Intratemporal facial nerve neurofibroma causing facial paralysis in an infant: Case report and review of the literature.

This article describes the first recorded case of intratemporal neurofibroma in an infant. A literature review of all other existing cases of intratemporal neurofibroma is performed, finding that the majority of cases involve multiple segments and can be found in the mastoid segment most often. Most common symptoms described included facial paralysis, otalgia, and conductive hearing loss, respectively.

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.

Natural history of facial paraganglioma with 2 decades of follow-up: A case report and literature review.

OBJECTIVE: To report 20 years of natural history data for a facial paraganglioma and provide a comprehensive review of the existing literature. PATIENT: 81-year-old female with a remote history of cardiac arrest while under anesthesia who elected to observe her facial paraganglioma for 20 years. INTERVENTIONS: Observation, clinical documentation, radiographic surveillance. MAIN OUTCOME MEASURES: Tumor progression, patient symptomatology, and review of management options. RESULTS: The initial presentation of the facial paraganglioma was facial spasm. Over the course of observation, symptoms progressed to include complete facial nerve paralysis, pulsatile tinnitus, and otalgia on the affected side. Radiologic surveillance demonstrated incremental growth and erosion of surrounding structures, including the posterior external auditory canal, stylomastoid foramen, and lateral semicircular canal with near-dehiscence. Twenty-four cases of facial paraganglioma were identified in the extended literature search and are summarized herein. CONCLUSIONS: This unique case contributes to the scarce literature surrounding facial paragangliomas by reporting the extended natural history of this disease.

Facial nerve palsy in neurosarcoidosis: clinical course, neuroinflammatory accompaniments, ancillary investigations, and response to treatment.

BACKGROUND: Facial nerve palsy is a cardinal manifestation of neurosarcoidosis, but dedicated studies of this disease feature have not been conducted. We sought to clarify the impact of facial palsy on the diagnosis of neurosarcoidosis, its subsequent clinicoradiographic evolution, and eventual treatment decisions. METHODS: A single-center retrospective analysis of patients with neurosarcoidosis and facial palsy was conducted over the preceding 10 years (01/01/2011-08/12/2021). RESULTS: 23/218 (10.6%) patients with neurosarcoidosis developed facial neuropathy. It was the inaugural manifestation of neurosarcoidosis in 17/23 (73.9%) and presented in isolation of other neurologic deficits or extra-facial MRI abnormalities in 12/23 (52.2%). At onset, facial palsy was unilateral in 20/23 (87.0%), and multiple cranial neuropathies were seen in 8/23 (34.8%). Non-facial inflammatory MRI abnormalities were observed in 6/15 (40.0%) patients at onset with leptomeningitis being most common (5/15, 33.3%). 13/23 (56.5%) experienced a second attack of neurosarcoidosis at a median of 8 months, including 3/23 (13.0%) with recurrent facial palsies. In the 12 patients with isolated facial paresis at onset, 4/12 (33.3%) remained free of new deficits or neuroimaging abnormalities by last follow-up. 17/23 (73.9%) eventually required initiation of steroid-sparing immunosuppressants, almost all for development of non-facial disease. The final median House-Brackmann score was 1. CONCLUSION: Facial neuropathy occurred less commonly than historically reported, and it often acts as a forerunner to systemic sarcoidosis and more widespread neurologic disease. Recurrent attacks of neurosarcoidosis occur early at high frequency following facial palsy. Recovery of facial nerve function is typically excellent.

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.

Post-Irradiation Facial Neuromyotonia/Myokymia: A Hemifacial Spasm Mimic.

Background: Hemifacial spasm is diagnosed on a clinical base, with certain atypical features alerting the physician for mimics. Phenomenology shown: Hemifacial neuromyotonia/myokymia characterized by tonic hemifacial contraction followed by multifocal undulating hemifacial twitches. Educational value: These features are a red flag for (post-irradiation) facial neuromyotonia/myokymia which generally responds well to low dose carbamazepine.

Hearing Preservation After Cochlear Reimplantation Using Electrocochleography: A Case Report.

Studies have shown that hearing preservation is possible in the context of reimplantation, but residual hearing could not be predicted or expected in these cases. We describe a case in which a patient with mild to profound sensorineural hearing loss who underwent cochlear implantation with a lateral wall array and had hearing preserved postoperatively. She developed facial nerve stimulation which was unresponsive to reprogramming. Using electrocochleography to measure intracochlear trauma during the insertion process, the patient underwent reimplantation with a perimodiolar electrode and hearing was preserved postoperatively. This case demonstrates the potential to use electrocochleography for hearing preservation during reimplantation. Laryngoscope, 131:2348-2351, 2021.

[VI and VII cranial nerve injury secondary to intracranial idiopathic hypertension in a pediatric patient]. / Afectación del VI y el VII par craneal secundaria a hipertensión intracraneal idiopática en un paciente pediátrico.

TITLE: Afectación del VI y el VII par craneal secundaria a hipertensión intracraneal idiopática en un paciente pediátrico.

Investigating Facial Nerve Stimulation After Cochlear Implantation in Adult and Pediatric Recipients.

OBJECTIVES/HYPOTHESIS: Facial nerve stimulation (FNS) can occur after cochlear implantation for a small number of recipients. This study aimed to investigate if a correlation exists between the variables involved in FNS. STUDY DESIGN: Retrospective cohort review. METHODS: There were 32 out of 1,100 cochlear implant recipients who experienced FNS in our clinic between 2010 and 2019. The following variables were recorded from a retrospective chart review: grade of FNS, onset of FNS, the number of channels stimulating FNS, and radiological findings of abnormalities in the inner ear. Statistical analyses were performed to identify a correlation between any of the variables involved. The techniques used to reduce FNS were analyzed. RESULTS: Eleven adult ears had progressive hearing loss, three had idiopathic sudden sensorineural hearing loss (SNHL), and one congenital SNHL. All pediatric ears were diagnosed with congenital SNHL, except for one ear with idiopathic sudden SNHL. The grade of FNS ranged from mild stimulation or slight motion in the eye, mouth, nasolabial, or forehead regions (n = 8) to total severe stimulation of the facial musculature and/or severe pain (n = 3). The onset of FNS occurred immediately after activation for nine ears, and up to 16 months later for the other subjects. A significant correlation was observed between the number of channels stimulating FNS, the grade of FNS, and the radiological findings of the inner ear. FNS was completely resolved for 30 ears and partially resolved for two ears. CONCLUSIONS: FNS can occur any time after cochlear implantation and can affect both adult and pediatric. However, it can be effectively resolved using specific fitting techniques. LEVEL OF EVIDENCE: 2c Laryngoscope, 131:374-379, 2021.

Case Report: Ocular Tilt Reaction with Internuclear Ophthalmoplegia and Multiple Cranial Nerve Palsies.

SIGNIFICANCE: Ocular tilt reaction (OTR) is an abnormal eye-head postural reaction that consists of skew deviation, head tilt, and bilateral ocular torsion. Understanding of the pathway of the vestibulo-ocular reflex (VOR) is essential because this will help to localize the pathology. PURPOSE: The aim of this study was to report a case of OTR with contralateral internuclear ophthalmoplegia (INO) and fifth and seventh cranial nerve palsies. CASE REPORT: A 51-year-old gentleman with underlying diabetes mellitus presented with sudden onset of diplopia for 3 days. On examination, his visual acuity was 20/30 bilaterally without a relative afferent pupillary defect. He had a right OTR consisting of a right head tilt, a skew deviation with a left eye hypertropia, and bilateral ocular torsion (right excyclotorsion and left incyclotorsion) with nystagmus. He also had a left adduction deficit and right abduction nystagmus consistent with a left INO. Ocular examination revealed evidence of proliferative diabetic retinopathy bilaterally. Two days after the initial presentation, the patient developed left seventh and fifth cranial nerve palsies. MRI showed left pontine infarction and multiple chronic lacunar infarctions. There was an incidental finding of a vascular loop compression on cisternal portions of the left trigeminal, facial, and vestibulocochlear nerves. Antiplatelet treatment was started on top of a better diabetic control. The diplopia was gradually resolved with improved clinical signs. In this case, the left pontine infarction had likely affected the terminal decussated part of the vestibulocochlear nerve from the right VOR pathway, medial longitudinal fasciculus, and cranial nerve nuclei in the left pons. CONCLUSIONS: The OTR can be ipsilateral to the lesion if the lesion is before the decussation of the VOR pathway in the pons, or it can be contralateral to the lesion if the lesion is after the decussation. In case of an OTR that is associated with contralateral INO and other contralateral cranial nerves palsy, a pathology in the pons that is contralateral to the OTR should be considered. Neuroimaging study can hence be targeted to identify the possible cause.

Optic, trigeminal, and facial neuropathy related to anti-neurofascin 155 antibody.

OBJECTIVE: To characterize the frequency and patterns of optic, trigeminal, and facial nerve involvement by neuroimaging and electrophysiology in IgG4 anti-neurofascin 155 antibody-positive (NF155+ ) chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Thirteen IgG4 NF155+ CIDP patients with mean onset age of 34 years (11 men) were subjected to neurological examination, blink reflex, and visual-evoked potential (VEP) testing, and axial and/or coronal T2-weighted head magnetic resonance imaging (MRI). RESULTS: Among 13 patients, facial sensory impairment, facial weakness, and apparent visual impairment were observed in three (23.1%), two (15.4%), and two (15.4%) patients, respectively. All 12 patients tested had blink reflex abnormalities: absent and/or delayed R1 in 11 (91.7%), and absent and/or delayed R2 in 10 (83.3%). R1 latencies had strong positive correlations with serum anti-NF155 antibody levels (r = 0.9, P ≤ 0.0001 on both sides) and distal and F wave latencies of the median and ulnar nerves. Absent and/or prolonged VEPs were observed in 10/13 (76.9%) patients and 17/26 (65.4%) eyes. On MRI, hypertrophy, and high signal intensity of trigeminal nerves were detected in 9/13 (69.2%) and 10/13 (76.9%) patients, respectively, whereas optic nerves were normal in all patients. The intra-orbital trigeminal nerve width on coronal sections showed a significant positive correlation with disease duration. INTERPRETATION: Subclinical demyelination frequently occurs in the optic, trigeminal, and facial nerves in IgG4 NF155+ CIDP, suggesting that both central and peripheral myelin structures of the cranial nerves are involved in this condition, whereas nerve hypertrophy only develops in myelinated peripheral nerve fibers.

Takayasu's Arteritis Presenting Atypically in a Female Nigerian.

Takayasu's arteritis (TA) is an idiopathic form of large vessel granulomatous vasculitis that mainly affects the aorta and its major branches, most frequently in young women under 50 years. While traditionally, it is a disease found commonly in Asia continent, it has also been reported from different parts of the world, albeit with a few reports from Sub-Sahara Africa. The clinical presentations are variable and are commonly from systemic inflammation, vascular occlusive diseases and aneurysm. Asymptomatic cases of TA have been documented and are usually discovered incidentally on physical examination. Common vascular symptoms from different series include vascular claudication, reduced or absent pulse, carotid bruit, hypertension and headache. Facial mononeuropathy and retinal ischaemic changes are rare findings in TA. However, occlusive disease of ulnar artery has not been reported in TA despite our extensive literature search. Here, we present a 48-year-old woman, who was admitted via the medical emergency with community acquired pneumonia but was incidentally diagnosed with Takayasu arteritis with lower motor neuron facial nerve palsy, unilateral blindness, and ulnar artery occlusion. Multidisciplinary management was instituted and patient was discharged after resolution of community acquired pneumonia, vascular claudication, and chronic headache. TA often presents asymptomatically and sometimes with atypical features and thus we suggest high index of suspicion and detailed cardio-vascular examination in young individuals with unexplained chronic headache, facial nerve palsy and visual symptoms.

Intratemporal facial nerve schwannomas: multicenter experience of 80 cases.

PURPOSE: To provide more data on the clinical presentation and natural evolution of facial nerve schwannomas and to provide guidance for therapeutic decision making. METHODS: A retrospective case review of eighty patients diagnosed with a facial nerve schwannoma between 1990 and 2018 in ten tertiary referral centers in Europe was performed. Patients' demographics, symptomatology, audiometry, anatomical site (segments involved), size and whenever possible volume measurement were registered. RESULTS: At presentation, transient or persistent facial palsy was the most common symptom, followed by hearing loss. The schwannoma involved more than one segment in the majority of the patients with the geniculate ganglion being most commonly involved. Initial treatment consisted of a wait and scan approach in 67.5%, surgery in 30% and radiation therapy in 2.5% of the patients. Tympanic segment schwannomas caused mainly conductive hearing loss and were more prone to develop facial palsy at follow-up. Internal auditory canal or cerebellopontine angle schwannomas presented with significantly more sensorineural hearing loss. CONCLUSIONS: Although modern imaging has improved diagnosis of this tumor, choosing the best treatment modality remains a real challenge. Based on the literature review and current findings, more insights into the clinical course and the management of facial nerve schwannomas are provided.

Sarcoidosis of the central nervous system: Safety and efficacy of treatment, and experience of biological therapies.

OBJECTIVES: Neurological complications of sarcoidosis are uncommon and the natural history and optimal treatments under-researched. With the advent of biological therapies, it is important to define the clinical characteristics and immunopathology of the disease. PATIENTS AND METHODS: Patients referred to and treated within the Centre for Neurosarcoidosis over a 15 year period who had biopsy proven "highly probable" disease of the central nervous system were studied prospectively. RESULTS: Corticosteroids were used effectively in all patients, immunosuppression in 79 % and TNFα antagonists in 23 %. Treatment with steroids alone inevitably led to relapse, and low dose immunosuppression was ineffective in those with severe forms of the disease. Use of biological therapies substantially improved outcome. Patients with cranial neuropathy had an excellent outcome. Those with pachymeningitis had marked radiological abnormalities but less disablement. Those with leptomeningitis had an invasive, destructive disease which responded well to treatment but with residual neurological impairments. Treatment was required for many years, but the risk of relapse following treatment withdrawal was low. Infective complications arose in six. There were two deaths, neither directly related to the neurological disease, nor its treatment. CONCLUSIONS: This prospective study of the natural history and treatment response in neurosarcoidosis provides evidence that the use of high dose immunosuppression and early and prolonged use of biological therapies is associated with greatly improved outcomes and lower mortality. The data may be used to plan further studies and treatment trials, and provide class IV evidence for the effectiveness of biological agents in the treatment of Neurosarcoidosis.