Abducens Research Articles

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.

A 39-Year-Old Woman with Oculodermal Melanocytosis: A Case Report

Introduction: Nevus of Ota, also known as oculodermal melanocytosis (ODM), is characterized by macular pigmentation which localized to the forehead and periocular area. It usually shows a dermatomal distribution from the first two branches of the trigeminal nerve. We reported a case of oculodermal melanocytosis (ODM).
 Case presentation: A 39-year-old woman, came with complaints of her left eye rolling inwards for the past 5 years accompanied by a flat black spot on her forehead that spread to her left cheek. She was done macular OCT and orbital ultrasonography of the left eye. She was diagnosed with oculodermal melanocytosis in the left eye, moderate NPDR in both eyes, juvenile cataracts in both eyes, and esotropia in the left eye due to left-eye cranial nerve VI paralysis. Follow-up at 6 months was advised to the patient.
 Conclusion: Although this is a benign condition, patients are at risk of developing complications such as increased intraocular pressure and melanoma of the skin and/or uvea, ongoing evaluation with imaging methods is highly recommended even if only skin lesions may be repaired using laser for cosmetic reasons.

Experience in treating children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle and systematic review.

To investigate the treatment plan and prognosis of children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle. In this retrospective study, the clinical information of 10 consecutively collected children with ocular dyskinesia and hemifacial spasm secondary to pontine tumours adjacent to the fourth ventricle was analyzed. All 10 children underwent pontine tumour resection through a trans-cerebellomedullary fissure approach; 4 children underwent preoperative diffusion tensor imaging scans to determine the relationship between the tumour and facial nerve nucleus, and the other 6 children underwent intraoperative deep electroencephalography (EEG) tumour monitoring, in which the tumour electrical discharge activity of the tumour was recorded. A voxel distribution map was established to describe the distribution of the tumour location, and patient prognosis was evaluated through clinical and imaging follow-up. All 10 children achieved total tumour resection; 9 tumours were pathologically suggested to be ganglioglioma (WHO grade I), and 1 was a hamartoma. The symptoms of the original ocular dyskinesia and hemifacial spasm disappeared immediately after the operation. The children were followed up for 4-75months, and none of the symptoms recurred; four cases with preoperative diffusion tensor imaging showed that the tumour was close to the facial nerve. Four in six intraoperative electrophysiological monitoring showed that the tumour had electrical discharge behaviour, and the tumour distribution map indicates a high density of tumour presence in the facial nerve nucleus and the nucleus of the abducens nerve. In paediatric patients, the facial symptoms are related to the location and abnormal electrical discharge of the tumour. There is no significant correlation between ocular dyskinesia and the location of the tumour. Conventional antiepileptic therapy for this disease is ineffective, and early surgical intervention for total tumour resection can achieve a clinical curative effect.

Pituitary metastasis arising from hepatocellular carcinoma: a case report and update of the literature.

Hepatocellular carcinoma (HCC) is the sixth most common neoplasia and the third leading cause of cancer-associated deaths worldwide. Most cases arise in patients with cirrhosis, and early detection through periodic screening can make it potentially curable. The presence of extrahepatic metastases (EHM) affects treatment decisions and curability. The lungs are the most common site for EHM, followed by lymph nodes, bones, and the adrenal glands. Interestingly, approximately only 15 cases of HCC metastasizing to the pituitary gland have been reported so far.The most common symptoms of pituitary metastasis (PM) arising from HCC are nerve palsies affecting the third, fourth, and sixth cranial nerves. Other symptoms, such as diabetes insipidus or pituitary insufficiencies, are present in a minority of cases. Detecting PM is difficult given its rarity. Gold-standard treatments for these patients have not yet been established, but the prognosis is dismal, with a median overall survival of only 4.5 months. In this paper, we present an interesting case of PM as the first symptom of an HCC in a 75-year-old female. We also present an overview of all cases reported to date with emphasis on symptom presentation and survival after diagnosis.Given the improvement of systemic therapy, more cases are diagnosed in both oligometastatic and palliative conditions. Therefore, better approaches and treatment modalities for extrahepatic metastases due to HCC should be defined.

Comparative characteristics of ophthalmological symptoms of orbitocranial and orbital cavernous venous malformation

BACKGROUND: Cavernous venous malformation of the orbit, formerly known as cavernous hemangioma of the orbit, refers to vascular malformations with slow blood flow, and is a frequent lesion of the orbit in adults. The spread of cavernous venous malformation of the orbit into the cranial cavity is extremely rare.
 AIM: The aim of this study is a comparative analysis of ophthalmological manifestations of orbitocranial and orbital cavernous venous malformation.
 MATERIALS AND METHODS: The analysis was performed on 50 patients operated in N.N. Burdenko National Scientific and Practical Center for Neurosurgery from 2004 till 2023. Two groups of patients with сavernous venous malformation of the orbit were identified: group 1 — with malformation spreading into the cranial cavity, group 2 —with malformation localized in the orbit only.
 RESULTS: In 29 patients, cavernous venous malformation of the orbit spread into the cranial cavity through superior orbital fissure, inferior orbital fissure, optic canal and/or through combinations thereof; in 21 patients, the malformation was located in the orbit only. Females prevailed in both groups; the average age was 44 years. The first group included patients with the following features: visual impairments were more frequent and more prominent, oculomotor disorders caused mainly by the involvement of oculomotor and abducens nerves; optic nerve atrophy, slight proptosis. The second group was identified by more prominent proptosis, more mild visual impairments, oculomotor disorders caused by the presence of the malformation in the orbit, at the eye fundus, optic nerve head edema prevailed.
 CONCLUSIONS: Ophthalmic symptoms due to topographic and anatomical variants of a cavernous malformation.

Special clinical features with a novel mutation site of CHN1 gene in a Chinese family with Duane retraction syndrome

ABSTRACT Purpose This study is to describe the special clinical and genotypic features of a Chinese family with variant types of Duane retraction syndrome and to present our experience on managing these cases. Methods Four individuals from one family were reviewed by ophthalmologic examinations, in which two affected and two unaffected individuals were revealed. MRI scans were performed on the two patients. Relevant gene mutations were screened by the next-generation sequencing technology and confirmed by Sanger sequencing technology. Results The six-year-old proband presented with special clinical features of severe horizontal gaze dysfunction, exotropia and mild scoliosis. His mother showed significantly limited binocular abductions, with retraction of eyeballs in adduction. From MRI scans, abducens nerves were not observed in both patients and the oculomotor nerve was slightly thin in the proband. The proband and his mother shared the same CHN1 gene mutation site (c. 62A>G; p.Y21C). Strabismus surgery was performed on the proband to correct the primary gaze exotropia. (NM_001822: exon3 or NM_001025201: exon4: c. 62A>G; p.Y21C) Conclusions A novel CHN1 gene mutation was revealed from a Chinese family with Duane retraction syndrome. Remarkably, the proband and his mother presented different clinical features of ocular motility disorder. Strabismus correction surgery and amblyopia training helped to improve the appearance and visual function of the proband.

Surgical implications of anatomical relationships among the Meckel’s cave, foramen ovale, foramen spinosum and VI cranial nerve in the anterior transpetrosal approach.

Surgical implications of anatomical relationships among the Meckel’s cave, foramen ovale, foramen spinosum and VI cranial nerve in the anterior transpetrosal approach.

Cranial nerve palsies and intracranial aneurysms: A narrative review of patterns and outcomes.

Cranial nerve palsy (CNP) in patients with intracranial aneurysms (IAs) can impose significant burdens on a patient's quality of life. The literature has a paucity of reviews addressing patterns of overall reported cranial nerve (CN) involvement and outcomes in patients with IA. The literature systematically reviewed CNP at presentation in the setting of IA using PubMed, Web-of-Science, and Scopus according to the PRISMA guidelines. Fifty-two studies reported a total of 513 patients with IA and 630 CNPs observed at presentation: oculomotor (58.25%), abducent (15.87%), optic (12.06%), trochlear (8.7%), and trigeminal (1.9%). Most common aneurysms are located in a posterior communicating artery (46%) and cavernous internal carotid artery (29.2%). Trends of CNP based on the rupture status of IAs showed that 80% were associated with unruptured IAs and 20% with ruptured IAs. Post-treatment of IA, 55% of patients had complete resolution of CNP, with most (89%; n = 134) resolving within the first 6 months. Stratified by CNP type: Complete resolution rate is 100% in CN VII-IX, 60% in CN VI, 59% in CN IV, 54% in CN III, 45% in CN V, and 43% in CN II. In patients with cranial nerve palsies attributed to IAs, the location and rupture status of the aneurysm could determine the type and severity of the nerve palsy. Most patients experienced favorable outcomes in terms of their resolution and long-term function of the CNP after treatment of the IA.

Establishing the Precise Location of Apanga Marma and defining its Structural Component through Cadaveric Study

Background: Apanga marma is a Jatrurdhwagata marma that is positioned within half angula dimension below the tail end of the eyebrow and outer to the lateral canthus of the eye. Its injury causes andhya and dristiupaghata. Sushruta classifies it as Sira structurally, however Vagbhata classifies it as Snayu marma. The precise position, radiance, and conflicts in opinion in identifying the structural entity and applied importance of this marma must be clarified. The objective of this study was to locate and ascertain the position and structural entity of Apanga marma. Materials and Methods: Study was conducted in two steps: Conceptual study by reviewing the literatures and cadaveric observational study by dissection over 4 cadavers. Results and Discussion: The Apanga marma was discovered to be positioned on the anterior section of the superior temporal fossa, near the lateral canthus of the eye. Extracranial, intracranial, and intraorbital neurovascular structures were observed within the designated area. Structures in the area include the Middle cerebral artery, the Middle meningeal artery, and the Abducent nerve, which can cause visual problems or total blindness if injured. Conclusion: Apanga marma was found to be located in the anterior section of the superficial temporal fossa, lateral to the lateral orbital edge, beneath the tail end of the eyebrow, and above the zygomatic arch. The middle meningeal and middle cerebral arteries were discovered as a specific anatomical entity in the marma area, resulting in blindness or vision impairment upon injury.

Microsurgical Procedures of Microvascular Decompression for Trigeminal Neuralgia

Microvascular decompression for trigeminal neuralgia was successfully performed following the secure surgical steps. The most crucial step involves creating a safe operative field in the dural opening. The petrotentorial junction should be identified without cerebellar retraction before proceeding to the deeper areas. Dissecting the petrosal vein and opening the horizontal fissure contributed to the expansion of the operative field. Bleeding often occurs from the dorsal cerebellar bridging vein and junction of the petrosal vein into the superior petrosal sinus. Transposition of the most common offenders, the superior and anterior inferior cerebellar arteries, is effectively achieved by dissecting both the proximal and distal sides of the neurovascular compression site. Teflon should be placed at a sufficient distance to prevent contact with the nerve, which can lead to recurrence. Treating vertebrobasilar artery-related cases is challenging and involves the risk of cranial nerve injuries. Multiple offending vessels are commonly involved. In such cases, it is essential to be aware of the course of the trochlear and abducens nerves during decompression. Applying the most effective and least risky maneuver is necessary for treating cases involving the vertebrobasilar artery.

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.

Retrobulbar haemorrhage and globe perforation after botulinum toxin injection into the medial rectus

Aims/Purpose: The aim of this communication is to show a recent case of both retrobulbar haemorrhage (RH) and probable globe perforation (GP) after botulinum toxin (BT) injection in the medial rectus of the right eye.Methods: We selected a recent case seen at our hospital. A 5u BT injection was performed under topical anaesthesia and guiding electromiography into the medial rectus due to a VI cranial nerve palsy. 2 min later the patient claimed intense pain and we evidenced a sudden growing periocular oedema and progressing exophthalmos. Retrobulbar haemorrhage was diagnosed and immediate canthotomy and cantholisys were performed, with consequent haemorrhage drainage. In the 24 h follow‐up visit, our patient claimed blurry vision and floaters. VA RE was 0.3 (before BT 0.7). Important corneal oedema and 3–4 + tyndall was observed. In the funduscopic exploration severe hemovitreous was observed; and an ocular ecography was performed, which showed no signs of retinal disruption. As vitreous started clearing and VA and floaters improved, observational treatment was decided. When we became able to see the retina with detail a possible perforation hole was seen in the infero‐medial quadrant, with no signs of traction or retinal elevation.Results: 3 months later, VA was 0.8 e° 1. Funduscopy showed an applied retina, with only a few persistent fibrine remainders in the inferior quadrant. Good‐coloured optic disc, with physiologic excavation and no signs of atrophy.Conclusions: Eventhough RH and GP are quite rare complications after BT infection, we should always take them into consideration. We also wanted to highlight these complications treatment. RH: Immediate canthotomy and cantholysis must be performed GP: close observation must be followed in order not to miss a retinal detachment. Plus, to avoid them we should be careful to perform the right technique and to use the electromiograph to guide us into the muscle.

Vogt‐Koyanagi‐Harada‐like findings as the first manifestation of neuroleukaemia

Aims/Purpose: To report a well‐documented case of a 62‐year‐old woman presenting with VKH‐like findings as the first manifestation of neuroleukaemia.Methods: Case study.Results: A 52‐year‐old woman who had received a diagnosis of acute myeloid monoblastic leukaemia (AML) one month prior and was in remission after the induction phase presented with floaters and gradual visual loss in both eyes. Visual acuity was 20/200 and 20/25, respectively. Anterior segment exam was normal in both eyes. Funduscopy showed reduced foveal reflex. On spectral‐domain OCT, subretinal fluid was found in both eyes. Fluorescein angiography showed hypofluorescent areas compatible with multifocal serous retinal detachments and multiple hyperfluorescent dots surrounding the detached areas. Days after, the patient debuted with facial palsy and binocular diplopia due to bilateral VI cranial nerve (CN) palsy, left VII CN palsy and right complete III CN palsy. An MRI was performed with no signs of infiltration. A cerebrospinal fluid sample was drawn by lumbar puncture and its analysis denoted infiltration of AML cells with similar immunophenotype to that on initial bone marrow biopsy. Our patient presented with an atypical case of Vogt‐Koyanagi‐Harada (VKH) with multiple leakages along the periphery of the detached retinal area. This sparing of the macula should be considered a suspicious pattern, and combined with the finding of CN palsies was enough to suspect a relapse in the form of CSF infiltration, making the diagnosis of neuroleukaemia possible.Conclusions: Exudative retinal detachment can be a primary presentation of AML or denote a relapse. VKH is a diagnosis of exclusion, as one of the main criteria for its diagnosis is the absence of other systemic diseases. This becomes especially important in atypical presentations and older patients. So as not to miss life‐threatening underlying conditions, leukaemia and lymphoma should be considered in the differential diagnosis of VKH.

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

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.

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.

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

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.

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.