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Rationale: Hypertrophic pachymeningitis (HP) is a rare and complex condition marked by inflammation and thickening of the dura mater. It can occur either on its own or as a result of various underlying medical issues. This type of granulomatous meningitis is extremely rare and poorly understood, making diagnosis and treatment particularly challenging. Patients with HP may experience severe headaches and cranial nerve defects, but in some cases, the condition can occur without any symptoms, making it undetected. We detail the case of patient who was diagnosed with HP after presenting with abducens nerve palsy, a condition that significantly affected her vision and quality of life. Remarkably, she showed substantial recovery following a course of methylprednisolone pulse therapy, coupled with careful radiographic diagnosis and follow-up assessments. The decision to report this case stems from its rarity and the diverse clinical manifestations associated with this condition, which can vary widely among patients., Patient Concerns: A 26-year-old Asian female, sought medical attention at The First Affiliated Hospital of Zhejiang Chinese Medical University, where she reported experiencing diplopia, a troubling symptom indicative of abducens nerve palsy., Diagnosis: To establish a definitive diagnosis, second-generation sequencing biological detection was employed to rule out any infectious causes, while radiographic imaging provided confirmation of HP., Interventions: In terms of intervention, the patient was promptly initiated on a regimen of methylprednisolone pulse therapy, followed by a carefully monitored oral sequential reduction of the medication. Throughout her treatment, she underwent regular liver function tests to ensure her safety, and liver protective drugs were also administered as a precautionary measure., Outcomes: As of now, the patient has successfully completed her treatment and is reported to be doing well, marking a positive outcome in her recovery journey., Lessons: This case underscores the critical importance of imaging examinations in the clinical diagnosis of patients presenting with diplopia, as they play a vital role in both diagnosing and differentiating between various diseases. Furthermore, it is essential to rule out any infection-related factors in patients diagnosed with HP before commencing treatment with glucocorticoids, ensuring a comprehensive approach to patient care., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: With the SARS-CoV-2 pandemic (COVID-19), data on central and peripheral nervous system involvement, including those causing cranial nerve 6 (CN6) palsy, have been limited to case reports. To extract clinically relevant features of COVID-19-related CN6 palsy, we report on a recurrent pediatric case and analysis of reported cases associated with infection or immunization., Methods: A PubMed search revealed 18 cases of isolated CN6 palsy in addition to the index case (n = 19). Clinical characteristics, workup, and temporal associations between systemic symptoms onset or vaccination, symptoms onset, and resolution were compiled and analyzed., Results: The median age of CN6 onset was 43 years (interquartile range [IQR]: 28-52). Sixteen cases (84.2%) were associated with COVID-19 illness and 3 (15.8%) were associated with COVID-19 vaccination. Four cases (23.5%) had positive neuroimaging findings. The median latency from first COVID-19 symptoms or vaccination to onset of CN6 palsy was 6 days (IQR: 2.3-16), and the median time from onset to resolution was 30 days (IQR: 14-60). Latency to onset of CN6 palsy was significantly and directly associated with time to resolution (R 2 = 0.401, P = 0.010). Patients who had a positive SARS-CoV-2 antibody test had significantly longer days from symptoms to onset (6.0 vs 24.5, P = 0.030), and patients with a positive SARS-CoV-2 polymerase chain reaction test had a significantly shorter time to resolution (17.50 vs 90, P = 0.042)., Conclusions: Isolated CN6 palsy from COVID-19 is rare, can occur in infants as young as 7 months, and can be recurrent. Longer latency from systemic symptoms onset portends greater recovery times, and this relationship may reflect multiple mechanisms by which COVID-19 (and/or an immune response thereto) causes cranial neuropathies with direct clinical relevance., Competing Interests: Dr. Gaier reports Luminopia, Inc (advisor, patent, equity) and Stoke Therapeutics, Inc (consultant). The other authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Background: This retrospective, observational cohort study aimed to determine recovery rate and recovery time of ocular motor nerve palsies (OMP) of third (CN III), fourth (CN IV), or sixth cranial nerves (CN VI)-and associated prognostic factors-in meningioma and pituitary adenoma (PA) patients., Methods: A total of 25 meningioma (28 eyes) and 33 PA patients (36 eyes), treated at the Leiden University Medical Center in the Netherlands from January 1, 1978 to January 31, 2021, were included. OMPs were evaluated according to a newly created recovery scale using on-clinical and orthoptic examinations, which were performed every 3-4 months until palsy recovery, or at 18 months follow-up., Results: Recovery rates of CN III (meningioma 23.5% vs PA 92.3%), CN IV (meningioma 20% vs PA 100%), and CN VI (meningioma 60% vs PA 100%) palsies were observed at 18 months follow-up, with differences between the 2 tumor types being observed in the treated patients only. Median recovery time of all OMPs combined was significantly longer in meningioma patients (37.9 ± 14.3 months vs 3.3 ± 0.1 months; P < 0.001). No significant protective or risk factors for recovery rate or time were identified., Conclusions: OMP recovery rates in treated patients were more favorable in patients with PA compared with patients with meningiomas, independent of OMP cause. With these new insights in OMP recovery, more accurate prognoses and appropriate follow-up strategies can be determined for meningioma and PA patients with OMPs., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the North American Neuro-Ophthalmology Society.)

Background: Arachnoid cysts (ACs) are benign lesions typically believed to not cause neurologic defects in the adult population and are most often found incidentally on imaging. We describe 2 patients with ACs potentially leading to isolated cranial nerve (CN) dysfunction., Methods: We describe 2 patients, 1 with a fourth nerve palsy and the other with a sixth nerve palsy found to have ACs on MRI brain imaging in locations that potentially caused a compressive CN palsy. We review previous literature of ACs presenting with CN III, IV, or VI palsy., Results: Patient 1 was a 62-year-old man who presented with a 22-year history of diplopia with strabismus examination consistent with a congenital CN IV palsy. Despite multiple surgeries, his CN IV palsy insidiously worsened. An AC in the posterior fossa with mass effect on the quadrigeminal plate and asymmetric atrophy of the right superior oblique was identified on imaging. Patient 2 was an 80-year-old man who presented with an 18-year history of diplopia and was found to have a left esotropia and abduction deficit consistent with complete CN VI palsy. An AC in the left cavernous sinus was identified on imaging. He underwent strabismus surgery with satisfactory resolution of diplopia. We identified a total of 18 previously published cases: 8 reports of CN III palsy, 4 reports of CN IV palsy, and 6 reports of CN VI palsy. Patient ages ranged from 1 to 67 with a median of 34.5. In 16/18 (89%) cases, the diagnosis of ACs was made within 1 year of symptom onset. Surgical removal of the AC was successful in resolving diplopia in 7/12 (58%) cases. In no case was strabismus surgery performed as primary treatment., Conclusions: Although ACs are typically congenital asymptomatic lesions, we present a case series of 2 patients with ACs in anatomic locations that potentially caused chronic, progressive, isolated CN palsies leading to strabismus. Our literature review revealed that most published cases detailing this clinical scenario resulted in neurosurgical fenestration of ACs with mixed results. Our cases represent 2 patients with AC-associated CN palsy treated with strabismus surgery., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by North American Neuro-Ophthalmology Society.)

Competing Interests: Disclosures None.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Significance: Intracranial hypotension is a condition that occurs from a cerebrospinal fluid leak. Various visual symptoms have been associated with this condition. Cranial nerve VI (CN VI) palsies are the most common ocular manifestation, as the abducens nerve is prone to injury because of its intracranial anatomical course., Purpose: This case report presents a CN VI palsy secondary to intracranial hypotension from ventriculoperitoneal shunt overfiltration. Diagnosis, treatment, and management considerations are discussed. No identifiable health information was included in this case report., Case Report: A 70-year-old White man was referred to the eye clinic for evaluation of binocular horizontal diplopia. The patient had a recent history of a left ventriculoperitoneal shunt for a persistent cerebrospinal fluid leak after complex mastoid surgery. The patient was also symptomatic for positional headaches, which improved in a recumbent position. He was diagnosed with a left CN VI palsy secondary to intracranial hypotension from a ventriculoperitoneal shunt overfiltration. The patient was followed up by neurosurgery for shunt adjustments to resolve the overfiltration. Binocular horizontal diplopia was managed conservatively with Fresnel prism., Conclusions: Intracranial hypotension should be considered in patients presenting with cranial nerve palsies and positional headaches. Obtaining neuroimaging and comanaging with neurology or neurosurgery are advised to make prompt diagnosis and treatment. Careful clinical monitoring and conservative diplopia therapy are recommended as visual symptoms improve upon resolution of the cerebrospinal fluid leak., Competing Interests: Conflict of Interest Disclosure: The author has reported no financial conflict of interest., (Copyright © 2023 American Academy of Optometry.)

Abstract: A previously healthy 2-year-old boy presented with a left sixth cranial nerve palsy. There was a family history of multiple sclerosis and optic neuritis. Neuroimaging showed multiple foci of T2/FLAIR hyperintense signal abnormality in both cerebral hemispheres and in the brainstem. The initial diagnosis was suspicious for demyelinating disease. However, there was no clinical improvement after a course of corticosteroids, and there was no change in his follow-up MRI. He later developed bilateral sixth nerve palsies, with esotropia addressed with bilateral medial rectus botulinum toxin injections. A brain biopsy was planned. However, his 3-month-old sister was separately admitted for fever and pancytopenia. She had markedly elevated ferritin, D-dimer, triglycerides, sIL-2R, CXCL9, and IL-18 and low fibrinogen. Her bone marrow biopsy showed hemophagocytosis. Genetic testing of both siblings revealed biallelic mutations in the PRF1 locus. The final diagnosis of familial hemophagocytic lymphohistiocytosis Type 2 was made. Both siblings underwent chemotherapy. The boy's sixth nerve palsies and MRI abnormalities resolved. Both siblings then went on to undergo bone marrow transplant., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by North American Neuro-Ophthalmology Society.)

Background: Imaging diagnosis of clival cancer may be difficult, in part because of normal variation in marrow signal with aging. Identifying whether clival cancer has damaged the sixth cranial nerve is a further challenge because minimal clival abnormalities could impinge on the nerve, which travels very close to the clivus., Methods: Two neuroradiologists, who were unaware of previous imaging and clinical diagnoses, reviewed MRI studies of 25 patients with cancer but no clival involvement and no sixth nerve palsy, 24 patients with clival cancer but without sixth nerve palsy, and 31 patients with clival cancer and sixth nerve palsy. The radiologists were tasked with determining whether there was clival cancer, whether there was a sixth nerve palsy and its laterality, and with indicating the pulse sequences used to make those determinations., Results: Both neuroradiologists correctly identified all 25 cases with a normal clivus. In about half of those cases, they depended on finding a homogeneously bright marrow signal; in the remaining cases, they excluded cancer by determining that the clivus was not expanded and that there were no focal signal abnormalities. Both neuroradiologists correctly identified clival cancer in 54 (98%) of the 55 cases with and without sixth nerve palsy. In doing so, they relied mostly on clival expansion but also on focal signal abnormalities. Both neuroradiologists were at least 80% correct in identifying a sixth nerve palsy, but they often incorrectly identified a palsy in patients who did not have one. When there was a one-sided signal abnormality or the clivus was expanded in one direction, both neuroradiologists were accurate in identifying the side of the sixth nerve palsy., Conclusion: Current MRI pulse sequences allow accurate differentiation of a normal from a cancerous clivus. When the marrow signal is not homogeneously bright in adults, cancer can be diagnosed on the basis of clival expansion or focal signal abnormalities. MRI is less accurate in predicting the presence of a sixth nerve palsy. However, the side of a unilateral palsy can be predicted when the clivus is clearly expanded in one direction or there is a focal signal abnormality on one side., Competing Interests: The authors report no conflicts of interest., (Copyright © 2022 by North American Neuro-Ophthalmology Society.)

Background: Covid-19 has serious sequelae that may be poorly understood, underreported, and, as a result, not diagnosed promptly, such as variations in clinical manifestations of hyperinflammation among people infected with SARS-CoV-2. ophthalmoplegia can be one of these manifestations., Methods: We are reporting a 55-year-old male patient with unilateral diplopia considering it as a case of multisystem inflammatory syndrome in adults. We also reviewed the literature systematically for the previously reported studies/cases with third, fourth and sixth cranial nerve palsies due to or after Covid-19., Results: The literature search yielded 17 studies reporting 29 patients. 71.4% of the patients were males with a mean age of 42.23 years. Ophthalmological symptoms took 9.7 days to appear after the respiratory involvement. All patients had diplopia as part of their visual symptoms. 41.4% of the patients had unilateral sixth nerve palsy, 24% had bilateral sixth nerve involvement, 17% had fourth nerve involvement, and 27.6% had third nerve involvement., Conclusion: Ophthalmoplegia is considered presenting symptom of Covid-19. Further research is needed to detect all neuro-ophthalmological manifestations of Covid-19., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2022 the Author(s). Published by Wolters Kluwer Health, Inc.)

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Introduction: The pandemic of coronavirus disease 2019 (COVID-19) stands as a major global health and social burden. As cases are growing, several other symptoms, besides the typical respiratory ones, are emerging. The involvement of the nervous system is increasingly recognized with manifestations ranging from hyposmia to meningoencephalitis and cranial neuropathies., Case Report: We report the case of a 41-year-old female patient who presented to the emergency department complaining of diplopia and headache over the last 2 days. She denied any medical history, as well as any other neurological or respiratory symptom. A detailed neurological and ophthalmological examination revealed a limitation to the abduction of the right eye due to palsy of the right lateral rectus muscle causing painless, horizontal diplopia in the right gaze. The computed tomography of the brain was normal. Based on the detected lymphopenia, she was tested for COVID-19 and was positive. The cerebrospinal fluid analysis showed no abnormalities, while also a repeated head computed tomography was similarly normal. The patient received no specialized medical treatment, and after 6 days, she was discharged home having a minimal degree of persistent diplopia. Two weeks later, brain magnetic resonance imaging was performed that was similarly unrevealing., Conclusions: Isolated abducens nerve palsy can be the only presenting symptom in COVID-19. Although several pathophysiological mechanisms have been proposed, the exact nature of this manifestation has not been clarified yet. Vigilance is required by neurologists to detect and manage patients with such subtle clinical presentations., Competing Interests: The authors declare no conflict of interest., (Copyright © 2021 Wolters Kluwer Health, Inc. All rights reserved.)

Significance: Lyme disease can have widespread and long-lasting systemic implications, and ocular manifestations of Lyme disease may be the only presenting symptoms of infection. As such, eye care providers can play a critical role in facilitating the diagnosis and treatment of this disease., Purpose: This case report describes an isolated sixth nerve palsy secondary to Lyme disease, which is a rare neuro-ophthalmic presentation. This report emphasizes the importance of considering Lyme disease in the setting of atypical nerve palsies to allow for appropriate treatment and prevent long-term complications of untreated disease., Case Report: A 63-year-old man presented with new-onset, painful diplopia for 1 day and was diagnosed with a right sixth nerve palsy. His medical history was significant for relapsing polychondritis with associated ocular complications, so his nerve palsy was presumed secondary to a flare of his systemic disease. However, secondary to complaints of new night sweats, infectious etiologies were also considered. The patient was initially treated with oral steroids with no improvement in his diplopia after being admitted to the hospital for further workup. Approximately 1 week after presentation, Lyme titers returned positive, and the diagnosis was revised to abducens palsy secondary to Lyme disease. The patient's nerve palsy rapidly resolved after oral therapy with doxycycline., Conclusions: Lyme disease is a systemic illness that can have widely varying manifestations, including ocular findings. Eye involvement may be the only presenting symptom, allowing eye care providers to serve an important role in disease recognition and management., Competing Interests: Conflict of Interest Disclosure: None of the authors have reported a financial conflict of interest., (Copyright © 2022 American Academy of Optometry.)

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.)

Background: During the surgical resection of petroclival meningiomas, preserving the cranial nerves is crucial. The abducens nerve is particularly vulnerable during surgery. However, the preoperative risk factors and postoperative prognosis of abducens nerve palsy (ANP) are poorly understood., Methods: We retrospectively analyzed 70 patients who underwent surgery for petroclival meningiomas between May 2010 and December 2019, divided into gross-total resection (GTR) and subtotal resection (STR) groups. The relationship of preoperative clinical factors with the incidence and recovery of postoperative ANP was analyzed., Results: Postoperative ANP was observed in 23 patients (32.9%). Multivariable logistic regression revealed that the tumor-to-cerebellar peduncle T2 imaging intensity index (TCTI) (P < 0.001) and internal auditory canal invasion (P = 0.033) contributed to postoperative ANP. GTR was achieved in 37 patients (52.9%), and 10 (27.0%) of them showed ANP. STR was achieved in 33 patients (47.1%), and 13 (39.4%) of them showed ANP. Recovery from ANP took a median of 6.6 months (range, 4.5-20.3 months). At 6 months after the operation, recovery of the abducens nerve function was observed in 16 patients (69.0%); of whom, 4 (40.0%) were in the GTR group and 12 (92.3%) were in the STR group (P = 0.025)., Conclusions: TCTI and internal auditory canal invasion were the risk factors for postoperative ANP. Although intentional STR did not prevent ANP immediately after the operation, recovery of the abducens nerve function after surgery was observed more frequently in the STR group than in the GTR group., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Background: Acquired sixth nerve (CN6) palsies in children may be benign or associated with an underlying neurologic condition. In children who presented with isolated (no associated neurologic or ophthalmic symptoms or signs) CN6 palsies, the rate of newly diagnosed neurologic disorders (such as tumors) is unclear. Moreover, the factors associated with spontaneous resolution and amblyopia in children with acquired CN6 palsies are unknown., Methods: We retrospectively reviewed the charts of all children younger than 18 years diagnosed with CN6 palsy at our institution from 2010 to 2020. We recorded ophthalmologic and neurologic history and examination findings, neuroimaging results, etiology of CN6 palsy, and outcomes including spontaneous resolution and amblyopia. We assessed etiologies of isolated and nonisolated CN6 palsies as well as frequency and factors associated with spontaneous resolution and amblyopia (in children ≤7 years)., Results: One hundred seventy-two children met inclusion criteria. Twenty CN6 palsies (12%) were isolated at presentation. Most isolated cases were presumed postviral or postvaccination (50%) or idiopathic (30%), but 2 cases (10%) were associated with newly diagnosed tumors. Spontaneous resolution occurred in 59% of CN6 palsies at a median of 12.3 weeks and was associated with older age (P = 0.03) and nontumor etiology (P = 0.006). Amblyopia developed in 18% of children at risk, exclusively in those with anisometropia, pre-existing strabismus, or younger than 12 months., Conclusions: Our findings and chart reviews suggest that approximately 10% of isolated acquired pediatric CN6 palsies are associated with a newly diagnosed brain tumor. This risk must be discussed with parents when considering immediate vs delayed neuroimaging. In addition, infants and children ≤7 years with secondary amblyogenic risk factors (anisometropia or pre-existing strabismus) require close follow-up to monitor and treat amblyopia., Competing Interests: The authors report no conflicts of interest., (Copyright © 2021 by North American Neuro-Ophthalmology Society.)

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Competing Interests: The authors report no conflicts of interest.

Abstract: Primary pancraniosynostosis is a rare variant of craniosynostosis in which the major cranial sutures prematurely fuse. Single-suture craniosynostosis is often recognized early in life due to an abnormal head shape. In contrast, primary pancraniosynostosis may be diagnosed later in life due to a grossly normal head shape and size. As such, these children can present with symptoms related to chronically elevated intracranial pressure (eg, vision loss or cognitive impairment). This report highlights a patient with primary pancraniosynostosis associated with unique neurologic sequelae-namely, bilateral abducens nerve palsy. A 9-year-old boy presented to the ophthalmologist with a 1-month history of double vision, drifting of his right eye toward the nasal bridge, and intracranial hypertension evident with papilledema. Physical examination was notable for mild bitemporal narrowing. A computed tomography study demonstrated radiologic thumbprinting, diffuse osseous sclerosis, and fusion of the bilateral coronal, sagittal, metopic, and lambdoid sutures. The patient underwent emergent cranial vault expansion with fronto-orbital advancement. Papilledema had resolved 4 months following surgery. At 2-year follow-up, abducens nerve palsy and head shape were significantly improved. This study brings attention to an unreported presenting symptom of pancraniosynostosis (bilateral abducens nerve palsy). This information may lead to quicker diagnosis and treatment of pancraniosynostosis-induced intracranial hypertension, which is critical to prevent long-term sequelae., Competing Interests: The authors report no conflicts of interest., (Copyright © 2020 by Mutaz B. Habal, MD.)

Rationale: Immunoglobulin G4 (IgG4)-related hypophysitis is a rare disorder which often requires invasive pituitary gland biopsy to confirm its diagnosis. We present a case whereby peripheral organ lesion biopsy and imaging findings were sufficient for the diagnosis., Patient Concerns: A 77-year-old man with diplopia was referred to our department by an opthomologist who had diagnosed the patient with right abducens nerve palsy., Diagnoses: Head magnetic resonance imaging revealed enlargement of the pituitary gland and pituitary stalk, while hormonal analysis revealed panhypopituitarism, thereby indicating a diagnosis of hypophysitis. Abdominal computed tomography imaging revealed a solid mass that encompassed the left kidney ureter. Although the patient did not have an increase in serum IgG4, a biopsy of the periureteral mass revealed infiltrating plasma cells that were positive when stained for IgG4., Interventions: The patient was given corticosteroid pulse therapy (methylprednisolone: 1 g × 3 days), followed by oral corticosteroids (prednisolone, 0.5 mg/kg/d)., Outcomes: The right abducens nerve palsy improved and the pituitary lesion shrank after the initiation of corticosteroid treatment., Conclusion: Based on the diagnosis of IgG4-related disease in the retroperitoneal organ and response to corticosteroid treatment, this patient was diagnosed with IgG4-related hypophysitis. This hypophysitis caused enlargement of the pituitary gland with resulting nerve compression, causing abducens nerve palsy. When IgG4-related hypophysitis is suspected, a thorough examination of other organ lesions and biopsy should be considered.

An 83-year-old man presented with a 1-year history of abducens palsy. Brain MRA revealed arterial dolichoectasia of most cerebral vessels, including the basilar, internal carotid, and middle cerebral arteries. Abducens nerve palsy as a sole manifestation of diffuse intracranial dolichoectasia is unusual but may occur in diffuse intracranial dolichoectasia.

Background: Unruptured intracranial aneurysms that cause isolated sixth nerve palsies are usually located extradurally in the cavernous sinus or intradurally as part of fusiform enlargement of the parent artery. Intradural saccular aneurysms of the vertebrobasilar circulation are a rare but life-threatening cause of isolated sixth nerve palsy. We provide documentation of 2 such cases., Methods: Case records of the Neuro-ophthalmology Clinics at the University of Michigan., Results: A 56-year-old woman with an arteriosclerotic risk profile reported new diplopia and had an isolated left sixth nerve palsy caused by an unruptured saccular aneurysm of the anterior inferior cerebellar artery. It was successfully coiled, and the sixth nerve palsy markedly improved. A 62-year-old woman with an arteriosclerotic risk profile and new diplopia had an isolated right sixth nerve palsy caused by a large unruptured vertebral artery aneurysm. It was coiled and later stented and recoiled. These procedures isolated the aneurysm and relieved the sixth nerve palsy., Conclusions: Isolated sixth nerve palsies in these 2 older adult patients with arteriosclerotic risk profiles were caused by unruptured intradural vertebrobasilar aneurysms. This report adds documentation to a rare phenomenon that must be taken into consideration in deciding whether brain imaging is needed in isolated sixth nerve palsy.

Objective: Otitis media, facial pain in trigeminal region, and ipsilateral abducens nerve palsy clinically define Gradenigo syndrome, a rare but serious complication of suppurative middle ear infection. Radiological investigation is required to confirm petrous apex involvement and to exclude further consequences as sinus thrombosis, meningitis, and intracranial abscess., Methods/results: We report the case of an 8-year-old child referred to our emergency department for recurrent headache and sudden strabismus. Clinical evidence of suppurative otitis media raised the suspicion for Gradenigo syndrome, definitively confirmed at computed tomography and magnetic resonance imaging scans. Conservative treatment alone enabled complete clinical and radiological remission, without long-term sequelae., Conclusions: Despite being a rare condition, Gradenigo syndrome should be taken into account as potential differential diagnosis in children referred to emergency department for recurrent headache and strabismus. An accurate anamnesis to document recent ear infection is mandatory to orientate the diagnosis and focus radiological investigations. Early recognition and timely intervention may allow conservative management to succeed, avoiding the need for surgery and serious sequelae.

An 84-year-old woman reported onset of headaches, diplopia, and blurred vision. On examination, she was found to have a left sixth nerve palsy and an incongruous right homonymous hemianopia. Brain MRI demonstrated a left petroclival meningioma, causing this unusual combination of clinical findings. The patient was treated with radiation therapy and has remained stable over 4 years of follow-up.

Rationale: Herpes zoster is characterized by unilateral vesicular eruption and it most often affects the trigeminal nerve. We would like to report a rare case of abducens and vagus nerves palsy caused by varicella zoster virus (VZV) without the typical vesicular rash., Patient Concerns: A 71-year-old woman presented with diplopia. Three days previously, she had experienced sore throat and hoarseness., Diagnosis: At presentation, the prism cover test revealed esotropia of 10 prism diopters at primary gaze, and abduction was restricted in the right eye. No vesicular rash was observed on the patient's face, and magnetic resonance imaging of the brain showed no pathology. Flexible fiberoptic laryngoscopy revealed multiple ulcerations on the right side of the larynx-from the epiglottis to the arytenoid. After 1 day of hospitalization, a diffuse skin rash occurred on the patient's trunk, and polymerase chain reaction for VZV DNA was positive at the skin lesion. The patient was diagnosed as having herpes zoster associated with vagus and sixth nerve palsy., Intervention and Outcomes: She received famciclovir at a dose of 500 mg 3 times daily for 7 days and coadministered methylprednisolone. At the 4-month follow-up, her diplopia and eye movement had resolved completely., Lessons: In patients with abducens nerve palsy without typical vesicular lesion, herpes zoster may not be detected early. In that case, systemic examination is very important for diagnosing herpes zoster.

Rationale: Convergent strabismus fixus is an ocular motor abnormality in which the eye is fixed in adduction. This condition is mostly associated with high myopia and is caused by a displacement of the extraocular muscles. We report a nonmyopic woman with convergent strabismus fixus due to aneurysms., Patient Concerns: A 79-year-old woman complained of progressive worsening of esotropia about 50 years prior to her visit. Neuroimaging showed that the eye was not dislocated, and the extraocular muscles were not displaced. However, aneurysms were found bilaterally from the intracavernous carotid arteries and the location was on both abducens nerves., Diagnoses: Chronic bilateral abducens nerve palsies due to aneurysms., Interventions: Endovascular treatment was successfully performed for the aneurysms., Outcomes: Convergent strabismus fixus still remained., Lessons: Chronic abducens nerve palsies may develop to nonmyopic convergent strabismus fixus without displacement of extraocular muscles, and mass lesions in the brain including aneurysms should be ruled out when orbital MRI cannot explain the condition.

Gradenigo syndrome was defined as a clinical triad of otitis media, severe orbito-facial pain, and ipsilateral sixth cranial nerve palsy. It may occur as a complication of either acute or chronic otitis media. Herein, we will report a case of Gradenigo syndrome induced by suppurative chronic otitis media that was promptly diagnosed and aggressively treated.

Background: Late recurrence of visual symptoms associated with carotid-cavernous fistula (CCF), including diplopia, is uncommon and raises concern for new or recurrent fistula formation., Methods: We report 2 patients with traumatic CCFs, where cranial nerve paresis resolved after endovascular CCF treatment only to reappear years later., Results: No evidence of recurrent or new fistula formation was found. Both were treated successfully with strabismus surgery., Conclusions: Although the cause of delayed onset diplopia after successful treatment is still unknown, theories include late compression of cranial nerves within the cavernous sinus due to coil mass that can cause chronic ischemia, delayed inflammation due to a thrombophilic nidus created by the coil mass, or injury to the cranial nerves that manifests later due to decompensated strabismus.

Rationale: Bilateral abducens nerve deficits caused by vertebral artery (VA) aneurysm have been reported, but there have been no reports regarding accompanying urinary retention., Patient Concerns: In this report, we describe an unusual case with bilateral abducens nerve palsy and urinary retention due to rupture of a vertebral aneurysm., Diagnoses: Subarachnoid hemorrhage caused by the rupture of a left VA aneurysm., Interventions: The VA aneurysm was successfully controlled by coil embolization., Outcomes: Urinary retention was improved after embolization and recovered by the day the patient left hospital. The bilateral abducens nerve palsy gradually recovered 6 months later., Lessons: This case emphasizes the importance of maintaining a broad, open mind in approaching the diagnosis and management of urinary retention associated with cranial nerve symptoms and reacting quickly to the clinical developments., (Copyright © 2017 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.)

Introduction: Sarcoidosis is a chronic, systemic, inflammatory disorder that is characterized by the formation of noncaseating granulomas. Patients may present with cranial nerve palsy, paresthesia, paresis, pyramidal signs, progressive cognitive decline, urinary retention, seizures, or hypothalamic-pituitary syndrome. Although the diagnosis of neurosarcoidosis can be challenging, neurological manifestations of sarcoidosis occur more frequently than previously described., Case Report: A 23-year-old African American man presented to our emergency department with diplopia, which was worsened on left horizontal gaze. On the day of admission, he had a witnessed seizure. Laboratory studies were significant only for mild leukopenia and erythrocyte sedimentation rate of 17 mm/h. Brain magnetic resonance imaging revealed diffuse thickening and enhancement of the dura, mild mass effect, and soft tissue enhancement through the foramen rotundum and left orbital apex. The patient was treated with intravenous methylprednisolone and discharged on 60 mg oral prednisone daily followed by a taper over a 2-month period., Conclusions: Our case demonstrates that mild neurological deficits can be the initial presentation of neurosarcoidosis in patients with undiagnosed or proven sarcoidosis.

Objectives: Sixth cranial nerve (SCN) palsy is an uncommon but important neurological problem in patients admitted to pediatric emergency department. The underlying etiology of SCN palsy has a wide range from viral infections to intracranial tumors; therefore, a careful and systematic approach is necessary while examining these patients., Methods: Fourteen patients who presented with acute SCN paralysis to pediatric emergency department during the last 10 years were examined., Results: The age at the time of admission ranged between 14 months and 16 years (median, 9.5 years). Of the 14 patients, 5 were girls and 9 were boys. A total of 3 of the 14 patients had bilateral cranial nerve VI paralysis, and 9 patients had additional abnormal findings on neurological examination. Neuroimaging studies included cranial tomography (n = 3) and brain magnetic resonance imaging in all patients. The underlying etiology was malignancy (n = 3); glioma, medulloblastoma, acute lymphoblastic leukemia, and dural sinus thrombosis (n = 2); as well as Guillain-Barre syndrome (n = 2), multiple sclerosis (n = 1), pseudotumor cerebri (n = 1), and meningitis (n = 1). The remaining 4 patients had miscellaneous benign etiologies., Conclusions: Other lesions of primary brain tumors causing increased intracranial pressure constitute 50% of the underlying etiology, followed by Guillain-Barre syndrome (14.2%). However, these patients had neurological symptoms signs, in addition to diplopia or SCN paralysis. Patients admitted to pediatric emergency department with acute SCN paralysis should be examined in detail to disclose the underlying etiology especially if they present with additional clinical signs or symptoms.

The combination of abducens nerve palsy and ipsilateral Horner syndrome was first described by Parkinson and considered as a localizing sign of posterior cavernous sinus lesions. The authors present a case with right abducens nerve palsy with ipsilateral Horner syndrome in a patient with carotid-cavernous fistula because of head trauma. The patient was referred to the ophthalmology clinic with diplopia complaint after suffering a head trauma during a motorcycle accident. Cerebral angiography showed low-flow carotid-cavernous fistula.

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated sensory and motor demyelinating polyneuropathy that typically presents as a relapsing-remitting or progressive disorder. Cranial neuropathies infrequently occur in association with other more typical symptoms of CIDP., Case Report: We report a case of CIDP with recurrent isolated sixth nerve palsy. Her physical examination showed a right sixth nerve palsy and absent deep tendon reflexes as the only indicator of her disease. Magnetic resonance imaging revealed thickening without enhancement of the trigeminal and sixth cranial nerves. Nerve conduction study (NCS) revealed a sensory and motor demyelinating polyneuropathy with conduction block and temporal dispersion in multiple nerves consistent with CIDP. Cerebrospinal fluid demonstrated albuminic-cytologic dissociation. She had a remarkable response to intravenous immunoglobulin and remains asymptomatic without any additional immunomodulating therapy., Conclusions: Isolated cranial neuropathies can rarely occur as the sole manifestation of relapsing-remitting CIDP. The profound demyelination found on NCS in this case demonstrates that there can be a dramatic discordance between the clinical and electrodiagnostic findings in some patients with this disorder.

Ocular motor cranial nerve palsies of viral etiology are uncommon and, when accompanied by skin lesions, zoster ophthalmicus is the most frequent diagnosis. We describe the case of a 68-year-old woman who developed fourth and sixth nerve palsies 3 days after appearance of a painful vesicular skin rash on the left side of her forehead. Neuroimaging was normal but polymerase chain reaction (PCR) testing of the cerebrospinal fluid was positive for Herpes Simplex 1 and negative for Varicella Zoster. The patient was treated with intravenous acyclovir, and the cranial nerve palsies resolved over 7 weeks. Although the similarity of the cutaneous vesicular eruption in our patient to that seen with zoster might have led to an incorrect diagnosis, acyclovir seems to be safe and effective for both viral etiologies.

This was a case of a 21-year-old female patient with a very rare and unexpected symptom "diplopia occurring due to the idiopathic sixth nerve palsy" encountered after 2 weeks following bimaxillary surgery performed for the correction of class III malocclusion deformity.