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Background: Limited research has suggested that trigeminal neuralgia (TN), an often-idiopathic pain disorder affecting the head and face, may arise from compression of the cervical spinal cord due to involvement of the spinal trigeminal tract. We hypothesize that adults with TN will have a greater likelihood of concurrent degenerative cervical myelopathy (DCM) compared to matched adults without TN. Methods: We will retrieve de-identified data from a United States network (TriNetX, Inc.) including medical records and medical/pharmacy claims, of >120 million patients attending academic healthcare institutions, with a data range spanning the previous 20 years. We will create two groups of adults (≥18 years old) based on the presence/absence of TN, excluding individuals with predisposing conditions for TN (e.g., multiple sclerosis, ophthalmic and oral/ maxillofacial surgery) and propensity match for potential sources of selection bias (e.g., age, sex, body mass index, diabetes mellitus, hypertensive diseases, migraine, osteoporosis). Results: The prevalence of DCM per group and likelihood of DCM will be calculated using an odds ratio and reported with 95% confidence intervals and P-value both before and after matching. Conclusions: This will be the largest study to our knowledge to examine for a potential association between TN and DCM.

Purpose: To evaluate the efficacy of early neuroimaging in patients with acute isolated 3rd, 4th, and 6th nerve palsy.Methods: Neuroimaging tests were performed in patients >50 years old with acute isolated 3rd, 4th, and 6th nerve palsy. The frequencies and types of abnormal findings were evaluated. All patients were divided into ischemic and non-ischemic groups and differences in clinical characteristics between groups were analyzed.Results: Of the 55 patients, nine (16.4%) had abnormal findings on brain imaging. Among 26 patients with 6th nerve palsy, four (15.4%) were found to have a cause including brain tumor, cerebrovascular compression, carotid cavernous fistula. Superior oblique muscle atrophy was found in two of 19 patients (10.5%) with 4th nerve palsy. Three of 10 patients (30%) with 3rd nerve palsy had abnormal findings and there were cases of pituitary apoplexy, posterior communicating, and cerebrovascular compression.Conclusions: In patients with acute isolated oculomotor paralysis, early neuroimaging tests play an important role in differential diagnosis and evaluation of causes that may lead to mortality.

Diplopia is a rare complication of spine surgery. The abducens nerve is one of the cranial nerves most commonly related to diplopia caused by traction injury. We report a case of a 71-year-old woman who presented with diplopia developing from abducens nerve palsy after C1-C2 fixation and fusion due to atlantoaxial subluxation with cord compression. As soon as we discovered the symptoms, we suspected excessive traction by the instrument and subsequently performed reoperation. Subsequently, the patient's symptoms improved. In other reported cases we reviewed, most were transient. However, we thought that our rapid response also helped the patient's fast recovery in this case. The mechanisms by which postoperative diplopia develops vary and, thus, remain unclear. We should pay attention to the fact that the condition is sometimes an indicator of an underlying, life-threatening condition. Therefore, all patients with postoperative diplopia should undergo thorough ophthalmological and neurological evaluations as well as careful observation by a multidisciplinary team.

Granulomatosis with polyangiitis (GPA, formerly Wegener’s) is a rare form of vasculitis, commonly affecting the upper and lower respiratory tract with simultaneous glomerulonephritis. Ear, nose and throat (ENT) manifestations account for the majority of presentations. The presence of antineutrophil cytoplasmic antibody is a recognized hallmark of GPA, but clinicians should remain cautious of false negative results. We describe a rare case of GPA presenting with concurrent middle ear disease and multiple lower cranial nerve palsies. Clinical judgment was affected by repeated negative autoimmune screens, and a definitive diagnosis was only achieved following renal biopsy. Reported cases of GPA presenting with mastoiditis or cranial nerve involvement are typically seropositive, with seronegative GPA following a less aggressive process. This case highlights the importance of clinical suspicion in the face of treatment resistant ENT pathology, and the need for early histopathological analysis. Early diagnosis and treatment are crucial in limiting disease progression.

Case report - Introduction Bisphosphonates are known to rarely cause multi-system inflammation, including multiple cranial neuropathies. This is possibly via provoking transient cytokine storm. The literature reports bisphosphonate-associated orbital inflammatory syndrome, and one case of retrobulbar optic neuritis following zoledronate. Bisphosphonate manufacturers report conjunctivitis, blurred vision, scleritis, orbital inflammation, uveitis and episcleritis as ocular side effects. Separately, neurological sequalae, including cranial neuropathies, are reported following COVID-19 infection and vaccination. Here, we report the first case of cavernous sinus inflammation temporally related to both zoledronate infusion, and more remotely, to Pfizer-BioNTech COVID-19 vaccination. Case report - Case description A 76-year-old white man developed fever, bony leg pain – which rendered him unable to walk – and frontal headache, within 8 hours of his first zoledronate infusion for osteoporosis. A few weeks earlier he received his first Pfizer-BioNTech COVID-19 vaccine. His General Practitioner commenced a short course of low-dose oral prednisolone for the episode. One week later, off prednisolone, the headache localised around the left eye. He developed horizontal diplopia associated with abduction deficit. He was diagnosed with left VIth nerve palsy. He was started on high-dose steroids and clopidogrel (with PPI) with neuroimaging to exclude stroke or venous sinus thrombosis. Two weeks later, the diplopia worsened over 4 days, with new left adduction deficit (-2 limitation), left ptosis 1-2mm and anisocoria 0.5-1mm R>L suggestive of partial third nerve palsy and early Horner’s syndrome. Ocular and neurological examinations were otherwise normal. He wore varifocals and had migraines, osteoporosis, and asthma, for which he used inhalers. He worked in visual arts and was an ex-smoker (>50 years) with moderate alcohol intake. Blood results revealed CRP 38mg/L, but otherwise normal inflammation/vasculitis/infection screen; anti-thyroglobulin antibodies were >4000 U/ml; GQ1P, Creatinine Kinase, anti-ganglioside, and Anti-AChR/MuSK antibodies were normal. CT head and Optical Coherence Tomography were unremarkable. An enhanced MRI of the brain and orbits revealed abnormal thickening and T2 hyper-intensity of the left oculomotor nerve, most notably involving the left canalicular portion. The left cavernous sinus also appeared asymmetrically bulky with a rind of abnormal enhancing soft tissue in the left cavernous sinus. Subtle STIR hyper-intensity was also observed in the ipsilateral CN III-innervated extra-ocular muscles. After a 6-week course of tapering prednisolone, the vertical diplopia and leg swelling persisted; the horizontal diplopia and headaches had resolved. By 3 months, there was resolution with mild residual visual changes. Case report - Discussion We report a constellation of symptoms relating to multi-system inflammatory syndrome involving the cavernous sinus. There is a lack of epidemiological data on the incidence of this rare presentation in the population. This case has close temporal association to bisphosphonate infusion ( This case highlights a wider issue relating to the challenging possibility of ascertainment bias and increased ‘Yellow Card’ reporting of rare presentations during this historic global coronavirus pandemic, which may or may not have any true causal association to vaccination. There is difficulty in disentangling a true vaccine reaction from an unrelated presentation of a rare condition with an unknown baseline incidence rate. This is especially topical given that the majority of the population are receiving the coronavirus vaccination at this time. We also question what a plausible cut-off point would be to propose a temporal relationship for an adverse reaction; in the literature, adverse reactions have been postulated to develop beyond 1 month after the provoking agent. Case report - Key learning points

Background: Nervus intermedius neuralgia (NIN), known as geniculate neuralgia (GN), is an uncommon cranial nerve disease caused by an offending vessel compressing the nervus intermedius (NI). Microvascular decompression (MVD) has now become a valued treatment approach for NIN because it can resolve neurovascular conflict (NVC) at the root entry zone of the NI. In the era of continuously optimizing and improving the surgical technique of MVD, further minimization of all possible postoperative complications is not only welcome but also necessary.Objective: The aim of this work is to assess the postoperative outcome of direct visualization of the NI during the MVD procedure.Methods: This study retrospectively reviewed the clinical records of a group of seven consecutive patients with NIN who underwent MVD in the period of 2013–2020 in our clinic and 16 studies reported NIN patients who underwent MVD in the period of 2007–2020.Results: In total, 91.3% of all patients experienced immediate and complete relief of cranial neuralgia after MVD. Six of 23 patients have experienced direct visualization of the NI intraoperatively, and 66.7% of those patients had complications such as facial paralysis, dysacousia, or a combination of these conditions postoperatively. Slight surgical approach-related complications such as complaints associated with excessive drainage of cerebrospinal fluid (CSF), nausea and vertigo, and delayed wound union were observed in 80% of the remaining 15 patients, and these symptoms are totally relieved in the telephone and outpatient follow-up after 6 months.Conclusion: Our case series shows that MVD produced immediate pain relief in the majority of NIN patients. MVD carries surgical risk, especially in patients who experience direct visualization of the NI after mechanical stretch and blunt dissection in surgical procedures. Attempts to avoid mechanical stretch and blunt dissection of the compressed nerve were important for intraoperative neuroprotection, especially facial nerve protection

Opsoclonus myoclonus syndrome (OMS) is a rare immune-mediated paraneoplastic or para/-post-infectious syndrome characterized by “dancing” eye movements, myoclonus, and ataxia. Neuropsychiatric symptoms have also been reported. Without treatment, OMS may progress to further neurological impairment and even death. Autoimmune attack of CNS structures in OMS is most commonly mediated by anti-Ri (also known as ANNA2) IgG antibodies, with additional findings implicating antibodies targeting various neurotransmitter receptors. Prompt immunotherapy and neoplasm treatment may result in improvement. We report a novel association of Contactin-Associated Protein-Like 2 (Caspr2) antibodies occurring in association with paraneoplastic OMS. While breast cancer and small cell lung cancer (SCLC) are more commonly associated with OMS among adults, we characterize a novel association between Caspr2 antibody in a patient with mixed non-small cell and small cell lung carcinoma.

Background: Recurrent painful ophthalmoplegic neuropathy (RPON), formerly known as ophthalmoplegic migraine (OM), is a poorly understood condition that presents with recurrent unilateral headaches and at least one ocular cranial nerve (CN) palsy, generally in childhood. There has been ongoing debate about whether the etiology of this disorder is neuropathic or related to migraines. The Case: We present a case about a 10-year-old male with his third presentation of RPON, repeatedly affecting his right oculomotor nerve. His treatment choices are discussed, along with associated outcomes. The patient was treated with topiramate with resolution of his symptoms occurred within one month. Conclusion: As the annual incidence of RPON is rare at fewer than 1 case per million people, clear documentation of observed cases with treatment failures and successes is key to building evidence for future management.

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.

Dr. Dinkin et al. reported 2 cases with MRI confirmation of cranial nerve inflammation within several days of systemic symptoms of the novel coronavirus disease 2019 (COVID-19). Both patients tested positive for SARS-CoV-2 by PCR from a nasal swab, and both were treated with hydroxychloroquine (one also with intravenous immunoglobulin), with mild short-term improvement. The authors suggest the inflammatory cranial neuropathies could have been because of a postviral immune-related response. Dr. Machado wonders whether the cranial neuropathies could have resulted from direct viral invasion, given their onset shortly after the viral symptoms started. As noted by Gutierrez-Ortiz et al. (Dinkin et al., reference 2 below) 2 patients developed evidence of cranial nerve inflammation and autoimmune neuropathy within 3–5 days of COVID-19 symptom onset. Each of these 4 cases highlight the accelerated time course of inflammatory cranial neuropathies after systemic manifestations of COVID-19, which seems to be more rapid than has been typically reported for variants of Guillain-Barre syndrome after other infectious illnesses. These observations may also support that there is an asymptomatic phase of this viral infection during which an immune response may be generated. Dr. Dinkin et al. reported 2 cases with MRI confirmation of cranial nerve inflammation within several days of systemic symptoms of the novel coronavirus disease 2019 (COVID-19). Both patients tested positive for SARS-CoV-2 by PCR from a nasal swab, and both were treated with hydroxychloroquine (one also with intravenous immunoglobulin), with mild short-term improvement. The authors suggest the inflammatory cranial neuropathies could have been because of a postviral immune-related response. Dr. Machado wonders whether the cranial neuropathies could have resulted from direct viral invasion, given their onset shortly after the viral symptoms started. As noted by Gutierrez-Ortiz et al. (Dinkin et al., reference 2 below) 2 patients developed evidence of cranial nerve inflammation and autoimmune neuropathy within 3–5 days of COVID-19 symptom onset. Each of these 4 cases highlight the accelerated time course of inflammatory cranial neuropathies after systemic manifestations of COVID-19, which seems to be more rapid than has been typically reported for variants of Guillain-Barre syndrome after other infectious illnesses. These observations may also support that there is an asymptomatic phase of this viral infection during which an immune response may be generated.

BackgroundNecrotising otitis externa is a progressive infection of the external auditory canal which extends to affect the temporal bone and adjacent structures. Progression of the disease process can result in serious sequelae, including cranial nerve palsies and death. There is currently no formal published treatment guideline.ObjectiveThis study aimed to integrate current evidence and data from our own retrospective case series in order to develop a guideline to optimise necrotising otitis externa patient management.MethodsA retrospective review of necrotising otitis externa cases within NHS Lothian, Scotland, between 2013 and 2018, was performed, along with a PubMed review.ResultsPrevalent presenting signs, symptoms and patient demographic data were established. Furthermore, features of cases associated with adverse outcomes were defined. A key feature of the guideline is defining at-risk patients with initial intensive treatment. Investigations and outcomes are assessed and treatment adjusted appropriately.ConclusionThis multi-departmental approach has facilitated the development of a succinct, systematic guideline for the management of necrotising otitis externa. Initial patient outcomes appear promising.

OBJECTIVES: Acute nystagmus (AN) is an uncommon neurologic sign in children presenting to pediatric emergency departments. We described the epidemiology, clinical features, and underlying causes of AN in a large cohort of children, aiming at identifying features associated with higher risk of severe underlying urgent conditions (UCs). METHODS: Clinical records of all patients aged 0 to 18 years presenting for AN to the pediatric emergency departments of 9 Italian hospitals in an 8-year period were retrospectively reviewed. Clinical and demographic features and the underlying causes were analyzed. A logistic regression model was applied to detect predictive variables associated with a higher risk of UCs. RESULTS: A total of 206 patients with AN were included (male-to-female ratio: 1.01; mean age: 8 years 11 months). The most frequently associated symptoms were headache (43.2%) and vertigo (42.2%). Ataxia (17.5%) and strabismus (13.1%) were the most common neurologic signs. Migraine (25.7%) and vestibular disorders (14.1%) were the most common causes of AN. Idiopathic infantile nystagmus was the most common cause in infants CONCLUSIONS: AN should be considered an alarming finding in children given the risk of severe UCs. Cranial nerve palsy, ataxia, and strabismus should be considered red flags during the assessment of a child with AN.

Importance Postviral olfactory loss is a common cause of olfactory impairment, affecting both quality of life as well as overall patient mortality. It is currently unclear why some patients are able to recover fully after a loss while others experience permanent deficit. There is a lack of research on the possible association between postviral olfactory loss and other cranial neuropathies. Objective To evaluate the incidence of other cranial nerve deficits in patients with postviral olfactory loss and determine if there is an association with neurologic injury in this group. This study also sought to determine if other known risk factors were associated with postviral olfactory loss. Design, Setting, and Participants A case-control study was conducted at a tertiary care rhinology clinic from January 2015 to January 2018 to review the incidence of cranial neuropathies in 2 groups of patients, those with postviral olfactory loss and those with chronic rhinosinusitis without olfactory loss used as a control group. Exposures The Stanford Translational Research Integrated Database Environment (STRIDE) system was used for patient identification and data extraction. Patients with a history of olfactory loss or chronic rhinosinusitis as well as incidence of cranial neuropathies were identified by usingInternational Statistical Classification of Diseases and Related Health Problems, Tenth Revision (ICD-10) codes. Main Outcomes and Measures This study reviewed incidence of postviral or idiopathic cranial neuropathies in both patient groups, while also evaluating for any difference in demographic characteristics, comorbidities, or other patient-related factors. Results There were 91 patients in the postviral olfactory loss group and 100 patients in the control group, which were age and sex matched as closely as possible. Of the 91 patients with postviral olfactory loss, mean (SD) age was 56.8 (15.3), and 58 (64%) were women; for the control group, the mean (SD) age was 57.5 (15.6) years, and 63 (63%) were women. Racial breakdown was similar across cases and controls, with white individuals making up 59% to 65%; Asian individuals, 20% to 24%; black individuals, approximately 3%; Hispanic individuals, approximately 1%; and the remaining patients being of other race/ethnicity. The incidence of other cranial neuropathies in the postviral olfactory loss group was 11% compared with 2% within the control group (odds ratio, 6.1; 95% CI, 1.3-28.4). The study also found 2 cases of multiple cranial neuropathies within a single patient within the olfactory group. Family history of neurologic disease was associated with more than 2-fold greater odds of cranial nerve deficit (odds ratio, 3.05; 95% CI, 0.59-15.68). Conclusions and Relevance Postviral olfactory loss appears to be associated with a higher incidence of other cranial neuropathies. It is possible that there is an inherent vulnerability to nerve damage or decreased ability for nerve recovery in patients who experience this disease process.

Congenital cranial dysinnervation disorders are a group of complex strabismus syndromes that present as congenital and non-progressive ophthalmoplegia. The genetic defects are associated with aberrant axonal targeting onto the motoneurons, development of motoneurons, and axonal targeting onto the extraocular muscles. We describe here the surgical management of a 16-year-old boy who presented with complex strabismus secondary to hypoplasia of the third cranial nerve and aberrant innervation of the upper ipsilateral eyelid.

Objective: To determine the frequency, clinical characteristics, associations, and outcomes of different types of peripheral nervous system (PNS) disease in a multiethnic/multiracial, prospective inception cohort of systemic lupus erythematosus (SLE) patients. Methods: Patients were evaluated annually for 19 neuropsychiatric (NP) events including 7 types of PNS disease. SLE disease activity, organ damage, autoantibodies, and patient and physician assessment of outcome were measured. Time to event and linear regressions were used as appropriate. Results: Of 1,827 SLE patients, 88.8% were female, and 48.8% were white. The mean ± SD age was 35.1 ± 13.3 years, disease duration at enrollment was 5.6 ± 4.2 months, and follow-up was 7.6 ± 4.6 years. There were 161 PNS events in 139 (7.6%) of 1,827 patients. The predominant events were peripheral neuropathy (66 of 161 [41.0%]), mononeuropathy (44 of 161 [27.3%]), and cranial neuropathy (39 of 161 [24.2%]), and the majority were attributed to SLE. Multivariate Cox regressions suggested longer time to resolution in patients with a history of neuropathy, older age at SLE diagnosis, higher SLE Disease Activity Index 2000 scores, and for peripheral neuropathy versus other neuropathies. Neuropathy was associated with significantly lower Short Form 36 (SF-36) physical and mental component summary scores versus no NP events. According to physician assessment, the majority of neuropathies resolved or improved over time, which was associated with improvements in SF-36 summary scores for peripheral neuropathy and mononeuropathy. Conclusion: PNS disease is an important component of total NPSLE and has a significant negative impact on health-related quality of life. The outcome is favorable for most patients, but our findings indicate that several factors are associated with longer time to resolution. (Less)

Imaging evaluation of patients with cranial nerve disorders is performed in daily practice. The complexity of the anatomy and the function of all 12 pairs of cranial nerves are challenging. Patient’s history, clinical, and biological data are mandatory to select the best imaging modality and protocol. Imaging interpretation has to be careful in order to identify intraaxial, extraaxial intracranial, and extracranial lesions. A wide variety of diseases are presented, including vascular, infections, inflammations, benign and malignant tumors, trauma, and neural degeneration.

Cranial nerve disease outside the skull base is a common cause of facial and/or neck pain, which causes significant disability for patients and frustration for clinicians. Neuropathy in this region can be traumatic, idiopathic, or iatrogenic secondary to dental and surgical procedures. MR neurography is a modification of conventional MRI techniques dedicated to evaluation of peripheral nerves and is being increasingly used for imaging of peripheral neuropathies at various sites in the body. MR neurography facilitates assessment of different causes of craniofacial pain and cranial nerves and allows elegant depiction of a multitude of regional neuropathies. This article discusses the anatomy, pathologic conditions, and imaging findings of the commonly implicated but difficult to image infratentorial nerves, such as the peripheral trigeminal nerve and its branches, facial nerve, glossopharyngeal nerve, vagus nerve, hypoglossal nerve, and greater and lesser occipital nerves. ©RSNA, 2018.

Background Herpes zoster of the head and neck commonly presents with Ramsay Hunt syndrome. However, vesicular eruptions may occur on the pharyngeal or laryngeal area with multiple lower cranial-nerve (CN) palsy. Case Report We report on the case of a 54-year-old man with herpes zoster of the pharynx and larynx with multiple CN palsy and persistent hiccups. He initially developed progressive dysphagia, hoarseness, and persistent hiccups (CN IX and X). After admission, Dizziness, hearing impairment, and peripheral facial palsy (CN VII and VIII) were complicated. The results of a polymerase chain reaction test of saliva and vesicular fluid from the ear and throat were strongly positive for varicella zoster virus. The progression of CN palsy was in an ascending sequence. Conclusion We suggest that the sequence of CN palsy may be either ascending or descending, depending on the initial site of involvement.

To evaluate the bilateral patterns and motor function of the extralaryngeal branches (ELB) of the recurrent laryngeal nerve(RLN). This study included 500 consecutive patients who underwent total thyroidectomy. Intraoperative nerve monitoring (IONM) was used in 230 patients. Demographic data, indications for surgery, the bilateral patterns of ELB of the RLN, electromyographic activity of the ELB, distance between the branching point to the entrance into the larynx, and the rate of postoperative morbidity were analyzed. The overall rate of ELB was 27.6% (276/1000). A single trunk of the RLN on both sides was found in 269 (54%) patients, whereas ELB on both sides was observed in 45 (9%) patients. The rates of ELB on the left and right sides were 26.6 and 28.6%, respectively. Of the 89 branched nerves which were dissected using IONM, an evoked motor response was present in 100% of the anterior branches and 5.6% of the posterior branches. The mean branching distance of the RLN was significantly greater in female patients than in male patients on the left side (p = 0.031). The patterns of ELB showed no significant difference in male and female patients. The rates of postoperative transient and permanent hypoparathyroidism and unilateral RLN palsy were 21.6 and 2.8%, and 3.2 and 0.8%, respectively. The rate of RLN palsy was higher in branched nerves compared to those with a single trunk (0.75 vs 0.3%; p = 0.2). Unilateral ELB of the RLN might be observed in approximately 1/4 of the patients, while bilateral branching is rare. A few number of posterior branches of the RLN can have motor function. The RLN’s with ELB might have a higher risk of injury compared to those with a single trunk.

Background and purpose Zika virus (ZIKV) infection has been associated with an increased incidence of Guillain-Barre syndrome (GBS) but the relative frequency of acute inflammatory demyelinating polyradiculoneuropathy (AIDP) and axonal GBS subtypes is controversial. Methods Twenty GBS patients diagnosed according to the Brighton criteria during the ZIKV outbreak in Cucuta, Colombia, were evaluated clinically and electrophysiologically. The electrodiagnosis of GBS subtypes was made according to a recently described criteria set that demonstrated a high diagnostic accuracy on the basis of a single test. The electrophysiological features of 34 Italian AIDP patients were used as control. Results All patients had symptoms compatible with ZIKV infection before the onset of GBS and ZIKV infection was laboratory confirmed through a plaque reduction neutralization test (PRNT90 ) in 100% of patients. The median time from onset of ZIKV infection symptoms to GBS was 5 days (interquartile range 1-6 days). Cranial nerve palsy was present in 85% of patients (facial palsy in 75%, bulbar nerve involvement in 60%), autonomic dysfunction in 85%, and 50% of patients required invasive mechanical ventilation. AIDP was diagnosed in 70% of patients. 40% of nerves of AIDP patients showed a prevalent distal demyelinating involvement but this pattern was not different from the Italian AIDP patients without ZIKV infection. Conclusions Guillain-Barre syndrome associated with ZIKV infection in Cucuta is characterized by a high frequency of cranial nerve involvement, autonomic dysfunction and requirement of mechanical ventilation indicating an aggressive and severe course. AIDP is the most frequent electrophysiological subtype. Demyelination is prevalent distally but this pattern is not specific for ZIKV infection.

A compressão vasculonervosa é relacionada a inúmeras enfermidades dos nervos cranianos, sendo a ressonância magnética (RM) o exame de escolha para o adequado planejamento cirúrgico. São ilustrados quatro casos de compressão vasculonervosa na RM e sua importância como método de investigação complementar

Background/Aims: The need for totally implantable central venous access devices (TICVADs) has increased with increased opportunities in the use of chemotherapy and parenteral nutrition. This study aimed to determine the outcomes of TICVAD implantation and use in patients aged ≥85 years. Methods: Between January 2010 and August 2016, 117 patients underwent TICVAD implantation and their records were retrospectively reviewed. Results: Participants were divided into 2 groups (plus-85 and sub-85 groups). Fifty-five patients (47.0%) had solid organ cancer alone; 35 patients (29.9%) had cerebrovascular or cranial nerve disease. The average follow-up period was 201 (2–1,620) days. Major complications were identified in 6 (14.6%) plus-85 patients and 11 (14.5%) sub-85 patients (p = 0.9813). Catheter-related infections developed in 3 plus-85 (7.3%) and 4 sub-85 patients (5.3%; p = 0.6549). There were no significant group differences in hematoma, pneumothorax, occlusion, and removal rates. In plus-85 patients examined just before surgery and a month after surgery, increased rates of serum albumin and Onodera’s prognostic nutritional index were observed in 48% (14/39) and 41% (12/39), respectively. Conclusions: The use of TICVADs in the plus-85 group resulted in effective outcomes. The results of this retrospective study support the wider use of TICVADs in patients aged ≥85 years.

Background Intracranial saccular aneurysms are acquired lesions that often present with neuro-ophthalmologic symptoms and signs. Recent advances in neurosurgical techniques, endovascular treatments, and neurocritical care have improved the optimal management of symptomatic unruptured aneurysms, but whether the chosen treatment has an impact on neuro-ophthalmologic outcomes remains debated. Evidence acquisition A review of the literature focused on neuro-ophthalmic manifestations and treatment of intracranial aneurysms with specific relevance to neuro-ophthalmologic outcomes was conducted using Ovid MEDLINE and EMBASE databases. Cavernous sinus aneurysms were not included in this review. Results Surgical clipping vs endovascular coiling for aneurysms causing third nerve palsies was compared in 13 retrospective studies representing 447 patients. Complete recovery was achieved in 78% of surgical patients compared with 44% of patients treated with endovascular coiling. However, the complication rate, hospital costs, and days spent in intensive care were reported as higher in surgically treated patients. Retrospective reviews of surgical clipping and endovascular coiling for all ocular motor nerve palsies (third, fourth, or sixth cranial nerves) revealed similar results of complete resolution in 76% and 49%, respectively. Improvement in visual deficits related to aneurysmal compression of the anterior visual pathways was also better among patients treated with clipping than with coiling. The time to treatment from onset of visual symptoms was a predictive factor of visual recovery in several studies. Few reports have specifically assessed the improvement of visual deficits after treatment with flow diverters. Conclusions Decisions regarding the choice of therapy for intracranial aneurysms causing neuro-ophthalmologic signs ideally should be made at high-volume centers with access to both surgical and endovascular treatments. The status of the patient, location of the aneurysm, and experience of the treating physicians are important factors to consider. Although a higher rate of visual recovery was reported with neurosurgical clipping, this must be weighed against the potentially longer intensive care stays and increased early morbidity.

Granulomatosis with polyangiitis (GPA), previously known as Wegener's granulomatosis (WG), is a rare systemic disease characterized by necrotizing granulomas and vasculitis that usually presents in the respiratory tract and renal system. Here, we report a case of anti-neutrophil cytoplasmic antibody (ANCA)-negative GPA with diagnostic difficulties. Neurologic involvement in GPA is not uncommon, with up to 50% of patients manifesting with neurologic defects; however, it is usually a late event. This patient was a 50-year-old man whose first manifestation was multiple neurologic defects without respiratory or renal symptoms. Since multiple neurologic deficits were the first manifestation and there were no pulmonary or renal symptoms, our patient was treated for 1 year for suspected infectious or inflammatory diseases such as non-tuberculous mycobacterial infection or immunoglobulin G4 (IgG4)-related disease with brain involvement; however, his symptoms would wax and wane. Reanalysis of the laboratory findings including biopsies, finally revealed this case to be compatible with GPA. Here, we report a case of GPA, focusing on the diagnostic pitfalls.

Introduction It can be challenging to accurately identify patients with brain metastases who have very poor prognosis and are unlikely to benefit from radiation (RT). We characterized factors of patients who died within 30 days of receiving RT for brain metastases. Methods Patients who received whole brain RT (WBRT) or stereotactic radiosurgery (SRS) for brain metastases between 1/1/2017–9/30/2020 at a single institution were identified. Patient, tumor, treatment, and death variables were collected. Characteristics between those who did and did not die within 30 days were compared using the Wilcoxon Rank-Sum or Chi-Square test. Survival was estimated with Kaplan-Meier method. Results 636 patients received WBRT (n=117) or SRS (n=519). Median age was 61. Median survival was 6 months (95% CI 5–7 months). 75 (12%) died within 30 days of RT. Patients who died within 30 days had worse median KPS (50 vs 80, p Discussion Patients who died within 30 days of RT had worse KPS, intracranial/extracranial disease burden, and neurologic symptoms. Future analyses will assess whether these factors can inform a prognostic model to identify patients with poor prognosis who may be appropriate for supportive care alone.

A healthy middle-aged man presents with symptoms of dysphagia and dysphonia following an upper respiratory infection, and is diagnosed and treated for complications of pharyngitis. He presents for evaluation at a tertiary care hospital after symptoms fail to resolve, with the final diagnosis being a carotid artery dissection with compressing pseudoaneurysm. This patient’s constellation of symptoms and physical examination findings are consistent with Collet-Sicard syndrome, a rare disorder caused by cranial nerve compression at the skull base. Understanding the morbidity of missing, or delaying, a diagnosis of carotid artery pathology, such as Collet-Sicard syndrome, underscores the importance of an accurate diagnosis. A review of cranial nerve anatomy, surrounding structures and potential mechanism of injury to the carotid artery are emphasised as key learning points.

Here, we report a patient who developed diplopia secondary to a right cranial nerve III and IV palsy, as well as fever and headache. Cerebrospinal fluid analysis (CSF) showed high varicella-zoster virus (VZV)-DNA viral load (>300,000,000 copies/ml). VZV antibodies in CSF was ≥1:16. Diagnosis of neurological reactivation of VZV infection was made without the presence of characteristic vesicular rash. Quantitative real-time PCR for VZV and intrathecal dosage of VZV IgM and IgG should be performed in cases suspected for viral encephalitis and also in all patients with not otherwise attributable cranial nerve lesions.

BACKGROUND Surgical exposure of the jugular foramen (JF) is challenging given its complex regional anatomy and proximity to critical neurovascular structures. OBJECTIVE To describe the anatomical basis, surgical technique, and outcomes of a group of patients who underwent the endoscopic endonasal approach to the JF. METHODS Five silicon-injected anatomical specimens were prepared for dissection. Additionally, a chart review was conducted through our patient database, searching for endonasal exposure of the JF. Demographic data, clinical presentation, pathological findings, extent of resection in the JF, and occurrence of complications were analyzed. RESULTS The endonasal exposure of the JF requires 3 sequential steps: a transpterygoid, a "far-medial," and an "extreme-medial" approach. Mobilization or transection of the cartilaginous portion of the eustachian tube (ET) is necessary. In the clinical series, cranial neuropathies were the presenting symptoms in 16 patients (89%). Eighteen tumors (10 chondrosarcomas, 7 chordomas, 1 adenocarcinoma) extended secondarily into the JF. Total tumor resection was achieved in 10 patients (56%), near total (≥90%) in 6 (33%), and subtotal (

Purpose To evaluate changes in corneal endothelial cell density over time in patients with dry eye disease (DED) and to correlate endothelial cell loss with corneal subbasal nerve density. Methods This retrospective study included 40 eyes of 20 patients with DED. Laser in vivo confocal microscopy had been performed in the central cornea of both eyes at an initial visit and repeated after a mean follow-up of 33.2 ± 10.2 months. The densities of corneal endothelial cells and subbasal nerves were measured in both visits and compared with 13 eyes of 13 normal age-matched controls. Results At the initial visit, the DED group had lower densities of corneal endothelial cells (2620 ± 386 cells/mm) and subbasal nerves (17.8 ± 7.5 mm/mm) compared with the control group (2861 ± 292 cells/mm and 22.8 ± 3.0 mm/mm, with P = 0.08 and P = 0.01, respectively). At the end of follow-up, although there was no significant change in subbasal nerve density (16.7 ± 7.2 mm/mm, P = 0.43), the mean corneal endothelial cell density significantly decreased to 2465 ± 391 cells/mm (P = 0.01), with a mean corneal endothelial cell loss of 2.1 ± 3.6% per year. The endothelial cell loss showed a statistically significant negative correlation with the initial subbasal nerve density (Rs = -0.55, P = 0.02). Conclusions Patients with DED have an accelerated corneal endothelial cell loss compared with that reported in the literature for normal aging. Those with lower subbasal nerve density, in particular, are at a higher risk for endothelial cell loss over time.

Leprosy is a debilitating disease that usually involves the peripheral branches of the cranial nerves leading to anesthetic/hypoesthetic skin lesions and thickened peripheral nerves. However, the involvement of the central nervous system (CNS) is extremely rare. To the best of the author's knowledge, the involvement of the cranial nerve nuclei by leprosy has not been reported in the literature and the present case is the first report of involvement of the facial nerve nuclei by leprosy.

Summary Although horses are affected by cranial nerve disease, our understanding of these structures' imaging anatomy is limited, and the optimal modality for imaging of each of these nerves is unclear. The aim of this study was to describe the imaging appearance of the equine cranial nerves on high-resolution 1.5T magnetic resonance imaging (MRI) and computed tomography (CT) scans of a cadaver head, and with these as standards, examine the utility of MRI and CT performed in clinical cases. High-resolution MRI and CT images were prospectively acquired of the head of a normal Thoroughbred gelding following euthanasia. Ten clinical cases undergoing high-field MRI under general anaesthesia and 10 clinical cases undergoing CT in the standing horse under sedation were retrospectively evaluated by three reviewers to assess cranial nerve visibility. On high-resolution, thin-slice, MRI scans of the normal cadaver head, each of the 12 cranial nerves and their topographic location could be appreciated. On high-resolution cadaver CT, cranial nerves II, V and VII were clearly visible, but others were less easily identified; osseous structures were clearly visualised. Clinical MRI and CT allowed for variable visualisation of the cranial nerves, dependent on the sequence and the orientation of scan planes. High-field MRI allowed excellent visualisation of equine cranial nerves, whereas CT allowed for more detailed visualisation of the osseous canals and foramina. In live horses, the ability to identify all 12 nerves is challenging with either MRI or CT; however, high-field MRI enables better visualisation of the nerve bundles than CT.

In this paper, a new type of EEG recording and electrical stimulation medical device with self-feedback function which using a microelectrode array is developed for the diagnosis and treatment of cranial nerve diseases. The main research of this paper is as follows: (1) Weak brain signal acquisition technology. It uses a noise suppression method without power frequency notch to obtain the true synchronous pulse width EEG waveform in this device. (2) Microcurrent stimulation technology. This device is designed with triple hardware protection and precision constant current source to meet clinical safety and high precision requirements. (3) An expert system for autonomous feedback control of stimulating currents. In this system, it uses a filter with infinite impulse response to preprocess the signal. And it uses wavelet transform combined with mathematical statistics method to extract EEG time-frequency information, integrated fuzzy c-means clustering search algorithm and expert system to control stimulation current. There are the functions of evoked potentials by using EEG nerve electrical stimulation and of using micro-current stimulation to feedback therapy in this device. It can be used for precise location of epilepsy lesions and clinical diagnosis of brain diseases such as Parkinson’s disease. It can also be used for neurological rehabilitation treatments such as depression, insomnia, and brain function disorders. This equipment has important scientific significance and application value for the research of cranial nerve clinical medicine, brain nerve function loop foundation and brain-computer interface

Pseudotumor cerebri syndrome is puzzling because it results in elevated intracranial pressure with no identifiable underlying cause. Ocular motor nerve palsies, other than a unilateral or bilateral sixth cranial nerve palsy, are rarely seen in patients with this condition. We report here on a 25-year-old female patient with bilateral fourth cranial nerve palsy caused by pseudotumor cerebri syndrome, whose ocular and systemic signs and symptoms of nerve palsy were completely resolved after medical treatment. We infer that fourth nerve palsy could be associated with pseudotumor cerebri syndrome; therefore, clinicians should consider rare ocular motor nerve palsies, even bilaterally, in patients with pseudotumor cerebri syndrome. RESUMO A sindrome do pseudotumor cerebral é uma síndrome enigmática caracterizada por aumento da pressão intracraniana sem causa definida. Na síndrome do pseudotumor cerebral, as paralisias de nervo oculomotor além da paralisia unilateral ou bilateral do sexto nervo craniano são raramente vistas. Nós reportamos o caso de uma paciente feminina de 25 anos com paralisia bilateral de quarto nervo craniano como resultado da síndrome do pseudotumor cerebral. Após tratamento médico para síndrome do pseudotumor cerebral, os sinais e sintomas oculares e sistêmicos da paralisia nervosa foram resolvidos. Em conclusão, a paralisia de quarto nervo craniano pode estar associada com síndrome de pseudotumor cerebral, portanto médicos devem considerar paralisias raras de nervo oculomotor, mesmo bilateralmente, em pacientes com síndrome do pseudotumor cerebral.

Facial paralysis is vulgo of facial neuritis and facioplegia, which is also called Bell disease [1]. It is non-purulent inflammation of facial nerve, which usually occur in winter and spring. The pathological change is neural edema, degeneration of myelin sheath and axon to varying degrees. The patients are recovered to varying degrees in 2 months after disease attack and some patients are recovered 1 year later. Some got persistent sequelae. 1. Causes: Facial neuritis can be induced by various factors. Facial neuritis usually is induced by cranial nerve diseases. This may be because facial nerve canal is a long bony pipeline. If petrosal bone develops abnormally, there usually is facial nerve canal stenosis, which is the internal pathogenic factor of facial neuritis. The external pathological factor of facial neuritis remains unclear. The early pathological change is edema of facial nerve, degeneration of myelin sheath and axon to varying degrees. The reason may be that cold air blast induces nutritional microvascular spasm of facial nerve and then lead to ischemia and anoxia of local tissue. Someone think that it is related to virus infection [2]. However, until now, no one can isolate virus. In recent years, some reports think that it may be a immunoreaction. Ramsay-Hunt Syndrome is induced by infection of herpes zoster virus and inflammation of geniculate ganglion and facial nerve. In short, the factors that can affect functional nucleus of facial nerve and neural axon functions can lead to dysfunction of facial muscles dominated by them such as infectious, compressive, traumatic, physical and neurogenic factors. Infectious factor accounts for 42.5% of the total number. It is the herpes zoster virus in dormant state, which usually hides in sensory ganglion of facial nerve. The virus is activated when local immunity is low. It can also be induced by inflammatory diseases such as meningitis, mumps, influenza, scarlet fever, malaria, multiple cranial neuropathy, local infection and otogenic infection (otitis media, labyrinthitis, mastoiditis). Compressive factor can induce facial neuritis, which is usually observed in tumors such as acoustic neuroma, parotid gland and primary acquired cholesteatoma and accounts for 5.5% of the total number. The common traumatic factors of facial paralysis include temporal bone fracture, facial trauma, surgery and injection of neurotoxic drug in facial nerve distribution area and account for 8.2% of the total patients. Neurogenic facial paralysis accounts for 13.5% of the total patients, which is usually induced by cerebrovascular disease, encephalic and non-traumatic neurogenic disease. If cold wind blows the face for a long time, undercooling in face can directly stimulate local nerve trophic vessels and induce spasm, which can induce ischemia and edema of neural tissue and dysfunctions. It is the common factor to induce facial paralysis. In addition, poisoning, metabolic disturbance such as diabetes mellitus and vitamin deficiency, vascular function insufficiency and congenital facial nucleus hypoplasia is one of the pathological factors. 2. Types: Facioplegia is divided into peripheral type and central type. The majority is peripheral type. (a) Central type is paralysis of muscle in lower part of the face on the offside of lesion induced by injury of supranuclear tissue includes cortex, cortex and brainstem fiber, medullary triangle and pons, which are usually observed in cerebrovascular disease, brain tumor and encephalitis. (b) Peripheral type is paralysis of all facial muscles in the ipsilateral of the lesion induced by injury of facial nerve nucleus or facial nerve, which is usually observed in catching cold, ear or meninx infection, peripheral facioplegia induced by neurofibroma. In addition, there is dysgeusia in the front two third of tongue and unclear speaking. 3. Dysfunctions mainly are motor and sensory dysfunctions of facial muscle. According to the motor dysfunction part of facial muscle, there are different clinical manifestations such as palpebral fissure enlargement, unable to frown or close eyes, forehead wrinkle disappearance, grinningly, agitating cheek and unable to whistle. There are also clinical manifestations such as forehead wrinkle disappearance, palpebral fissure enlargement, nasolabial groove becoming shallow, droop, stretching face to offside, lacrimation or salivation. The dysfunctions are different in different injured part of facial nerve. It can be summarized as follows: (a) Facial nerve branch below stylomastoid foramen is injured, which can induce peripheral facial paralysis. The main manifestations are forehead wrinkle disappearance in injured side, unable to crinkle forehead and frown, palpebral fissure enlargement, unable to close or close incompletely, Bell syndrome (eyeball rotate upward and outside when closing eyes and white sclera is exposed), nasolabial groove becoming shallow, corner of the mouth skews to uninjured side, corner of the mouth deviate to uninjured side when exposing teeth, air leakage in agitating cheek or whistle, food retention in teeth buccal space when eating. (b) Facial nerve branch above tympanic cord is injured. Besides peripheral facial paralysis, there is dysgeusia in two third front of tongue in the ipsilateral. (c) Facial nerve branch above stapedius is injured. Besides peripheral facial paralysis and dysgeusia in two third front of tongue in the ipsilateral, there is hyperakusis. (d) Geniculate ganglion is injured. Besides peripheral facial paralysis, dysgeusia in two third front of tongue in the ipsilateral and hyperakusis, there is pain in mastoidea, hypaesthesia of auricle and external auditory canal, herpes of external auditory canal or tympanic membrane, which is called Hunt syndrome [3]. 4. Sequelae: Facial dysfunction still exists half a year after the disease. The main signs include no raising eyebrow movement or incomplete raising eyebrow movement, incomplete opening eyes, corner of the mouth going upward when raising eyebrow, stretching the corner of the mouth when closing eyes, eyes becoming small when agitating mouth (synkinetic movement of mouth and eye), shallow nasolabial groove, facial stiffness, food retention, tears, secondary prosopospasm and denervated muscle atrophy. Sequelae are due to delay of the illness or improper therapy method. Some reports deem that half a day after attach is the best therapeutic opportunity. Nerve in injured side is not recovered timely, which lead to incomplete conduction of neural impulse or conduction generalization. In addition, some sequelae are due to irreversible injury of facial nerve.