CHN1 and duane retraction syndrome: Expanding the phenotype to cranial nerves development disease.

Duane retraction syndrome is a congenital eye movement disorder characterized by a failure of abducens nerve to develop normally, resulting in restriction or absence of abduction, adduction, or both, and narrowing of the palpebral fissure and retraction of the globe on attempted adduction. There is a genetic heterogeneity in Duane retraction syndrome (DURS). DURS maps to chromosome 8q13 in some patients, and pathogenic variants in CHN1 and MAFB genes are known to lead to DURS. We report here a child and his father with Duane retraction syndrome, associated to swallowing difficulties and unilateral trapeze aplasia. A whole exome sequencing revealed a heterozygous missense variant in CHN1 gene. This gene encodes GTPase-activating protein and is involved in the assembly of neuronal locomotor circuits. A patient with a 8q deletion has previously been described with a Duane retraction syndrome associated to trapeze aplasia. We provide an additional description to support the role in cranial nerves development of the CHN1 gene.

Activity limitations and participation restrictions in patients with peripheral facial palsy: a cross-sectional study over a six-year period.

BACKGROUND: Peripheral facial palsy is a pathological condition caused by a wide range of etiologies. A damage of VII cranial nerve produces facial disfigurement and limitations in daily life activities, such as drinking, eating and speaking. As a consequence, patients may experience psychological distress and social isolation. To counsel and design a patient-tailored rehabilitation for patients affected by peripheral facial palsy, physical and social limitations should be considered. Moreover, the knowledge of factors associated with disability plays a key role in the early identification and adequate care of patients with higher risk to develop psychological distress and participation restrictions. AIM: To evaluate activity limitations, psychological distress and participation restrictions of patients affected by peripheral facial palsy seeking for rehabilitation and to identify individual and disease-specific factors associated to disability. DESIGN: Cross-sectional study. SETTING: Consultation hour dedicated to facial palsy patients in the outpatient clinic of a Rehabilitation Unit. POPULATION: One hundred eighty-six outpatients with recent or chronic peripheral facial palsy at the first assessment at our Rehabilitation Unit. METHODS: Using multiple linear regression models, we evaluated the association between the two subscales of Facial Disability Index (FDI) and the composite score of Sunnybrook Facial Grading System, as well as the association between the two FDI subscales and other clinical and demographic variables. RESULTS: Activity limitations correlate with the severity of palsy, while psychological distress and participation restrictions do not correlate with the neurological impairment. The correlation between the severity of palsy and both activity limitations and participation restrictions is influenced by palsy etiology. Activity limitations decrease with time from palsy onset, while psychological distress and participation restrictions are more severe in women. CONCLUSIONS: Beyond severity, also etiology, time from onset and gender influence disability after facial palsy. These factors should be considered in counselling and planning a patient-tailored multidisciplinary rehabilitative treatment. CLINICAL REHABILITATION IMPACT: Our study highlights the individual and pathology-associated factors related to activity limitations and participation restrictions in patients with peripheral facial palsy. These elements should be considered in the definition of a patient-tailored rehabilitative plan and in the organization of a multidisciplinary care.

Cranial nerves impairment in post-acute oropharyngeal dysphagia after COVID-19.

BACKGROUND: According to literature, after COVID-19, patients may require rehabilitation care because of different degrees of physical impairments. Neurologic disorders are often described but no specific data about postacute cranial nerves involvement and possible correlation with dysphagia development are yet available. CASE REPORT: The patient is a 69-year-old man who presented acquired weakness and dysphagia with clinical cranial nerves impairment of lingual, IX, X and XII after SARS-CoV-2 infection, without electrophysiological alterations. He underwent rehabilitation program for two months, with slow recovery. However, at discharge residual hypoglossal nerve deficit sign was present. CLINICAL REHABILITATION IMPACT: This single case expands knowledge about clinical picture after SARS-CoV-2 disease. Is important to notice that cranial, particularly bulbar nerves could be involved as late complications. Thus, we discuss about risk factors, the nature of the damage and the impact in dysphagia pathophysiology and recovery. If supported by further studies, this case may help to understand dysphagia features in these patients.

Reduced genioglossus muscle activity caused by fluid overload in anesthetized rats.

INTRODUCTION: Although the precise cause of obstructive sleep apnea (OSA) remains unknown, various anatomical or structural factors are thought to influence upper airway patency. Recent clinical studies show that OSA is frequently observed among patients with fluid-retaining states, such as heart/renal failure and postsurgery. It is important to note that a cause-effect relationship is not yet established, and our understanding of the effects of fluid overload is limited. The goal of this study was to investigate an animal model that can characterize the physiological changes that occur in response to fluid overload. METHOD: Acute nonsurvival experiments were conducted in 16 Sprague-Dawley rats. Rats were initially anesthetized by inhaled isoflurane, while the femoral vein was cannulated and urethane (1.2-1.5 g/Kg body weight) was gradually delivered intravenously to induce anesthesia. Additional doses of urethane were delivered as necessary to maintain a surgical plane of anesthesia. A surgical incision was made on the cervical area to catheterize carotid artery to measure blood pressure. A pair of stainless-steel wires was injected into the tongue to measure genioglossus muscle activity (GGEMG). All physiological measurements were recorded as intravenous infusion of saline was provided to the rat (infusion rate = 22 ml/kg over 30 min). RESULTS: Acute saline overloading resulted in a 33% decrease in GGEMG, when compared to baseline. There was also a gradual drop in the respiratory rate (13% decrease) that reached statistical significance at 10 min after infusion was stopped. The blood pressure exhibited a 14% increase which subsequently returned to baseline within 40 min stopping infusion. There were no significant changes in the heart rate. CONCLUSION: The results of this study indicate that systemic fluid overload can affect significant changes in different physiological systems including reduction in genioglossus muscle activity, increase in blood pressure, and change autonomic nervous system function.

Happy Hypoxemia in COVID-19-A Neural Hypothesis.

Many COVID-19 patients are presenting with atypical clinical features. Happy hypoxemia with almost normal breathing, anosmia in the absence of rhinitis or nasal obstruction, and ageusia are some of the reported atypical clinical findings. Based on the clinical manifestations of the disease, we are proposing a new hypothesis that SARS-CoV-2 mediated inflammation of the nucleus tractus solitarius may be the reason for happy hypoxemia in COVID-19 patients.

Electrical stimulation of cranial nerves in cognition and disease.

The cranial nerves are the pathways through which environmental information (sensation) is directly communicated to the brain, leading to perception, and giving rise to higher cognition. Because cranial nerves determine and modulate brain function, invasive and non-invasive cranial nerve electrical stimulation methods have applications in the clinical, behavioral, and cognitive domains. Among other neuromodulation approaches such as peripheral, transcranial and deep brain stimulation, cranial nerve stimulation is unique in allowing axon pathway-specific engagement of brain circuits, including thalamo-cortical networks. In this review we amalgamate relevant knowledge of 1) cranial nerve anatomy and biophysics; 2) evidence of the modulatory effects of cranial nerves on cognition; 3) clinical and behavioral outcomes of cranial nerve stimulation; and 4) biomarkers of nerve target engagement including physiology, electroencephalography, neuroimaging, and behavioral metrics. Existing non-invasive stimulation methods cannot feasibly activate the axons of only individual cranial nerves. Even with invasive stimulation methods, selective targeting of one nerve fiber type requires nuance since each nerve is composed of functionally distinct axon-types that differentially branch and can anastomose onto other nerves. None-the-less, precisely controlling stimulation parameters can aid in affecting distinct sets of axons, thus supporting specific actions on cognition and behavior. To this end, a rubric for reproducible dose-response stimulation parameters is defined here. Given that afferent cranial nerve axons project directly to the brain, targeting structures (e.g. thalamus, cortex) that are critical nodes in higher order brain networks, potent effects on cognition are plausible. We propose an intervention design framework based on driving cranial nerve pathways in targeted brain circuits, which are in turn linked to specific higher cognitive processes. State-of-the-art current flow models that are used to explain and design cranial-nerve-activating stimulation technology require multi-scale detail that includes: gross anatomy; skull foramina and superficial tissue layers; and precise nerve morphology. Detailed simulations also predict that some non-invasive electrical or magnetic stimulation approaches that do not intend to modulate cranial nerves per se, such as transcranial direct current stimulation (tDCS) and transcranial magnetic stimulation (TMS), may also modulate activity of specific cranial nerves. Much prior cranial nerve stimulation work was conceptually limited to the production of sensory perception, with individual titration of intensity based on the level of perception and tolerability. However, disregarding sensory emulation allows consideration of temporal stimulation patterns (axon recruitment) that modulate the tone of cortical networks independent of sensory cortices, without necessarily titrating perception. For example, leveraging the role of the thalamus as a gatekeeper for information to the cerebral cortex, preventing or enhancing the passage of specific information depending on the behavioral state. We show that properly parameterized computational models at multiple scales are needed to rationally optimize neuromodulation that target sets of cranial nerves, determining which and how specific brain circuitries are modulated, which can in turn influence cognition in a designed manner.

Multiple cranial nerve injury in Ramsay Hunt Syndrome: a case report.

Herpes zoster oticus (Ramsay Hunt Syndrome) is characterized by facial nerve paralysis, ear pain and auricular skin rash. It occurs as a result of reactivation oflatent varicella zoster virus infection in the geniculate ganglion of the facial nerve. Major clinical symptoms include 7th nerve paralysis or cranial nerve paralysis and vesicles along the nerve with cocomitant ear pain. Other cranial nerve involvement although uncommon, can be found in some cases. In this study, a 74-year-old female patient had ipsilateral 8th, 9th and 10th cranial nerves injury. Cranial nerve paralysis accompanied with injury has been repor ted in R amsay Hunt Syndrome.

Novel method of intraoperative ocular movement monitoring using a piezoelectric device: experimental study of ocular motor nerve activating piezoelectric potentials (OMNAPP) and clinical application for skull base surgeries.

Intraoperative monitoring systems that utilize various evoked potentials for the detection and/or preservation of cranial nerves have become increasingly common due to recent technical and commercial developments, particularly during skull base surgeries. We established a novel system for the intraoperative monitoring of the extraocular motor nerves (eOMNs) using a piezoelectric device capable of detecting imperceptible vibrations induced by ocular movement, with sensors placed on the eyelids alone. We first evaluated the efficacy and reliability of this device for the intraoperative monitoring of eOMNs in two Beagle dogs. Based on the results, we then determined the appropriate stimulation parameters for use in human surgical cases involving removal of various skull base tumors. Animal experiments revealed that a 0.4 mA monopolar electrical stimulation was required to elicit significant responses and that these responses were not inferior to those obtained via the electrooculogram/electromyogram. Significant responses were also detected in preliminary clinical investigations in human patients, following both direct and indirect monopolar electrical stimulation of the oculomotor and abducens nerves, although obtaining responses from the trochlear nerve was difficult. Intraoperative monitoring using a piezoelectric device provides a simple and reliable method for detecting eOMNs, especially the oculomotor and abducens nerves. This monitoring system can be adapted to various surgeries for skull base tumor.

Cranial nerve outcomes in regionally recurrent head & neck melanoma after sentinel lymph node biopsy.

OBJECTIVE: Characterize long-term cranial nerve (CN) outcomes following sentinel lymph node biopsy (SLNB) based management for head and neck cutaneous melanoma (HNCM). METHODS: Longitudinal review of HNCM patients undergoing SLNB from 1997-2007. RESULTS: Three hundred fifty-six patients were identified, with mean age 53.5 ± 19.0 years, mean Breslow depth 2.52 ± 1.87 mm, and 4.9 years median follow-up. One hundred five (29.4%) patients had SLNB mapping to the parotid basin. Eighteen patients had positive parotid SLNs and underwent immediate parotidectomy / immediate completion lymph node dissection (iCLND), with six possessing positive parotid non-sentinel lymph nodes (NSLNs). Fifty-two of 356 (14.6%) patients developed delayed regional recurrences, including 20 total intraparotid recurrences: five following false negative (FN) parotid SLNB, three following prior immediate superficial parotidectomy, two following iCLND without parotidectomy, and the remaining 12 parotid recurrences had negative extraparotid SLNBs. Parotid recurrences were multiple (4.9 mean recurrent nodes) and advanced (n = 4 extracapsular extension), and all required salvage dissection including parotidectomy. Immediate parotidectomy/iCLND led to no permanent CN injuries. Delayed regional HNCM macrometastasis precipitated 16 total permanent CN injuries in 13 patients: 10 CN VII, five CN XI, and one CN XII deficits. Fifty percent (n = 10) of parotid recurrences caused ≥1 permanent CN deficits. CONCLUSIONS: Regional HNCM macrometastases and salvage dissection confer marked CN injury risk, whereas early surgical intervention via SLNB ± iCLND ± immediate parotidectomy yielded no CN injuries. Further, superficial parotidectomy performed in parotid-mapping HNCM does not obviate delayed intraparotid recurrences, which increase risk of CN VII injury. Despite lack of a published disease-specific survival advantage in melanoma, early disease control in cervical and parotid basins is paramount to minimize CN complications. LEVEL OF EVIDENCE: 4 (retrospective case series) Laryngoscope, 130:1707-1714, 2020.

Septic cavernous sinus thrombosis in a paediatric patient with undiagnosed thrombophilia.

A previously healthy 8-year-old girl presented to the hospital with right periorbital and forehead swelling in the setting of a 1-week history of upper respiratory symptoms. Contrast-enhanced CT revealed pansinusitis with orbital and frontal abscesses and thrombosis of the anterior portion of the superior sagittal sinus. She was treated with surgical drainage, antibiotics and anticoagulants. After 2 days she developed cranial nerve (CN) VI palsy. Contrast-enhanced MRI revealed small epidural abscess, right-sided thrombi of the cavernous sinus, internal carotid artery and internal jugular vein, in addition to the superior sagittal sinus. Thrombophilia workup revealed heterozygosity for factor V Leiden. She underwent 6 weeks of parental antibiotic therapy, 3 months of anticoagulation therapy and 7 days of steroids. With treatment, her visual acuity returned to baseline, CN VI palsy resolved and repeat imaging showed resolution of thrombi and epidural abscess, but persistent narrowing of the internal carotid artery.

Brainstem Glioma: Clinical Profile and Challenges of Management in a Developing Country.

BACKGROUND AND OBJECTIVES: Brainstem gliomas are relatively rare tumours of the central nervous system which have varying presentations and clinical course. This study aims to analyse the clinical profile and challenges of management of these tumours in a resource-limited country. METHIODS: We retrospectively analysed the data from the records of the patients managed for briainstem glioma between January 2010 and July 2017. RESULTS: There were 11 patients in the study (7 males and 4 females). The median age at diagnosis was 9 years. Eight of the patients were less than 15 years. The duration of symptoms ranged from 1 month to 2 years. All the patients had cranial nerve deficits at presentation, while 7 patients had cerebellar signs. Hydrocephalus was present in 4 patients. The lesion was pontine in 9 patients and tectal in 2. Three of the patients with hydrocephalus had ventriculoperitoneal shunt insertion while one patient refused surgery. Only one of the patients had radiotherapy. None of the patients received chemotherapy. A patient was dishcarged against medical advice. One patient is still alive after 4 years while another patient is alive after 2 years. The other 9 patients are dead with a mean survival period of 6 months. CONCCLUSION: Most of the tumours in this series were located in the pons and ran aggressive courses. Majority of our patients did not have access to radiotherapy while none had chemotherapy.

Preliminary design and validation of the "6-K-scale" for bulbar symptoms evaluation in SBMA.

BACKGROUND: Spinal and bulbar muscular atrophy (SBMA) is a late onset, X-linked neuromuscular disease. Bulbar symptoms are a main characteristic of the disease but a tool for their clinical evaluation still does not exist. The aim of this study was to design and test a new scale (6-K-scale) for evaluation of bulbar function in SBMA. METHODS: We considered 60 genetically confirmed SBMA patients and built a scale to evaluate the V, VII, IX, X, and XII cranial nerves (CN) and the ansa cervicalis. Functional status was evaluated through the Spinal and Bulbar Muscular Atrophy Functional Rating Scale (SBMAFRS), 6-min-walk-test (6MWT), Adult Myopathy Assessment Tool (AMAT) scale, and FVC%. Twenty patients underwent a re-test after 3 weeks, while 31 were tested longitudinally after 6 months. Validation of the scale included reliability assessment and factorial analysis. To evaluate convergent validity, correlations between the 6-K-scale and functional parameters were performed. RESULTS: Internal consistency as measured by Cronbach's alpha was high (0.85) as was test-retest reliability. Principal component analysis yielded a six-factor solution accounting for 71.7% of the variance. The scale score was strongly correlated with the functional parameters. CONCLUSION: In conclusion, we designed and validated a new scale for bulbar evaluation in SBMA patients. This scale will be a useful tool in the clinical practice as well as a possible outcome measure in clinical trials.

Cranial nerve involvement in patients with MOG antibody-associated disease.

Objective: To describe clinical and radiologic features of cranial nerve (CN) involvement in patients with myelin oligodendrocyte glycoprotein antibodies (MOG-IgG) and to assess the potential underlying mechanism of CN involvement using a nonhuman primate (NHP) model. Methods: Epidemiologic, clinical, and radiologic features from a national cohort of 273 MOG-IgG-positive patients were retrospectively reviewed for CN involvement between January 2014 and January 2018. MOG-IgG binding was evaluated in CNS, CN, and peripheral nerve tissues from NHP. Results: We identified 3 MOG-IgG-positive patients with radiologic and/or clinical CN involvement. Two patients displayed either trigeminal or vestibulocochlear nerve lesions at the root level, and the remaining patient had an oculomotor nerve involvement at the root exit and at the cisternal level. Additional CNS involvement was found in all 3 patients. None of the 3 patients' sera recognized MOG expression in CN of NHP. Conclusion: Craneal nerve involvement can coexist in patients with MOG antibody disease, although the underlying pathophysiology remains elusive.

Observations on the Natural History of Camurati-Engelmann Disease.

Camurati-Engelmann disease (OMIM 31300) is a rare cranio-tubular bone dysplasia characterized by osteosclerosis of the long bones and skull caused by dominantly-inherited mutations in the transforming growth factor beta 1 (TGFB1) gene. A wide variation in phenotype has been recognized, even within families carrying the same mutation. In addition, aspects of the natural history of the disorder, in particular whether it is always progressive or can remit spontaneously, remain uncertain. In a large kindred carrying a TGFB1 gene mutation (c.653G > A; p.R218H) we have attempted to clarify the extent of phenotypic variability and the natural history of the disease through detailed individual histories of symptoms, and skeletal imaging by both radiography and scintigraphy. Only one subject had the classical childhood onset with bone pain in the legs and gait disturbance. Eight subjects reported the onset of leg pain in their teenage years that, by their early 20s, had either resolved or persisted at a low level. Two of these eight later developed cranial nerve palsies. There was a wide variation in the radiographic appearance in adults, but disease extent and activity in long bones, as assessed by scintigraphy, was inversely correlated with age (p < 0.025). In younger subjects the radiographic and scintigraphic appearances were concordant, but in older subjects the scintigram could be quiescent despite florid radiographic changes. Sequential scintigrams in two subjects showed reduced activity in the later scan. One subject had suffered meningoencephalitis in early childhood that resulted in paresis of one arm. The affected arm showed markedly less disease involvement, implicating mechanical or growth factors in its etiology. Our data suggest that the natural history of Camurati-Engelmann disease can be benign, and that disease activity commonly attenuates in adulthood. Severe cases of childhood onset and/or with cranial nerve involvement, may occur only in a minority of mutation carriers. © 2019 American Society for Bone and Mineral Research.

Vestibular impairment in chronic inflammatory demyelinating polyneuropathy.

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a common, treatable, autoimmune peripheral neuropathy considered to produce imbalance by weakness and proprioceptive impairment rather than vestibular impairment. We measured semicircular canal vestibular function in 21 CIDP patients (15M/6F) by the video head impulse test and postural stability with a battery comprising the modified Clinical Test of Sensory Integration and Balance, the Berg Balance Scale, the Dynamic Gait Index, the Fall Efficiency Scale, and the International Cooperative Ataxia Rating Scale. Of the 21 patients, 16 had vestibular impairment, ranging from mild-affecting just a single semicircular canal, to severe-affecting all 6 canals. Although the severity of the vestibular impairment did not correlate either with the severity of the postural imbalance or of the peripheral neuropathy, our data show that vestibular impairment is an additional challenge to balance that some CIDP patients will face.

Functional Recovery of Cranial Nerves in Patients with Traumatic Orbital Apex Syndrome.

OBJECTIVE: Traumatic orbital apex syndrome (TOAS) is a rare disease characterized by the damage of cranial nerves (CNs) II, III, IV, and VI. The aim of our study was to analyze the functional recovery of CNs in TOAS and discuss the management of these patients. METHODS: We retrospectively reviewed 28 patients with TOAS treated in the Department of Neurosurgery, Shanghai Changzheng Hospital from February 2006 to February 2016. Functional recovery of CNs was evaluated based on extraocular muscle movement and visual perception. Follow-up duration was at least 6 months. RESULTS: There were 26 males and 2 females with a mean age of 35.3 years. The most common cause of TOAS was traffic accident. CN IV suffered the lightest injury among CNs III, IV, and VI. CN II achieved obvious improvement at 3-month follow-up, while other CNs enjoyed evident improvement at 6-month follow-up. There was no significant difference between conservative treatment and surgical decompression. CONCLUSION: CNs passing through orbital apex region might recover to different degrees several months after proper management. Clinical decision should be individualized and surgical decompression could be considered with evidence of fracture, hematoma, or deformation.

Diplopía binocular en un hospital terciario: etiología, diagnóstico y tratamiento / Binocular diplopia in a tertiary hospital: Aetiology, diagnosis and treatment

Objetivo: Estudiar las causas, el diagnóstico, el tratamiento y los resultados de una serie de casos con diplopía binocular. Material y método: Estudio retrospectivo de pacientes referidos a consulta de diplopía del servicio de oftalmología durante un año. La diplopía se clasificó en aguda ≤1mes desde su inicio, subaguda (1-6 meses) y crónica (>6). La resolución de la diplopía se consideró espontánea si desaparecía sin necesidad de tratamiento, parcial cuando quedaba intermitente y no resolución espontánea cuando se necesitó tratamiento. Se consideró buen resultado cuando desaparecía por completo la diplopía con/sin tratamiento o era intermitente sin afectar a la calidad de vida del paciente. Resultados: Un total de 60 casos fueron incluidos. La edad media fue 58,65 años (60% mujeres). El 60% fueron agudas o subagudas. La evolución media de la diplopía fue 82,97 semanas. La etiología más frecuente fue isquémica en el 45%. La parálisis del sexto nervio fue el diagnóstico más frecuente: 38,3%, después estrabismos descompensados: 30%. Se encontraron lesiones en las pruebas de neuroimagen en un 17,7%. La resolución espontánea se produjo en un 28,3%. El 53,3% presentó un buen resultado con desaparición de la diplopía al final del estudio. Conclusiones: Las causas más frecuentes de diplopía binocular fueron las parálisis de los nervios craneales, especialmente del vi, seguidas de estrabismos descompensados. Se encontraron lesiones estructurales en las pruebas de imagen en un porcentaje importante. Solo en un tercio de los pacientes la diplopía se resolvió espontáneamente y la mitad tuvo un mal resultado a pesar del tratamiento (AU)

Objective: To study the causes, diagnosis and treatment in a case series of binocular diplopia. Material and method: A retrospective chart review was performed on patients seen in the Diplopia Unit of a tertiary centre during a one-year period. Diplopia was classified as: acute ≤ 1 month since onset; subacute (1-6 months); and chronic (>6 months). Resolution of diplopia was classified as: spontaneous if it disappeared without treatment, partial if the course was intermittent, and non-spontaneous if treatment was required. It was considered a good outcome when diplopia disappeared completely (with or without treatment), or when diplopia was intermittent without significantly affecting the quality of life. Results: A total of 60 cases were included. The mean age was 58.65 years (60% female). An acute or subacute presentation was observed in 60% of the patients. The mean onset of diplopia was 82.97 weeks. The most frequent aetiology was ischaemic (45%). The most frequent diagnosis was sixth nerve palsy (38.3%), followed by decompensated strabismus (30%). Neuroimaging showed structural lesions in 17.7% of the patients. There was a spontaneous resolution in 28.3% of the cases, and there was a good outcome with disappearance of the diplopia in 53.3% at the end of the study. Conclusions: The most frequent causes of binocular diplopia were cranial nerve palsies, especially the sixth cranial nerve, followed by decompensated strabismus. Structural lesions in imaging tests were more than expected. Only one third of patients had a spontaneous resolution, and half of them did not have a good outcome despite of treatment (AU)

[Idiopathic cranial nerve failure]. / Idiopathische Hirnnervenausfälle.

Cranial nerve lesions require a thorough diagnostic work-up and known etiologies have to be excluded before the term idiopathic can be considered. The focus of the present review is on idiopathic peripheral facial nerve paralysis (Bell's palsy) for which this terminology has been established. For all other cranial nerve lesions the typical clinical signs, established etiologies and possible diagnostic pitfalls are discussed.

Bilateral painful tic convulsif.

'Painful tic convulsif' (PTC) describes the coexistence of hemifacial spasm and trigeminal neuralgia. In this report, we describe a unique presentation of bilateral PTC in a man with bilateral hemifacial spasm and trigeminal neuralgia secondary to neurovascular conflict of all four cranial nerves. Following failed medical and radiofrequency therapy, microvascular decompression of three of the four involved nerves was performed, where the offending vessels were mobilised and Teflon used to prevent conflict recurrence. He continues to respond to Botox for right hemifacial spasm. Since surgery, he remains pain free bilaterally and spasm free on the left.