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This study aims to establish the final definite etiology among patients with long-term follow-up for painful ophthalmoplegia. The data of 44 cases (16 females, 28 females) were examined. In the first diagnosis, subjects were scanned in terms of benign and secondary etiologies. Clinical and radiological follow-up results of patients were recorded. During the follow-up period, data on clinical outcomes (relapse or progression), treatment responses, and final diagnoses were evaluated In total, 49 episodes of painful ophthalmoplegia (44 patients) were evaluated. Secondary etiologies were identified in 21 patients benign/secondary tumours causes in 10, inflammatory in 1, infectious in 3, vascular in 3, demyelinating disease in 1, autoimmune in 2, drug-related cause in 1. 23 patients with benign etiologies; 11 had Tolosa-Hunt syndrome (THS), 2 had Recurrent Painful Ophthalmoplegic Neuropathy (RPON), and 10 had diabetic ophthalmoparesis (DO). 7 of 11 patients with THS met the International Classification Headache Disorders 3rd edition (ICHD-3 beta) criteria, 4 were with a normal MRI, and 1 had a recurrence. 9 of 10 patients with benign/secondary tumours causes were malignant, and 7 died due to disease progression during the treatment process. One of ten patient was followed with diabetic ophthalmoparesis and was diagnosed with cavernous sinus involvement of B-cell lymphoma as a result of clinical progression during follow-up. Painful ophthalmoplegia is a complex clinical condition with a broad differential diagnosis with malignant and benign etiologies. A detailed clinical examination, imaging, and long-term follow-up are essential for accurate diagnosis and treatment management. [ABSTRACT FROM AUTHOR]

The superior orbital fissure contains cranial nerves III, IV, VI, and V1 with their three branches: frontal, lacrimal, and nasociliary. Superior orbital fissure syndrome (SOFS) is rare and can occur as a result of compression of these nerves due to trauma, bleeding, or inflammation in the retrobulbar space, but no cases of SOFS after deep lateral orbital wall decompression (DLOWD) have been reported. The aim of this paper is to describe this pathology, its possible causes, management, and outcome. Retrospective study of 575 DLOWD in patients with disfiguring exophthalmos due to Graves' ophthalmopathy performed in our hospital between 2010 and 2023. Three cases of postoperative SOFS were identified based on clinical presentation, history, physical examination, and radiological study. All patients were observed for a minimum of 12 months. SOFS was diagnosed with the presence of ophthalmoplegia, ptosis, fixed and dilated pupils, hypo/anesthesia of the upper eyelid and forehead, loss of corneal reflex, and no loss of vision after DLOWD. Fractures, edema, and hemorrhages were excluded. They were treated with high-dose intravenous steroids and the patients recovered completely. DLOWD challenges orbital surgeons because it requires removing bones near the globe or neurovascular structures. SOFS may occur due to the proximity and increased pressure on these structures. [ABSTRACT FROM AUTHOR]

Myogenic transcription factors with a basic helix–loop–helix (bHLH) such as MYOD, myogenin, MRF4, and MYF5 contribute to muscle differentiation and regulation. The MYF5 gene located on chromosome 12 encodes for myogenic factor 5 (MYF5), which has a role in skeletal and extraocular muscle development and rib formation. Variants in MYF5 were found to cause external ophthalmoplegia with rib and vertebral anomalies (EORVA), a rare recessive condition. To date, three homozygous variants in MYF5 have been reported to cause EORVA in six members of four unrelated families. Here, we present a novel homozygous MYF5 frameshift variant, c.596dupA p. (Asn199Lysfs*49), causing premature protein termination and presenting with external ophthalmoplegia, ptosis, and scoliosis in three siblings from a consanguineous family of Pakistani origin. With four MYF5 variants now discovered, genetic testing and paediatric assessment for extra-ocular features should be considered in all cases of congenital ophthalmoplegia. [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]

A 79-year-old man was admitted for 2 weeks of dizziness, followed by diplopia, involuntary movement and progressive gait disturbances. Neurologic examination revealed horizontal and vertical gaze paresis, bilateral choreiform movement with orofacial dyskinesia, and limb/truncal ataxia. MRI revealed fluid-attenuated inversion recovery image-hyperintense signal abnormalities in the dorsal midbrain, pontine and medulla. Within another few days, the patient developed type II acute respiratory failure requiring artificial invasive ventilation. Because autoimmune encephalitis was suspected, he received intravenous immunoglobulin therapy followed by intravenous methylprednisolone, but only his ophthalmoplegia improved minimally. Serological tests were positive for anti-Ri onconeural antibodies. CT-guided mediastinal lymph node biopsy was performed and revealed small cell lung carcinoma. We report the rare manifestation of anti-Ri antibody-associated paraneoplastic neurological syndrome (PNS), and this case can alert us to the importance of respiratory management in this diverse neurologic disease. Furthermore, PNSs positive for anti-Ri antibodies should be added to the list of differential diagnoses of chorea with orofacial dyskinesia. [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]

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. [ABSTRACT FROM AUTHOR]

Background: Chronic progressive external ophthalmoplegia (CPEO) is a rare disorder that can be at the forefront of several mitochondrial diseases. This review overviews mitochondrial CPEO encephalomyopathies to enhance accurate recognition and diagnosis for proper management. Methods: This study is conducted based on publications and guidelines obtained by selective review in PubMed. Randomized, double-blind, placebo-controlled trials, Cochrane reviews, and literature meta-analyses were particularly sought. Discussion: CPEO is a common presentation of mitochondrial encephalomyopathies, which can result from alterations in mitochondrial or nuclear DNA. Genetic sequencing is the gold standard for diagnosing mitochondrial encephalomyopathies, preceded by non-invasive tests such as fibroblast growth factor-21 and growth differentiation factor-15. More invasive options include a muscle biopsy, which can be carried out after uncertain diagnostic testing. No definitive treatment option is available for mitochondrial diseases, and management is mainly focused on lifestyle risk modification and supplementation to reduce mitochondrial load and symptomatic relief, such as ptosis repair in the case of CPEO. Nevertheless, various clinical trials and endeavors are still at large for achieving beneficial therapeutic outcomes for mitochondrial encephalomyopathies. Key Messages: Understanding the varying presentations and genetic aspects of mitochondrial CPEO is crucial for accurate diagnosis and management. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial encephalomyopathy that is characterized by progressive ptosis and impaired ocular motility. Owing to its nonspecific clinical manifestations, CPEO is often misdiagnosed as other conditions. Herein, we present the case of a 34-year-old woman who primarily presented with incomplete left eyelid closure and limited bilateral eye movements. During the 6-year disease course, she was diagnosed with myasthenia gravis and cranial polyneuritis. Finally, skeletal muscle tissue biopsy confirmed the diagnosis. Biopsy revealed pathological changes in mitochondrial myopathy. Furthermore, mitochondrial gene testing of the skeletal muscle revealed a single chrmM:8469-13447 deletion. In addition, we summarized the findings of 26 patients with CPEO/Kearns-Sayre syndrome who were misdiagnosed with other diseases owing to ocular symptoms. In conclusion, we reported a rare clinical case and emphasized the symptomatic diversity of CPEO. Furthermore, we provided a brief review of the diagnosis and differential diagnosis of the disease. [ABSTRACT FROM AUTHOR]

Introduction: Streptococcal meningoencephalitis (SME) is a rare, and frequently lethal, acute infection, and inflammation of the central nervous system parenchyma, with associated meningeal involvement. Bacterial meningoencephalitis is generally associated with high rates of morbidity and mortality, despite available antimicrobial and corticosteroid treatments. While Streptococcus pneumoniae is well recognised to cause bacterial meningitis, direct extension into the central nervous system parenchyma is rare. Case Presentation: A previously well 49-year-old man presented with sudden onset severe headache, fevers, neck stiffness, and reduced consciousness. The manifestations of SME in this patient were bilateral pupil-involving third-nerve palsies, wall-eyed bilateral internuclear ophthalmoplegia (WEBINO), bilateral blindness, bilateral deafness, a right lower motor neuron facial palsy, and upper motor neuron signs in his limbs. Initially, a partial response to high dose intravenous antibiotics occurred, but with administration of intravenous corticosteroids, further substantial resolution of the patient's neurological and neuro-ophthalmological deficits occurred. Conclusion: This case highlights the benefit of multidisciplinary diagnostic and therapeutic interventions in a case of SME complicated by bilateral pupil-involving third-nerve palsies, WEBINO, bilateral blindness, bilateral deafness, a right lower motor neuron facial palsy, and upper motor neuron signs. It appears to be the first reported case of SME with this rare collection of neuro-ophthalmological abnormalities. [ABSTRACT FROM AUTHOR]

Background: Progressive external ophthalmoplegia (PEO) is a common subtype of mitochondrial encephalomyopathy. Objective: The study aimed to investigate the relationship between mitochondrial DNA (mtDNA) abnormalities, muscle pathology, and clinical manifestations in Chinese patients with single large‐scale mtDNA deletion presenting with PEO. Methods: This is a retrospective single‐center study. Patients with PEO who had a single large deletion in mitochondrial DNA were included in this study. The associations were analyzed between mtDNA deletion patterns, myopathological changes, and clinical characteristics. Results: In total, 155 patients with mitochondrial PEO carrying single large‐scale mtDNA mutations were enrolled, including 137 chronic progressive external ophthalmoplegia (CPEO) and 18 Kearns–Sayre syndrome (KSS) patients. The onset ages were 9.61 ± 4.12 in KSS and 20.15 ± 9.06 in CPEO. The mtDNA deletions ranged from 2225 bp to 9131 bp, with m.8470_13446del being the most common. The KSS group showed longer deletions than the CPEO group (p = 0.004). Additionally, a higher number of deleted genes encoding respiratory chain complex subunits (p = 0.001) and tRNA genes (p = 0.009) were also observed in the KSS group. A weak negative correlation between the mtDNA deletion size and ages of onset (p < 0.001, r = −0.369) was observed. The proportion of ragged red fibers, ragged blue fibers, and cytochrome c negative fibers did not correlate significantly with onset ages (p > 0.05). However, a higher percentage of abnormal muscle fibers corresponds to an increased prevalence of exercise intolerance, limb muscle weakness, dysphagia, and cerebellar ataxia. Conclusion: We reported a large Chinese cohort consisting of mitochondrial PEO patients with single large‐scale mtDNA deletions. Our results demonstrated that the length and locations of mtDNA deletions may influence onset ages and clinical phenotypes. The severity of muscle pathology could not only indicate diagnosis but also may be associated with clinical manifestations beyond the extraocular muscles. [ABSTRACT FROM AUTHOR]

This document, published in the Journal of Neurology, discusses a case study of a 54-year-old man who presented with dizziness and oscillopsia. The patient had previously experienced a right dorsal pontine infarction and subsequent right internuclear ophthalmoplegia (INO). The examination revealed conjugate torsional ocular oscillation and palatal tremor. The patient was diagnosed with oculopalatal tremor (OPT), which is a delayed complication of damage to the dentatorubro-olivary pathway. The study suggests that torsional pendular nystagmus may occur as a delayed complication of a focal lesion affecting the central tegmental tract adjacent to the medial longitudinal fasciculus (MLF). [Extracted from the article]

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]

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]

The internist Hermann Nothnagel (1841–1905) took a special interest in the cerebellum. In an early experimental study on rabbits conducted in 1876, he demonstrated the involvement of the vermis in the pathophysiology of motor ataxia. Between 1879 and 1889, he reported four cases of tectal tumors that clinically manifested with bilateral ophthalmoplegia and unilateral gait ataxia, culminating in the Cerebellar Classic highlighted here. Nothnagel attributed this clinical syndrome to lesions of the colliculi ("quadrigeminal bodies") and compression of the nuclei of the third cranial nerves, but also left open the possibility of the involvement of neighboring structures, such as the cerebellar vermis. Today, the ataxic component of Nothnagel syndrome is explained by a dorsal midbrain abnormality of either neoplastic or vascular origin, involving the superior cerebellar peduncles, besides the oculomotor nerves. [ABSTRACT FROM AUTHOR]

Intravascular large B-cell lymphoma (IVLBCL) is a rare type of lymphoma, involving the lumen of predominantly small blood vessels, especially capillaries. The orbit is an uncommon site of involvement for IVLBCL, and diagnosis before autopsy is even more rare as most cases are established post-mortem. Herein, the authors describe a 73-year-old male who presented with 3 weeks of progressive bilateral ptosis and ophthalmoplegia. Computed tomography (CT) and subsequent magnetic resonance imaging (MRI) revealed diffuse abnormal thickening and enhancement of bilateral orbital apices, superior orbital fissures, and cavernous sinus, along with persistent focal opacification of the left frontal and ethmoid sinuses. Infectious and inflammatory workup of serum and cerebrospinal fluid was negative. Ethmoidal sinus and middle turbinate biopsy confirmed intravascular large B-cell lymphoma and the patient was started on R-CHOP chemotherapy regimen. [ABSTRACT FROM AUTHOR]

• Titin related disorders comprises a wide spectrum phenotype. • The clinical phenotype has been expanded by reporting the association with ophthalmoplegia. • With advanced diagnostic techniques including next generation sequencing, novel phenotypes will be added to Titin related disorders. Titin-related myopathy is an emerging genetic neuromuscular disorder with a wide spectrum of clinical phenotypes. To date, there have not been reports of patients with this disease that presented with extraocular muscle involvement. Here we discuss a 19-year-old male with congenital weakness, complete ophthalmoplegia, thoracolumbar scoliosis, and obstructive sleep apnea. Muscle magnetic resonance imaging revealed severe involvement of the gluteal and anterior compartment muscles, and clear adductor sparing, while muscle biopsy of the right vastus lateralis showed distinctive cap-like structures. Trio Whole Exome Sequencing (WES) showed compound heterozygous likely pathologic variants in the TTN gene. (c.82541_82544dup (p.Arg27515Serfs*2) in exon 327 (NM_001267550.2) and c.31846+1G>A (p.?) in exon 123 (NM_001267550.2). To our knowledge, this is the first report of a TTN -related disorder associated with ophthalmoplegia. [ABSTRACT FROM AUTHOR]

Background: Chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial disease with slowly progressive bilateral ptosis and symmetric ophthalmoplegia due to a genetic mutation that results in defective oxidative phosphorylation. Common genes that are implicated in CPEO include POLG, RRM2B, ANT1 and PEO1/TWNK. Here, we report a case of a patient diagnosed with CPEO caused by a novel mutation in PEO/TWNK after suffering a right pontine stroke. Case presentation: A 70-year-old man with history of chronic progressive bilateral ptosis and ophthalmoplegia, as well as similar ocular symptoms in his father and grandfather, presented with acute onset of right hemifacial weakness and dysarthria. Brain MRI revealed an acute ischemic stroke in the right dorsal pons. The patient did not experience diplopia due to severe baseline ophthalmoplegia. Creatine kinase was elevated to 6,080 U/L upon admission and normalized over the course of one week; electromyography revealed a myopathic process. Genetic testing revealed a novel mutation c.1510G > A (p. Ala504Thr) in a pathogenic "hot spot" of the C10ORF2 gene (TWNK/PEO1), which is associated with CPEO. The mutation appears to be deleterious using several pathogenicity prediction tools. Conclusions: This case report describes a patient with late-onset CPEO caused by a novel, likely pathogenic, mutation in the TWNK gene. Although the patient presented with a pontine stroke, it manifested with solely new onset facial palsy, as he had a severe underlying ophthalmoplegia secondary to his CPEO. [ABSTRACT FROM AUTHOR]

Lemierre's syndrome is a triad consisting of oropharyngeal infection, internal jugular vein thrombophlebitis, and systemic embolisation typically involving lung and brain. Orbital involvement in this life-threatening condition is rare but potentially blinding and may be an indicator of intracranial involvement. We describe a case of odontogenic Lemierre's syndrome complicated by extensive orbital and intracranial septic venous thrombosis, with optic and cranial neuropathy resulting in monocular blindness and ophthalmoplegia. A multidisciplinary approach with abscess drainage, antibiotic and antithrombotic therapy, and close radiological monitoring was critical for preserving contralateral vision and neurological function. [ABSTRACT FROM AUTHOR]

Migraine increased the risk of Bell palsy in the total population. Among migraine patients, between =30 and <60 years old are at an increased risk of Bell palsy. A migraine is a primary headache characterized by recurrent headache attacks triggered by various factors. As much as 10% of the global population is thought to experience migraine headaches. It was earlier considered that migraine headaches were triggered by the dilation of cerebral vessels, and the recent evidence supports that migraine attacks can also occur in the absence of vasodilation. According to the researchers, the direct neural effects from the trigeminal nerve to the facial nerve could contribute to the risk of facial palsy among patients with migraine. An alteration of the trigeminovascular function has been suggested to trigger migraines. The neurogenic inflammation of the facial nerve trunk caused by its proximity to the dilated posterior auricular/stylomastoid/occipital and superficial temporal arteries during a migraine attack leads to a temporary lower motor neuron type of paresis of the muscles supplied by the facial nerve. We herewith report a rare case of migrainous left Bell's palsy after migrainous right external ophthalmoplegia, treated with Sumatriptan. [ABSTRACT FROM AUTHOR]

A 60-year-old man with longstanding bilateral asymmetrical ptosis presented with a partial third nerve palsy. His diplopia improved following an ice pack test. He did not report any symptoms related to the coronavirus disease 2019 (COVID-19), and nasopharyngeal swab was negative. Initial head imaging and blood work-up were normal except for a high titer of anti-GQ1b antibodies. The patient was subsequently diagnosed with acute ophthalmoparesis without ataxia which is part of the anti-GQ1b antibody syndrome spectrum. He made a spontaneous recovery over the following months without the need for immunotherapy. Clinical features, pathophysiology and a review of the literature are discussed herein. It is important to consider anti-GQ1b antibody syndrome in patients with symptoms of diplopia, ptosis or suspected ocular myasthenia. [ABSTRACT FROM AUTHOR]

Facial paralysis decreases eye protection mechanisms leading to ocular problems up to corneal ulceration, and blindness. This study aimed to evaluate the outcomes of periocular procedures for recent facial paralysis. Medical records of patients with unilateral recent complete facial palsy who did periocular procedures at the Maxillofacial Surgery Department of San Paolo Hospital (Milan, Italy) between April 2018 and November 2021 were retrospectively reviewed. 26 patients were included. All patients were evaluated 4 months after surgery. The first group included 9 patients who underwent upper eye lid lipofilling and midface suspension with fascia lata graft; they had no ocular dryness symptoms and no need for eye protection measures in 33.3% of cases, significant reduction of ocular symptoms and need for eye protection measures in 66.6% of patient, 0–2 mm lagophthalmos in 66.6% and 3–4 mm lagophthalmos in 33.3%. The second group of 17 patients who underwent upper eyelid lipofilling, midface suspension with fascia lata graft and lateral tarsorrhaphy, had no ocular dryness symptoms and no need for eye protection measures in 17.6% of patient, significant reduction of ocular symptoms and need for eye protection measures in 76.4% of patient, 0–2 mm lagophthalmos in 70.5%, 3–4 mm lagophthalmos in 23.5% and one patient 5,8%had 8 mm lagophthalmos and persistent symptoms. No ocular complication, cosmetic complain or donner site morbidity were reported. Upper eyelid lipofilling, midface suspension with fascia lata graft and lateral tarsorrhaphy decrease ocular dryness symptoms and need for eye protection measures and improve lagophthalmos: the association of the reinnervation with these complementary techniques is therefore highly recommended in order to immediately protect the eye. [ABSTRACT FROM AUTHOR]

• Three individuals with myopathy and a novel MYH2 splice-site variant. • Myopathy had autosomal dominant inheritance in a large four-generation pedigree. • EMG, muscle MRI, and muscle biopsy consistent with MYH2- associated myopathy. • RNAseq on fibroblasts demonstrated that the variant alters splicing. • Lack of ophthalmoplegia and congenital contractures expands the phenotype. MYH2 encodes MyHCIIa, a myosin heavy chain found in fast type 2A fibers. Pathogenic variants in this gene have previously been implicated in dominant and recessive forms of myopathy. Three individuals reported here are part of a family in which four generations of individuals are affected by a slowly progressive, predominantly proximal myopathy in an autosomal dominant inheritance pattern. Affected individuals in this family lacked classic features of an MYH2- associated myopathy such as congenital contractures and ophthalmoplegia. A novel variant, MYH2 c.5673+1G>C, was detected in the proband and subsequently found to segregate with disease in five additional family members. Further studies demonstrated that this variant affects splicing, resulting in novel transcripts. These data and muscle biopsy findings in the proband, indicate that this family's MYH2 variant is causative of their myopathy, adding to our understanding of the clinical and molecular characteristics of the disease. [ABSTRACT FROM AUTHOR]

While patients with infectious origin tended to show complete clinical remission after adequate therapy, patients with neoplastic aetiology did not recover [[12]]. Neither the cerebral or spinal MRI, nor the CSF results indicated Multiple Sclerosis (MS) specific findings CSF findings. The prevalence among patients with Multiple Sclerosis (MS) has been estimated between 17 and 41% [[6]]. In addition, the patient suffered from a COVID-19 infection 2 months before onset of ocular symptoms (at time of incubation, the patient had her first COVID-vaccination with Spikevax© (Moderna) administered) and an adequately substituted hypothyroidism. [Extracted from the article]

Soft tissue filler injection is the second most common nonsurgical cosmetic procedure. Despite the safety of fillers, as use has grown, so has the number of patients affected by adverse events. Ophthalmoplegia following cosmetic filler injection is a rare complication, mostly occurring after injection to the glabella, nasolabial fold, periorbital, and lateral nasal site. In all cases where ophthalmoplegia has been reported following fillers, patients have simultaneously experienced vision loss and other ocular symptoms. We report a case of isolated acute ophthalmoplegia following hyaluronic acid injection solely in the temple region. A 40-year-old woman, 3 hours after the procedure, presented to our hospital with left eye ophthalmoplegia, ptosis, and hypotropia. Treatment started with hyaluronidase, steroids, and anticoagulants. After 4 weeks, left eye ophthalmoplegia remained unchanged, and through a 10-week follow-up, all left ocular movements improved, and only mild hypotropia and ptosis persisted. This case report shows that ophthalmoplegia may also happen with temple region filler injections. We also review available prevention techniques and treatments to avoid such complications when performing soft tissue fillers for gaunt appearance correction. [ABSTRACT FROM AUTHOR]

Keywords: abetalipoproteinemia; ophthalmoplegia; ataxia; MTTP EN abetalipoproteinemia ophthalmoplegia ataxia MTTP 514 517 4 03/23/23 20230301 NES 230301 Abetalipoproteinemia is an uncommon potentially treatable cause of early-onset ataxia associated with retinitis pigmentosa and acanthocytes. Deep brain stimulation for the treatment of tremor and ataxia associated with abetalipoproteinemia. However, atypical retinal pigmentation retinopathy and diarrhea with fat malabsorption favored abetalipoproteinemia over other diagnostic possibilities. [Extracted from the article]

Background: Video-oculography (VOG) is used to quantify functional deficits in internuclear ophthalmoplegia (INO), whereas MRI can detect the corresponding structural lesions in the medial longitudinal fasciculus (MLF). This study investigates the diagnostic agreement of MRI compared to VOG measurements. Methods: We prospectively compared structural MRI findings and functional VOG measures of 63 MS patients to assess their diagnostic agreement for INO. Results: MRI detected 12 true-positive and 92 true-negative MLF lesions for INO compared to VOG (12 true-positive and 38 true-negative patients) but identified one-third of the MLF lesions on the wrong side. MRI ratings were specific (92.0%) to detect MLF lesions but not sensitive (46.2%) for diagnosing INO (86.4% and 63.2% by patient). Accordingly, MRI has a high positive likelihood ratio of 5.77 but a modest negative likelihood ratio of 0.59 for the probability of INO (4.63 and 0.43) with an accuracy of 82.5% (79.4%). Conclusion: MRI assessments are highly specific but not sensitive for detecting INO compared to VOG. While MRI identifies MLF lesions in INO, VOG quantifies the deficit. As a simple, quick, and non-invasive test for diagnosing and tracking functional INO deficits, it will hopefully find its place in the diagnostic and therapeutic pathways of MS. [ABSTRACT FROM AUTHOR]

Tolosa-Hunt Syndrome is an idiopathic granulomatous inflammatory disease of the cavernous sinus, superior orbital fissure, or orbit which is manifested by ocular pain and ophthalmoplegia. Granulomatous inflammation of the dural wall of the cavernous sinus and/or superior orbital fissure is the basis of the pathological process. The disease is clinically manifested by orbital pain, diplopia, exophthalmos, and/or oculomotor abnormalities. The syndrome is also called painful ophthalmoplegia with a key symptom being neuropathic periorbital or retro-orbital pain, and paresthesias along the first branch of the trigeminal nerve. We present a case of this syndrome. The patient was administered hormonal therapy, Medrol at a daily dose of 1 mg/kg body weight/day intravenously for five days, with subsequent transition within a month to oral Medrol. The pain in the eye completely relieved and restoration of normal ocular motility was observed in the presence of hormonal therapy. Tolosa-Hunt syndrome is a clinical diagnosis of exclusion (i.e., it is diagnosed after excluding other causes of painful ophthalmoplegia). [ABSTRACT FROM AUTHOR]

Sphenoid sinuses mucocele (SSM) is an uncommon cause of orbital apex syndrome (OAS). Diagnosis of neurological complications in SSM might be delayed when the expansion of mucocele beyond the sinuses is not evident in conventional sinuses imaging. We present a case of a 76-years old man with spared-pupil ophthalmoplegia associated with ptosis caused by a unilateral left SSM in which internal carotid artery Doppler ultrasound showed distal sub-occlusion waves pattern. Sinus occupation was noted in the magnetic resonance imaging (MRI) and was further evaluated in computed tomography (CT) scan and MR angiography. Nor CT or MR angiography showed clear evidence of neighboring structures compression. Doppler ultrasound of internal carotid showed high-resistance waveforms and decreased wave velocities helping diagnosis. Structures compression was confirmed intra-operatively and the patient was discharged asymptomatic after sphenoid sinus drainage. In this first report of carotid Doppler ultrasound findings in a patient with a neurological presentation of a sphenoid sinus mucocele, a high-resistance waveform of the internal carotid may help differentiate uncomplicated sinusitis from invasive mucocele. [ABSTRACT FROM AUTHOR]

Ophthalmoplegia is the diagnostic hallmark of anti-GQ1b antibody syndrome. This study aimed to define the patterns of acute comitant strabismus in patients with anti-GQ1b antibody syndromes. We retrospectively analyzed the ocular motor findings in 84 patients with anti-GQ1b antibody-associated ophthalmoplegia during the acute phases. Of the 84 patients, 11 (13%) showed acute comitant strabismus. Compared to those without, patients with acute comitant strabismus frequently showed abnormal ocular motor findings that included gaze-evoked (n = 8), spontaneous (n = 4) and positional nystagmus (n = 4), saccadic hypermetria (n = 3), head-shaking nystagmus (n = 2), pulse-step mismatch (n = 1), and impaired visual cancellation of the vestibulo-ocular reflex (n = 1, p < 0.001). On the contrary, iridoplegia (p = 0.029) and ptosis (p = 0.001) were more commonly observed in patients with paralytic (incomitant) strabismus than in those with acute comitant strabismus. Comitant strabismus can manifest during the acute phase of anti-GQ1b antibody syndromes in association with other central ocular motor abnormalities. These findings implicate that the cerebellum and/or brainstem can be the primary target of the anti-GQ1b antibodies. [ABSTRACT FROM AUTHOR]

Orbital apex is a region involving bone, vascular and neural structures. Pathologies involving this region may lead to several symptoms and signals and to orbital apex syndrome. It is a rare syndrome that is characterized by signals involving nervous ocular motor nerves through the optic foramen and the superior orbital fissure. This can affect the oculomotor, the third canal and abducens nerves; the ophthalmic division of the fifth cranial nerve and the optic nerve. Its causes include neoplastic, vascular, traumatic, infectious, inflammatory, and idiopathic conditions. We often deal with conditions with no treatment, so it is necessary to control the symptoms and prevent late implications. The purpose of this case series is to report on the causes of orbital apex disease and its potential chronic implications. [ABSTRACT FROM AUTHOR]

Objective: Describe and discuss a typical case with recurrent painful ophthalmoplegic neuropathy (RPON), and demonstrate a likely extrinsic compression of the sixth cranial nerve by a vascular loop. Background: RPON is a rare, but well-known entity, characterized by attacks of ophthalmoplegia associated with ipsilateral headache. The etiology is unknown, but a neurovascular conflict (NVC) has been suggested as a cause. Method: A 62-year-old woman presented in the emergency department with a horizontal diplopia that developed a few hours after the onset of a left temporal headache. In the past she had presented to the clinic with the same problem several times. She had been thoroughly examined, but no cause had been found. The symptoms had resolved by themselves. In addition to a classic finding in RPON, gadolinium enhancement and thickening of a cranial nerve, the new MRI scanning detected an NVC. Herein we discuss the relevance of this finding. Conclusion: A neurovascular conflict causing inflammatory demyelination of a single cranial nerve and relapsing remitting painful ophthalmoplegic neuropathy is an alluring idea. However, it is hard to conceive theoretically, and more reported cases with compelling data are needed to give credibility to such an idea. [ABSTRACT FROM AUTHOR]

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A clinical case of a patient with progressive supranuclear palsy (PSP) and wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) syndrome identified using video-oculography and ophthalmological examination is presented. WEBINO syndrome is a combination of bilateral internuclear ophthalmoplegia and exodeviation. Few cases of such oculomotor disorder in PSP patients have been earlier described. However, a closer look at the pathogenesis of co-existing internuclear ophthalmoplegia and strabismus reveals its common features with the origins of typical oculomotor disorders seen in PSP. Therefore, this horizontal eye movement impairment might be not so uncommon in PSP patients. Diplopia as one of the subjective manifestations of WEBINO is a treatable phenomenon. Its management is capable of improving the gait function, severely affected in PSP. Practical recommendations are given for neurologists to improve the detection of horizontal gaze dysfunction in PSP and the role of instrumental methods of examination is explained. Awareness of clinical practitioners of WEBINO syndrome will not only elevate the quality of help for PSP patients, but also expand the understanding of pathogenesis of oculomotor abnormalities in this type of atypical parkinsonism. [ABSTRACT FROM AUTHOR]

Tolosa-Hunt Syndrome is an uncommon condition characterized by painful ophthalmoplegia affecting the third, fourth, and/or sixth cranial nerves, which is caused by an unknown etiology of non-specific inflammation in the cavernous sinus or superior orbital fissure. a 60-year-old diabetic male patient who recovered from covid-19, 15 days before, came with complain of sharp and intense unilateral headache, ptosis, ophthalmoplegia and loss of vision in right eye in the past 10 days diagnosed with Tolosa-Hunt Syndrome was presented. He was suffering from a right-sided headache, periorbital pain, and double vision. Right-sided ptosis, right-sided trochlear and abducens nerve palsy, and partial right-sided oculomotor nerve palsy with hypoesthesia in the ocular division of the trigeminal nerve were discovered during the examination. The optic nerve and lateral rectus muscle revealed abnormal signal intensity variations on magnetic resonance imaging showed the minimal proptosis noted on the right side with mild edema in the right periorbital soft tissues and small enhancing soft tissue in the right cavernous sinus region which suggested the possibility of Tolosa Hunt Syndrome of the head and orbit. Pain and ptosis were greatly reduced after 72 hours of steroid treatment. Tolosa-Hunt Syndrome is an exclusionary diagnosis with a clinical presentation, normal tests, and magnetic resonance imaging. Recurrent unilateral orbital pain, ipsilateral oculomotor paralysis, and a quick reaction to steroids characterize Tolosa-Hunt syndrome, a severe ophthalmoplegia Treated with intravenous methyl prednisolone (40 mg) once a day with other supportive care therapy. Yet, there was no reversal of vision loss and ptosis. However, the headache subsided and the patient was discharged after 7 days. In Vadodara, Gujarat, India this is most likely the first occurrence and reported case of Tolosa-Hunt syndrome. [ABSTRACT FROM AUTHOR]

Background: Food-borne botulism is a rare neuromuscular junction disorder due to the effect of toxins released from Clostridium botulinum ingested by eating improperly stored food. Its classic manifestation is a rapidly evolving descending symmetrical flaccid paralysis with dysautonomia.Case Presentation: We have described a case of type B food-borne botulism with a benign clinical course characterized by an initially unilateral tonic mydriatic pupil. An extensive neurophysiological evaluation inclusive of pilocarpine eye drop(s) test, facial and limbs nerve stimulation and sudomotor tests, was decisively leading the diagnostic process.Conclusions: The importance of what has been described here lies in underlining that it is always advisable to consider food-borne botulinum intoxication, even in the case of unilateral/asymmetrical internal ophthalmoplegia without generalized progressive involvement of the voluntary muscles. [ABSTRACT FROM AUTHOR]

Background: Acute motor sensory axonal neuropathy (AMSAN) is a severe form of Guillain–Barré syndrome, which typically is not related to ocular palsy, and is pathologically associated with antibodies against GM1 and GD1a gangliosides. Case presentation: We present a patient with a severe, drug‐resistant form of AMSAN with ophthalmoplegia related to antiganglioside (anti‐GD1a, ‐GD1b, ‐GM1) antibodies in the serum and cerebrospinal fluid, and with a poor long‐term outcome. Conclusions: Rare cases of AMSAN with ophthalmoplegia are described in the literature. The present patient is a case of an unusual clinical phenotype of AMSAN, the characteristics of which warn of a serious clinical course that requires timely aggressive treatment. [ABSTRACT FROM AUTHOR]

Chronic progressive external ophthalmoplegia (CPEO) is a clinical manifestation of mitochondrial diseases that causes progressive ptosis and impaired eye movements. Diagnosing CPEO can be challenging without pathologic or genetic confirmation, particularly when patients present with asymmetrical involvement. This study aimed to investigate the clinical and genetic characteristics of CPEO, which would aid in early identification. A total of seven patients pathologically and/or genetically confirmed to have CPEO were included. Long-range PCR encompassing the entire mitochondrial DNA (mtDNA) and nuclear DNA (nDNA) was carried out. In the cases with mtDNA deletion, the exact deletion ranges of mtDNA were identified by sequencing. A full ophthalmologic examination including prism and alternate cover test in the primary position, evaluation of ductions and versions, and binocularity was performed. In five patients (71%), ophthalmoplegia and ptosis were asymmetric between both eyes, while two patients (29%) showed symmetric ophthalmoplegia. Five patients were confirmed to have mtDNA deletion (c.8470_13447del(4977bp)) associated with CPEO, and one was confirmed to have an nDNA missense mutation (SLC25A4 , c.311A>G). Genetic diagnosis was unsuccessful in the remaining patient, who had a pathologic diagnosis of CPEO. This study shows that CPEO can commonly manifest asymmetric ophthalmoplegia and ptosis, which contradicts previously established knowledge. The most common genetic abnormality is single large-scale deletions of mtDNA. A limited number of patients require further confirmatory studies. [ABSTRACT FROM AUTHOR]

Background: Miller Fisher Syndrome is a variant of Guillain-Barre Syndrome, classically characterized by the triad of ataxia, areflexia and ophthalmoplegia. However, many conditions can mask the presentation of Miller Fisher Syndrome, potentially leading to grave consequences due to delayed diagnosis or even misdiagnosis. Case Report: We describe a case of Miller Fisher Syndrome mimicking acute sphenoid sinusitis with intracranial complications in an otherwise healthy 19-year-old man. Accurate diagnosis with prompt treatment led to full clinical recovery of our patient. Conclusion: Sphenoid sinusitis warrants great vigilance and thorough neurological examination due to its proximity to structures such as the cavernous sinus and its associated cranial nerves. This case highlights its potential to mask more devastating conditions like Miller Fisher Syndrome and the successful role of medical management without the need for sphenoidotomy. [ABSTRACT FROM AUTHOR]

Miller Fisher syndrome (MFS) is frequently encountered variant of Gillian Barre Syndrome (GBS). It has distinct clinical and serological features. Here we describe an atypical GQ1b seronegative case with significantly elevated anti-glutamic acid decarboxylase antibody (GAD-Ab). A 24-year-old previously healthy Caucasian male presented with rapidly progressive ascending weakness, binocular diplopia and autonomic instability for 2 days. Examination was remarkable for asymmetrical facial weakness (L > R), opthalmoplegia and truncal ataxia without areflexia. MRI brain was normal. CSF analysis showed elevated protein. Electromyography/Nerve Conduction Study (EMG/NCS) within the first week was normal. Antiganglioside antibodies were negative. Extended serological and neoplastic workup revealed negative anti-GQ1b antibody, but significant increase of GAD-Ab, Voltage Gated Calcium Channel (VGCC) Ab, and mild elevation of TPO Ab IgG and Thyroglobulin (Tg) Ab IgG. Clinical diagnosis of partial MFS was made. He received a course of IVIg (2 g/kg over 5 days) and had complete recovery in 3 months. There are incomplete or atypical forms of MFS. Recognition of its various clinical presentations is essential for early diagnosis and optimal management. Further investigation is needed to elucidate the role of anti-GAD-ab and other autoimmune antibodies in the pathogenesis of GQ1b-seronegative MFS patients. [ABSTRACT FROM AUTHOR]

Maxillary swing approaches provide excellent exposure of the anterior, mid, and anterolateral skull base offering a wide window to approach nasopharyngeal neoplasms; however, they are also associated with complications. The present study aimed to evaluate the results of a modified total maxillary swing (TMS) approach developed to minimize postoperative complications. The modified TMS approach was used to treat five patients who had extensive juvenile nasopharyngeal angiofibromas between March and October 2019 at our tertiary care center. Surgical technique, preoperative image findings, and intra-operative findings were recorded. In the postoperative follow-up, patients were examined to rule out all possible complications associated with the procedure according to the literature. A retrospective analysis was performed to assess tumor extensions, surgical modifications, and postoperative complications. All tumors had orbital and infratemporal (lateral limit) involvement while four had intracranial involvement. No per-operative complications were reported, and postoperative clinical and endoscopic evaluation was performed at 1, 2, and 3 months. There was no evidence of complications including residue, recurrence, maxillary necrosis, ophthalmoplegia, epiphora, palatal fistula, or jaw malocclusion. Besides, minor complications such as infraorbital margin skin retraction, infraorbital serous collection, maxillo-zygomatic abscess, and unsightly scar were also not seen. Only one case presented with maxillary osteomyelitis which was resolved with mini-plate removal and antibiotics. A modified TMS approach is a prudent option to ensure complete removal of juvenile nasopharyngeal angiofibromas with negligible complications. [ABSTRACT FROM AUTHOR]

Mucoceles are benign, encapsulated, expansible, locally invasive masses within a paranasal sinus filled with mucus and lined by epithelium. Sphenoid sinus mucoceles are relatively rare, accounting for only 1% of all paranasal sinus mucoceles. Usually presenting with headache but can rarely result in visual defect, diplopia and at times, ptosis. We present two cases of sphenoid sinus mucocele where patient presented with ophthalmoplegia, ptosis and/or sudden onset vision loss. With the help of imaging studies like CT scan and MRI of paranasal sinus diagnosis was made and immediate surgical intervention was planned which resulted in acceptable improvement of symptoms and vision. [ABSTRACT FROM AUTHOR]

Eight-and-a-half syndrome (EHS) is a neuro-ophthalmological condition characterised by horizontal gaze palsy, internuclear ophthalmoplegia, and ipsilateral facial palsy. Albeit rare, EHS is a well reported condition in the literature, with several reports presenting multiple aetiologies. Infarcts are the cause in more than half the cases. Human immunodeficiency virus (HIV)-related cases are rare, and are probably underreported in low- and middle-income countries. In this report, we describe EHS secondary to neurotoxoplasmosis in a 40-year-old HIV-positive Brazilian man. EHS secondary to neurotoxoplasmosis is a challenging diagnosis, with important differential diagnoses, notably for HIV patients. [ABSTRACT FROM AUTHOR]

Purpose: To investigate changes in peripapillary and macular vessel density (VD) in vascular paralytic strabismus using optical coherence tomography angiography (OCTA). Methods: Medical records of patients who recovered from monocular vascular paralytic strabismus were retrospectively analyzed. Age, sex, presence of underlying diseases, strabismus type and severity, time to recovery, and visual acuity at diagnosis were evaluated. VD in the optic disc area and macular capillary plexus density were estimated using OCTA. The effect of paralytic strabismus on intraocular VD was investigated by comparing VD between the paralysis and contralateral eyes. To analyze hemodynamic changes, VD changes in the paralysis eye during the attack and recovery were compared. Results: Thirty-one patients (mean age, 64.1±13.0 years; 21 males, 10 females) were included and mean recovery time was 3.0±1.6 months. The most common paralysis was sixth nerve palsy (54.8%). When comparing OCTA results between the paralysis and contralateral non-paralysis eyes, foveal VD in the superficial capillary plexus (SCP) was significantly lower in the paralysis eye (P = 0.034); however, VD in the optic disc area was not different. In the paralysis eye, foveal VD in the SCP significantly increased after paralysis recovery (P = 0.04). During attack, the maximal deviation angle and severity of duction limitation were significantly related to foveal VD in SCP. The greater the deviation angle and the more severe the eye movement restriction, the lower the foveal VD in SCP. Conclusions: Transient retinal ischemia of the paralysis eye was observed in a patient with paralytic strabismus, which corresponded to the degree of deviation angle and ocular motor restriction. Ischemic factors, which are the etiology of vascular paralytic strabismus, affect intraocular blood flow. [ABSTRACT FROM AUTHOR]

Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is an uncommon ocular motility disorder that encompasses the following clinical signs: bilateral adduction deficits, bilateral abducting nystagmus, convergence lost, and a large angle exotropia in primary gaze. Here we report a case of a 55-year-old man presenting with atypical WEBINO syndrome with unilateral exotropia. The coverage test was used to record the patient's alternating exotropia. The patient experienced diplopia and ophthalmoplegia and was admitted to our hospital 3 days after the onset of the double vision. Neurologic examination showed left eye exotropia and bilateral internuclear ophthalmoplegia with impaired convergence. Vertical saccades of the left eye were also limited. Consequently, an MRI scan suggested an acute infarction in the left of the pontine tegmentum. The patient was finally diagnosed with pons infarction and was treated with anticoagulation and anti-platelet aggregation therapy. [ABSTRACT FROM AUTHOR]

In central as well as peripheral vestibular lesions, right-beating horizontal nystagmus is almost always associated with clockwise (top poles of the eyes beating to the right ear) torsional nystagmus when observed and vice versa (concordant nystagmus). This study aimed to determine the etiologies and mechanisms of horizontal and torsional nystagmus beating in the opposite directions (discordant nystagmus). We reviewed the medical records of 16 consecutive patients with discordant horizontal–torsional nystagmus who had been evaluated at the dizziness clinics of Seoul National University Bundang Hospital (n = 11, from March 2003 to March 2021) and Korea University Medical Center (n = 5, from March 2019 to March 2021). The underlying etiologies included inferior vestibular neuritis (n = 7), Meniere's disease (n = 4), internuclear ophthalmoplegia (n = 3), medullary hemorrhage (n = 1), and normal pressure hydrocephalus (n = 1). The torsional nystagmus decreased during the gaze in the same direction (for instance, during rightward gaze in clockwise nystagmus) and increased during the gaze in the opposite direction. Head-impulse tests (HITs) were positive for the ipsilesional posterior canal (PC) in all 11 patients with unilateral peripheral vestibulopathy and two of the three patients with unilateral central vestibulopathy. Discordant horizontal–torsional nystagmus may be observed in peripheral as well as central lesions. Given the findings of HITs and modulation of spontaneous nystagmus during lateral gazes, discordant horizontal–torsional nystagmus may be ascribed to selective damage of the excitatory or inhibitory pathway from the PC that innervates the ipsilateral superior oblique and contralateral inferior rectus muscles. [ABSTRACT FROM AUTHOR]

Introduction  Pituitary apoplexy is an uncommon clinical condition that can require urgent surgical intervention, but the factors resulting in recurrent apoplexy remain unclear. The purpose of this study is to determine the risks of a recurrent apoplexy and better understand the goals of surgical treatment. Methods  A retrospective chart review was performed for all consecutive patients diagnosed and surgically treated for pituitary apoplexy from 2004 to 2021. Univariate analysis was performed to identify risk factors associated with recurrent apoplexy. Results  A total of 115 patients were diagnosed with pituitary apoplexy with 11 patients showing recurrent apoplexy. This occurred at a rate of 2.2 cases per 100 patient-years of follow-up. There were no major differences in demographic factors, such as hypertension or anticoagulation use. There were no differences in tumor locations, cavernous sinus invasion, or tumor volumes (6.84 ± 4.61 vs. 9.15 ± 8.45 cm, p  = 0.5). Patients with recurrent apoplexy were less likely to present with headache (27.3%) or ophthalmoplegia (9.1%). Recurrent apoplexy was associated with prior radiation (0.0 vs. 27.3%, p  = 0.0001) and prior subtotal resection (10.6 vs. 90.9%, p  = 0.0001) compared with first time apoplexy. The mean time to recurrent apoplexy was 48.3 ± 76.9 months and no differences in overall follow-up were seen in this group. Conclusion  Recurrent pituitary apoplexy represents a rare event with limited understanding of pathophysiology. Prior STR and radiation treatment are associated with an increased risk. The relatively long time from the first apoplectic event to a recurrence suggests long-term patient follow-up is necessary. [ABSTRACT FROM AUTHOR]