinesis after the 6th month from onset of facial palsy. CONCLUSION: Physical rehabilitation was shown to prevent significant deterioration in synkinesis in female and younger patients with facial nerve palsy. Careful follow-up with regard to synkinesis is required in cases in which the facial nerve damage is thought to be severe.

A Rare Presentation of Cranial Polyneuropathy Without Rash Caused by Varicella Zoster Virus.

INTRODUCTION: Varicella Zoster Virus (VZV) is associated with many disorders of the central and peripheral nervous systems including neuralgia, meningitis, meningoencephalitis, cerebellitis, vasculopathy, myelopathy, Ramsay-Hunt syndrome, and polyneuritis cranialis. Cranial nerves V, VI, VII, VIII, IX, X, XI, and/or XII may be affected. The neurological disorders caused by VZV usually present with rash, but may rarely present without rash. CASE REPORT: We herein present a case of polyneuritis cranialis without rash caused by VZV affecting cranial nerves VII, VIII, IX, and X. After excluding other causes of the condition, we diagnosed VZV infection based on VZV DNA in the CSF and an elevated anti-VZV IgG level in serum. The patient responded well to antiviral therapy. CONCLUSION: VZV infection should be kept in mind during the differential diagnosis of polyneuritis cranialis; it is important to note that VZV re-activation may occur without rash.

Continuous hemifacial myokymia as the revealing symptom of demyelinating disease of the CNS.

Facial myokymia (FM) is an uncommon involuntary movement, disorder of the musculature supplied by the facial nerve and, characterized by spontaneous undulating, vermicular movements beneath the, skin. It has rarely been described as a form of presentation of multiple, sclerosis. We describe a 31-year-old man presenting with continuous, unilateral facial myokymia as the revealing symptom of a demyelinating, disorder of central nervous system. Brain magnetic resonance imaging, showed an ipsilateral pontine T2/FLAIR hyperintensity close to the, postgenu course of facial nerve, suggestive of a segmental demyelination, of facial nerve causing facial nuclear hyperactivity and resulting in FM., Facial myokymia must raise the possibility of MS in adults under the age, of 40.

Efficiency of ultrasound and water capsule-guided local injection of botulinum toxin type A treatment on patients with facial spasm.

OBJECTIVE: To study the efficacy of ultrasound and water capsule-guided local injection of botulinum toxin type A (BTX-A) treatment on patients with facial spasm. PATIENTS AND METHODS: One hundred and fifty-seven cases of facial spasm were randomly divided into oral drug treatment group (group A) (78 cases) and ultrasound and water capsule-guided local injection of botulinum toxin type A treatment group (group B) (79 cases). Cohen, Acbert spasm strength grade scores in each case with facial spasm were recorded. Therapeutic effect, duration, significant efficiency, and muscle spasm strength were compared before and three after treatment. RESULTS: The muscle spasm strength showed no significant change in group A after the treatment. However, the muscle spasm strength was decreased significantly in group B after treatment (p < 0.01). CONCLUSIONS: Ultrasound and water capsule-guided local injection of botulinum toxin type A treatment is a safe, effective, and simple treatment for patients with facial spasm.

Otitis complicated by Jacod's syndrome with unusal facial nerve involvement: Case report and review of literature.

Otitis media is a well-known condition and its infra-temporal and intracranial complications are extremely rare because of the widespread usage of antibiotic treatment. We report a case of 63-year-old female with complaints of right-sided facial pain and diplopia. She had a history of acute otitis media before 4 months of admission to our neurology unit. Neurological examination showed that total ophthalmoplegia with ptosis, mydriasis, decreased vision and loss of pupil reflex on the right side. In addition, there was involvement of 5th and 7th cranial nerves. Neurological and radiological follow-up examinations demonstrated Jacod's Syndrome with unusual facial nerve damage and infection in aetiology. Sinusitis is the most common aetiology, but there are a few cases reported Jacod's Syndrome originating from otitis media.

The effects of aminoguanidine, methylprednisolone, and melatonin on nerve recovery in peripheral facial nerve neurorrhaphy.

BACKGROUND: The medications may enhance the recovery after nerve paralysis. We aimed to evaluate the effects of aminoguanidine (AG), melatonin, and methylprednisolone on peripheral facial nerve neurorrhaphy. METHODS: The buccal branch of the facial nerve was transected and autografted in 32 New Zealand rabbits. Subjects were divided into 4 groups equally (AG, melatonin, methylprednisolone, and control). After the medical treatment latency and amplitude were measured with nerve conduction study at 3, 6, and 10 weeks. Then, coapted segments of nerve were examined microscopically. The groups were compared with each other. RESULTS: The latent period was shortened, and the amplitudes were increased in the AG group; the latent period was shortened, and the amplitudes did not show significant change in the melatonin group with the time. There were no significant differences between the amplitudes at 3 to 6 and 3 to 10 weeks in the methylprednisolone group, and the latent period was shortened. There was no significant difference between the amplitude values at 3, 6, and 10 weeks in the control group. In the histological examination, AG had the best influence on preventing myelin degeneration and reducing the accumulation of myelin debris. Considering the increase in collagen fibers, the best results were achieved in the melatonin group. The degree of myelin-axonal degeneration was higher in the methylprednisolone group. The degree of collagen fiber increase, axonal degeneration, myelin degeneration, and the accumulation of myelin debris were detected quite high in the control group. CONCLUSIONS: Aminoguanidine and melatonin alone achieved an increase in regeneration after peripheral facial nerve neurorrhaphy, but methylprednisolone did not. The best healing was determined in the AG group.

[Pediatric reversible acute velar palsy: A case report]. / Paralysie vélaire aiguë réversible de l'enfant : à propos d'un cas.

INTRODUCTION: Approximately 40 cases of acute idiopathic velopharyngeal reversible paralysis in the pediatric population have been reported in the literature. OBSERVATION: We present the case of a 12-year-old boy who had consulted in pediatric emergency departments for symptomatology including rhinolalia, nasal regurgitation, and deviation of the labial commissure. Paraclinical explorations helped diagnose rhombencephalitis with enterovirus. The introduction of oral corticosteroids was followed by rapid clinical improvement in 3 days. Monitoring 1 month later showed complete regression of symptoms. DISCUSSION: Similar cases in the literature describe the occurrence of nasal regurgitation and rhinolalia, sometimes associated with other cranial nerve impairment. The pathogenesis is rarely highlighted and the imaging results are always normal. Ad integrum recovery with or without corticosteroids is the rule. In light of this literature review, it is possible to conclude that the occurrence of such a suggestive clinical picture should limit the often costly and unnecessary additional tests.

Comparison of facial synkinesis at 6 and 12 months after the onset of peripheral facial nerve palsy.

OBJECTIVE: To investigate the time course of synkinesis as a sequela of facial nerve palsy so that we are able to determine an appropriate time for deciding the outcome of recovery in patients with facial nerve palsy. METHODS: Nineteen consecutive patients with peripheral facial nerve palsy who developed synkinesis were enrolled. We compared the degree of synkinesis at 6 and 12 months after the onset of palsy. Our investigation consisted of (1) scoring using the Sunnybrook facial grading system and (2) computing the asymmetry (%) in eye opening width. We also judged whether all 19 cases were cured or not based on the treatment outcome criteria of the Japan Society of Facial Nerve Research at 6 and 12 months. RESULTS: The synkinesis score based on Sunnybrook facial grading system and the degree of asymmetry in eye opening width during mouth movement deteriorated significantly between the 6th and 12th month after the onset of palsy. One of the cases regarded as "cured" at the 6th month was later judged to be "non-cured" due to deterioration in the synkinesis score at the 12th month. CONCLUSION: From our results, synkinesis deteriorated after the 6th month from the onset of palsy. Therefore, we should follow up the degree of synkinesis until at least the 12th month, and the outcome of recovery in patients with facial synkinesis should be evaluated at least 12 months after the onset.

Clinical characteristics and outcomes of delayed facial palsy after tympanoplasty.

CONCLUSION: Delayed facial palsy after tympanoplasty had no predisposition for gender or side. It seemed that prednisolone alone without antiviral drugs was enough for the treatment of delayed facial palsy after tympanoplasty. OBJECTIVE: To analyze the clinical characteristics and prognosis of delayed facial palsy after tympanoplasty. METHODS: The cases with delayed facial palsy after tympanoplasty treated from January 2005 to January 2014 at our hospital were included in the study, and their clinical characteristics and outcomes were analyzed. RESULTS: The incidence of delayed facial palsy after tympanoplasty was 1.1% (16/1420 cases). Facial palsy occurred between 5 and 14 days (average 7.4 ± 1.8 days) after surgery. The incidence of delayed facial palsy was not different between males and females (p > 0.05). Also there was no significant difference between left and right side (p > 0.05). All patients were treated by prednisolone alone, and their facial nerve recovered to HB grade I after 3 weeks to 2 months without any sequelae.

Subjective and objective outcome measures in the treatment of facial nerve synkinesis with onabotulinumtoxinA (Botox).

PURPOSE: To evaluate the Sunnybrook Facial Grading System (SFGS) and Facial Clinimetric Evaluation Scale (FaCE Scale) instrument outcome measures pre- and 30-day posttreatment of facial nerve synkinesis with botulinum toxin with attempts to correlate the 2 scales. METHODS: An IRB approved retrospective review of 22 patients with facial nerve synkinesis where the surgeon completed the SFGS and the patient completed the FaCE prior to receiving onabotulinumtoxinA therapy, the SFGS, and FaCE scales were completed again 1 month later. RESULTS: Of the 22 patients, 9 complete datasets were analyzed. Mean patient age was 59.8; 8 (89%) women and 1 (11%) men. Overall SFGS composite score decreased from 57.6 ± 20.9 to 45.2 ± 13.5, (p = 0.001). SFGS subdomain synkinesis significantly improved (p < 0.001), while voluntary movement significantly decreased (p = 0.002). A difference in the resting symmetry was not statistically significant (p = 0.08). The FaCE scale composite score significantly improved from 40.9 ± 9.5 to 47.6 ± 11.9, (p = 0.03). FaCE subdomains facial comfort (p = 0.005) and social function (p = 0.009) significantly improved, while oral function, eye comfort, facial movement, and lacrimal control did not. The Δ pre/post-SFGS composite score did not correlate with the Δ pre/post-FaCE composite score (rs= -0.318). Subdomain analysis demonstrated significant negative correlation between Δ pre/post-SFGS synkinesis score and Δ pre/post-FaCE eye comfort score (rs = -0.826, p < 0.01). CONCLUSIONS: Significant improvement was seen in objectively reported synkinesis following botulinum toxin therapy. An improvement was noted in the overall subjective facial nerve functioning following therapy along with improvement in social functioning and facial comfort. A meaningful negative correlation was noted when comparing the SFGS "synkinesis" subdomain with the FaCE scale subdomain "eye comfort", implying improvement in eye comfort with control of synkinesis.