Do infants with isolated congenital sixth nerve palsy require comprehensive work-up? A retrospective cohort and review of the literature.

PURPOSE: The purpose of this study is to describe a case series of infants with isolated congenital sixth nerve palsy (ICSNP) and suggest a management algorithm based on our experience and a review of the literature. METHODS: A retrospective cohort design was used. The clinical database of a single tertiary medical center was reviewed to identify all patients diagnosed with ICSNP from January 2020 to November 2022. Data were collected as follows: demographic parameters, age at initial presentation, presenting symptoms and signs, findings on ophthalmic and neurologic examinations, findings on follow-up, and outcome. RESULTS: Six patients were included. All were born at term. The average gestational weight was 3675.7 ± 262.7 g. Three mothers had gestational diabetes. Five deliveries necessitated labor induction either by oxytocin (n = 4) or by membrane stripping followed by oxytocin (n = 1). One had also gone a forceps assisted delivery. Symptoms were noticed in all newborns by their parents within the first week of life. Ophthalmological and neurological examinations were otherwise unremarkable apart of one patient with a head turn to the side of the involved eye. Four patients underwent brain imaging that were unremarkable. All abduction deficits resolved by 1 to 3 months of age. Follow up examinations were unremarkable (mean follow up 14.3 ± 5.0 months, range 4-23). CONCLUSIONS: This case series, together with previous reports, support ICSNP's benign nature. We suggest an initial basic work-up that solely includes ophthalmological and neurological examinations which will be elaborated in case of any additional pathologic findings or if ICSNP does not fully resolve by 3 months.

A Rare Case of Abducens Nerve Palsy Caused by Primary Squamous Cell Carcinoma of the Middle Ear.

Abducens nerve palsy is the most common ocular motor nerve palsy, and its possible aetiologies are numerous and diverse. Primary malignancy rarely occurs in the middle ear, with most cases associated with long-standing ear discharge and peak age of presentation in the sixties. We report a rare case of a 64-year-old male who presented with right abducens nerve palsy, which led to the diagnosis of primary squamous cell carcinoma of the right middle ear, and to our knowledge, this has not been reported previously in English literature.

Etiologic distribution of isolated abducens nerve palsy: Analysis of 807 patients and literature review.

BACKGROUND AND PURPOSE: The etiologies of abducens nerve palsy have shown a large variability among studies. This study aimed to establish the clinical features and underlying etiologies of isolated abducens nerve palsy by recruiting patients from all departments in a referral-based university hospital. METHODS: We reviewed the medical records of 807 patients with a confirmed diagnosis of isolated abducens nerve palsy at all departments of Seoul National University Bundang Hospital, Seongnam, Republic of Korea, from 2003 to 2020. We also compared the proportion of etiology with that of the patients pooled from the previous studies. RESULTS: The most common etiology was microvascular (n = 296, 36.7%), followed by idiopathic (n = 143, 17.7%), neoplastic (n = 115, 14.3%), vascular anomalies (n = 82, 10.2%), inflammatory (n = 76, 9.4%), and traumatic (n = 35, 4.3%). Patients were mostly managed by ophthalmologists (n = 576, 71.4%), followed by neurologists (n = 479, 59.4%), emergency physicians (n = 278, 34.4%), neurosurgeons (n = 191, 23.7%), and others (n = 72, 8.9%). The proportion of etiology significantly differed according to the age and sex of the patients and the specialties involved in the management (p < 0.001). Compared to the pooled data from the previous reports, the current study showed a higher prevalence of microvascular cause but a lower occurrence of traumatic and neoplastic causes. CONCLUSIONS: The results of previous studies on etiologic distribution of isolated abducens nerve palsy should be interpreted with consideration of the demographic features of patients recruited and the specialties involved.

Bilateral sixth nerve palsy with subdural hematoma: a unique presentation of B12 deficiency.

Vitamin B12 is inextricably associated with the development and maintenance of neuronal functions. It is classically associated with subacute combined degeneration and peripheral neuropathy; however, cranial neuropathy is uncommon. We observed the rarest neurological manifestation of B12 deficiency. A 12 months infant had history of lethargy, irritability, anorexia, paleness, vomiting, and neurodevelopmental delay for 2 months. He also developed inattention and altered sleep pattern. His mother noticed bilateral inward rotation of both eyes. On examination, the infant had bilateral lateral rectus palsy. The infant was found to have anemia (7.7g/dL) and severe B12 deficiency (74pg/mL). On MRI, there was cerebral atrophy, subdural hematoma (SDH) and wide cisternal spaces and sulci. On supplementation with cobalamin, he improved clinically though mild restriction of lateral gaze on the left side persists. Follow up MRI showed significant improvement in cerebral atrophy with resolution of SDH. To date, such clinical presentation of B12 deficiency has never been reported. The authors suggest B12 supplementation for at risk population esp at antenatal stage and lactating mothers in national programs. The treatment of this condition should be initiated early to prevent long term sequelae.

Vertebrobasilar dolichoectasia and other arterial abnormalities leading to abducens nerve palsy.

INTRODUCTION: Dolichoectatic vessels can cause cranial nerve dysfunction by either direct compression or ischemia. Abducens nerve palsy due to neurovascular compression by elongated, enlarged, tortuous or dilated arteries is an uncommon but important cause. AIM: To highlight neurovascular compression as a cause of abducens nerve palsy and discuss various diagnostic techniques. METHODS: Manuscripts were identified using the National Institutes of Health PubMed literature search system. Search terms included abducens nerve palsy, neurovascular compression, dolichoectasia and arterial compression. Inclusion criteria required that the articles were written in English. RESULTS: The literature search identified 21 case reports where abducens nerve palsy was due to vascular compression. Out of these 18 patients were male and the mean age was 54 years. Eight patients had unilateral right abducens nerve involvement; eleven patients had unilateral left nerve involvement and two patients had bilateral involvement. The arteries causing the compression were basilar, vertebral and anterior inferior cerebellar arteries. A compressed abducens nerve is not usually clearly detected on CT (Computed Tomography) or MRI (Magnetic Resonance Imaging). MRA (Magnetic Resonance Angiography), Heavy T2- WI (weighted imaging), CISS (constructive interference in steady state) and FIESTA (Fast Imaging Employing Steady-state Acquisition) are essential to demonstrate vascular compression of the abducens nerve. The various treatment options included controlling hypertension, glasses with prisms, muscle resection and microvascular decompression.

A Case of Mycoplasma Infection with an Atypical Presentation of Abducens Nerve Palsy, Erythema Multiforme and Polyarthritis without Respiratory Manifestations.

Mycoplasma pneumoniae is a self-propagating microorganism that commonly causes respiratory tract infections. It can also cause a variety of extrapulmonary symptoms with or independently of respiratory symptoms, such as skin lesions, arthralgia, myalgia, hemolysis, cardiac lesions, gastrointestinal symptoms, and central nervous system lesions, which are rare manifestations reported in approximately 0.1% of cases. In this study, we present a unique case of Mycoplasma-related abducens nerve palsy, polyarthritis, and erythema multiforme without respiratory disease. The patient was a 69-year-old woman who presented to our hospital with a skin rash, fever, arthralgia, and diplopia without respiratory symptoms. Brain magnetic resonance imaging showed optic neuritis on the right side, suggesting the diplopia was caused by right abducens nerve palsy. However, the etiologies of abducens nerve palsy were not revealed by the physical examination, blood biochemistry tests, or bacteriological examinations, including the cerebrospinal fluid examination obtained at admission. Mycoplasma infection was suspected from erythema multiforme revealed by a skin biopsy and polyarthralgia, and it was finally diagnosed according to elevated Mycoplasma particle agglutination (PA) antibodies in paired serum. Though minocycline did not improve her diplopia, the daily administration of 30 mg of prednisolone gradually improved her symptoms, and the Mycoplasma PA antibody titer, which was regularly measured in the clinical course, also decreased, suggesting a relationship between Mycoplasma infection and abducens nerve palsy. This is the first case of isolated abducens nerve palsy, which was reported as the only central neurological symptom in an adult patient with Mycoplasma infection. The mechanism or pathogenesis of CNS manifestations caused by Mycoplasma pneumoniae remains to be elucidated, and further investigation is needed. Hence, Mycoplasma infection is a common disease. Clinicians should be aware of the diverse manifestations, including abducens nerve palsy, of Mycoplasma infection and should consider Mycoplasma infection even in the absence of typical respiratory symptoms.

Simultaneous Unilateral Abducens Nerve Palsy and Contralateral Anterior Ischaemic Optic Neuropathy as the Presenting Signs of Giant Cell Arteritis.

Ischaemic optic neuropathy is the most common, feared, and recognised ocular manifestation of giant cell arteritis (GCA), while extraocular muscle palsy rarely occurs in the disease. Overlooking the diagnosis of GCA in aged patients with acquired diplopia and strabismus is not only sight- but also life-threatening. Here, we present, for the first time, a case of unilateral abducens nerve palsy and contralateral anterior ischaemic optic neuropathy as the presenting signs of GCA in a 98-year-old woman. Prompt diagnosis and treatment prevented further visual loss and systemic complications and allowed for rapid resolution of the abducens nerve palsy. We also aim to discuss the possible pathophysiological mechanisms of diplopia in GCA and to emphasise that acquired cranial nerve palsy must raise suspicion of this severe disease in elderly patients, particularly in association with ischaemic optic neuropathy.

Persistent Trigeminal Artery Causing an Abducens Nerve Palsy: A Case Report.

We present a case of a 50-year-old female who was diagnosed with an isolated right abducens nerve palsy and was found to have a persistent trigeminal artery (PTA). The trigeminal artery is the most common persistent embryological carotid-vertebrobasilar anastomosis. A PTA can be picked up as an incidental finding on magnetic resonance imaging (MRI) or angiography. It has been reported that a PTA can be found in 0.1 to 0.6% of all cerebral angiograms. PTA has been linked to several rare abnormalities such as vascular aneurysms and cranial nerve compression. Our patient presented with diplopia and was found to have a paresis of the right lateral rectus muscle consistent with a right abducens nerve palsy. MRI found a right-sided PTA indenting the ventral surface of the pons. This case investigates and highlights that neurovascular compression from a PTA can cause an isolated abducens nerve palsy. Further research is required to investigate if surgical intervention for non-aneurysmal PTA would be beneficial for patients.

Traumatic retroclival epidural hematoma associated with reversible ipsilateral internal carotid artery stenosis.

INTRODUCTION: Traumatic retroclival epidural hematoma is rare. It is more common in pediatrics than in adults. Although it has been known that these cases are frequently associated with abducens nerve palsy, internal carotid artery stenosis is rarely found with those hematomas. CASE REPORT: An 8-year-old girl was transferred to our hospital following a traffic accident. She had clear consciousness with right abducens nerve palsy. Computed tomography revealed the left side of both retroclival hematoma without clival fracture and subarachnoid hemorrhage at the Sylvian fissure. She also had a fracture of left femoral neck and ipsilateral lung contusion. Magnetic resonance imaging revealed a retroclival hematoma located in the epidural space and severe stenosis of left internal carotid artery (ICA) from the cavernous to supraclinoid portion without evidence of brain contusion. She was managed conservatively, and her right abducens nerve palsy recovered completely without deterioration of other neurological findings. Neuroradiological findings suggested this ICA stenosis as traumatic dissection. She was discharged home 2 months after the traffic accident. CONCLUSION: Retroclival epidural hematoma without clival fracture associated with ipsilateral ICA stenosis is extremely rare. Although the exact mechanism of the ICA stenosis remains unclear, cerebral vascular events should be considered in the cases with traumatic retroclival hematoma.

Initial Presentation of Granulomatosis with Polyangiitis as Progressive Skull Base Osteomyelitis.

A healthy man in his 30s presented with a 2-week history of severe bitemporal pain and pressure. He was initially treated for presumed acute rhinosinusitis, but his symptoms continued to worsen and underwent endoscopic sinus surgery at an outside community facility. He developed left abducens nerve palsy postoperatively, and magnetic resonance imaging (MRI) demonstrated evidence of extensive skull base osteomyelitis. He was initiated on intravenous (IV) broad-spectrum antibiotics but was subsequently found to have prostatic and submandibular sterile fluid collections. The patient subsequently developed new right abducens and left vagal nerve palsies and underwent revision endoscopic sinus surgery. Pathology revealed extensive inflammation, necrotizing granulomas, and evidence of small and medium vessel vasculitis. Extensive laboratory workup was negative, except for anti-PR-3 antibody positivity. Given the characteristic findings on pathology and laboratory findings, the patient was diagnosed with granulomatosis with polyangiitis (GPA). High-dose glucocorticoid therapy as well as rituximab infusion were promptly initiated. He had marked improvement in his symptoms and resolution of his right CN VI palsy but left-sided CN VI and CN X palsies persisted. This patient presented without the typical rhinologic manifestations of GPA, and rather presented with progressive sinusitis, skull base osteomyelitis with associated cranial neuropathies, and aseptic systemic abscesses. Prompt diagnosis of GPA is particularly important in those with otorhinolaryngological manifestations, as early initial immunosuppressive therapy has been linked to lower relapse and mortality rates. Vigilance and early differentiation between GPA and other forms of sinusitis is of critical importance, particularly when symptoms are refractory to standard rhinosinusitis therapies.

Scrub Typhus Presenting as Unilateral Abducens Nerve Palsy.

Scrub typhus, an acute febrile infectious disease prevalent in the "Tsutsugamushi Triangle", is a mite-born rickettsial zoonosis, caused by Orientia tsutsugamushi. Although the clinical presentation is protean, it rarely causes abducens nerve palsy. We report a 14-year-old previously healthy Indian girl who presented with a recent onset right abducens nerve palsy and headache, but without fever and without the classic dermatological manifestation ("eschar") of the disease. After exclusion of common infectious, autoimmune, and neoplastic causes, she was finally diagnosed with scrub typhus associated with an abducens nerve palsy, which responded to doxycycline therapy.

Cranial nerve palsies due to incidental durotomy in lumbar Spine surgery: a case report.

We present a 65-year old man who underwent a partial laminectomy at L4. During surgery an incidental durotomy (ID) appeared. Postoperatively he developed cranial nerve palsies. Subsequent to surgical closure of the ID, symptoms completely resolved within three months.

Vertical rectus muscle union combined with lateral rectus plication for complete abducens nerve palsy.

PURPOSE: The aim of this study was to report our postoperative results concerning the vertical rectus (VR) muscle union combined with lateral rectus (LR) plication for the treatment of large-angle esotropia (ET) in complete abducens nerve palsy. METHODS: Medical records from 36 patients who had undergone the VR union procedure for ET treatment due to sixth-nerve palsy between July 2014 and July 2018 at Beyoglu Eye Research and Training Hospital were reviewed. One week before surgery, all patients underwent a 4-IU botulinum toxin A (btx) injection into the ipsilateral medial rectus (MR). All patients then underwent a VR muscle union procedure. A non-absorbable suture was inserted through the lateral muscular margin of each VR muscle at approximately 1/5 the width from the edge at 10 mm distance from the VR insertion. Both sutures were then tied to each other above the LR. Plication of the LR muscle using a non-absorbable suture was performed in all cases. RESULTS: The study population consisted of 14 (38.9%) females and 22 (61.1%) males. The mean age was 36.31 ± 19.16 years. The mean preoperative deviation angle in primary gaze into distance was 47.77 ± 18.48 prism diopter (PD). The mean deviation angle 1 year after surgery was - 1.0 ± 6.62 PD. Abduction improved from - 4.27 ± 0.46 to - 1.88 ± 0.96. CONCLUSION: The VR muscle union in combination with LR plication appears to be an effective treatment procedure for complete abducens nerve palsy patients.

A child with Gradenigo syndrome presenting with meningism: a case report.

BACKGROUND: The symptoms of meningitis which include fever, headache, photophobia and irritability along with abducens nerve palsy pose a diagnostic dilemma requiring urgent attention. Here we report how such a dilemma was methodically and sequentially resolved using anatomical knowledge supported by neuroimaging and the eventual diagnosis of Gradenigo syndrome was made. CASE PRESENTATION: A 6-year-old previously healthy boy from Sri Lanka presented with high grade fever, headache, photophobia and left eye pain for 10 days and diplopia for 2 days duration. Neurological examination was unremarkable except for left sided abducens nerve palsy. He had high inflammatory markers and white blood cell count. A tentative differential diagnosis of acute bacterial meningitis complicated by cerebral oedema, acute hydrocephalus or cerebral abscess was made. However, non-contrast CT brain, cerebrospinal fluid analysis and electroencephalogram were normal leading to a diagnostic dilemma. MRI brain with contrast performed 3 days later due to limited resources revealed left mastoiditis extending to petrous temporal bone confirming Gradenigo syndrome. CONCLUSION: This case report highlights the importance of a thorough physical examination in children presenting with unrelated neurological symptoms and signs. Unilateral abducens nerve palsy raises the suspicion of increased intracranial pressure and neuroimaging is vital in diagnostic uncertainties. Gradenigo syndrome emphasises the importance of incorporating anatomical knowledge into clinical practice.

Unilateral Abducens Nerve Palsy with Ipsilateral Horner's Syndrome as an Initial Manifestation of Recurrent Nasopharyngeal Carcinoma.

A 62-year-old man was admitted to our clinic with a two-week history of double vision. Neuro-ophthalmological examination showed a left abducens nerve palsy with an ipsilateral Horner's syndrome. Neuro-imaging identified recurrent nasopharyngeal carcinoma explaining the clinical findings. Co-existence of unilateral abducens nerve palsy and ipsilateral Horner's syndrome is very rare and localises to the posterior cavernous sinus. Therefore, clinicians should be aware of this rare co-existence and perform appropriate neuro-imaging to clarify the underlying aetiology.

An Incidental Finding of Ecchordosis Physaliphora in a Case of Abducens Nerve Palsy: Case Report.

Ecchordosis physaliphora (EP) is a rare non-malignant mass that originates from remains of the notochord and is typically asymptomatic. A 42-year-old man presented with sudden onset of painless horizontal diplopia and his neurological exam showed sixth cranial nerve palsy. Magnetic resonance imaging (MRI) identified a non-enhanced retroclival mass (EP) with increased signal intensity on T2 and decreased signal intensity on T1-weighted sequences. He was treated with methylprednisolone, completely recovered in four weeks and has remained symptom free. Conservative management should be attempted before surgery in all cases since symptoms can resolve spontaneously and EP could be an incidental finding.

Gradenigo's syndrome: A common infection with uncommon consequences.

Acute otitis media is a common diagnosis encountered by emergency medicine providers. With appropriate antibiotic treatment, patients with otitis media, in general, have minimal long-term sequela from their underlying infection (Limb et al., 2017 [1]). However, untreated cases can develop life-threatening complications that require prompt intervention. We report a case of an 8-year-old that developed Gradenigo's syndrome, a condition characterized by the triad of otitis media, facial pain in the distribution of the trigeminal nerve, and abducens nerve palsy (Yeung and Lustig, 2016; Janjua et al., 2016; Kantas et al., 2010; Motamed and Kalan, n.d.; Vita Fooken Jensen et al., 2016 [2-6]). Signs and symptoms are often subtle, so a high-level of suspicion is required in order not to miss this potentially fatal process.

One-Sided Headache Is a Symptom Suggesting Aneurysmal Lesion in Patients with Isolated Abducens Nerve Palsy.

An isolated unilateral abducens nerve palsy or headache alone usually yields negative findings on neuroimages. The authors report an individual with right abducens nerve palsy who developed a sudden, persistent headache on the right side. Magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) revealed a giant aneurysm of the cavernous carotid artery that was located along the course of the right abducens nerve. The findings in this case of isolated abducens nerve palsy suggest that headaches should be considered as important signs for intracranial aneurysmal lesions.

Bilateral abducens nerve palsy in an infant case of fulminant acute disseminated encephalomyelitis: a case report.

BACKGROUND: Sixth (abducens) nerve palsy (ANP) is far less frequent in children and has not been reported as a sign of acute disseminated encephalomyelitis (ADEM). We present an infant case of ADEM with bilateral abducens nerve palsy (BANP). CASE PRESENTATION: We report one case of BANP in a 15-month-old boy of fulminant ADEM. The patient underwent physical examinations and brain MRI scan three times during about six months follow-up. The patient had BANP and developmental regression when he regained consciousness from a coma, and the signs had persisted for 6-months. CONCLUSIONS: BANP can be a symptom of ADEM.

Isolated traumatic retroclival hematoma: case report and review of literature.

BACKGROUND: Retroclival hematomas are a rare entity. The pathology can be categorized into epidural hematoma or subdural hematoma based on the anatomy of the tectorial membrane. Frequently, the etiology is related to accidental trauma, though other mechanisms have been observed, including coagulopathy, non-accidental trauma, and pituitary apoplexy. There have been only 2 prior cases where both epidural and subdural hematoma co-present. CASE PRESENTATION: An 8-year-old male was involved in a high-speed motor vehicle accident. He presented with a Glasgow Coma Score (GCS) of 14 with bilateral abducens nerve palsies. Computed tomography (CT) revealed a hemorrhage along the dorsum sella, clivus, and dens. Magnetic resonance imaging (MRI) demonstrated the retroclival hematoma in both the subdural and epidural space. At discharge, 19 days after the accident, the abducens nerve palsies had resolved without medical or operative intervention. CONCLUSION: Retroclival hematoma may present after trauma. Although most cases exhibit a benign clinical course with conservative management, significant and profound morbidity and mortality have been reported. Prompt diagnosis with close observation is prudent. Surgical management is indicated in the presence of hydrocephalus, symptomatic brainstem compression, and occipito-cervical instability.