A Rare Case of Isolated Abducens Nerve Palsy With Contemporaneous Thalamic Infarct.

We report a case of isolated left abducens nerve palsy accompanying a right thalamic infarct. The patient, a 43-year-old Malay male with newly diagnosed hypertension, diabetes mellitus, and dyslipidemia, initially reported binocular diplopia on left lateral gaze persisting for five weeks. Subsequently, he experienced acute left-sided body weakness and slurred speech for over one day. Clinical examination revealed restricted left eye lateral gaze (-3) with no relative afferent pupillary defect. Additionally, decreased power (4/5) was noted in the left upper and lower limbs. Brain magnetic resonance imaging (MRI) revealed restricted diffusion in the right thalamus extending to the right posterior internal capsule, left anterior cingulate gyrus, and left caudate nucleus. The patient was initiated on antiplatelet, antihypertensive, and oral hypoglycemic agents, resulting in symptom improvement. This rare neuroophthalmological finding has not been reported previously.

A Case of Epstein-Barr Virus Encephalitis and Orbital-Face Inflammation.

Epstein-Barr virus (EBV) can cause follicular conjunctivitis, keratitis, oculoglandular syndrome, meningitis, and encephalitis. We report a 54-year-old Hispanic male who presented with right pupil-involved complete ophthalmoplegia, orbital and masticatory muscle inflammation, trigeminal enhancement, and new corneal infiltrate highly suggestive of EBV. Labwork was negative except for positive EBV polymerase chain reaction (PCR) in serum. Magnetic resonance imaging (MRI) of his brain and orbits with contrast showed enhancement of the right ganglion of the trigeminal nerve, oculomotor nerve, all extraocular muscles in the right orbit, and right masticatory and temporalis muscles and a right subacute lacunar infarct. The patient was diagnosed with encephalitis and orbital-face inflammation secondary to EBV infection. The patient improved with systemic steroids.

Cerebral Venous Sinus Thrombosis Complicating Herpes Zoster Ophthalmicus Ophthalmoplegia.

Cerebral venous sinus thrombosis (CVST) is a rare cause of strokes and is most common in younger patients particularly those less than 50 years of age. It is more common in females than in males and is known to be associated with pregnancy, puerperium, oral contraception, congenital and acquired thrombophilia, and malignancy. Less commonly, it has been shown to be associated with infections and more recently has been found to be associated with COVID-19 infection with thrombocytopenia and the COVID-19 vaccine AstraZeneca. Rare cases have been reported in association with varicella zoster virus (VZV) infection (chickenpox) and its reactivated version of herpes zoster virus (HZV) infection (shingles). We report the case of a 68-year-old lady with herpes zoster ophthalmicus ophthalmoplegia who developed cerebral venous thrombosis (CVT).

Painful Eyes in Neurology Clinic: A Guide for Neurologists.

Eye pain is a common complaint among patients presenting to the neurology clinic. It can be related to neurologic diseases, but it can also be a localized eye condition. Such disorders can be misleading, as their benign appearance might mask more grave underlying conditions, potentially leading to misdiagnoses or delayed treatment. Clinicians should be aware of the specific neurologic or systemic disorders (eg, demyelinating diseases or vascular abnormalities) that might first manifest as eye pain. Formal ophthalmic consultation is recommended for patients presenting with eye pain as the predominant complaint especially when red flags for more serious pathology are present.

Internuclear ophthalmoplegia following radial artery cardiac catheterization approach: An unusual presentation.

Internuclear ophthalmoplegia (INO) may happen following percutaneous coronary intervention and angiography. However, no reports of INO during radial artery angioplasty were reported yet. We report a rare case in a 47-year-old man presenting with diplopia after radial artery angioplasty. Although the symptoms were resolved after 60 days, diagnosing this obstacle is necessary to reduce the patient and physician's anxiety.

Recurrent Headache With Diplopia: A Common Presentation of an Uncommon Entity.

Recurrent painful ophthalmoplegic neuropathy (RPON) is a rare headache syndrome, the diagnosis of which can be daunting to those who are not familiar with it. It presents characteristically with recurrent ocular motor weakness and ipsilateral head pain without an underlying etiology and often has unique imaging findings. Even after the successful diagnosis of this entity, there are no published management guidelines. Here, we present the case of a 31-year-old man whom we diagnosed with RPON following two episodes of unilateral headache with ophthalmoplegia over a three-month period and treated successfully with high-dose steroids on both occasions. We highlight the lack of prior migraine history and seeming antecedent viral infection as potential supporting evidence that this condition has a unique pathophysiology different from migraine. We also highlight his dramatic and reproducible response to steroids as additional evidence that steroids are good acute treatment options for this condition. Finally, as our patient lacked the expected cranial nerve imaging abnormalities on head MRI, we suggest that cranial nerve thickening and/or enhancement on MR imaging is not a sine qua non for this diagnosis, contrary to the opinion of some experts.

Uraemic brainstem encephalopathy mimicking ocular myasthenia: a case report.

BACKGROUND: Uraemia causes a generalised encephalopathy as its most common neurological complication. Isolated brainstem uraemic encephalopathy is rare. We report a case of fatigable ptosis and complex ophthalmoplegia in brainstem uraemic encephalopathy. CASE PRESENTATION: A 22-year-old Sri Lankan man with end stage renal failure presented with acute onset diplopia and drooping of eyelids progressively worsening over one week. The patient had not complied with the prescribed renal replacement therapy which was planned to be initiated 5 months previously. On examination, his Glasgow coma scale score was 15/15, He had a fatigable asymmetrical bilateral ptosis. The ice-pack test was negative. There was a complex ophthalmoplegia with bilateral abduction failure and elevation failure of the right eye. The diplopia did not worsen with prolonged stare. The rest of the neurological examination was normal. Serum creatinine on admission was 21.81 mg/dl. The repetitive nerve stimulation did not show a decremental pattern. Magnetic resonance imaging (MRI) of the brain demonstrated diffuse midbrain and pontine oedema with T2 weighted/FLAIR hyperintensities. The patient was haemodialyzed on alternate days and his neurological deficits completely resolved by the end of the second week of dialysis. The follow up brain MRI done two weeks later demonstrated marked improvement of the brainstem oedema with residual T2 weighted/FLAIR hyperintensities in the midbrain. CONCLUSIONS: Uraemia may rarely cause an isolated brainstem encephalopathy mimicking ocular myasthenia, which resolves with correction of the uraemia.

Management of Ptosis in Kearns-Sayre Syndrome: A Case Report and Literature Review.

Kearns-Sayre syndrome (KSS) is a rare mitochondrial disease that affects young adults, due to a deletion of mitochondrial DNA and characterized by the triad: age of onset lower than 20 years, chronic progressive external ophthalmoplegia, and an atypical pigmentary retinopathy. It is also characterized by other endocrine, neurological, and especially cardiac impairment with a very high risk of cardiac complications during surgical procedures under all types of anesthesia. We report a case of KSS revealed by severe bilateral ptosis and confirmed by a muscle biopsy with "ragged red fibers." The ptosis was surgically managed by cautious Frontal suspension under local anesthesia "Frontal nerve block." Through this case, we discuss challenges in the management of KSS patients.

Leiomioma orbitario gigante en un paciente pediátrico: reto diagnóstico y terapéutico / Giant orbital leiomyoma in a pediatric patient: Diagnostic and therapeutic challenge

Este manuscrito presenta un caso de un leiomioma orbitario de larga evolución en un joven de 14años. El tumor era inusualmente grande y causaba una proptosis severa y una afectación importante de la musculatura ocular. El paciente presentaba amaurosis, una oftalmoplejía completa, dolor ocular espontáneo e incapacidad para cerrar los párpados. Debido al tamaño del tumor y a su progresión, se realizó una exenteración orbitaria derecha para eliminar todo el contenido orbitario, incluyendo el tumor y el globo ocular. El procedimiento quirúrgico tenía como objetivo prevenir la recurrencia del tumor y mejorar la calidad de vida del paciente. El análisis histopatológico confirmó el diagnóstico de leiomioma orbitario. Este caso presenta un interés particular por el grado de evolución que ha alcanzado. La extirpación completa del tumor y un seguimiento a largo plazo son necesarios para prevenir la recurrencia y garantizar resultados óptimos para el paciente. Además, este caso refleja las grandes diferencias en el acceso a la sanidad en las diferentes regiones del mundo. (AU)

This manuscript describes an exceptional case of a long-standing orbital leiomyoma in a 14-year-old male. The tumor was unusually large, causing severe proptosis and significant involvement of the ocular muscles. The patient presented with amaurosis, complete ophthalmoplegia, spontaneous eye pain, and the inability to close the eyelids, leading to psychological distress. Due to the tumor's size and progression, a right orbital exenteration was performed to remove all orbital contents, including the tumor and the eyeball. The surgical procedure aimed to prevent tumor recurrence and improve the patient's quality of life. The histopathological analysis confirmed the diagnosis of orbital leiomyoma. This case presents a particular interest due to the degree of evolution it has reached. Complete tumor excision and long-term follow-up are necessary to prevent recurrence and ensure optimal patient outcomes. This report underscores global healthcare disparities and the complexity of managing rare orbital neoplasms in diverse country settings. (AU)

One-and-a-Half Syndrome in a Case of Brainstem Bleed.

One-and-a-half syndrome (OHS) is a horizontal gaze palsy in one direction with internuclear ophthalmoplegia (INO) in the other. The only eye movement possible is the abduction of the contralateral eye with nystagmus. The usual structures affected are the medial longitudinal fasciculus and paramedian pontine reticular formation or the abducens nucleus. Most commonly, the OHS is caused by ischemia and demyelinating lesions. The other causes include infectious, neoplastic, and rarely traumatic. We report a case of a 42-year-old non-compliant hypertensive female who presented with giddiness, projectile vomiting, and right-sided hemiparesis and was found to have OHS on cranial nerve examination in the emergency department (ED). In the ED, the presence of complete horizontal gaze palsy in one direction with INO in the other direction should raise suspicion of a brainstem pathology.

Recurrent Tolosa-Hunt syndrome: a case report.

Introduction and importance: Tolosa-Hunt syndrome is a rare condition with unknown aetiology that manifests clinically as unilateral orbital pain and ophthalmoplegia. It is a diagnosis of exclusion that resolves spontaneously but can recur and respond dramatically to systemic steroids. Case presentation: The authors herein report a case of a 38-year-old male who presented with horizontal diplopia, limited outward movement of the right eye, and blurry vision for two days which was managed with oral Prednisolone. The patient visited 3 months later with progressive ptosis and vertical diplopia with periorbital pain over the right eye. It was eventually diagnosed via magnetic imaging resonance studies and successfully treated for Tolosa-Hunt syndrome with IV methylprednisolone followed by oral prednisolone. Clinical discussion: Hence, the typical clinical presentation of the case with significant response to steroids, exclusion of other conditions from investigation and imaging, and subsequent recurrence of similar symptoms were crucial for making the diagnosis of Tolosa-Hunt syndrome. Conclusion: Tolosa-Hunt syndrome is a syndrome of painful ophthalmoplegia which responds well to steroid therapy but has a tendency to recur. Hence, patients must be adequately informed about the reoccurrence and kept under follow-up.

Mitochondrial myopathies diagnosed in adulthood: clinico-genetic spectrum and long-term outcomes.

Mitochondrial myopathies are frequently recognized in childhood as part of a broader multisystem disorder and often overlooked in adulthood. Herein, we describe the phenotypic and genotypic spectrum and long-term outcomes of mitochondrial myopathies diagnosed in adulthood, focusing on neuromuscular features, electrodiagnostic and myopathological findings and survival. We performed a retrospective chart review of adult patients diagnosed with mitochondrial myopathy at Mayo Clinic (2005-21). We identified 94 patients. Median time from symptom onset to diagnosis was 11 years (interquartile range 4-21 years). Median age at diagnosis was 48 years (32-63 years). Primary genetic defects were identified in mitochondrial DNA in 48 patients (10 with single large deletion, 38 with point mutations) and nuclear DNA in 29. Five patients had multiple mitochondrial DNA deletions or depletion without nuclear DNA variants. Twelve patients had histopathological features of mitochondrial myopathy without molecular diagnosis. The most common phenotypes included multisystem disorder (n = 30); mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (14); limb myopathy (13); chronic progressive external ophthalmoplegia (12); and chronic progressive external ophthalmoplegia-plus (12). Isolated skeletal muscle manifestations occurred in 27%. Sixty-nine per cent had CNS and 21% had cardiac involvement. Mutations most frequently involved MT-TL1 (27) and POLG (17); however, a wide spectrum of established and novel molecular defects, with overlapping phenotypes, was identified. Electrodiagnostic studies identified myopathy (77%), fibrillation potentials (27%) and axonal peripheral neuropathy (42%, most common with nuclear DNA variants). Among 42 muscle biopsies available, median percentage counts were highest for cytochrome C oxidase negative fibres (5.1%) then ragged blue (1.4%) and ragged red fibres (0.5%). Skeletal muscle weakness was mild and slowly progressive (decline in strength summated score of 0.01/year). Median time to gait assistance was 5.5 years from diagnosis and 17 years from symptom onset. Thirty patients died, with median survival of 33.4 years from symptom onset and 10.9 years from diagnosis. Median age at death was 55 years. Cardiac involvement was associated with increased mortality [hazard ratio 2.36 (1.05, 5.29)]. There was no difference in survival based on genotype or phenotype. Despite the wide phenotypic and genotypic spectrum, mitochondrial myopathies in adults share similar features with slowly progressive limb weakness, contrasting with common multiorgan involvement and high mortality.

Bilateral Sequential Abducens Nerve Palsy After Pfizer-BioNTech COVID-19 Vaccine (BNT162b2): A Case Report and Literature Review.

This case report details the occurrence of bilateral sequential abducens nerve palsy in a previously healthy 42-year-old woman two days after receiving her first dose of the Pfizer-BioNTech COVID-19 vaccine (BNT162b2). Despite the widespread global administration of COVID-19 vaccines, instances of abducens palsy following vaccination are limited in the available literature. Considering the temporal association between vaccination and symptom onset, the absence of underlying medical conditions predisposing to such neurological manifestations, normal brain imaging results, the occurrence of other cranial palsies post-vaccination, and analogous occurrences after different vaccinations, we propose a plausible connection between the patient's abducens palsy and the COVID-19 vaccination. Our findings contribute to the growing body of evidence regarding the side effects and safety profile of COVID-19 vaccines. Importantly, the resolution of symptoms with conservative management and the uneventful administration of the second vaccine dose suggest that the observed abducens palsy may be a transient and isolated reaction.

Treatment Response to Acute Total Ophthalmoplegia in Primary Pituitary Lymphoma: A Case Report and Review of the Literature.

Primary pituitary lymphoma (PPL) is an extremely rare localized lymphoma without systemic involvement. The most common clinical presentations of PPL are hypopituitarism, headaches, and ophthalmoplegia. Diagnosing PPL without a biopsy is almost impossible. There is no study that has specifically investigated and reviewed treatment responses to the ophthalmological symptoms of PPL patients. Herein, we present a 66-year-old female patient who had acute-onset total ophthalmoplegia and headache as admission symptoms, which was diagnosed as PPL after subtotal resection. In the present study, we discussed the response of ophthalmological symptoms to treatment with a review of the literature. Only 18 reported cases had postoperative ophthalmological examination, and in 94.4% of these cases, ophthalmoplegia resolves after surgery and chemotherapy. The complete resection rate of the PPL in the literature was found to be as low as 12.3% in this region because of the hard and adhesive nature of the tumor. Our review unveiled that complete recovery of ophthalmoplegia can be achieved even in the late phase of the symptoms. In the present case, ophthalmoplegia resolved completely following subtotal resection and rituximab, high-dose methotrexate, and cytarabine treatment.

Giant orbital leiomyoma in a pediatric patient: diagnostic and therapeutic challenge.

This manuscript describes an exceptional case of a long-standing orbital leiomyoma in a 14-year-old male. The tumor was unusually large, causing severe proptosis and significant involvement of the ocular muscles. The patient presented with amaurosis, complete ophthalmoplegia, spontaneous eye pain, and the inability to close the eyelids, leading to psychological distress. Due to the tumor's size and progression, a right orbital exenteration was performed to remove all orbital contents, including the tumor and the eyeball. The surgical procedure aimed to prevent tumor recurrence and improve the patient's quality of life. The histopathological analysis confirmed the diagnosis of orbital leiomyoma. This case presents a particular interest due to the degree of evolution it has reached. Complete tumor excision and long-term follow-up are necessary to prevent recurrence and ensure optimal patient outcomes. This report underscores global healthcare disparities and the complexity of managing rare orbital neoplasms in diverse country settings.

Recurrent Third Nerve Paresis with Migraine: A Case Report and Review of the Literature.

Ophthalmoplegic migraine (OM), first described by Charcot in 1870, is a disorder characterised by recurrent episodes of migraine associated with ophthalmoplegia. It has been extensively described in children and is rarer in adults. Commonly, the third nerve is affected with pupillary involvement and, more rarely, the fourth or the sixth nerve. OM is now believed to be an inflammatory demyelinating neuropathy. However, in the largest series of OM so far, by Lal et al. it most commonly involved the sixth nerve, started with a crescendo migraine and was accompanied by no enhancement of the cranial nerves. This has led to a rethink about the role of migraine, in the pathogenesis of OM. We describe a 14-year-old boy, with a 10-year history of intermittent headache followed by drooping of right eyelid and diplopia. The current episode started with a migrainous headache, which increased in severity over 3 days, followed by right third nerve paresis with pupillary involvement. Contrast-enhanced magnetic resonance imaging (MRI) of the brain with contrast showed nodular thickening at the root entry zone of the right oculomotor nerve with bright enhancement. The child responded to oral prednisolone, which was tapered over a month. Migraine prophylaxis with propranolol was concurrently added. His repeat MRI brain showed complete disappearance of enhancement of the lesion at 1 year.

Using AI Text-to-Image Generation to Create Novel Illustrations for Medical Education: Current Limitations as Illustrated by Hypothyroidism and Horner Syndrome.

Our research letter investigates the potential, as well as the current limitations, of widely available text-to-image tools in generating images for medical education. We focused on illustrations of important physical signs in the face (for which confidentiality issues in conventional patient photograph use may be a particular concern) that medics should know about, and we used facial images of hypothyroidism and Horner syndrome as examples.

Microsurgical Clip Reconstruction of a True Posterior Communicating Artery Aneurysm.

Approximately 25% of intracranial aneurysms originate at the internal carotid artery and posterior communicating artery (PCoA) junction.1 In contrast to typical PCoA aneurysms, which are usually saccular, a subset known as true PCoA aneurysms arise directly from the PCoA. These represent about 1.3% of all intracranial aneurysms and 6.8% of PCoA aneurysms.1 The first report of a true PCoA aneurysm was in 1979.2Video 1 illustrates the microsurgical clipping of a true PCoA aneurysm in a 27-year-old man with subarachnoid hemorrhage and left-sided ophthalmoplegia. Computed tomography angiography revealed a large true patient consent, Our surgical strategy included 1) an extended pterional approach, 2) early brain relaxation through basal cisterns and third ventricle opening, 3) Sylvian fissure dissection, 4) partial uncus resection, 5) tracing the PCoA to the aneurysm, 6) pilot clipping and thrombectomy, and 7) careful aneurysm dissection and definitive clipping. The patient had an uncomplicated recovery and was discharged on postoperative day 5 with resolved third nerve dysfunction. A literature review from 2022 documented only 47 cases of true PCoA aneurysms, predominantly manifesting with rupture.3 Some studies suggest that these aneurysms may have a higher rupture risk than typical internal carotid artery-PCoA junction aneurysms.4 Microsurgical clipping is a primary treatment, often in cases associated with a fetal posterior cerebral artery variant.5 Ensuring the patency of the PCoA and thalamoperforating arteries is crucial, with careful visualization of the clip's distal ends to avoid impacting nearby neurovascular structures.

Nystagmus and Abducens Nerve Palsy as an Early Presentation of Non-alcoholic Wernicke Encephalopathy.

Wernicke encephalopathy (WE) is a rare but life-threatening syndrome that is commonly associated with chronic alcoholism. It has also been found to be associated with malnutrition, prolonged parenteral nutrition, hemodialysis, hyperemesis gravidarum, gastroplasty, and AIDS. It usually presents as a clinical triad of confusion, ophthalmoplegia, and gait ataxia. Nystagmus is usually the most common and earliest ophthalmologic sign. We report a case of non-alcoholic WE in a patient who had prior bariatric surgery and was treated for malnutrition and sepsis, with nystagmus being the initial presentation. The MRI of the brain was normal. The diagnosis of WE was made clinically and was supported by the patient's symptomatic and clinical recovery following intravenous thiamine treatment. It is essential to highlight that a high level of suspicion is needed to diagnose non-alcoholic WE to allow the commencement of appropriate treatment and reduce morbidity and mortality rates related to this condition.

Internuclear ophthalmoplegia as a presentation of procedural stroke: a case report.

BACKGROUND: Cardiac catheterization and endovascular procedures are extensively used in modern medicine, and procedural stroke is one of the major complications that the catheterization laboratory team may face in their everyday work. Recognizing the signs and symptoms of procedural stroke is crucial to ensuring appropriate management. We herein report a case of internuclear ophthalmoplegia that caused blurred vision, diplopia, and dizziness on lateral gaze as an unusual presentation of procedural stroke. CASE PRESENTATION: A 60-year-old Thai woman underwent right partial colectomy and was diagnosed with stage IV diffuse large B-cell lymphoma. Pre-chemotherapy echocardiography revealed mild left ventricular systolic dysfunction, and she therefore underwent diagnostic catheterization. Coronary angiography revealed normal coronary arteries, leading to a diagnosis of non-ischemic cardiomyopathy. After the procedure, she immediately developed dizziness and diplopia. During the right lateral gaze, she exhibited impaired adduction of the left eye and horizontal nystagmus of the right eye. A diagnosis of left internuclear ophthalmoplegia was made. Magnetic resonance imaging revealed a tiny area exhibiting characteristics of an acute infarct in the left paramedian midbrain, including the left medial longitudinal fasciculus, which explained the clinical picture. Another region of restricted diffusion indicating an acute infarct was detected in the right inferior cerebellar hemisphere. Magnetic resonance angiography revealed no significant cerebral artery disease. The patient achieved full neurological recovery 6 weeks after symptom onset. CONCLUSION: This report describes an uncommon presentation of procedural stroke that is likely to be misdiagnosed, especially by medical staff unfamiliar with internuclear ophthalmoplegia. Despite the good prognosis of internuclear ophthalmoplegia, appropriate stroke care is crucial in patients with procedural stroke because of the risk of multiple brain infarcts.