Sixth Nerve Palsy Research Articles

Secondary pseudotumor cerebri in the pediatric population: clinical features, treatment, and prognosis

Purpose: Pseudotumor cerebri syndrome (PTSC) is characterized by elevated intracranial pressure (ICP) without intracranial mass, hydrocephalus, or abnormalities in cerebrospinal fluid composition. In secondary PTSC (sPTSC), there is a reason that increases the CSF pressure. The aim of this study is to evaluate the diagnosis, treatment, and follow-up of pediatric patients diagnosed with secondary PTC.
 Materials and methods: This is a retrospective study conducted in a single-center tertiary pediatric hospital. We included patients aged 1-17 years who were diagnosed with sPTSC in a tertiary hospital between 2018 and 2023 and met the current diagnostic criteria for PTSC. We evaluated the complaints, etiology, ophthalmological evaluations, and treatment results of the cases.
 Results: Seventeen patients with a diagnosis of PTCS were included in the study. The mean age was 9.82(±4.6). Of the patients, 9(56.2%) were male and 8(43.7%) were female. The most common symptoms were headache in 10 patients(62.5%), nausea/vomiting in 6 patients(37.5%), and double vision in 5 patients(31.2%). All patients had papilledema and 7(43.7%) patients had sixth nerve palsy. Recurrence was observed in 3(16%) patients. Optic nerve fenestration was performed in three patients as a second-line treatment.
 Conclusion: In cases that do not respond to medical treatment, optic nerve sheath fenestration may be a good treatment option.
 Keywords: optic nerve, child, pseudotumor cerebri

Pseudotumor cerebri in the paediatric population: clinical features, treatment and prognosis

IntroductionPseudotumor cerebri (PC) in prepubertal patients displays certain characteristics that differentiate it from its presentation at the postpubertal stage. The aim of this study is to describe the characteristics of paediatric patients diagnosed with PC at our centre and to compare them according to their pubertal status. Patients and methodsWe included patients aged between 1 and 18 years who were diagnosed with PC in a tertiary-level hospital between 2006 and 2019 and who met the updated diagnostic criteria for PC. They were classified according to body weight and pubertal status. Subsequently, we analysed results from lumbar punctures, neuroimaging studies, ophthalmological assessments, and treatments received during follow-up. ResultsWe included 28 patients, of whom 22 were of prepubertal age and 6 were of postpubertal age. The mean age (standard deviation) was 9.04 (2.86) years. Among the postpubertal patients, 83.3% were boys, 66.7% of whom presented overweight/obesity. In the group of prepubertal patients, 27% were boys, 31.8% of whom were overweight. The most frequent symptoms were headache (89.9%) and blurred vision (42.9%). All patients presented papilloedema, and 21.4% manifested sixth nerve palsy. Possible triggers were identified in 28.6% of cases. Nineteen percent of patients presented clinical recurrence, all of whom were prepubertal patients. Complete clinical resolution was achieved in 55.6% of patients. ConclusionPrepubertal patients with PC show lower prevalence of obesity, higher prevalence of secondary aetiologies, and higher recurrence rates than postpubertal patients.

Multiple Primary Malignancies and Bilateral Vocal Cord Paralysis Confusing the Management of Each Other

AbstractDouble primary malignancy though uncommon, we often encounter in our clinical practice. The lung malignancy is known to cause left vocal cord paralysis. Bilateral abductor paralysis secondary to adenocarcinoma of the lung with concurrent basal cell carcinoma of the face is not common. Proper counseling and timely management are needed in these cases of multiple primary malignancies. Early evaluation in all cases of hoarseness can help in early diagnosis and management.

Bilateral 6th nerve palsy due to intracranial hypertension after methyl methacrylate cranioplasty: a case report

Bilateral 6th nerve palsy due to intracranial hypertension after methyl methacrylate cranioplasty: a case report

Pseudotumorcerebri – a rare complication of ATRA based treatment of Acute Promyelocytic Leukaemia

Acute promyelocytic leukaemia is a highly curable sub type of acute myeloid leukaemia and about 90% of these patients have good chance of survival with current all transretinoic acid based arsenic tri oxide or chemotherapy. However, a rare complication, pseudotumour cerebri may arise following All Trans Retinoic Acid (ATRA) therapy, which may provide obstacle to continue this promising therapy. We present the case report of a young female patient with acute promyelocytic leukaemia treated with Arsenic trioxide (ATO) and ATRA that subsequently develop abrupt onset of lateral rectus palsy and decreased visual acuity due to pseudotumour cerebri. We treated the patient with acetazolamide and kept her at frequent follow up. There was significant improvement of her lateral rectus palsy as well as visual acuity following treatment with acetazolamide that allowed us to continue the primary All Trans Retinoic Acid (ATRA) based treatment. J Bangladesh Coll Phys Surg 2024; 42: 105-108

A Case of Neurosyphilis Presenting with Isolated Abducens Nerve Palsy.

We herein report a case of neurosyphilis that presented with isolated bilateral abducens nerve palsy. A 39-year-old man was referred to our department with diplopia. He had a history of homosexual relationships and showed only bilateral abducens nerve palsy upon a neurological examination. Positive syphilis tests in the serum and cerebrospinal fluid and a contrasting effect on the abducens nerve on magnetic resonance imaging (MRI) confirmed the diagnosis of active neurosyphilis. When a patient manifests isolated abducens nerve palsy, neurosyphilis can be a differential diagnosis, although rare, and contrast-enhanced MRI may help diagnose the disease.

An overview of neuro-ophthalmic disorders at Jenna Ophthalmic Center, Baghdad, Iraq (2021-2022).

Neuro-ophthalmic disorders are often documented individually for each illness, with little data available on their overall incidence and pattern. The overall incidence of neuro-ophthalmic illnesses in Iraq is still not recorded. This study aimed to evaluate the clinical, demographic, and etiological features of patients seeking consultation at an Iraqi neuro-ophthalmology clinic. A prospective cross-sectional observational research was conducted at the Janna Ophthalmic Center in Baghdad, Iraq. The center serves a diverse patient population from various governorates. All newly diagnosed patients with neuro-ophthalmic disorders who visited the neuro-ophthalmological clinic, regardless of gender or age group, were included. The neuro-ophthalmologist established a diagnosis for each case by reviewing the patient's medical history, doing physical examinations, administering specific tests, and, in certain cases, using neuroimaging methods. The duration of the study was extended from March 2021 to November 2022. Among the 6440 patients evaluated, 613 cases were confirmed at the neuro-ophthalmology clinic. Ischemic optic neuropathy (NAION, AION, and PION) was the most prevalent diagnosis, accounting for 17.61% of newly reported cases in the field of neuro-ophthalmology. This was followed by sixth nerve palsy. Diabetes mellitus affected 42.7% of the cases, followed by hypertension, which affected 39.3% of the participants. The incidence of neuro-ophthalmic diseases tended to be high. Ischemic optic neuropathy and sixth nerve palsy, traumatic/compressive optic neuropathy, and papilledema were the most common neuro-ophthalmic disorders reported.

Abducens Nerve Palsy as the Only Presenting Symptom for Solitary Cerebellar Tuberculoma in an Immunocompetent Young Adult

Abducens Nerve Palsy as the Only Presenting Symptom for Solitary Cerebellar Tuberculoma in an Immunocompetent Young Adult

Diplopia as the presenting symptom of a post‐transplant lymphoproliferative disorder in a bilateral inferior lim transplanted young adult

Aims/Purpose: To present a unique case of post‐transplant lymphoproliferative disorder (PTLD) after bilateral inferior limb transplantation, which is still controversial due to the absence of precedents in the literature and the unclear risk–benefit ratio.Methods: Case report.Results: A 22‐year‐old male presented to the emergency room complaining of double vision. He had a history of bilateral inferior limb amputation 8 years ago and received a bilateral lower limb transplantation 2 years later. He had normal graft function and was receiving cytomegalovirus prophylaxis. During neurological examinations left abducens nerve (CN VI) palsy was noted. Other cranial nerves affected produced facial hypoesthesia (CN V) and mild paresis (CN VII), hearing loss and tinnitus in left side (CN VIII) and soft palate asymmetry (CN X). He was otherwise neurologically intact, alert and orientated. MRI revealed a lesion located at the facial colliculus at the floor of 4th ventricle. In the context of immunosuppression, the differential diagnosis included abscess versus PTLD. Biopsy of the lesion was performed. Morphologically atypical lymphocytic cells suggestive of B lymphoma were identified. Monoclonal rearrangement for heavy chain Igen and immunoglobulins were identified with molecular biology techniques. The histology features were consistent with a diagnosis of CNS B lineage primary lymphoma. Two days later, bilateral supracondylar amputation was performed. Following the explant, immunosuppression was withdrawn. His underlying condition was finally diagnosed as PTLD, for which he started a chemotherapeutic regimen with radical intention (methotrexate and cytosine arabinoside). The patient recovered fully and up to date, 10 years later, is doing well.Conclusions: Due to its diverse aetiologias, a proper workup must be done of the patients presenting with diplopia to the emergency room. PLTD must be a condition to always be considered in patients with history of solid organ transplant; since the early diagnosis is crucial for a successful management of this often‐fatal condition.

Outcomes and Factors Associated with Successful Strabismus Surgery for Abducens Nerve Palsies: A Retrospective Study and Literature Review [Response to Letter

Outcomes and Factors Associated with Successful Strabismus Surgery for Abducens Nerve Palsies: A Retrospective Study and Literature Review [Response to Letter

Isolated Abducens Nerve Palsy: A Rare Manifestation of Recurrent Extramedullary Myeloma

Isolated Abducens Nerve Palsy: A Rare Manifestation of Recurrent Extramedullary Myeloma

Abducens palsy with facial nerve palsy: A rare clinical presentation

Abstract Multiple cranial nerve palsies are commonly due to brainstem lesions and the presence of abducens nerve palsy with peripheral facial nerve palsy is very rare and the exact etiologies are not defined. Very few reports of pontine infarct are associated with the condition. We report a young male presented with sudden onset diplopia with left-side abduction deficit and inadequate eyelid closure of the left eye. Upon evaluation, it was found to have high blood pressure recordings and imaging revealed the presence of an ipsilateral pontine infarct. The patient was managed with antihypertensives and antiplatelet agents and symptomatic resolution was found after 2 months of follow-up. This report describes a very rare association of abducens and facial nerve palsy in pontine infarct and to our best knowledge only a few reports are available on the same.

Parkinson’s syndrome – The syndrome within a syndrome – A case report

Horner’s syndrome is caused by damage to the sympathetic pathway anywhere along its course. The unique association of the sixth nerve palsy with ipsilateral Horner’s syndrome has a localizing value, known as Parkinson’s syndrome. The patient described here had left ptosis, miosis of pupil, and abduction limitation on left gaze. A diagnosis of Parkinson’s syndrome was made. Magnetic resonance angiography confirmed a cavernous lesion. High suspicion is essential for diagnosis of such a variant of Horner’s syndrome.

Abducens Nerve Palsy - A Rare Complication of Spinal Cord Stimulator Insertion: Case Report and Literature Review

BACKGROUND: Spinal cord stimulation (SCS) is an emerging therapeutic intervention for intractable chronic pain. However, SCS surgery is not exempt from complications such as lead migration, infections, hardware malfunction and neurological deficits. CASE REPORT: We present this case report on bilateral abducens nerve palsy, as a rare iatrogenic complication of SCS system insertion in a 65-year-old man with intractable chronic pain. After failing conservative treatment and explant, he underwent corrective strabismus surgery and achieved symptom control. CONCLUSION: The case presents a diagnostic and management challenge due to the nature of his presentation, neuroimaging pitfalls and limited high-quality evidence for management. We recognise cranial nerve palsy as a potential, yet real, complication of SCS surgery, of which abducens nerve palsy presents significant detriment to patients lives. As SCS continues to expand in clinical practice, inevitably, so will its iatrogenic sequela signalling the need for greater recognition and formation high-quality evidence for efficacious management. KEY WORDS: Spinal cord stimulation insertion, abducens nerve palsy, chronic pain, case report

Left Sixth Nerve Palsy and Subdural Hygroma Following Intrathecal Morphine Pump Placement: A Case Report

BACKGROUND: An intrathecal (IT) pump is an effective drug delivery system that assists patients with chronic pain management. However, due to the invasive nature of the procedure, a complication that could arise is cerebrospinal overdrainage or leak, which could lead to adverse effects. CASE REPORT: The patient underwent an IT morphine pump implant for treatment of his chronic, intractable low back pain. Following the procedure, he developed persistent headaches and diplopia and was found to have left lateral rectus palsy. Imaging studies showed the development of bilateral subdural hygromas. His condition was resolved with surgical intervention and time. CONCLUSIONS: After an extensive literature search and review, to our knowledge, this is the first reported case of sixth nerve palsy and subdural hygromas following the implant of a drug delivery system. KEY WORDS: Abducens palsy, IT pump, subdural hydrgomas, intrathecal drug delivery system, lateral rectus palsy

Abducens Nerve Palsy Caused by Cavernous Sinus Meningioma Mimicking Tolosa-Hunt Syndrome

Purpose: To report a case of abducens nerve palsy caused by cavernous sinus meningioma mimicking Tolosa-Hunt syndrome (THS).Case summary: A 59-year-female visited to the ophthalmic department for sudden onset of horizontal diplopia for 20 days. The extraocular examination showed 10 prism diopters (PD) esotropia at distant fixation and ortho at near fixation without definitive abduction limitation. At 2 months of follow-up, she showed the 25 PD left esotropia with abduction limitation of the left eye. The ocular pain and headache were newly developed. Magnetic resonance imaging (MRI) with contrast was performed and revealed the enlargement and enhancement of left cavernous sinus. THS was suspected and high dose steroid treatment was performed. The ocular findings and headache were improved after treatment. These symptoms showed waxing and waning with steroid tapering during follow-up. The follow-up MRI showed no definitive improvement of cavernous sinus lesion compared with previous study. The cavernous sinus meningioma was considered. There was no improvement of ocular motility findings during follow-up and the strabismus surgery was performed.Conclusions: The abducens nerve palsy with headache can occur caused by cavernous sinus meningioma. It may be difficult to differentiate it from THS in certain cases.

Botulinum Toxin Injection for the Treatment of Third, Fourth, and Sixth Nerve Palsy: A Meta-Analysis.

The efficacy of botulinum toxin injection for the treatment of third, fourth, and sixth nerve palsy was evaluated. PubMed, Scopus, EMBASE, Web of Science, and Google Scholar databases were searched. Data about the duration of palsy (acute vs chronic), cause of the palsy, type of toxin used, mean dose, and other background characteristics were collected. Outcome variables were success rate (defined by alleviation of diplopia or reduction in eye deviation) and standardized mean difference of prism diopter and abduction deficit before and after injection. The Joanna Briggs Institute checklist was implemented for the risk of bias assessment. The analysis included 38 articles, comprising 643 patients. The overall treatment success rate in acute and chronic nerve palsy was 79% and 33%, respectively. The success rate was not significantly different between different subgroups of age, type of botulinum toxin, pre-injection prism diopter, etiology of the palsy, duration of follow-up, and mean dose of botulinum toxin injection. However, in both acute and chronic palsy, diabetes etiology was accompanied by the highest success rate. Overall symptomatic response to botulinum injection was 84% (95% CI: 67% to 96%), whereas functional response was observed in 64% (95% CI: 47% to 79%) of the patients. The odds ratio for the success rate of treatment of palsies with botulinum toxin versus expectant management was 2.67 (95% CI: 1.12 to 6.36) for acute palsy and 0.87 (95% CI: 0.17 to 4.42) for chronic palsy. Botulinum toxin can be used for the treatment of acute third, fourth, and sixth nerve palsy, especially in patients with acute palsy and more severe tropia. [J Pediatr Ophthalmol Strabismus. 2024;61(3):160-171.].

Case Report: Tolosa-Hunt syndrome, a neuroinflammatory origin of painful ophthalmoplegia

Background Tolosa-Hunt syndrome is a rare neuroinflammatory disease that affects the cavernous sinus and superior orbital fissure, resulting in symptoms of neuropathy. Case presentation We report a case of a 35-year-old male patient with no past medical history who presented to the emergency department (ED) complaining of a four-day history of progressively increasing headaches associated with right eye pain and double vision. A physical examination showed findings suggestive of abducens nerve palsy. A computed tomography (CT) scan showed subtle asymmetric thickening of the right cavernous sinus, which prompted further investigation by magnetic resonance imaging (MRI) scan. The MRI scan showed intensely enhanced soft tissue thickening in the right cavernous sinus region, slightly extending to the right orbital apex, which caused enlargement of the cavernous sinus and appeared isointense to the adjacent muscles on T1 and T2. The diagnosis of Tolosa-Hunt syndrome was made after the exclusion of all other possible pathologies. The patient received prednisolone therapy; his symptoms improved, and he was discharged after three days. Two weeks later, the patient presented again to the ED with the same symptoms and was treated symptomatically. Conclusions This case highlights a very rare etiology for painful ophthalmoplegia, which is Tolosa-Hunt syndrome. Thickening of the cavernous sinus on MRI images with a background history of painful ophthalmoplegia and headache should raise clinician’s suspicion towards the disease. In addition, exclusion of other possible causes is very important, as Tolosa-Hunt syndrome is a diagnosis of exclusion.

Abducens Nerve Palsy Due to Coronavirus Disease (COVID-19)

The main causes of N.VI paralysis in adults are microvascular disease, vasculopathy, tumors, and inflammatory conditions. However, recent studies suggest that neurotropic viruses may be the etiology of N.VI paralysis. COVID-19 also has neurotropic and neuroinvasive capabilities that make the eye susceptible to bilateral optic neuritis, papilledema, and cranial nerve paralysis, the most common of which is abducens nerve palsy. The aim of this literature review is to review studies that describe the occurrence of abducens nerve palsy in COVID-19 patients. The literature search began in the period April-May 2022 from the Google Scholar database (https://scholar.google.com/). This mini review used the AMSTAR (Assessment of Multiple Systemic Review 2, including randomized and nonrandomized studies) checklist and reviewers independently agreed on the selection of eligible studies and reached consensus regarding which studies to include with inclusion criteria. This mini review found that 75% of cases were strongly associated with viral infection and 25% of cases were strongly associated with COVID-19 vaccination. Only 50% of cases report improvement in abducens nerve palsy. This has the implication that with the increasing prevalence of COVID-19 worldwide, ophthalmologists should be aware that abducens nerve palsy may be part of COVID-19.

10249-PEDT-8 CURRENT STATUS AND ISSUES OF PEDIATRIC DIFFUSE INTRINSIC PONTINE GLIOMA (DIPG)

Abstract that predominantly occurs in childhood, with a median survival of less than one year. Herein we present a recent case of DIPG and discuss the current status and future challenges. The patient was a 7-year-old boy who complained of tinnitus, headaches, and unstable gait. The MRI showed diffuse enlargement of the pons, and the contrast-enhanced T1-weighted image exhibited an irregular ring-like enhancement. Neurologically, he presented with left abducens nerve palsy, horizontal gaze nystagmus, significant left ataxia, and truncal ataxia. As the lesion extended to the left cerebellar peduncle, a stereotactic biopsy was performed. However, postoperative bleeding occurred, resulting in transient consciousness disturbance, left facial palsy, speech disturbance, and dysphagia, necessitating enteral nutrition management. The pathological diagnosis was high-grade glioma, IDH wild type, H3K27M mutation (-), TERTp wild type, BRAF V600E mutation (-), BRAF fusion (-), and MGMT unmethylated. After surgery, the patient received radiation therapy (54Gy/30fr), and his symptoms improved, allowing him to resume oral intake. Post-irradiation, the lesion shrank, and he was discharged home without assistance. However, six months later, his symptoms worsened, and he started taking temozolomide orally, but the effect was limited. Nine months later, he became unable to take oral medication, and a central venous port was placed. We coordinated with home care physicians to provide intravenous and palliative treatments, but respiratory and consciousness disturbances gradually worsened, and he died 10 months after the initial diagnosis. The median survival of DIPG patients treated at our hospital is currently 10.5 months, and this case followed a typical course. The development of novel treatments is desired, but due to the rarity of the disease, conducting comparative trials is challenging. Hence, registry data forming a control group are necessary. Additionally, transitioning to home-based palliative care and sharing information on supportive therapies are also critical tasks.