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Purpose : Minimally invasive and surgical spine procedures are commonplace with various risks and complications. Cranial nerve palsies, however, are infrequently encountered, particularly after procedures such as lumbar punctures, epidural anesthesia, or intrathecal injections, and are understandably worrisome for clinicians and patients as they may be interpreted as secondary to a sinister etiology. However, a less commonly considered source is a pneumocephalus which may, in rare cases, abut cranial nerves and cause a palsy as a benign and often self-resolving complication. Here, we present the case of a patient who underwent an intrathecal methotrexate infusion for newly diagnosed non-Hodgkin's T -cell lymphoma and subsequently developed an abducens nerve palsy due to pneumocephalus. We highlight the utility of various imaging modalities, treatment options, and review current literature on spinal procedures resulting in cranial nerve palsies attributable to pneumocephalus presenting as malignant etiologies., Competing Interests: Declaration of conflicting interestsThe authors have no conflicts of interest to declare that are relevant to the content of this article.

The anti-GQ1b IgG antibody is often accompanied by other anti-ganglioside antibodies, which induces various neurological symptoms. We herein report a patient with anti-ganglioside antibodies, including anti-GQ1b IgG and anti-GT1a IgG antibodies, showing bilateral ophthalmoplegia, facial nerve palsies, dysarthria, dysphagia, dysesthesia in both hands, and enhancement of the bilateral oculomotor, abducens, and facial nerves on gadolinium (Gd)-enhanced T1-weighted brain magnetic resonance imaging (MRI). He was first treated with intravenous immunoglobulin, which improved ophthalmoplegia, bulbar palsies, and dysesthesia of hands, but the facial nerve palsies worsened, and Gd enhancement of the brain nerves persisted. High-dose methylprednisolone therapy subsequently improved the facial nerve palsies and Gd enhancement of the cranial nerves. This is the first case with anti-ganglioside antibodies presenting with multiple cranial nerve palsies that was followed to track the changes in the Gd enhancement of cranial nerves on MRI.

Rationale: Hypertrophic pachymeningitis (HP) is a local or diffuse fibrous thickness of the dura mater of the brain or spinal cord, caused by infection or connective tissue disease. Headache is the most common clinical symptom, followed by various cranial nerve disorders such as visual impairment, diplopia, and hearing loss. HP can be classified into secondary and idiopathic. Here, we report a case of bilateral progressive profound sensorineural hearing loss diagnosed in a patient with idiopathic HP, where a cochlear implant was effectively used., Patient Concerns: The patient was a 77-year-old woman. Hearing loss gradually progressed bilaterally, and magnetic resonance imaging showed a space-occupying lesion with a continuous contrast enhancement in the bilateral internal auditory canals, and diffused dural thickening from the middle to the posterior cranial fossa., Diagnoses: A trans-labyrinthine biopsy was conducted, and a definite diagnosis of idiopathic HP was made. Thickening of the dura mater in the bilateral internal auditory canals was thought to cause profound hearing loss., Interventions and Outcomes: A cochlear implant was implemented 4 months after biopsy, and a favorable hearing response was obtained postoperatively., Lessons: This is the first report of a cochlear implant in a patient with idiopathic HP. Cochlear implantation was considered a good treatment for profound hearing loss due to idiopathic HP, which provides a reference for patients to receive timely and correct treatment., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Cranial nerve palsies after gunshot injury are not uncommon. We report the mechanism of isolated hypoglossal nerve paralysis caused by a gunshot. We report a 74 years old patient in whom a bullet entered through the right nostril and then ended up right occipital condyle. The only neurologic deficit was tongue deviation which resolved in one week. The bullet was not removed. The effect of clival slope may have an importance in this type of injury.

Patients with inflammatory bowel disease (IBD) may present with extraintestinal manifestations. Neurological symptoms associated with IBD are infrequent. Thus, any unexplained neurological symptom that occurs in patients with IBD should raise the suspicion of a link between the two disorders. We report a case of a man in his 60s, who was diagnosed with Crohn's disease and developed ptosis and diplopia. Neurological examination revealed oculomotor nerve palsy, sparing the pupil. MRI and magnetic resonance angiography of the brain were insignificant and no other cause was determined. He was treated with oral corticosteroids and symptoms gradually subsided. Cranial nerve palsies associated with IBD have been rarely reported. They usually involve the optic and acoustic nerve and are attributed to a common dysimmune base. This is the first reported case of oculomotor nerve palsy (III cranial nerve) associated with IBD. Clinicians treating patients with IBD should be alert for unusual neurological complications and treat them appropriately., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. No commercial re-use. See rights and permissions. Published by BMJ.)

Sphenoid sinusitis is a clinically important entity as it may be associated with catastrophic complications including cavernous sinus thrombosis (CST), cerebral abscess or infarction, meningitis, base of skull osteomyelitis and cranial nerve palsies. We report a case of occult sphenoid sinusitis presenting as Streptococcus intermedius bacteraemia, bilateral jugular vein and CST, cranial nerve palsy and base of skull osteomyelitis., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2023. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Traumatic chiasmal syndrome and traumatic third nerve palsies are rare entities usually caused by severe, high-speed, closed-head road accidents that require a thorough diagnostic work-up and complex medical and surgical management. This report presents the case of a young adult who was involved in a motorcycle accident and sustained blunt frontal head trauma, resulting in optical chiasmal syndrome and complete unilateral third nerve palsy. Ophthalmological examination demonstrated a right complete ptosis, a downward and outward position of the right eye with a fixed and dilated pupil, and bitemporal hemianopsia. In addition, funduscopy revealed bilateral optical nerve atrophy. After stabilisation and during follow-up, strabismus surgery was performed with improvement of ocular alignment in the primary position. Subsequently, eyelid surgery was carried out with good amplification of the visual field, particularly on the left side. Although challenging, surgical intervention in these cases should be considered for both functional and cosmetic reasons., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2022. No commercial re-use. See rights and permissions. Published by BMJ.)

Background: Idiopathic hypertrophic pachymeningitis (IHP) is a rare inflammatory disease that causes focal or diffuse thickening of the dura mater. However, longitudinal follow up studies are still lacking for these patients., Objective: To investigate the clinical characteristics, neuroimaging findings, treatment response and outcome of IHP., Method: A retrospective case series of 30 patients admitted Beijing Tiantan Hospital were screened via Hospital Information System from January 1st, 2011, to January 31st, 2021. All patients' clinical symptoms, imaging, and treatment response were collected via a standardized form. We compared the effects of high-dose and low-dose corticosteroids on headache, impaired vision, and MRI remission during acute onset. The effects of different immunosuppressants on preventing relapses were also compared., Results: Headache (93.3%) and multiple cranial neuropathy (66.7%) were the most common symptoms of IHP. Cerebral spinal fluid test showed that protein levels were elevated in 17 (56.7%) patients, and white blood cells were increased in seven patients. MRI demonstrated that diffuse (60%) and focal (40%) enhancement occurred in the dura mater, especially in the tentorium cerebellum (80%). High-dose and low-dose corticosteroids reduced headache and dural enhancement during the acute phase. The high dose corticosteroid significantly relieved the headache than the low dose group (p = 0.041). Patients treated with mycophenolate mofetil and cyclophosphamide might achieve longer remission (months, p = 0.428)., Conclusion: Headache and multiple cranial neuropathy are the most common clinical manifestations of IHP. In this study, almost all patients had a good initial response to corticosteroid therapy during the acute phase. Mycophenolate mofetil and cyclosporine may be effective for preventing relapses., Competing Interests: Conflict of Interest The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2022 Elsevier B.V. All rights reserved.)

Introduction: There are various etiologies for isolated third, fourth and sixth cranial nerve palsies. The most common etiology in adults aged 50 years and older with vascular risk factors such as diabetes, hyperlipidemia, and hyperlipidemia, is microvascular ischemia. The role of early neuroimaging in older patients with vascular risk factors presenting with acute isolated ocular motor nerve palsy is controversial., Aims: We present six cases of patients aged 50 years and older, with vascular risk factors, who suffered from acute isolated paralysis of the sixth nerve. All cases were diagnosed with a non-microvascular ischemia etiology., Results: The identified etiologies included petroclival meningioma with involvement of the cavernous sinus, metastatic hepatoma, plasmacytoma, venous thrombosis, arterial-venous malformation and aneurysm., Conclusions: Despite the high cost and presumed low yield, early neuroimaging can change the clinical management in certain cases., Discussion: Previous studies have found the yield of early neuroimaging in older vasculopathic patients with acute 4th or 6th cranial nerve palsies to be 1-15%. In spite of these reports, our cases demonstrate the need for early onset imaging in these cases.

Background: Multiple cranial neuropathies carry a wide range of differential diagnoses, and when combined with cerebrospinal fluid monocytosis they often suggest an infective etiology. Reactivation of varicella zoster virus has been associated with a wide range of neurological complications. Among the cranial nerves, the upper cranial nerves (trigeminal and facial nerves) are more commonly affected; there have been some reports of lower cranial polyneuropathies resulting from varicella zoster virus reactivation. However, polyneuropathy involving both the cranial and cervical nerves is rarely reported., Case Presentation: This report highlights the case of a 64-year-old Chinese man presenting with an acute, severe dysphagia and dysphonia secondary to herpes zoster-associated progressive polyneuropathy involving the lower cranial and upper cervical nerves, without any mucocutaneous manifestations., Conclusions: To our knowledge, this is the first case of varicella zoster virus-associated cranial and cervical polyneuropathy in the literature. The report also highlights the poor sensitivity of varicella zoster virus DNA detection by polymerase chain reaction in the cerebrospinal fluid, and proposes that serology be routinely performed in such polymerase chain reaction-negative cases without a clear diagnosis., (© 2022. The Author(s).)

Ocular cranial nerve palsy of presumed ischemic origin (OCNPi) is the most common type of ocular cranial nerve palsy (OCNP) in patients aged ≥ 50 years; however, no definite diagnostic test exists. As diagnostic criteria include clinical improvement, diagnoses are often delayed. Diagnostic biomarkers for OCNPi are required; we hypothesized that cerebral small vessel disease is associated with OCNPi. We analyzed 646 consecutive patients aged ≥ 50 years with isolated unilateral OCNP who underwent work-ups at two referral hospitals. White matter hyperintensities (WMHs), silent infarctions, and cerebral microbleeds (CMBs) were assessed. In multivariate analyses, mild (grades 1-3) and moderate to severe (grades 4-6) WMHs were significantly associated with OCNPi compared to OCNP of other origins (odds ratio [OR] 3.51, 95% confidence interval [CI] 1.91-6.43, P < 0.001; OR 3.47, 95% CI 1.42-8.48, P = 0.006, respectively). Silent infarction and CMBs did not remain associated (OR 0.96, 95% CI 0.54-1.70, P = 0.870; OR 0.55, 95% CI 0.28-1.08, P = 0.080, respectively). Associations between WMH and OCNPi remained after excluding patients with vascular risk factors. In conclusion, the presence of WMH could independently predict ischemic origin in patients with isolated unilateral OCNP at early stage of admission., (© 2022. The Author(s).)

To assess association between migraines and development of ocular motor cranial nerve palsy (CNP) and finding risk factors using the National Sample Cohort database from the Korea National Health Insurance Service. Data was analyzed from 4,234,341 medical screening examinees aged 20-90 years in 2009. Cox proportional hazard regression analysis was used to the adjusted hazard ratios (HR) for ocular motor CNP according to presence of migraine. Subgroup analysis was performed to evaluate effect of other factors on association of migraine with ocular motor CNP. A total of 5806 participants (0.14% of subjects) developed ocular motor CNP and were assigned to CNP group, 4,048,018 were assigned to control group, with an average of 8.22 ± 0.93 years of follow-up. Incidence of ocular motor CNP increased in migraine group compared to control. After adjusting potential confounding variables, HR for ocular motor CNP was 1.166 (confidence interval [CI] 1.013-1.343) in migraine group. Subgroups of relatively younger age less than 65 years (HR = 1.267, 95% CI 1.067-1.504), male gender (HR = 1.228, 95% CI 1.000-1.122), smokers (HR 1.426, 95% CI 1.127-1.803), and diabetes mellitus patients (HR = 1.378, 95% CI 1.045-1.378) showed a stronger association between migraines and development of ocular motor CNP. Our population-based cohort study demonstrated a significant association between presence of migraines and incidence of ocular motor CNP. Especially, relatively younger age, males, smokers, and diabetes patients with migraines could have a higher risk of developing ocular motor CNP., (© 2022. The Author(s).)

Background: Coronavirus disease 2019 (COVID-19) has a vast array of presentations and associations with neuro-ophthalmic diseases. There has been a recent surge in ophthalmic manifestations secondary to fungal sinus infections in India especially in diabetic patients who were given systemic steroids. We present our COVID-19-related cranial neuropathies presenting in our clinic., Methods: This is a retrospective case series of 10 patients affected with COVID-19 disease and who presented with cranial nerve palsies at the neuro-ophthalmic department of a tertiary eye care hospital in South India. An analysis of electronic medical records data was performed, including their comorbidities, symptoms, cranial nerves involved, ocular and neuroimaging findings, site of lesion, etiology, and prognosis., Results: Most of the patients (7 of 10) presented with multiple cranial nerve palsies (MCNP) with poor visual acuity. 2 of the 10 cases succumbed to death due to the intracranial involvement. All MCNP cases had uncontrolled diabetes with a history of systemic steroids, and neuroimaging of these cases showed sinusitis of varying severity most of which were suggestive of fungal invasive type., Conclusion: Our study emphasizes the need to screen for fungal involvement in COVID-19 cases presenting with MCNP especially on diabetic patients on systemic steroids so that an early diagnosis may reduce visual loss and mortality. Physicians treating COVID-19 cases need to be aware of this dreadful complication., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by North American Neuro-Ophthalmology Society.)

This study aimed to assess the associations between liver enzymes including γ-glutamyl transferase (GGT) and the development of ocular motor cranial nerve palsy (CNP) using the National Sample Cohort database from Korea's National Health Insurance Service. We analyzed data from 4,233,273 medical screening examinees aged 20 years or more in 2009. Study participants were followed up until December 31, 2018. A Cox proportional hazard regression analysis was performed for quartiles of liver enzymes to determine the linkage between each value and ocular motor CNP using quartile 1 as a reference after adjusting for potential confounders. A total of 5,807 (0.14%) patients developed ocular motor CNP during the follow-up period of 8.22 ± 0.94 years. The incidence of ocular motor CNP gradually increased as the GGT levels increased. The highest quartile of the GGT group had hazard ratio (HR) of 1.245 (95% confidence interval [CI], 1.136-1.365). Regarding alanine aminotransferase (ALT), the highest quartile of the ALT group had HR of 1.141 (95% CI, 1.049-1.241). However, the incidence of ocular motor CNP did not gradually increase as the ALT levels increased. The coexistence of the increased level of GGT, metabolic syndrome, and obesity showed a stronger association with ocular motor CNP development (HR, 1.331; 95% CI, 1.173, 1.511) compared to having a single factor or two factors. In conclusion, our population-based cohort study demonstrated a significant association between serum GGT level and the incidence of ocular motor CNP, suggesting that GGT could be a new clinical marker for predicting the occurrence of ocular motor CNP., (© 2021. Fondazione Società Italiana di Neurologia.)

Background: Ramsay Hunt Syndrome (RHS) is a neurotological disorder involving the reactivation of the varicella-zoster virus (VZV) in the geniculate ganglion of the facial nerve (Sweeney and Gilden in J Neurol Neurosurg Psychiatry 71:149-154, 2001). The characteristic presentation involves ipsilateral lower motor neuron type facial paresis, auricular pain with or without hearing impairment, and vesicular lesions of the external auditory canal and outer ear. Involvement of the facial and vestibulocochlear nerve is typical in RHS, whilst multiple cranial neuropathies are rare and associated with poorer prognosis and systemic complications (Arya et al. in Am J Case Rep 19:68-71, 2017; Shinha and Krishna in IDCases 2:47-48, 2015; Shim et al. in Acta Otolaryngol 131:210-215, 2011; Coleman et al. in J Voice 26:e27-e28, 2012; Morelli et al. in Neurol Sci 29:497-498, 2008;). Likely mechanisms involved in the pathogenesis of cranial polyneuropathy include direct peri-neural and trans-axonal spread of viral inflammation between contiguous cranial nerves and haematogenous dissemination between nerves with shared blood supply. Impairments in speech, swallowing, hearing, and oculo-protection can contribute to morbidity and requires a multidisciplinary approach to patient care., Methods: We present a rare case of RHS with multiple cranial neuropathies followed by a comprehensive review of current literature with regard to the pathophysiology, diagnostic workup, and the management strategies employed in these patients., Conclusion: RHSs with multiple cranial neuropathies are important to recognise as they are associated with significant morbidity and poor prognosis. A multidisciplinary approach to patient management is required to address the several complications that can arise from cranial nerve deficits, especially in regard to speech and swallow., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Ocular motor nerve palsies (OMNP) frequently cause patients to present in an emergency room. In the following study, we report the differential diagnosis of OMNP by use of magnetic resonance imaging (MRI) and CSF examination as a standard., Method: We performed a data analysis of N = 502 patients who presented with oculomotor, trochlear, and/or abducens nerve palsy in the emergency room of the Department of Neurology, University of Ulm, between January 2006 and December 2019. We report clinical and MRI scan findings in all patients; furthermore, the CSF of 398 patients has been analysed., Results: Abducens nerve palsies were most common (45%), followed by palsies of the oculomotor (31%) (CNP III) and trochlear nerve (15%). Multiple OMNPs were seen in 9% of our cohort. The most common causes included inflammations (32.7%), space-occupying lesions, such as aneurysms or neoplasms (17.3%), diabetes mellitus (13.3%), and brainstem infarctions (11%). Still 23.4% of the patients could not be assigned to any specific cause after differential diagnostic procedures and were described as idiopathic. One of three patients with an inflammation and 39% of the patients with space-occupying lesions showed additional cranial nerve deficits., Conclusion: Inflammation and space-occupying processes were the most frequent causes of OMNP, although brainstem infarctions also play a significant role, in particular in CNP III. The presence of additional CNPs increases the probability of an inflammatory or space-occupying cause., (© 2021. The Author(s).)

Background: Isolated ischemic ocular cranial nerve palsies (OCNP) involving the 3rd, 4th and 6th cranial nerves (CN) are prevalent conditions in ophthalmic practice. However, it is not clearly established whether such patients are at increased risk of stroke after onset of OCNPs., Methods: Medline, PubMed, Embase and Cochrane Central registers were systematically searched for eligible studies comparing isolated ischemic OCNPs against matched controls on the subsequent development of stroke with at least two years of follow up. Case reports and series were excluded. Appropriate studies were entered for meta-analysis to determine hazard ratios. Search and data extraction was completed on 22 Feb 2021. Random effect models were used to generate pooled hazard ratios (HRs) and 95% confidence intervals (CIs)., Results: Three studies were suitable for meta-analysis (total n = 2,756 OCNP cases and 21,239 matched controls). The meta-analysis demonstrated a hazard ratio of 5.96 (4.20-8.46 95% CI) of subsequent stroke after isolated OCNP within the first year. The hazard ratio reduced to 3.27 (2.61-4.10 95% CI) after five years although remains raised at 2.49 (1.53-4.06 95% CI) up to 12 years. The highest risk was demonstrated with 3rd cranial nerve palsies. Two additional studies assessed the risk of stroke with newly diagnosed diabetics and compared OCNPs against lacunar stroke. These studies did not demonstrate a significant increased risk of stroke, although they may be statistically underpowered., Conclusion: Ischemic OCNPs represent a significant risk factor for development of subsequent stroke in a similar magnitude to transient ischemic attack within the first year., (Copyright © 2021. Published by Elsevier Ltd.)

Abstract: The objective of this study was to investigate the difference in clinical features according to age and factors affecting recovery of acquired sixth cranial nerve (CN6) palsy.A total of 156 patients with acute CN6 palsy between March 2016 and August 2021 who were followed up for at least 3 months were included in this study. Etiology, rate of recovery, and factors associated with recovery were retrospectively investigated.The average age of patients with CN6 palsy was about 60years and the mean duration of recovery was about 2.5 months. Of 156 patients, 72 (46.15%) had a microvascular etiology and 25 (16.03%) patients had a brain vascular lesions. Brain neoplasm, trauma, and "others" were found in 10 (6.41%), 11 (7.05%), and 15 (9.62%) patients, respectively. Among the total of 156 patients, 28 (17.95%) failed to completely recover. Non-isolated CN6 palsy with other cranial nerve palsies were recorded in 29 (18.59%) cases. Comparison of age (<50years vs ≥50years), between recovery and non-recovery groups showed that etiology was significantly different.The recovery rate of acquired CN6 palsy was about 82% and about 27% of patients had brain lesions. Also, varying rates and duration of recovery were found according to etiology, so we should be pay attention to diagnosis of causative disease in CN6 palsy patients., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Competing Interests: Competing interests: None declared.

Background: Papilledema can be a manifestation of neurologic Lyme borreliosis (LB). The clinical manifestations and progression of these cases have not been comprehensively documented to date. We aimed to describe clinical and diagnostic features and to assess patient outcomes in cases of papilledema secondary to neurologic LB., Methods: We searched MEDLINE, EMBASE, and the Cochrane Database from inception to August 2019. We did not restrict our search by study design or by publication date, status, or language., Results: Twenty-eight studies describing 46 cases of papilledema secondary to neurologic LB were included. Common clinical features included cranial neuropathy (68%) and diplopia (61%). Most patients did not recall tick bite (71%) and were afebrile (74%). Brain imaging was normal in 64% cases. Cerebrospinal fluid analysis showed lymphocytic pleocytosis (77%). Initial treatment with intravenous ceftriaxone was given in 52% of cases and resulted in a 100% resolution rate. Concomitant treatment with acetazolamide resulted in favorable outcomes., Conclusions: For patients in endemic regions who describe symptoms suggestive of intracranial hypertension and papilledema, especially accompanied by facial nerve palsy and other cranial nerve palsies, underlying neurologic LB should be considered., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by North American Neuro-Ophthalmology Society.)

Abstract: An 11-year-old boy presented with 2 weeks of intermittent headache, right orbital pain, and constant diplopia. Brain MRI showed dural thickening and enhancement of the right lateral cavernous sinus, right orbital apex, and tentorium. Initial cerebral spinal fluid analysis showed only mild pleocytosis, and serum diagnostics were unrevealing. The working diagnosis was Tolosa-Hunt syndrome. His pain and sixth nerve palsy resolved with corticosteroids. Five months after initial presentation, he developed new numbness of the right cheek, complete right ophthalmoplegia, and weakness and numbness of his right hand and leg, all of which were responsive to steroids. Fifteen months later, he returned to the emergency department with 2 weeks of left-sided headaches and acute diplopia. On examination, he had a left cranial nerve 6 palsy. Dural biopsy showed diffuse mononuclear inflammatory cell reaction consisting mostly of lymphocytes with no signs of granuloma formation, nor any epithelioid or giant cells. His clinical course was consistent with an autoinflammatory condition of unknown etiology. Genetic testing with an immunodeficiency panel showed a risk allele in NOD2 (nucleotide-binding oligomerization domain 2) c.3019dup (p.Leu1007Prof*2) that is associated with an increased risk for Crohn disease. His clinical condition had similarities to central nervous system sarcoidosis. Because of the similarities between our patient's clinical, imaging, and genetic findings and neurosarcoidosis, he was switched to a more targeted therapy-infliximab. His condition has since been stable for nearly 2 years. In conclusion, genetic testing should be considered in patients with suspected occult autoimmunity., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Background: Cranial nerve (CN) palsy due to cancer involvement has been considered as an unfavorable prognostic factor for patients with nasopharyngeal carcinoma (NPC). We assessed the role of IMRT based treatment on the recovery of CN palsy and investigated the prognostic value of complete recovery of CN palsy., Methods: A total of 115 NPC patients with cancer-related CN palsy were included in the study. We referred CTCAE version 5.0 to evaluate the grade of CN palsy., Results: All patients with grade 1 CN palsy recovered completely during the 2 years of follow-up after definite treatment. Most grade 2 palsy could change gradually to grade 1 palsy or complete recovery during 2 years of follow-up. Patients with more than 2 symptoms of CN palsy had poor 3-year disease-free survival (DFS) than these with 1 or 2 symptoms (60.3% vs. 84.9%, HR 0.25, 95% CI 0.07-0.89, P = 0.001). There were no significant differences for PFS, OS, DMFS and LRFS between patients with complete recovery and non-complete recovery from CN palsy after receiving IMRT based comprehensive treatment., Conclusions: IMRT based comprehensive treatment could effectively promote the recovery of tumor-related CN palsy for NPC patient. More than 2 symptoms of CN palsy was a poor prognostic factor for DFS of NPC patients. The prognostic role of complete recovery of CN palsy was not identified in our study.

Background: An isolated oculomotor nerve (CN III) palsy is a diagnostic concern because of the potential for serious morbidity or life-threatening causes. We present 5 unusual causes of oculomotor nerve palsy that escaped initial diagnosis in order to raise awareness of their associated features that will facilitate correct diagnosis., Methods: This study consisted of a retrospective analysis of clinical features and imaging of 5 patients who were referred for neuro-ophthalmologic evaluation with presumed diagnosis of oculomotor nerve palsy of unknown reasons., Results: A complete CN III palsy and an inferior division CN III palsy were diagnosed with a schwannoma in the cavernous sinus and orbital apex portion, respectively; a middle-aged woman with aberrant regeneration was found to have a small meningioma; an adult man with ptosis was diagnosed with cyclic oculomotor paresis with spasms; and a patient after radiation was diagnosed with neuromyotonia., Conclusions: Localizing the lesion of oculomotor nerve palsy and careful examination of the imaging is crucial. Aberrant regeneration, cyclic pupil changes, and past medical history of amblyopia, strabismus, or radiation are also very helpful for diagnosis., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by North American Neuro-Ophthalmology Society.)

Purpose of Review: This article discusses the differential diagnosis, evaluation, and management of trigeminal neuralgia and reviews other neuralgias of the head and neck, including those that contribute to neuralgic ear pain., Recent Findings: Most cases of trigeminal neuralgia are related to vascular compression, a demyelinating plaque, or a compressive mass affecting the trigeminal nerve. However, recent studies have shown that up to 11% of patients have a family history of trigeminal neuralgia, suggesting that some patients may have a genetic predisposition to demyelination or nerve hyperexcitability. In these patients, trigeminal neuralgia may occur at a younger age, on both sides of the face, or in combination with other neuralgias., Summary: When a patient presents with neuralgic pain, the diagnosis is made by careful history and neurologic examination, with attention to the dermatome involved, the triggers, and the presence of any associated sensory deficit. All patients with new neuralgia or neuropathic facial pain warrant a careful evaluation for a secondary cause. The presence of sensory deficit on bedside examination is particularly concerning for an underlying secondary etiology., (Copyright © 2021 American Academy of Neurology.)

Abstract: This review of disorders of the fourth cranial nerve includes discussion on anatomy, examination techniques, congenital and acquired etiologies, differential diagnosis, and management options. The findings of the superior oblique muscle on orbital MRI in patients with fourth nerve palsy have had a major impact on our understanding of this cranial neuropathy. In addition, briefly reviewed are rare disorders of the fourth nerve: superior oblique myokymia, Brown syndrome, and ocular neuromyotonia. It behooves the clinician to have a clear understanding of the role that the fourth cranial nerve plays in a variety of neuro-ophthalmic conditions., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Objective: To report two cases of cranial multineuritis after severe acute respiratory syndrome caused by coronavirus-2., Methods: Patients' data were obtained from medical records of the clinical chart of dell'Angelo Hospital, Venice, Italy., Results: The first patient is a 42-year-old male patient who developed, 10 days after the resolution of coronavirus-2 pneumonia and intensive care unit hospitalization with hyperactive delirium, a cranial multineuritis with asymmetric distribution (bilateral hypoglossus involvement and right Claude Bernard Horner syndrome). No albumin-cytologic dissociation was found in cerebrospinal fluid; severe bilateral denervation was detected in hypoglossus nerve, with normal EMG of other cranial muscles, blink reflex, and cerebral magnetic resonance with gadolinium. He presented a striking improvement after intravenous human immunoglobulin therapy. The second case is a 67-year-old male patient who developed a cranial neuritis (left hypoglossus paresis), with dyslalia and deglutition difficulties. He had cerebrospinal fluid abnormalities (albumin-cytologic dissociation), no involvement of ninth and 10 th cranial nerves, diffuse hyporeflexia, and brachial diparesis., Discussion: Cranial neuritis is a possible neurological manifestation of coronavirus-2 pneumonia. Etiology is not clear: it is possible a direct injury of the nervous structures by the virus through olfactory nasopharyngeal terminations. However, the presence of albumin-cytological dissociation in one patient, the sparing of the sense of smell, and the response to human immunoglobulin therapy suggests an immune-mediated genesis of the disorder.

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.

An 18-year-old woman presented with a 1-week history of sore throat, lethargy and fevers. She was subsequently diagnosed with glandular fever and was managed conservatively. Following 1 week of conservative measures, she developed acute upper airway obstruction requiring emergency surgical tracheostomy insertion. Further investigation including electromyography demonstrated multiple cranial nerve neuropathies., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2020. No commercial re-use. See rights and permissions. Published by BMJ.)

BACKGROUND Coronavirus disease (COVID 19) is caused by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) and is the causative agent of a serious disease that is of great global public health concern. Palsy of the third cranial nerve is very rare in patients with confirmed 2019 novel coronavirus disease (COVID-19). We describe the case of a patient with an incomplete palsy of the left third cranial nerve sparing the pupils in the context of SARS-CoV-2 virus infection. CASE REPORT We report the case of a 24-year-old woman with confirmed COVID-19, which presented with acute onset of diplopia and strabismus of the left eye that occurred 3 days after the start of general symptoms. The patient had no significant medical history. Based on detailed ophthalmic and neurological examination, acute painless incomplete palsy of the third cranial nerve was suspected. Oculo-cerebral magnetic resonance angiography was unremarkable. Blood tests revealed mild normocytic regenerative anemia. According to the Moroccan recommendations, chloroquine and azithromycin were started. After what, a quick improvement of exotropia and diplopia was observed, and complete recovery was obtained by the sixth day of treatment. No adverse effects of the treatment were noted. CONCLUSIONS Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) can cause neurological complications such as cranial nerve palsy. The pathological mechanism remains unclear. Full recovery of the ocular motricity is possible, and prognosis depends on the severity of the respiratory illness.

Objective: Petrous internal carotid aneurysm (PA) concomitant with a mass lesion and cranial nerve palsy is relatively rare. Flow-diverter stent implantation is now widely used as an alternative treatment for PA. However, alternative treatments sometimes cannot be used because of tortuosity of the carotid artery, allergies to contrast material, and high costs. The outcomes of different treatment methods should therefore be assessed. Here, we review the available literature on treatments for PA., Methods: In a search using the terms "aneurysm", "carotid artery", and "petrous" on PubMed, MEDLINE, and databases such as OvidSP, 221 articles were identified. We also performed a literature review and discuss and compare the causes, symptoms, treatment methods, and clinical outcomes of PA., Results and Conclusions: Onset of secondary aneurysm was generally heralded by bleeding (p<0.001), while onset of primary aneurysm was heralded by cranial nerve deficit (p= 0.0014). Outcomes after treatment of 34 cranial nerve palsies in 25 patients are reported.

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 <1 year of age. UCs accounted for 18.9% of all cases, mostly represented by brain tumors (8.3%). Accordant with the logistic model, cranial nerve deficits, ataxia, or strabismus were strongly associated with an underlying UC. Presence of vertigo or attribution of a nonurgent triage code was associated with a reduced risk of UCs., 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., Competing Interests: POTENTIAL CONFLICT OF INTEREST: The authors have indicated they have no potential conflicts of interest to disclose., (Copyright © 2020 by the American Academy of Pediatrics.)

Aim: To investigate the magnetic resonance imaging (MRI) results of patients complaining from diplopia with ocular nerve palsy., Material and Methods: A routine ophthalmic examination was performed, a neurological consultation was requested, and cranial MRI was performed for all patients. The image results were sorted into four groups: ischemic lesions, demyelinating disease lesions, tumors, and no lesions. White matter gliosis and cerebral infarcts were included in the ischemic lesion group. The medical histories of the patients were acquired from medical records. The chi-squared test was used to analyze the relationship between age and cranial MRI images and to analyze the relationship between the image and paresis type. The statistical significance threshold was set at p < 0.05, unless otherwise stated., Results: Ischemic MRI images were the most common image type seen in our study. Third nerve paresis was significantly correlated with ischemic cerebral lesions observed by MRI (p=0.009). Furthermore, lesions were significantly correlated with patients aged above 50 years (p=0.004). There were no significant correlations between fourth or sixth nerve paresis and cranial ischemic images (p=0.680 and p=0.678, respectively). There were two instances of cerebral artery aneurysm, three instances of cerebral infarct, and one instance of intracranial mass, all in patients aged over 50 years., Conclusion: Although our patients had minimal or nonexistent neurological symptoms, some had serious cranial pathologies. These pathologies were commonly seen in patients aged over 50 years. We recommend performing MRI on all patients with binocular diplopia.

Congenital cranial dysinnervation disorders result from a maldevelopment of brainstem nuclei and/or cranial nerves. In some cases, specific genetic abnormalities have been identified. We expand the clinical phenotype of these disorders with the report of a 28-month-old girl who was initially evaluated for seizures and was found to have right sixth nerve palsy, small optic discs with reduced vision in her right eye. Her development was delayed. Brain MRI showed multiple abnormalities involving other cranial nerves, the optic chiasm and brainstem. Her developmental delay and seizure disorder suggest additional cortical involvement.

Binocular diplopia and right hemifacial numbness developed in a 52-year-old woman after resection of a right temporal lobe glioblastoma. Based on the Parks-Bielschowsky 3-step test, she was diagnosed with a right cranial nerve (CN) IV palsy in addition to right CN V dysfunction. Iatrogenic diplopia may result from temporal lobe surgery due to the intimate relationship of CN IV and CN III to the mesial temporal lobe. In addition, injury to CN V within Meckel cave is believed to be the cause of facial numbness in some patients after temporal lobe surgery. The anatomy of the intracranial portion of CN IV is reviewed, and the etiologies of CN IV palsy are discussed., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Objective: To retrospectively analyze the data of 72 patients with tuberculous meningitis (TBM) combined with cranial nerve palsy, to explore the incidence, clinical features, CSF assay changes and outcome in patients with TBM., Patients and Methods: A total of 72 patients were diagnosed as TBM with cranial nerve palsy. The gender, age, clinical manifestations, CSF examinations, cerebral magnetic resonance imaging (MRI) enhancement scan were collected. All these patients had completed at least 2 months of follow up after anti-tuberculous treatment., Results: This study retrospectively evaluated 486 patients; include 254 (52.3%) men and 232 (47.7%) women. The mean age was 35.2 ± 17.0 years. 72 patients (14.8%) were diagnosed as TBM with cranial nerve palsy. Among them, 38 cases (52.8%) had optic nerve palsy (the 2nd nerve), 41 cases (56.9%) had oculomotor nerve palsy (the 3rd nerve), 3 cases (4.2%) had abducens nerve palsy (the 6th nerve), and 10 cases (13.9%) had auditory nerve palsy (the 8th nerve). 16 patients (22.2%) had two groups of cranial nerve involvement (oculomotor nerve and optic nerve in 10 cases, optic nerve and auditory nerve in 5 cases, and optic nerve and abducens nerve in 1 case). Two patients (2.8%) had three groups of cranial nerve involvement (oculomotor nerve, optic nerve and abducens nerve). CSF MTB was detected by acid fast bacilli (no patient was positive), MTB DNA detection by multiplex polymerase chain reaction (PCR) (3 patients were positive) and MTB cultures (2 patients were positive). There was no significant difference of CSF cells and biochemistry investigations between the patients with or without cranial nerve palsy. Magnetic resonance imaging (MRI) enhancement scan were done in 66 (91.7%) patients after admission. It was abnormal in 57 (86.3%) patients. 15 cases (26.3%) had meningeral enhancement, 25 cases (43.9%) had tuberculoma, 11 cases (19.3%) had hydrocephalus and 6 cases (10.5%) with infarct. All patients were followed up after 2 months of anti-tuberculous treatment. 70 patients (97.2%, 70/72) with the cranial nerve palsy were fully recovered without obvious sequel., Conclusion: The complications of cranial nerve palsy in TBM patients are not uncommon, and the rate of misdiagnosis is high, which makes them vulnerable to emergencies such as disturbance of consciousness. Effective anti-tuberculous treatment can restore most cranial nerve palsy., (Copyright © 2019. Published by Elsevier B.V.)

Background: The aim of this study was to define the risk of ischemic stroke among newly diagnosed type 2 diabetes (T2D) patients who developed ocular motor cranial nerve (CN) palsies., Methods: From the National Health Insurance Service - National Sample Cohort database (2002-2013) of a random sample of 1 025 340 Koreans, patients with newly diagnosed T2D aged ≥20 years were included in the study. The incidence of ocular motor CN palsies was identified using diagnostic codes for third, fourth, and sixth CN palsies. To determine the effect of incident ocular motor CN palsy on subsequent ischemic stroke, covariate Cox regression was used (Model 1 included only ocular motor CN palsy as a time-varying covariate; Model 2 included ocular motor CN palsy and demographic information; Model 3 included all variables in Model 2 as well as comorbidity, concomitant medication, and the Charlson comorbidity index score)., Results: Of 45 820 T2D patients, 75 developed ocular motor CN palsy and 1411 had ischemic stroke. Four patients experienced ischemic stroke after the development of ocular motor CN palsy. Incident ocular motor CN palsy was associated with the subsequent risk of ischemic stroke in Models 1, 2 and 3 (hazard ratios [95% confidence intervals] 3.74 [1.40-9.98], 3.33 [1.25-8.89], and 2.96 [1.11-7.92], respectively). Male sex, older age, and lower income were associated with an increased risk of ischemic stroke. Among confounders, hypertension, atrial fibrillation, and congestive heart failure were associated with the risk of ischemic stroke., Conclusions: Physicians should pay more attention to manageable risk factors of ischemic stroke when diabetic patients suffer from ocular motor CN palsies., (© 2018 Ruijin Hospital, Shanghai Jiaotong University School of Medicine and John Wiley & Sons Australia, Ltd.)

Rationale: Rheumatoid arthritis (RA) shows a variable clinical expression in patients. Articular disease is common manifestation, but patients may rarely present with extra-articular manifestation such as cranial neuropathy. Also, primary hepatic lymphoma (PHL) has rarely been reported in patient treated with immunosuppressive drug such as methotrexate (MTX) for RA. We herein describe a case of cranial neuropathy and MTX-related PHL in a woman receiving MTX for RA., Patient Concerns: A 73-year-old women received MTX treatment for more than 5 years, presented with recurrent cranial neuropathies. During therapy of cranial neuropathies, liver enzyme levels were elevated., Diagnoses: The patient was diagnosed as RA by laboratory examination. A series of examinations had been launched to evaluate any possible cause of the extra-articular manifestation of the patient including ultrasound, computed tomography, magnetic resonance image (MRI) and positron emission tomography of the liver and MRI of the brain. Finally, the patient diagnosed as MTX-associated PHL and cranial neuropathy., Interventions: The patient underwent 4-year MTX therapy for RA at first with prednisolone. After that, she had been treated with cyclophosphamide therapy for cranial neuropathy. The liver biopsy was performed for hepatic lesion., Outcomes: MTX was discontinued, but no improvement of PHL and elevated liver enzyme was observed during the 3 weeks. The patient received 6 cycles of chemotherapy for 3 months and achieved complete remission including PHL and cranial neuronal lesion with symptom. No instances of relapse have occurred in 2 years of follow-up., Lessons: The present case is the extremely rare case in which MTX-related PHL and cranial neuropathy were involved together in the RA patient. It is necessary to examine long-term follow up hepatic and neurologic examinations that patient had a long history of receiving MTX therapy for RA.

The detection of pathological conditions related to the twelve cranial pairs represents a significant challenge for both clinicians and radiologists; imaging techniques are fundamental for the management of many patients with these conditions. In addition to knowledge about the anatomy and pathological entities that can potentially affect the cranial pairs, the imaging evaluation of patients with possible cranial pair disorders requires specific examination protocols, acquisition techniques, and image processing. This article provides a review of the most common symptoms and syndromes related with the cranial pairs that might require imaging tests, together with a brief overview of the anatomy, the most common underlying processes, and the most appropriate imaging tests for different indications., (Copyright © 2018 SERAM. Publicado por Elsevier España, S.L.U. All rights reserved.)

Background: In the list of named numerical neuro-ophthalmological syndromes, such as one-and-a-half syndrome and others, we report for the first time twenty-and-a-half syndrome, which is characterized by one-and-a-half syndrome with bilateral seventh and right fifth nerve palsy (1.5 + 7 + 7 + 5 = 20.5) in a patient with ischemic stroke., Case Presentation: A 45-year-old Asian Hindu woman presented with vomiting and imbalance of 1 day's duration. She had left-sided ataxic hemiparesis with one-and-a-half syndrome with bilateral seventh and right fifth nerve palsy. Magnetic resonance imaging of her brain revealed acute non-hemorrhagic infarct in the right posterolateral aspect of pons and medulla, with normal brain vessels angiography. We described her disorder as twenty-and-a-half syndrome. She was put on antiplatelet therapy., Conclusions: Twenty-and-a-half syndrome is reported for the first time. It is due to posterior circulation stroke; in our case, it was due to lacunar infarcts in the pons and medulla, manifesting as one-and-a-half syndrome with bilateral seventh and right fifth nerve palsy.

Purpose: To determine whether ocular motor cranial nerve (CN) palsy raises the risk of subsequent stroke in the general population., Methods: We investigated the association between ocular motor CN palsy and occurrence of stroke using the National Health Insurance Service-National Sample Cohort database from 2002 to 2013. We included individuals aged ≥ 20 years on January 1st, 2004, and excluded those having any paralytic strabismus, disorders in binocular movement, diplopia and any cerebrovascular diseases before entering the cohort. Incident ocular motor CN palsy was identified by diagnostic codes for third, fourth, and sixth nerve palsies. To determine the effect of incident ocular motor CN palsy on the occurrence of subsequent stroke, we used time-varying covariate Cox regression models. Model 1 included only incident third, fourth, and sixth nerve palsies as a time-varying covariate. Model 2 included Model 1 and defined demographic information. Model 3 included Model 2, comorbidity, co-medication, and the Charlson index score., Results: Among 727,689 individuals in the cohort, 1,633 patients developed ocular motor CN palsy and 17,657 patients suffered stroke. Cox regression models showed that development of ocular motor CN palsy was associated with an increased risk of subsequent stroke (hazard ratio [HR] = 4.65; 95% confidence intervals [CIs], 3.74-5.80 in Model 1), and the results were consistent even after adjusting for demographic factors and confounders in Model 2 and 3. Men, older age, and individuals not living in Seoul/Incheon area were associated with an increased risk of stroke, while individuals with higher income were associated with decreased risk of stroke in both Model 2 and 3. Sensitivity analyses using propensity score-based matching produced similar results in all three Models (HR = 1.95; 95% CI, 1.55-2.46 in Model 1, HR = 1.91; 95% CI, 1.52-2.41 in Model 2, and HR = 1.63; 95% CI, 1.29-2.06 in Model 3)., Conclusions: The occurrence of ocular motor CN palsy is a significant risk factor of subsequent stroke even after adjusting for demographic factors and confounders in the general population. Physicians may need to educate patients with ocular motor CN palsy regarding the higher risk of future stroke., Competing Interests: The authors have declared that no competing interests exist.

Objective: To evaluate cranial nerve (CN) outcomes after primary stereotactic radiosurgery (SRS) for petroclival, cavernous sinus, and cerebellopontine angle meningiomas., Methods: From our prospectively maintained database of 2022 meningioma patients who underwent Leksell stereotactic radiosurgery (SRS) during a 30-year interval, we found 98 patients with petroclival, 242 with cavernous sinus, and 55 patients with cerebellopontine angle meningiomas. Primary radiosurgery was performed in 245 patients. Patients included in this report had at least one CN deficit at the time of initial presentation and a minimum of 12 month follow up. Median age at the time of SRS was 58 years. Median follow up was 58 months (range 12-300 months), Median tumor volume treated with SRS was 5.9 cm 3 (range 0.5-37.5 cm 3 ), and median margin dose was 13 Gy (range 9-20Gy)., Results: Tumor control was achieved in 229 patients (93.5%) at a median follow up of 58 months. Progression free survival rate (PFS) after SRS was 98.7% at 1 year, 96.4% at 3 years, 93.7% at 5 years, and 86.4% at 10 years Overall, 114 of the 245 patients (46.5%) reported improvement of CN function. Patients with CP angle meningiomas demonstrated lower rates of CN improvement compared to petroclival and cavernous sinus meningioma patients. Deterioration of CN function after SRS developed in 24 patients (10%). The rate of deterioration was 2.8% at 1 year, 5.2% at 3 years, and 8% at 10 years., Conclusion: Primary SRS provides effective tumor control and favorable rate of improvement of preexisting CN deficit.

Purpose of Review: This article describes the clinical features and diagnostic criteria, pathophysiology (when known), and treatment strategies of the major cranial neuralgias., Recent Findings: Abnormal vascular loops compressing cranial nerves are the most common known pathogenesis associated with the primary neuralgias., Summary: The most frequently encountered primary neuralgias are trigeminal neuralgia, occipital neuralgia, and, rarely, glossopharyngeal neuralgia. Nervus intermedius neuralgia is even more rare. All neuralgias merit a careful workup for secondary causes. Drug treatment generally relies on antiepileptic drugs, antidepressants, and baclofen. OnabotulinumtoxinA can be useful in treating some cranial neuralgias. Surgical and invasive treatments include ablation, gamma knife treatment, and microvascular decompression.