Isolated pseudo­abducens palsy and contralateral occipital headache with thalamic stroke: A case report and mini­review of the literature.

The abducens nerve (sixth cranial nerve) is essential for lateral eye movement, and its malfunction can cause a variety of issues with vision. Pseudo-abducens palsy is a rare neurological condition that causes a limitation in eye abduction, while the abducens nerve is still functioning. Thalamic pain syndrome, a severe complication of cerebrovascular events, presents as intense neuropathic pain provoked by temperature fluctuations. Although thalamic strokes are infrequently associated with ocular abnormalities, some studies suggest an association between isolated pseudo-abducens palsy and thalamic infarctions. The present study describes the case of a 38-year-old male patient with 1-day progressive diplopia and occipital headache who had abducens palsy on the left side as a result of a right thalamic infarction. The patient had a 10-year history of smoking and a 1-year history of hypertension, which was poorly controlled. The diagnosis was supported by a neurological examination, imaging and stroke etiology investigations. The patient recovered well within 5 days, highlighting the good prognosis of an acute thalamic presentation. In addition, a mini-review of the literature was performed and two similar reports were identified upon searching the literature using the Embase, Google Scholar, Lilacs, Medline, SciELO and ScienceDirect databases. On the whole, the present study demonstrates that understanding the complex neuronal connections inside the thalamus is critical for a proper diagnosis and appropriate intervention strategies in patients with thalamic stroke with oculomotor impairments. Further research is required to elucidate the underlying causes and develop treatment techniques for thalamic infarction consequences.

Case report: Tolosa-Hunt syndrome-expanding the neuromyelitis optica spectrum disorder phenotype?

Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune astrocytopathy caused by the autoantibody of aquaporin-4 (AQP4). Herein, we report a case of Tolosa-Hunt syndrome presenting with abducens palsy and AQP4 antibodies. This was a rare case of AQP4-immunoglobulin G seropositivity in a patient with Tolosa-Hunt syndrome. Our findings may expand the clinical phenotype of NMOSD and indicate that clinicians should consider testing for AQP4 antibodies in patients with Tolosa-Hunt syndrome.

A Case of Unilateral Sixth Cranial Nerve (CN VI) Avulsion in the Prepontine Cistern After Severe Head Trauma.

The patient was a 46-year-old woman who presented with right sixth cranial nerve (CN VI) palsy following severe head trauma secondary to a motor vehicle collision one month prior. In this case report, we aim to add to the literature an additional example of unilateral CN VI avulsion as visualized by MRI secondary to head trauma. 3D T2 MRI was used to visualize the CN VI avulsion. CT was also used in the evaluation of head trauma. In our view, the force trajectory of the patient's impact with the vehicle dashboard, as evidenced by the right occipital lobe fracture, explains the etiology of the unilateral right CN VI avulsion. The combination of clinical and imaging findings was central to the analysis of this case.

Aetiologies of acquired pediatric sixth nerve palsies in a U.K. based population.

Due to the low incidence of sixth cranial nerve palsies in children, there has been limited evidence published on this subject, especially from a population based within the UK. The incidence of etiologies has been found to vary significantly within the literature, especially with regard to neoplasms. The main aim of this study is to present the etiologies of newly diagnosed pediatric sixth nerve palsies in a UK-based population. We also take into consideration if the palsies were isolated or associated with other neurological signs or symptoms. Retrospective data collection was carried out on the medical records of 50 pediatric patients with a new-onset sixth nerve palsy. They all presented to a large tertiary referral hospital in the South of the UK between 1 January 2007 and 31 December 2017. Data collected for each patient included age, gender, ethnicity, unilateral versus bilateral, other signs and symptoms, etiology, where the patient first presented, and whether the palsy was the first presenting feature. Thirty-three (66%) patients had a new-onset sixth nerve palsy in conjunction with other neurological signs or symptoms and were considered non-isolated. Seventeen cases (34%) were found to be isolated. Etiologies included high intracranial pressure (18%), neoplasm (14%), surgery for neoplasm (14%), viral (14%), infection (12%), trauma (8%), idiopathic (6%), benign space-occupying lesion (4%), congenital (2%), inflammation (2%), Alexander's disease (2%), Kawasaki syndrome (2%), and diabetes (2%). Our study found non-isolated sixth nerve palsies to be the most common presentation. These patients had a high number of potentially sinister etiologies, the most common being high intracranial pressure followed by post-surgery for neoplasm and neoplasm. Isolated sixth nerve palsies were more commonly due to viral or idiopathic etiology; however, two cases of benign space-occupying lesion and one of neoplasm were identified.

More Than Meets the Eye: Isolated Bilateral Abducens Nerve Palsy as the Initial Presentation of Multiple Sclerosis.

Multiple sclerosis (MA) is a chronic demyelinating disease of the central nervous system. Although the initial presentation of MS is widely variable, only rarely does it present with isolated bilateral cranial nerve involvement. With this article, we report a case of MS initially presenting as a clinically isolated syndrome of bilateral abducens nerve palsy.

Clinical profile and management of sixth nerve palsy in pediatric patients (0-15 years) in Southern India - A hospital-based study.

PURPOSE: This study was done to evaluate the clinical profile in pediatric patients (0-16 years) presenting with acute onset esotropia due to sixth nerve palsy and its management options in a tertiary care set up of Southern India. METHODS: A total of 12 patients presenting to our OPD with acute onset esotropia due to sixth nerve palsies were included in this retrospective study. All patients were observed for 6 months and managed with prism and/or patching while waiting for spontaneous resolution and later managed surgically. Neuroimaging was done in all cases. RESULTS: The mean deviation of esotropia at presentation was 30.17 ± 5.7 Prism Diopter (range 12-50 Prism Diopter 95% CI, SD 10.11). Mean age of the patients during presentation was 8.6 ± 2.4 years (range: 1-15 years, SD 4.27). Among the common causes of sixth nerve palsy in our study population were trauma and idiopathic intracranial hypertension followed by tumor and miscellaneous causes. Only three patients underwent surgical correction of residual deviation after a waiting period of 6 months for self-resolution. Spontaneous resolution was observed in 41.6% patients, and surgical correction (unilateral resection-recession) was done in 25% of the patients with good surgical outcome. CONCLUSION: At 1-year follow up, the motor outcome was satisfactory except for one patient who had diffuse pontine glioma and had worsening neurological symptoms on follow-up.

Oculomotor Cranial Neuropathies: Diagnosis and Management.

Ocular nerve palsies are among the most common cranial neuropathies in neurological practice. Nerves can get affected anywhere along their path from the brainstem to the orbit. There can be isolated involvement of multiple cranial nerves together. The etiologies differ according to the type of presentation. The steps toward the diagnosis need to be strategically planned and must be based on clinical localization. It is crucial to make proper localization to plan further investigations and thus treatment of the etiology. This review covers the approach toward the diagnosis, etiologies involved, and management of ocular cranial neuropathies.

A case of juvenile-onset amyotrophic lateral sclerosis with a de novo frameshift FUS gene mutation presenting with bilateral abducens palsy.

Fused in sarcoma (FUS) is the most common causative gene in juvenile-onset amyotrophic lateral sclerosis (jALS). We presented a case of a 15-year-old Chinese girl with atypical and extremely rare bilateral abducens palsy was caused by a heterozygous c.1520del (p.Gly507Alafs*22) pathogenic frameshift mutation in the FUS gene revealed by whole-exome sequencing. This is the first jALS case presenting with bilateral abducens palsy and carrying de novo FUS genetic variant.

Abducens Palsy and Anosmia Associated with COVID-19: A Case Report.

AIM: To report the case of acute isolated abducens nerve palsy and anosmia in a healthy 69-year-old female following SARS-CoV-2 infection. METHOD: This is a case report of a previously healthy 69-year-old Caucasian female who presented to the emergency eye centre with a four-day history of binocular, horizontal diplopia eight days after testing positive for SARS-CoV-2 infection. Anosmia was her isolated symptom of COVID-19. RESULTS: The patient was diagnosed with left abducens nerve palsy. Aetiology was presumed to be post-viral as the patient was not diabetic and had no pre-existing microvascular risk factors. Diplopia resolved within 3.5 weeks. Measurements confirmed complete spontaneous recovery of the abducens palsy within 6 weeks. CONCLUSION: Cranial nerve palsies may constitute part of the neurological spectrum of COVID-19 disease. This case report aims to raise awareness amongst clinicians of coronavirus-induced neurological symptoms. Research suggests SARS-CoV-2 infection can trigger an aberrant immune response in some individuals, causing inflammatory nerve damage leading to anosmia and neuropathy. This case report supports the hypothesis that direct or indirect virally mediated injuries along the routes of the cranial nerves can cause neuropathy and olfactory dysfunction. The longer latency effects of COVID-19 infection are not well understood. The long-term rehabilitation of patients exposed to COVID-19 is a major public health concern requiring multidisciplinary expertise. This case report highlights the value of the Orthoptist in the diagnosis and care of patients experiencing neuropathy following COVID-19 exposure.

Acute-onset binocular diplopia in neurological unit: Aetiological factors and diagnostic assessment.

OBJECTIVES: To investigate the aetiology of acute-onset binocular diplopia (AOBD) in neurological units and identify the key diagnostic procedures in this setting. MATERIALS AND METHODS: Clinico-demographic data from patients hospitalized for AOBD from 2008 to 2019 were retrospectively reviewed. AOBD due to an underlying neurological disorder known to cause diplopia was addressed as secondary diplopia. Ophthalmoparesis plus was defined when subtle neurological signs/symptoms other than ophthalmoparesis were detected during neurological examination. RESULTS: A total of 171 patients (mean age 57.6 years) were included in the study. A total of 89 subjects (52%) had an oculomotor disturbance consistent with sixth nerve palsy, and 42 (24.6%) showed multiple oculomotor nerve involvement. The most common cause of AOBD was presumed to be microvascular in 56 patients (32.7%), while a secondary aetiology was identified in 102 (59.6%). Ophthalmoparesis plus and multiple oculomotor nerve involvement significantly predicted a secondary aetiology in multivariable logistic regression analysis. Brain CT was never diagnostic in isolated ophthalmoparesis. A combination of neuroimaging examinations established AOBD diagnosis in 54.9% of subjects, whereas rachicentesis and neurophysiological examinations were found to be performant in the remaining cases. CONCLUSIONS: AOBD may herald insidious neurological disease, and an extensive diagnostic workup is often needed to establish a diagnosis. Neurological examination was pivotal in identifying patients at higher risk of secondary aetiology. Even in cases of apparently benign presentation, a serious underlying disease cannot be excluded. Brain MRI was found to perform well in all clinical scenarios, and it should be always considered when managing AOBD.

Unilateral Abducens Palsy and Headache in Postpartum Patient Presenting to Ophthalmology.

Postpartum patients rarely present to eye casualty. Here we report a case of a seven-day postpartum patient with sudden onset horizontal diplopia and an occipital headache from the perspective of the ophthalmology eye casualty in a tertiary hospital. Intracranial imaging ruled out any acute pathology. The patient required epidural anaesthesia during labour, and a diagnosis of a post-dural puncture headache (PDPH) with an abducens nerve palsy was reached. A blood patch was not provided in this case. The headache settled and the diplopia self-resolved three weeks postpartum. PDPH with extra-ocular muscle paresis is rare, and, as the diplopia onset usually follows the characteristic orthostatic headaches of PDPH, it is likely that these patients are followed up by obstetricians or anaesthetists. These patients rarely present to ophthalmology services to receive this diagnosis, therefore ophthalmologists might not be familiar with this pathology. To our knowledge, this is the first case report of PDPH with cranial nerve palsy that has been documented to present to an ophthalmology department.

Supramaximal Horizontal Rectus Recession-Resection Surgery for Complete Unilateral Abducens Nerve Palsy.

Purpose: To review the surgical procedures and outcomes of supramaximal horizontal rectus recession-resection surgery for abduction deficiency and esotropia resulting from complete unilateral abducens nerve palsy. Methods: A total of 36 consecutive cases diagnosed as complete abducens nerve palsy, receiving supramaximal medial rectus recession (8.5 ± 1.4 mm, range: 6-10) combined with a supramaximal lateral rectus resection (11.1 ± 1.7 mm, range: 8-14) as performed over the period from 2017 to 2020, were reviewed retrospectively. All surgeries were performed by a single surgeon. Pre- and post-operative ocular motility, ocular alignment, forced duction test, binocular vision, abnormal head posture, and surgical complications were assessed. Results: Of these 36 cases, 23 (63.8%) were followed up for greater than 2 months (Mean ± SD = 8.4 ± 6.0, range: 2-24) after surgery and the collected data was presented. Mean ± SD age of these patients was 41.7 ± 14.4 (range: 12-67) years with 73.9% being female. Trauma (52.2%, 12/23) and cerebral lesions (21.7%, 5/23) were the primary etiologies for this condition. Esodeviation in primary position improved from 55.5 ± 27.2 prism diopters (PD) (range: +25 to +123) to 0.04 ± 7.3 PD (range: -18 to +12) as assessed on their last visit. Pre-operative abduction deficits of -5.6 ± 1.0 (range: -8 to -4) reduced to -2.4 ± 1.4 (range: -4 to 0) post-operatively. The mean dose-effect coefficient of 2.80 ± 1.20 PD/mm (range: 1.07-6.05) was positively correlated with pre-operative esodeviation. Rates of overcorrection and ortho were 69.6 and 26.1%, respectively, on the first day after surgery, while on their last visit the respective levels were 4.3 and 82.6%. Conclusion: Supramaximal horizontal rectus recession-resection surgery is an effective treatment method for complete abduction deficiency. The dose-effect was positively correlated with pre-operative esodeviation. Overcorrection on the first day post-operatively is required for a long-term satisfactory surgical outcome.

Delayed diagnosed Gradenigo's syndrome associated with acute otitis media.

Gradenigo's syndrome presents as a triad of retroorbital pain, ipsilateral abducens palsy, and purulent otorrhea. If the otologic pathologies in Gradenigo's syndrome go unnoticed, the condition could be misdiagnosed with neurological diseases because of retroorbital pain and abducens palsy. Treatment of Gradenigo's syndrome remains controversial. Although some reports state that long-term antibiotic treatment is sufficient, we recommended that management ought to be guided on a case-by-case basis depending on patient and disease factors. Herein, we report a delayed diagnosed pediatric case of Gradenigo's syndrome associated with acute otitis media that was treated with ventilation tube insertion.

Bilateral Abducens Nerve Palsy Due to Extensive Cerebral Venous Sinus Thrombosis.

Cerebral venous sinus thrombosis (CVST) is a relatively rare condition. We present a case of an acute aseptic thrombosis of the sagittal, transverse and sigmoid sinus in a puerperium patient with protein S deficiency. The specifics of the case include high intracranial pressure (ICP) caused by sinus thrombosis with typical symptomatology and bilateral papilloedema, which also manifested in transient bilateral abducens nerve palsy and, consequently, bilateral horizontal diplopia. The recovery of the cranial nerve function occurred 3 to 4 weeks after it was initially reported. Prompt and adequate anticoagulant therapy contributed to the almost complete recanalization of the dural venous sinus thrombosis and a positive outcome of the disease.

Endoscopic-Assisted Infralabyrinthine Approach to Petrous Apex Cholesterol Granuloma / 대한이비인후과학회지

A cholesterol granuloma is the most common primary lesion of the petrous apex, and accounts for 40% of the pathologies that arise in this region. The primary treatment for symptomatic lesions is by surgery to decompress and drain or to completely remove the lesion. Here we describe the use of infralabyrinthine approach to access a lesion through the temporal bone and completely remove it with the assistance of a 0-degree endoscope. A 43-year-old man visited our clinic for diplopia. Magnetic resonance imaging revealed a 2.3-cm cholesterol granuloma located in the left petrous apex that caused deviation of the left abducens nerve. The tumor was completely removed using the endoscopic-assisted infralabyrinthine approach; the patient is currently being followed up, and there is no evidence of disease recurrence. This case report describes the successful completion of a petrous apex cholesterol granuloma that preserved the cochlear and vestibular systems.

Adjustable Posterior Fixation Suture Technique in Adjustable Superior Rectus Transposition.

BACKGROUND: Recently, the superior rectus transposition has been widely adopted for the treatment of complete abducens palsy and Duane syndrome. This procedure is useful in that there is a decreased risk of anterior segment ischemia compared to complete vertical rectus transposition, possibly decreased incidence of postoperative-induced vertical deviations than complete vertical rectus transposition, and improvement in abduction compared to simple medial rectus recession. One difficulty with this procedure is the lack of adjustability in most patients. Our group has adopted a new technique for an adjustable posterior fixation myopexy suture for use with patients under topical anesthesia. METHODS: The superior rectus muscle is temporally transposed to the insertion of the lateral rectus muscle. The corner of the superior rectus muscle that is placed adjacent to the lateral rectus muscle is placed on an adjustable suture. Then, a posterior fixation myopexy suture consisting of a single-armed 6-0 vicryl suture is secured between the superior and lateral rectus muscles, approximately 10 mm from the lateral rectus insertion to drag the superior rectus muscle temporally. This suture is also placed on an adjustable suture. The patient is then positioned sitting up, fixing at a target at approximately 10 feet away from the patient's head. Cover testing is utilized to determine whether any vertical deviation has been induced. If there is a vertical deviation, the posterior fixation suture may be loosened. RESULTS: We find that this technique to be useful if an induced vertical deviation or an overcorrection occur, and is thought to be due to the reported possible complication of restriction induced by the posterior fixation suture and the transposed rectus muscle. CONCLUSION: Our technique for performing superior rectus transposition with an adjustable posterior fixation myopexy suture may be useful to surgeons who wish to have an adjustable option as a way to decrease the risk of postoperative complications such as induced vertical deviations and overcorrections.

Adenoid cystic carcinoma of the hard palate presenting as ipsilateral sixth nerve palsy.

Adenoid cystic carcinoma (ACC) is an uncommon malignant neoplasm composed of basaloid epithelial and myoepithelial cells. The palate is the most commonly involved intraoral site for ACC. Here, we document the case of an advanced ACC arising from the hard palate that presented with right-sided sixth nerve palsy in a 75-year-old male with no other systemic illnesses. ACC of the head and neck involving the cavernous sinus and presenting as isolated sixth nerve palsy is exceedingly rare. In the absence of vasculopathic or ischemic risk factors, regardless of the age of the patient; neuroimaging should be performed in cases of isolated nontraumatic sixth nerve palsy.

Isolated abducens nerve palsy treated by immunoadsorption in a patient with diarrhea-associated hemolytic uremic syndrome / Parálisis ocular aislada del sexto par craneal tratada con inmunoadsorción en un paciente con síndrome urémico hemolítico asociado a diarrea

Shiga toxin-producing Escherichia coli (STEC) that causes a prodromal hemorrhagic enteritis is the main cause of hemolytic uremic syndrome (HUS) particularly in pediatric patients. It is characterized by acute kidney injury with microangiopathic hemolytic anemia and thrombocytopenia. The kidney and brain are the two major target organs, and neurological involvement is the most frequent cause of mortality. The time delay between bloody diarrhea and neurological symptoms ranges from few days to a month. Neurological disorders include disturbances in cognitive functions, focal neurological signs, epileptic seizures, myoclonus and neuropsychiatric symptoms. Cerebral magnetic resonance imaging reveals various patterns of hyperintensities distributed through cerebral matter or may be totally normal even the patient has severe neurological involvement. Electroencephalography usually show generalized or focal slowing of the background activity, spikes or sharp waves despite being normal in around 20% of patients. We present here an adult male patient referred to our center with requirement of hemodialysis due to diarrhea-associated HUS complicated by acute kidney injury. Later during the course of plasma exchange therapy the patient developed an isolated abducens nerve palsy. Complete renal recovery was achieved by plasma exchange therapy but abducens palsy remedied rescue introduction of immunoglobulin G (IgG) depletion by immunoadsorption

Escherichia coli, productor de toxina Shiga (STEC), que causa una enteritis hemorrágica en fase prodrómica, es la principal causa del síndrome urémico hemolítico (SUH), particularmente, en pacientes pediátricos. Se caracteriza por una lesión renal aguda con anemia hemolítica microangiopática y trombocitopenia. El riñón y el cerebro son los dos órganos principales a los que ataca, y la afectación neurológica es la causa más frecuente de mortalidad. El tiempo que transcurre entre la aparición de diarrea sanguinolenta y los síntomas neurológicos varía entre pocos días y un mes. Los trastornos neurológicos incluyen alteraciones en las funciones cognitivas, signos neurológicos focales, ataques epilépticos, mioclonías y síntomas neuropsiquiátricos. La resonancia magnética de cerebro revela varios patrones de hiperintensidades distribuidas a través de la materia cerebral o puede ser totalmente normal incluso si el paciente tiene un compromiso neurológico severo. El electroencefalograma generalmente muestra una disminución generalizada o focal de la actividad de fondo, picos u ondas agudas, a pesar de ser normal en alrededor del 20% de los pacientes. Presentamos un paciente adulto de sexo masculino, derivado a nuestro centro para ser tratado con hemodiálisis debido a SUH asociado a diarrea, complicado por insuficiencia renal aguda. Luego, durante el transcurso de la terapia de intercambio de plasma, el paciente desarrolló una parálisis ocular aislada del sexto par craneal. Se logró una recuperación renal completa por medio de la terapia de intercambio plasmático; no obstante, la parálisis del nervio motor ocular externo remedió la disminución de la inmunoglobulina G (IgG) mediante el tratamiento de rescate de inmunoadsorción