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Case Presentation: A 52-year-old female presented to the emergency department with four days of right periorbital pain, ipsilateral temporal headache, diplopia, and photophobia. Physical examination of the right eye revealed painful ophthalmoplegia, cranial nerves III and VI paresis, increased intraocular pressure, and mild proptosis. Magnetic resonance venogram and magnetic resonance imaging orbits with contrast demonstrated an abnormal signal surrounding the right cavernous sinus/petrous apex. Tolosa-Hunt syndrome (THS) was diagnosed. Per neurology recommendations, the patient was placed on a steroid regimen over the course of three weeks. She was discharged on hospital day nine following resolution of symptoms. She had no recurrence of symptoms or residual deficits noted at her two-week follow-up appointment.Discussion: With an estimated annual incidence of one case per million, THS is a sinister etiology of unilateral headache, painful ophthalmoplegia, and oculomotor palsy. Tolosa-Hunt syndrome is caused by granulomatous inflammation in the cavernous sinus and is highly responsive to corticosteroids. Magnetic resonance imaging studies of the cavernous sinus and orbital apex are highly sensitive for THS and characteristically show enlargement and focal-enhancing masses within the affected cavernous sinus.

This is a case report describing an unusual presentation of acute painful diplopia that led to the diagnosis of VEXAS syndrome. VEXAS (Vacuoles, E1 enzyme, X-linked, Autoinflammatory, Somatic) syndrome is an adult-onset monogenic auto-inflammatory disease due to somatic UBA1 gene mutation in haematopoietic progenitor cells. Our patient was a 67-year-old diabetic male who presented with painful eye movements associated with diplopia, left periorbital pain and swelling. Imaging revealed an inflammatory process involving multiple intra- and extra-orbital structures. The patient improved initially with a short course of intravenous steroids. However, two months later he re-presented with right facial swelling. Bone marrow biopsy demonstrated UBA1 gene mutation supporting the diagnosis of VEXAS syndrome. This case highlights a unique ocular presentation of VEXAS. [ABSTRACT FROM AUTHOR]

Vision loss secondary to aesthetic filler treatment is a rare but disastrous complication. The aim of this review was to update the published cases of blindness after filler injection that have occurred since our group published reviews of 98 cases in 2015 and an additional 48 cases in 2019. A literature review was performed to identify all cases of visual complications caused by filler injection published between September 2018 and March 2023. The cases were analyzed independently and in combination with previously reviewed cases. Analyses were based on the number of cases with data available. A total of 365 new cases of partial or complete vision loss after filler injection were identified. The sites that were highest risk were the nose (40.6%), forehead (27.7%), and glabella (19.0%). The filler injected was hyaluronic acid in 79.6% of cases. The most common associated signs were ptosis (56.2%), ophthalmoplegia (44.1%), pain (31.2%), and skin changes (73.2%). Strokelike features were seen in 19.2% of cases. Of the cases reporting visual outcomes (318), 6.0% experienced complete vision recovery, 25.8% had partial improvement in visual acuity, and 68.2% had no vision recovery. Partially preserved visual acuity at onset was a significant predictor of visual improvement (P <.001). The 3 most common treatments were subcutaneous hyaluronidase at or near the filler site (70.1%), systemic steroids (57.3%), and intraarterial thrombolytic therapy (56.0%). No treatments were significantly associated with visual improvement (P >.05). Although blindness and stroke from fillers is a rare complication, practitioners who inject filler should have a thorough knowledge of prevention and management strategies. Level of Evidence: 3 [ABSTRACT FROM AUTHOR]

Copyright of Revista Foco (Interdisciplinary Studies Journal) is the property of Revista Foco and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

Hepatocellular carcinoma (HCC) is the most common primary malignancy of the liver, among which around 18–64% metastasize, most frequently to lungs, regional lymph nodes and adrenal glands. Metastasis of HCC to the central nervous system represents a rare yet clinically significant phenomenon, often presenting diagnostic and therapeutic challenges. In this case report, we document a 35-year-old male who presented with a secondary headache and right ophthalmoplegia attributable to metastatic deposits secondary to HCC infiltrating the cavernous sinus and superior orbital fissure. Despite therapeutic interventions including local palliative radiotherapy and sorafenib, the patient succumbed to acute liver failure after 9 months. This case highlights the aggressive potential of HCC to involve the central nervous system and the importance of heightened clinical suspicion for early diagnosis and intervention in such rare but clinically impactful scenarios. [ABSTRACT FROM AUTHOR]

Abstract Background Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is an autosomal recessive disease associated with alterations in mitochondrial DNA (mtDNA). The typical age of onset of MNGIE is between the first and second decade of life. Diagnosis requires the presence of several key clinical features: sensorimotor neuropathy, external ophthalmoplegia, ocular ptosis, leukoencephalopathy, and gastrointestinal (GI) dysmotility. Unfortunately, MNGIE diagnosis is very challenging, and patients often undergo multiple diagnostic and surgical operations that are unnecessary. Case presentation This case is of a 51-year-old male presenting with a 2-year history of limb weakness, GI problems and cachexia. There was also a 1-year history of progressive ptosis and ophthalmoplegia. The patient’s uncle and brother had both died from GI-related issues prior to the age of 40. On physical examination, ocular motility was impaired in all directions and there was atrophy and reduction in power in both lower and upper extremities. FLAIR and T2-weighted sequences of brain MRI demonstrated diffuse cerebral white matter hyperintensity (leukoencephalopathy). On discharge, the patient was referred for genetic consultation for bone marrow transplantation and had regular follow-up with a gastroenterology specialist. Conclusion In patients presenting with chronic progressive ophthalmoplegia, severe gastrointestinal complications, sensorimotor neuropathy and white matter lesions on MRI, it is important to consider investigating for MNGIE.

Multiple oculomotor palsies are a rare condition. They typically present with ptosis, diplopia and preserved pupillary function. We present the case of a 65-year-old woman with a history of diabetes and arterial hypertension, initially presenting with headache and later developing diplopia, progressive left palpebral ptosis and fluctuating pupillary response. She was diagnosed with unilateral paralysis of cranial nerves III and IV. Extension studies were performed and, ultimately, the Neurology Department diagnosed microvascular ischemia due to diabetes. Metabolic and blood pressure therapy was adjusted; two months later, complete recovery was achieved. Multiple oculomotor palsy is a diagnostic challenge in the general medical practice. Fluctuating pupillary response, or anisocoria, may be caused by neoplasms or aneurysms in the posterior communicating artery, with potentially devastating consequences. Therefore, judicious clinical evaluation is crucial for etiological diagnosis and appropriate treatment.

We report a case of a 6-year-old boy with autism spectrum disorder presenting with new-onset squint and 'ptosis' following a recent infection. Clinical examination revealed ataxia and areflexia alongside a dilated pupil poorly reactive to light. Subsequently, his eye movements deteriorated to near-complete ophthalmoplegia at 1-week review. Further investigations inclusive of a magnetic resonance imaging (MRI) brain scan, a computed tomography (CT) venogram and a lumbar puncture were conducted to consider and rule out differential diagnoses. Cerebrospinal fluid analysis revealed an albuminocytologic dissociation. The clinical triad of progressive ophthalmoplegia, areflexia and areflexia alongside albuminocytologic dissociation led to the diagnosis of Miller Fisher syndrome. The patient was commenced on intravenous immunoglobulin and his symptoms showed significant improvement. We use this interesting case to provide context for key learning points about diagnosing Miller Fisher syndrome in children. [ABSTRACT FROM AUTHOR]

Background: Mitochondrial neurogastrointestinal encephalopathy (MNGIE) is an autosomal recessive disease associated with alterations in mitochondrial DNA (mtDNA). The typical age of onset of MNGIE is between the first and second decade of life. Diagnosis requires the presence of several key clinical features: sensorimotor neuropathy, external ophthalmoplegia, ocular ptosis, leukoencephalopathy, and gastrointestinal (GI) dysmotility. Unfortunately, MNGIE diagnosis is very challenging, and patients often undergo multiple diagnostic and surgical operations that are unnecessary. Case presentation: This case is of a 51-year-old male presenting with a 2-year history of limb weakness, GI problems and cachexia. There was also a 1-year history of progressive ptosis and ophthalmoplegia. The patient's uncle and brother had both died from GI-related issues prior to the age of 40. On physical examination, ocular motility was impaired in all directions and there was atrophy and reduction in power in both lower and upper extremities. FLAIR and T2-weighted sequences of brain MRI demonstrated diffuse cerebral white matter hyperintensity (leukoencephalopathy). On discharge, the patient was referred for genetic consultation for bone marrow transplantation and had regular follow-up with a gastroenterology specialist. Conclusion: In patients presenting with chronic progressive ophthalmoplegia, severe gastrointestinal complications, sensorimotor neuropathy and white matter lesions on MRI, it is important to consider investigating for MNGIE. [ABSTRACT FROM AUTHOR]

Bickerstaff brainstem encephalitis (BBE) is a variant of Guillain-Barré syndrome (GBS) characterized by inflammation of the brainstem and cranial nerves. We describe a 60-year-old man presenting with acute confusion, bilateral progressive facial drooping, difficulty swallowing, aphasia, and confusion over two days. Examination showed bilateral facial paralysis, impaired eye movements, ptosis, dysarthria, diminished gag reflex and fatigue. MRI brain was unremarkable. Intravenous immunoglobulin (IVIG) was promptly started with noticeable improvement in facial strength and swallowing by day three. At discharge two weeks later, he had near complete resolution. BBE likely involves autoantibodies triggered by infections causing nerve inflammation. Diagnosis is clinical based on bilateral facial and bulbar weakness. Early immunotherapy provides best recovery prospects. Further research can uncover immunological mechanisms to guide treatments. Optimized protocols tailored to BBE compared with GBS are needed. Distinguishing BBE facilitates prompt therapy and favourable outcomes from this rare syndrome. [ABSTRACT FROM AUTHOR]

The thalamus is a deep structure located in the diencephalon, whose arterial blood supply is mainly from four branches of posterior cerebral artery. The artery of percheron (AOP) is an infrequent variation of thalamic perfusion, occlusion of which presents with a heterogenous, atypical list of symptoms without focal signs. This is in contrast to the typical, easily recognizable focal neurology of other ischemic infarcts. Therefore, AOP infarctions may be misdiagnosed, delayed in diagnosis or missed altogether. It is a rare, but vital area of neurology that needs to be studied by clinicians to facilitate an overall care for patients. We report the case of a 59-year-old lady who presented to us with bilateral complete ptosis with bilateral vertical gaze palsy and nystagmus, whose initial NCCT brain was normal. Subsequent MRI showed bilateral thalamic and midbrain ischemic infarctions in the artery of Percheron distribution. Our case highlights the importance of high degree of suspicion, early diagnosis and role of MRI in the diagnosis when initial CT is normal.

Abstract 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.

Objective: Orbital apex syndrome (OAS) is a rare condition with multiple cranial nerve involvement caused by varied etiologies. It is not only a threat to the patient’s vision but also life-threatening due to the intracranial spread of infection, if not diagnosed early and treated accurately. To study the outcome of endoscopic sinus surgery (ESS) for OAS secondary to sinusitis concerning resolution of ptosis, improvement of ophthalmoplegia, visual prognosis, intracranial spread of infection, and mortality. Methods: A retrospective review of patients with OAS secondary to sinusitis who underwent ESS from 2011 to 2021 was tabulated and analyzed. Results: Twenty-seven patients (mean age: 55.11+/-16 years; male 62%) were included in this study. At presentation, blurring of vision (81%), headache (66%), diplopia (63%) ptosis (63%) were the most common symptoms, and ophthalmoplegia (100%) was the most common sign. Five patients had no perception of light and the rest had various degrees of vision impairment. The most common etiopathology of sinusitis was fungal sinusitis (12 mucormycosis and four aspergillus). The final visual prognosis at three months follow-up post-ESS showed vision stabilization (no improvement or worsening) in 13 (48%) patients, improvement in seven (26%) patients, and vision deterioration in two (7%) patients. There was a significant improvement in ptosis (70%) and ophthalmoplegia (85%). There was no intracranial spread of infection or recurrence with a mortality rate of 3.7% (one patient). Conclusion: ESS coupled with appropriate antimicrobials effectively treats OAS secondary to sinusitis with decreased morbidity and mortality.

Fat-soluble vitamins, including vitamins A, D, E and K, are molecules with no energy value that are essential to the body's functioning and to life. Their intake is almost exclusively exogenous, i.e. dietary. Deficiency or excess of fat-soluble vitamins is responsible for a variety of more or less specific clinical symptoms. The fact that the functions performed by these vitamins are both ubiquitous and vital explains the wide variety of clinical manifestations and their potential seriousness. Some syndromes are typical of a deficiency in fat-soluble vitamins, such as: the combination of ophthalmological and immunity impairment in the case of vitamin A; a haemorrhagic syndrome and osteopenia in the case of vitamin E; and osteomalacia, muscular weakness, even falls, and rickets in the case of vitamin D. Diagnosis of a deficiency in one of the fat-soluble vitamins is based on blood tests, which are not always essential for routine use. In this case, a therapeutic test may be suggested. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: The endoscopic endonasal approach (EEA) is a minimally invasive and promising modality for treating traumatic superior orbital fissure (SOF) syndrome (tSOFS). Recently, the endoscopic transorbital approach (ETOA) has been considered an alternative method for reaching the anterolateral skull base. This study accessed the practicality of using the ETOA to treat SOF decompression using both cadaveric dissection and clinical application. Methods: Bilateral anatomic dissections were performed on four adult cadaveric heads using the ETOA and EEA to address SOF decompression. The ETOA procedure for SOF decompression is described, and the extent of SOF decompression was compared between the ETOA and EEA. The clinical feasibility of the ETOA for treating SOF decompression was performed in two patients diagnosed with tSOFS. Results: ETOA allowed for decompression over the lateral aspect of the SOF, from the meningo-orbital band superolaterally to the maxillary strut inferomedially. By contrast, the EEA allowed for decompression over the medial aspect of the SOF, from the lateral opticocarotid recess superiorly to the maxillary strut inferiorly. In both patients treated using the ETOA and SOF decompression, the severity of ophthalmoplegia got obvious improvement. Conclusions: Based on the cadaveric findings, ETOA provided a feasible access pathway for SOF decompression with reliable outcomes, and our patients confirmed the clinical efficacy of the ETOA for managing tSOFS. [ABSTRACT FROM AUTHOR]

Objective: Orbital apex syndrome (OAS) is a rare condition with multiple cranial nerve involvement caused by varied etiologies. It is not only a threat to the patient's vision but also life-threatening due to the intracranial spread of infection, if not diagnosed early and treated accurately. To study the outcome of endoscopic sinus surgery (ESS) for OAS secondary to sinusitis concerning resolution of ptosis, improvement of ophthalmoplegia, visual prognosis, intracranial spread of infection, and mortality. Methods: A retrospective review of patients with OAS secondary to sinusitis who underwent ESS from 2011 to 2021 was tabulated and analyzed. Results: Twenty-seven patients (mean age: 55.11+/-16 years; male 62%) were included in this study. At presentation, blurring of vision (81%), headache (66%), diplopia (63%) ptosis (63%) were the most common symptoms, and ophthalmoplegia (100%) was the most common sign. Five patients had no perception of light and the rest had various degrees of vision impairment. The most common etiopathology of sinusitis was fungal sinusitis (12 mucormycosis and four aspergillus). The final visual prognosis at three months follow-up post-ESS showed vision stabilization (no improvement or worsening) in 13 (48%) patients, improvement in seven (26%) patients, and vision deterioration in two (7%) patients. There was a significant improvement in ptosis (70%) and ophthalmoplegia (85%). There was no intracranial spread of infection or recurrence with a mortality rate of 3.7% (one patient). Conclusion: ESS coupled with appropriate antimicrobials effectively treats OAS secondary to sinusitis with decreased morbidity and mortality. [ABSTRACT FROM AUTHOR]

Miller Fisher Syndrome (MFS) is a rare variant of Guillain-Barré Syndrome (GBS), characterised by a unique clinical triad of ophthalmoplegia, ataxia, and areflexia. MFS, which was first described in 1956 by Charles Miller Fisher , is frequently preceded by an infection, usually respiratory or gastrointestinal, and is believed to be triggered by an autoimmune reaction targeting peripheral nervous system components. However the MFS is further categorised into incomplete forms which can be present without the classical triad, such as acute ophthalmoparesis, acute ataxic neuropathy, acute ptosis and acute mydriasis. We report a case of a 50-year-old man presenting with dilated and unresponsive pupils, which progressed rapidly to ophthalmoplegia, ataxia, and areflexia. Given the clinical history supported by the cyto-protein dissociation in cerebrospinal fluid, the patient was diagnosed to have MFS and successfully treated with intravenous immunoglobulin. This case underscores the importance of recognising atypical features of MFS, such as primary mydriasis, and highlights the variable clinical spectrum within the syndrome. Clinicians should maintain a high index of suspicion for MFS, particularly when faced with unusual neurological presentations, to ensure timely intervention and optimal patient outcomes.

Abstract Objective To evaluate the efficacy and safety of peribulbar triamcinolone acetonide injection for treating ocular myasthenia gravis (OMG), with a comparison of traditional oral drug therapy. Methods A total of 22 patients with OMG who received periocular triamcinolone acetonide injection (initially 20 mg weekly, then once per month later if symptoms were improved) from July 2019 to July 2022 were evaluated by a comparison of symptom degree before and after treatment. Adverse reactions were also monitored during the period of treatment. The period of follow-up was more than 6 months. Additionally, a comparison of the treatment efficacy between this periocular injection and traditional oral administration was performed in OMG patients. Results After 4 weeks of treatment, the degree of ptosis in OMG patients decreased to -3.00 ± 0.69, compared to the value (-0.86 ± 1.32) before treatment. The degree of ophthalmoplegia also decreased from 3.12 ± 0.72 to 0.86 ± 0.88 (P

Giant cell arteritis (GCA) is the most common form of vasculitis in elderly with multiple cardinal clinical features, such as headache associated with acute visual loss, jaw claudication and high inflammatory markers. GCA is readily treatable with immunosuppressants. Yet, if treated late, GCA could result in significant morbidities, such as aortic aneurysm, dissection, rupture and permanent visual loss. Early initiation of diagnostic evaluations, such as ultrasound of the temporal and axillary arteries and temporal artery biopsy, is thus very important. We present a case of an 84-year-old man who was diagnosed with GCA who presented with solely painless-intermittent ophthalmoplegia. He was later confirmed to have GCA with temporal artery ultrasound and biopsy. This case serves to emphasise the importance of recognising uncommon presentations of GCA and early initiation of its diagnostic tests and treatment.

Carotid-cavernous fistulas (CCFs) are abnormal connections between the carotid arteries and the cavernous sinus, posing significant neuro-ophthalmologic risks. This report presents a rare case of bilateral post-traumatic CCFs, focusing on clinical presentation, diagnosis, and management. Symptoms mimic conjunctivitis, causing diplopia, exophthalmos, and ophthalmoplegia. Diagnosis relied on computed tomography, magnetic resonance angiography, and digital subtraction angiography. Management involved transarterial embolization with coils, achieving successful outcomes. This highlights the importance of timely intervention and comprehensive imaging to prevent complications. Teaching point: This case report details a rare instance of bilateral post-traumatic carotid-cavernous fistulas, emphasizing clinical presentation, diagnostic evaluation, and management.

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. [ABSTRACT FROM AUTHOR]

Background and purpose: Tolosa-Hunt Syndrome (THS) is a rare disorder, and detailed clinical information and treatment outcomes have yet to be fully elucidated. This study aims to investigate the clinical features and factors associated with the treatment outcomes of THS, as defined by the established diagnostic criteria. Methods: This study retrospectively recruited 91 patients with a diagnosis of THS from 2003 to 2020. We analyzed the clinical features and outcomes, the initial treatment response, recurrences, and the final treatment response. Results: Isolated ocular motor nerve palsy was the most common (82.4%) finding of ophthalmoplegia, involving the oculomotor nerve in more than half of the cases (52.0%). The MRI lesions were mostly observed in the cavernous sinus (94.5%) with an extracavernous extension in about one-third of them. Five patients showed only extracavernous lesions. A total of 25 (27.5%) patients experienced recurrence. Recurrence occurred during steroid tapering as part of the initial treatment in seven, while in 18 patients, it happened after the successful termination of the initial treatment. However, all patients achieved complete remission at the final. Age was associated with a decrease in initial symptom duration (HR = 1.023, CI = 1.004–1.044) as well as an increase in recurrence-free duration (HR = 0.944, CI = 0.911–0.978). High-dose corticosteroid treatment was associated with a decrease in initial symptom duration (HR = 1.642, CI = 1.001–2.695) and total treatment duration (HR = 2.203 CI = 1.302–3.730). Conclusions: THS can recur frequently especially in younger but have a favorable prognosis. High-dose corticosteroids can be an effective initial treatment and reduce the total treatment duration. [ABSTRACT FROM AUTHOR]

Objective: To evaluate the efficacy and safety of peribulbar triamcinolone acetonide injection for treating ocular myasthenia gravis (OMG), with a comparison of traditional oral drug therapy. Methods: A total of 22 patients with OMG who received periocular triamcinolone acetonide injection (initially 20 mg weekly, then once per month later if symptoms were improved) from July 2019 to July 2022 were evaluated by a comparison of symptom degree before and after treatment. Adverse reactions were also monitored during the period of treatment. The period of follow-up was more than 6 months. Additionally, a comparison of the treatment efficacy between this periocular injection and traditional oral administration was performed in OMG patients. Results: After 4 weeks of treatment, the degree of ptosis in OMG patients decreased to -3.00 ± 0.69, compared to the value (-0.86 ± 1.32) before treatment. The degree of ophthalmoplegia also decreased from 3.12 ± 0.72 to 0.86 ± 0.88 (P < 0.001) after treatment. The achievement rates of minimal manifestations status (MMS)for ptosis and ophthalmoplegia after 4 week-treatment were 86.3% and 75%, respectively, while they were 50% and 30% in patients with traditional oral administration. There was statistically significant difference only in MMS (rather than symptom relief rate and generalization conversion rate) between two groups. No serious complications (except for intraorbital hematoma) were found in OMG patients during the treatment period. Conclusion: Repeated peribulbar injection of triamcinolone acetonide can effectively alleviate the initial symptoms of OMG patients. However, the evaluation of its long-term efficacy is still needed. Clinical Trial Registry: This study has been clinically registered by Chinese Clinical Trial Registry (ChiCTR), first trial registration date:05/07/2019, registration number: ChiCTR1900024285. [ABSTRACT FROM AUTHOR]

Copyright of Hungarian Medical Journal / Orvosi Hetilap is the property of Akademiai Kiado and its content may not be copied or emailed to multiple sites or posted to a listserv without the copyright holder's express written permission. However, users may print, download, or email articles for individual use. This abstract may be abridged. No warranty is given about the accuracy of the copy. Users should refer to the original published version of the material for the full abstract. (Copyright applies to all Abstracts.)

The mass effect associated with large or giant intracranial aneurysms is difficult for traditional endovascular treatment. This study investigated whether flow diverters can relieve the aneurysmal mass effect caused by aneurysmal compression symptoms. Fifty-five patients with unruptured large and giant intracranial aneurysms treated by a flow diverter at our institution from January 2014 to February 2022 were retrospectively evaluated. In this study, 53 patients were included. Initially, 27 patients (51.9%), including 10 with compressive optic neuropathy, 12 with third nerve palsy, 2 with facial hyperesthesia, and 11 with sixth nerve palsy, were symptomatic. The symptom duration was shorter in the improved group (n = 2.2 ± 4.0 vs. n = 3.1 ± 3.9, P = 0.49). Thrombus formation following the flow diversion procedure was typically observed on magnetic resonance imaging (MRI) performed immediately and was not significantly associated with symptomatic improvement (OR = 0.395; 95% CI (0.058–2.698), P = 0.343). However, symptomatic improvement was seen in most patients when the aneurysm size decreased on MRI. A reduction in the aneurysm size on the MRI at the 3-month follow-up was correlated with symptomatic improvement in the multivariate analysis (OR = 0.08, 95% CI (0.013–0.485), P < 0.05). A flow diverter might help alleviate compression symptoms caused by large or giant intracranial aneurysms. Shrinkage of the aneurysm within 3 months postoperatively and a shorter duration of symptoms contribute to the favorable outcomes of mass effect. Ultimately, prompt treatment is crucial for improving symptomatic intracranial artery aneurysms. [ABSTRACT FROM AUTHOR]

This document provides information on the diagnosis and imaging techniques for giant cell arteritis (GCA), a chronic inflammatory disorder of the large-sized vessels. The importance of early diagnosis is emphasized, as a delay can lead to significant health issues. Ultrasound is recommended as a low-invasive and rapid method for diagnosing GCA, with high diagnostic value. The document also discusses other imaging techniques, such as high-resolution MRI and FDG-PET, and includes criteria for classifying GCA. It acknowledges the limitations and advantages of different imaging methods. [Extracted from the article]

Abstract Background Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome (GBS) which is characterized by the three components of ophthalmoplegia, ataxia, and areflexia. Some studies reported MFS as an adverse effect of the COVID-19 vaccination. We aimed to have a detailed evaluation on demographic, clinical, and para-clinical characteristics of subjects with MFS after receiving COVID-19 vaccines. Materials and methods A thorough search strategy was designed, and PubMed, Web of Science, and Embase were searched to find relevant articles. Each screening step was done by twice, and in case of disagreement, another author was consulted. Data on different characteristics of the patients and types of the vaccines were extracted. The risk of bias of the studies was assessed using Joanna Briggs Institute (JBI) tools. Results In this study, 15 patients were identified from 15 case studies. The median age of the patients was 64, ranging from 24 to 84 years. Ten patients (66.6%) were men and Pfizer made up 46.7% of the injected vaccines. The median time from vaccination to symptoms onset was 14 days and varied from 7 to 35 days. Furthermore,14 patients had ocular signs, and 78.3% (11/14) of ocular manifestations were bilateral. Among neurological conditions, other than MFS triad, facial weakness or facial nerve palsy was the most frequently reported side effect that was in seven (46.7%) subjects. Intravenous immunoglobulin (IVIg) was the most frequently used treatment (13/15, 86.7%). Six patients received 0.4 g/kg and the four had 2 g/kg. Patients stayed at the hospital from five to 51 days. No fatal outcomes were reported. Finally, 40.0% (4/15) of patients completely recovered, and the rest experienced improvement. Conclusion MFS after COVID-19 immunization has favorable outcomes and good prognosis. However, long interval from disease presentation to treatment in some studies indicates that more attention should be paid to MFS as the adverse effect of the vaccination. Due to the challenging diagnosis, MFS must be considered in list of the differential diagnosis in patients with a history of recent COVID-19 vaccination and any of the ocular complaints, ataxia, or loss of reflexes, specially for male patients in their 60s and 70s.

Carotid cavernous fistula (CCF) is an abnormal vascular shunt from the carotid artery to the cavernous sinus. The characteristic clinical features include chemosis, proptosis, ocular bruit, headache, diplopia, and ophthalmoplegia. The classical radiological features on magnetic resonance imaging (MRI) include proptosis, extraocular muscle enlargement, dilatation of the superior ophthalmic vein, and ipsilateral cavernous sinus enlargement. We report the case of a 66-year-old female who presented with headache, redness of the right eye, and double vision, who had features of CCF on MRI.

Orbital Apex Syndrome (OAS) is a spectrum of Orbital Apex Disorder (OAD) in which progressive vision loss occurs due to the involvement of oculomotor nerve at the orbital apex, resulting in optic neuropathy and ophthalmoplegia. Generally patients represents with the associated symptoms related to the structures involved, specifically the orbital fissure, orbital appex or cavernous sinus, collectively known as OAD. The present study reports a case of in 38-year-old female patient, detected to have carcinoma bronchus on further evaluation. The patient presented with bilateral progressive blurring of vision, diplopia, and headache. The Magnetic Resonance Imaging (MRI) brain and orbits revealed thickening of the intracanalicular portion of the right optic nerve, thickening of the intracranial portion of bilateral bilateral optic nerves, a soft tissue intensity lesion at the planum sphenoidale, pituitary gland with out a bright spot, nodular thickening of infundibulum, and thickening of the bilateral cavernous sinus showing near homogeneous postcontrast enhancement. A Chest X-ray (CXR) followed by High Resolution Computed Tomography (HRCT) thorax confirmed a soft tissue density mass lesion with spiculated margins in the posterior segment of the right upper lobe, along with an abrupt termination of the posterior segmental bronchus. Fibreoptic bronchoscopy revealed narrowing of the right upper lobe segmental bronchus. Bronchial lavage fluid revealed features of adenocarcinoma. A whole-body Positron Emission Tomography (PET) scan performed elsewhere showed a welldefined hypermetabolic, heterogeneously enhancing soft tissue in the posterior segment of the right upper lobe and at the right orbital apex. Tissue diagnosis could not be confirmed as the patient’s health deteriorated. The MRI brain and orbits with contrast is the most important modality in evaluating OAD. The OAS is rarely reported as the first symptom of an occult lung carcinoma.

Purpose: This is a case of myasthenic incomplete ophthalmoplegia mimicking a partial cranial nerve 3 palsy both subjectively and objectively improving after treatment with eculizumab. Observations: We chronicle a case of severe generalized myasthenia gravis including myasthenia masquerading as a partial cranial nerve 3 palsy, refractory to pyridostigmine, mycophenolate, prednisone, intravenous immunoglobulin and plasma exchange but responsive to eculizumab. Conclusions and importance: This case demonstrates ocular and generalized myasthenia gravis refractory to several other therapies but amenable to eculizumab infusions, suggesting this medication may be of significant value in these difficult cases, and should be further explored for refractory ocular myasthenia gravis.

Key Clinical Message: This is the case of a 51‐year‐old woman with chronic progressive external ophthalmoplegia (CPEO) alongside some comorbidities in a low‐resource setting. This report seeks to sensitise clinicians about this rare condition in order to increase their index of suspicion and avoid misdiagnosis. [ABSTRACT FROM AUTHOR]

Background: Appropriate management of ophthalmoplegia demands good anatomy knowledge of cranial nerves involved i.e., III, IV and VI. Where these nerves originate and where they end, clinical features associated with paralysis of each nerve all this knowledge help in proper management of cases. Objective: To study clinical and etiological factors associated with the ophthalmoplegia. Methods: Prospective, Hospital based observational study was carried out among 50 cases of ophthalmoplegia. Detailed history was recorded. Thorough clinical examination including examination of both the eyes was carried out in terms of laterality, pupils affected or not, Central nervous system involvement, cranial nerves III, IV and VI etc. Papilledema, blood pressure, fasting blood sugar were investigated for each case. Results: Most common age group affected was >50 years in 50%. Males were slightly more affected than females. Majority (72%) cases were bilateral. Only nine cases had affected pupils. In ten cases, central nervous system was affected. Hypertension was seen in 32% and diabetes in 44%. 14% had history of trauma. Inflammation, tumor and vascular affection was seen in three cases each. Four cases had viral infection. Among all factors, diabetes was significantly associated with III cranial nerve involvement (p<0.05). Hypertension was significantly associated with IV cranial nerve involvement (p<0.05). But no factor was significantly associated with VI cranial nerve involvement (p>0.05). Conclusion: We conclude that ophthalmoplegia mainly affects elderly and males. It is usually bilateral and sometimes pupils and CNS are affected. Hypertensives and diabetics are at an increased risk of ophthalmoplegia. [ABSTRACT FROM AUTHOR]

Mitochondrial enoyl CoA reductase protein-associated neurodegeneration (MEPAN) syndrome is an ultra-rare autosomal recessive disorder caused by loss-of-function mutations in the MECR gene. The syndrome is characterized by dystonia in early childhood, basal ganglia signal abnormalities on MRI, and subsequent optic atrophy, with relative sparing of cognition. We characterize the ophthalmic manifestations observed in a patient with MEPAN syndrome, as a detailed account of ocular findings has not been published to date. Case study of a patient with genetically confirmed MEPAN syndrome, with full ophthalmic evaluation including slit-lamp exam, sensorimotor exam, fundus photography, retinal ocular coherence tomography (OCT), electroretinography, visual evoked potentials, and visual field testing. The patient exhibited decreased visual acuity of 20/150 in both eyes with moderate dyschromatopsia on pseudoisochromatic plate testing, while peripheral vision was largely intact on Goldmann visual field testing. Fundus exam revealed bilateral optic atrophy with pallor most pronounced temporally, corresponding to OCT findings of diffuse retinal nerve fiber layer thinning most prominent in the papillomacular bundle region and severe ganglion cell layer thinning in the maculae. She also displayed a high frequency horizontal end-gaze nystagmus and symmetric bilateral external ophthalmoplegia. The pattern of bilateral optic atrophy in our patient with MEPAN syndrome shows predilection for the papillomacular bundle, similar to that seen in other mitochondrial disorders with optic neuropathy, such as Leber Hereditary Optic Neuropathy and Dominant Optic Atrophy. Our patient's external ophthalmoplegia is another neuro-ophthalmic finding that may be seen in patients with heritable mitochondrial disease, either as an isolated ocular phenotype or within a constellation of systemic manifestations [ABSTRACT FROM AUTHOR]

Background: Miller Fisher syndrome (MFS) is a subtype of Guillain-Barré syndrome (GBS) which is characterized by the three components of ophthalmoplegia, ataxia, and areflexia. Some studies reported MFS as an adverse effect of the COVID-19 vaccination. We aimed to have a detailed evaluation on demographic, clinical, and para-clinical characteristics of subjects with MFS after receiving COVID-19 vaccines. Materials and methods: A thorough search strategy was designed, and PubMed, Web of Science, and Embase were searched to find relevant articles. Each screening step was done by twice, and in case of disagreement, another author was consulted. Data on different characteristics of the patients and types of the vaccines were extracted. The risk of bias of the studies was assessed using Joanna Briggs Institute (JBI) tools. Results: In this study, 15 patients were identified from 15 case studies. The median age of the patients was 64, ranging from 24 to 84 years. Ten patients (66.6%) were men and Pfizer made up 46.7% of the injected vaccines. The median time from vaccination to symptoms onset was 14 days and varied from 7 to 35 days. Furthermore,14 patients had ocular signs, and 78.3% (11/14) of ocular manifestations were bilateral. Among neurological conditions, other than MFS triad, facial weakness or facial nerve palsy was the most frequently reported side effect that was in seven (46.7%) subjects. Intravenous immunoglobulin (IVIg) was the most frequently used treatment (13/15, 86.7%). Six patients received 0.4 g/kg and the four had 2 g/kg. Patients stayed at the hospital from five to 51 days. No fatal outcomes were reported. Finally, 40.0% (4/15) of patients completely recovered, and the rest experienced improvement. Conclusion: MFS after COVID-19 immunization has favorable outcomes and good prognosis. However, long interval from disease presentation to treatment in some studies indicates that more attention should be paid to MFS as the adverse effect of the vaccination. Due to the challenging diagnosis, MFS must be considered in list of the differential diagnosis in patients with a history of recent COVID-19 vaccination and any of the ocular complaints, ataxia, or loss of reflexes, specially for male patients in their 60s and 70s. [ABSTRACT FROM AUTHOR]

Primary optic nerve sheath meningioma is generally a benign tumor. In rare instances, however, the growth rate and intraocular and intracranial extensions can be highly aggressive, especially in children, leading to poor prognosis. Here, we reported a case of a 24-year-old woman who presented with left eye swelling for 3 years. This was associated with blurred vision, retrobulbar pain, and redness. On examination, the left eye was severely proptosed with complete ophthalmoplegia. Magnetic resonance imaging showed an extensive tumor occupying the whole left orbital cavity with a disfigured eyeball. However, no intracranial extension was observed. Interestingly, complete surgical excision was feasible via transconjunctival anterior orbitotomy without bone removal. The histopathological examination confirmed the diagnosis of optic nerve sheath meningioma. Adjunct radiotherapy was given. On a follow-up after 2 years, left enophthalmos with esotropia was observed. [ABSTRACT FROM AUTHOR]