Eight-and-a-Half Syndrome Secondary to Acute Brainstem Infarction.

Eight-and-a-half syndrome is a rare neuro-ophthalmologic condition, which is characterized by ipsilateral horizontal gaze palsy, internuclear ophthalmoplegia (INO), and ipsilateral lower motor neuron facial palsy. We report a case of eight-and-a-half syndrome secondary to acute brainstem infarction. A 55-year-old gentleman with underlying diabetes mellitus and hypertension presented with a sudden onset of double vision in the right lateral gaze for one day. On examination, there was a limitation in the left eye horizontal eye movement with limited right eye adduction. Further neurological examination revealed left lower motor neuron facial nerve palsy. Magnetic resonance imaging (MRI) of the brain showed an acute infarct involving the left side of the thalamus extending to the left side of the midbrain, pons, and medulla. He was diagnosed with eight-and-a-half syndrome secondary to acute brainstem infarction. The patient was referred to the neuromedical team, where he was treated with anti-platelet medications. He showed gradual improvement on follow-up and had complete resolution of ophthalmoplegia after three months. There was only minimal residual facial nerve weakness. Eight-and-a-half syndrome has a localizing value to the dorsal tegmentum of the pons. It requires thorough neurological examination and imaging studies for accurate diagnosis and management. This case highlights the potential for a significant recovery in patients with eight-and-a-half syndrome when timely and appropriate treatment is administered.

Neuro-Behçet's Syndrome Without Genital Ulcers: A Case Report and Literature Review.

Behçet's disease is a rare multisystemic vasculitis characterized by oral ulcers, genital ulcers, and skin and ocular lesions. Neuro-Behçet's syndrome is a condition in which individuals with Behçet's disease experience neurological symptoms that cannot be attributed to other neurological diseases. We present a rare case of neuro-Behçet's syndrome with acute internuclear ophthalmoplegia and deteriorating neurological function with a prior history of recurrent oral ulcers with pathergy-like features, acneiform papulopustular rash, retinal hemorrhages, and recurrent epididymitis without genital ulcers. Patient improved with cyclophosphamide. This case underscores the importance of diagnosing and managing neuro-Behçet's syndrome in the absence of genital ulcers.

Internuclear Ophthalmoplegia Following COVID-19 with Abnormal Findings on Neuroimaging; A Case Report.

COVID-19 had been reported to be associated with neuro-ophthalmic complications. However, there is a shortage of evidence regarding internuclear ophthalmoplegia (INO) presentation after COVID-19 infection with related brain magnetic resonance imaging (MRI). Herein, we report a case of INO in a 69-year-old woman with presentation of diplopia following COVID-19 infection, along with her neuroimaging. We concluded that it is important to recognize the possibility of INO in patients with diplopia after COVID-19 and to consider brain MRI as an informative tool in these patients.

Medial longitudinal fasciculus (MLF)-syndrome in a multimorbid patient with alcohol use disorder: a case report.

Background: Patients with multiple comorbidities can present as a diagnostic challenge as overlapping symptomatology complicates the discovery of emergent pathology. Symptoms of alcohol misuse or orthostatic hypotension may especially cloud the diagnosis of insidious neurological disease, such as posterior circulation infarct. With a growing elderly population, it is expected that the complex multimorbid patient will represent a growing challenge to prompt stroke detection and treatment. Case Description: Herein, we present a 69-year-old male with a history of alcohol abuse, chronic obstructive pulmonary disease, type 2 diabetes mellitus, paroxysmal atrial fibrillation, and congestive heart disease. The patient arrived at our emergency department with dizziness, ataxia, and diplopia. His symptoms had a sudden onset and gradual exacerbation over a span of 2 days, notably aggravated by standing and walking, but relieved when seated or supine. Notably, a month before admission, the patient had been treated with anti-congestive medications for severe congestive heart failure leading to a weight loss of 55 lbs over period of 2 weeks. The initial differential diagnoses were orthostatism, Wernicke's encephalopathy (WE), and ischemic stroke. Magnetic resonance imaging (MRI) revealed a subacute infarct in the medial longitudinal fasciculus (MLF). Conclusions: The case underscores the challenge in diagnosing neurological conditions in multimorbid individuals. The combination of various underlying conditions may drastically complicate the diagnosis. Successful diagnosis and treatment necessitates meticulous evaluation of clinical observations, medical history, current medications, and pertinent diagnostic evaluations to effectively narrow down the potential differential diagnoses.

[Wernicke encephalopathy : a neuro-ophthalmological side effect of bariatric surgery]. / L'encéphalopathie de Gayet-Wernicke : une complication neuro-ophtalmologique de la chirurgie bariatrique.

Due to an increase in the worldwide prevalence of obesity and the efficiency of bariatric surgery, this procedure is more often performed. Besides its benefits, it has also disadvantages and may be the cause of nutritional deficiencies. Thiamin deficiency is particularly important to diagnose and to treat early as it can lead to major sequelae and even to death. Wernicke's encephalopathy is the most frequent presentation associating confusion, ataxia, ophtalmoplegia and nystagmus. The full triad is not usually observed, which may lead to sub-diagnosis of this affection. The diagnosis is clinical, biological and radiologic thanks to the brain MRI. Intravenous thiamin supplementation therapy must be administered as fast as possible in order to avoid long-term damages. In the ophthalmological field, the potential sequelae are ophthalmoplegia, nystagmus and optic neuropathy. Therapeutics for nystagmus are pharmacological, surgical and/or optical. We illustrate this condition with a case report of an 18-year-old man developing Wernicke's encephalopathy as early as six weeks after a sleeve gastrectomy.

Suite à une augmentation de la prévalence de l'obésité dans le monde et à l'efficacité de la chirurgie bariatrique, cette technique est pratiquée de plus en plus fréquemment. Malgré ses avantages, elle n'est pas sans risque et peut être responsable de déficits nutritionnels multiples. Le déficit en vitamine B1 ou thiamine est particulièrement important à connaître et, à rapidement diagnostiquer en raison des nombreuses séquelles invalidantes, voire le décès du patient, dont il peut être responsable. Le tableau classique est l'encéphalopathie de Gayet-Wernicke associant confusion, ataxie et troubles oculomoteurs. Néanmoins, il n'est pas toujours complet, ce qui participe au sous-diagnostic de cette pathologie. Le diagnostic est clinique, biologique et/ou radiologique grâce à l'IRM cérébrale. La supplémentation vitaminique intraveineuse doit être instaurée le plus rapidement possible afin d'éviter des séquelles à long terme. D'un point de vue ophtalmologique, les séquelles potentielles sont les ophtalmoplégies, les nystagmus et les neuropathies optiques. Les thérapies envisageables du nystagmus, outre la supplémentation en thiamine en aigu, sont pharmacologiques, chirurgicales et/ou optiques. Nous illustrons cette pathologie par un cas clinique d'encéphalopathie de Gayet-Wernicke dès la 6ème semaine post-opératoire d'une chirurgie bariatrique de type «sleeve¼ chez un patient de 18 ans.

Exploring Treatments for a Rare Guillain-Barré Variant: A Case Report of Miller-Fisher Syndrome.

The symptoms of Miller-Fisher syndrome (MFS) are a triad of areflexia, ataxia, and ophthalmoplegia. The condition is a rare variant of Guillain-Barré syndrome (GBS), an acute immune-mediated nerve disorder. Both conditions involve abnormal autoimmune responses that may often be triggered by infections such as Campylobacter jejuni, human immunodeficiency virus, Epstein-Barr virus, and Zika virus, among others. As a result, the immune system mistakenly attacks the body's own nerve tissues. MFS is characterised by ophthalmoparesis, which can progress to complete external ophthalmoplegia and may include ptosis, facial nerve paralysis, sensory impairments, and muscle weakness. Diagnosis is supported by lumbar puncture, revealing albumin-cytologic dissociation, although initial tests may not always be indicative. A diagnostic marker for MFS is the presence of anti-GQ1b antibodies, which target the GQ1b ganglioside in nerves and affect oculomotor function in particular. Electrodiagnostic studies often show absent or reduced sensory responses without reduced conduction velocity. Treatment options include intravenous immunoglobulin therapy and plasmapheresis, which are both equally effective. This case study demonstrated significant clinical improvement in a patient undergoing plasmapheresis due to financial constraints, highlighting the efficacy of this treatment approach. A 50-year-old female presented with limb paraesthesia, progressive ptosis, imbalance, and transient diplopia following a recent fever. Examination revealed stable vitals, decreased deep tendon reflexes, reduced vibratory sensation, cerebellar ataxia, and cranial nerve abnormalities. Cerebrospinal fluid analysis showed elevated protein, suggesting MFS. Normal magnetic resonance imaging and nerve conduction studies indicated GBS, with positive anti-GQ1b antibodies. After five plasma exchange cycles, the patient improved substantially and was discharged with no residual symptoms after one month.

Painful Diplopia as an Initial Presentation of VEXAS Syndrome.

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.

Evaluation of Patients with Painful Ophthalmoplegia for Benign and Secondary Etiologies.

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.

Metastasis to the skull base involving the sphenoid and cavernous sinus in Hepatocellular carcinoma: A case report.

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.

Tolosa-Hunt Syndrome as the Initial Presentation of Systemic Lupus Erythematosus.

Tolosa-Hunt syndrome (THS), also known as painful ophthalmoplegia, recurrent ophthalmoplegia, or ophthalmoplegia syndrome, is described as severe and unilateral peri-orbital headaches associated with painful and restricted eye movements. THS is an uncommon disorder due to granulomatous inflammation of the cavernous sinus. Although THS is primarily idiopathic, it has rarely been reported in association with systemic lupus erythematosus (SLE). This case report describes a unique case of THS presenting as the initial manifestation of SLE, a multi-system autoimmune disease. We present a detailed case report of a 54-year-old female patient who presented with THS with the classical symptoms of THS including unilateral headache, double vision, and orbital pain. A cranial nerve examination revealed right oculomotor nerve palsy with the inability to adduct, raise, or depress her right eye. A detailed clinical examination revealed alopecia areata and erythematous macular lesions on her right earlobe. Laboratory investigations were unremarkable except for an increased erythrocyte sedimentation rate (ESR). Diagnostic investigations, including MRI and serological tests, were conducted to explore the underlying causes and systemic involvement. The patient's MRI showed characteristic findings consistent with THS, while serological tests revealed positive antinuclear antibodies, anti-ds-DNA antibodies, and anti-Smith antibodies and low complement levels leading to a concurrent diagnosis of SLE. There were no other systemic manifestations of lupus at the time of presentation.  Treatment with high-dose corticosteroids led to rapid improvement in ocular symptoms and headaches. Maintenance immunosuppressive therapy was initiated for the management of SLE. The patient had no relapses on follow-up. This case report underscores THS as a potential initial manifestation of SLE. It highlights the need for comprehensive diagnostic evaluation in patients presenting with atypical cranial neuropathy to consider systemic autoimmune disorders like SLE. Early diagnosis and management are crucial for improving outcomes in such intertwined pathologies. This case emphasizes the need for clinicians to be aware of the possibility of THS as the initial manifestation of SLE. This extended abstract provides a comprehensive overview of the article, laying out the significance of the case in broadening the clinical understanding of the overlap between localized inflammatory syndromes and systemic autoimmune conditions like SLE.

Vertical "Half-and-Half" Syndrome with Ipsilateral Pseudoabducens Palsy due to Rostral Midbrain Lacunar Infarction.

Vertical "half-and-half" syndrome, characterized by contralateral upward and ipsilateral downward gaze palsy, is a rare variant of vertical eye movement disturbance. Similarly, pseudoabducens palsy, manifesting as abductive palsy despite no lesion to the pons, constitutes another rare type of eye movement disturbance. Both conditions have been associated with lesions in the thalamo-mesencephalic junction. We present a rare case report detailing a patient exhibiting vertical "half-and-half" syndrome with ipsilateral pseudoabducens palsy following a left lacunar infarction of the thalamo-mesencephalic junction. Additionally, we discuss the potential underlying mechanisms contributing to this rare combination of eye movement disorders.

A Case of Congenital Idiopathic Enlargement of Extraocular Muscles.

The purpose of this case report is to report a case of congenital idiopathic enlargement of extraocular muscles. A four-month-old girl showed limitation of adduction and supraduction in the right eye. A computerized axial tomography (CAT) scan revealed hypertrophy of the lateral rectus muscle and inferior rectus muscle of the right eye. Thyroid hormone and antibody levels were normal. No inflammatory findings on magnetic resonance imaging (MRI). A traction test under general anesthesia revealed a strong limitation of supraduction and a mild limitation of adduction. Therefore, the inferior rectus muscle was recessed 4.5 mm at the age of six months. A partial biopsy of the inferior rectus showed no inflammatory cell infiltration. After the first surgery, the patient's limitation of supraduction improved, but the limitation of adduction persisted. So, a 5 mm recession of the right lateral rectus muscle was added at one year and one month. However, the hypertropia of the sound eye became stronger after treatment of amblyopia. Because of the strong limitation of supraduction, tenotomy of the inferior rectus was performed at the age of six years. Postoperatively, no impairment of infraduction occurred, and the limitation of supraduction was mildly improved. Since the findings on MRI were not changed through our observation period, we concluded that the patient had idiopathic external ophthalmoplegia.

Identification of a Novel Frameshift Variant in MYF5 Leading to External Ophthalmoplegia with Rib and Vertebral Anomalies.

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.

Successful Management of Acute Streptococcal Meningoencephalitis Complicated by Bilateral Third-Nerve Palsies, Wall-Eyed Bilateral Internuclear Ophthalmoplegia, Blindness, and Deafness: Case Report.

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.

Abducens Nerve Palsy as a Rare Complication of Herpes Zoster Ophthalmicus: A Case Report.

Herpes zoster ophthalmicus (HZO) is a condition resulting from the reactivation of dormant varicella zoster virus within the sensory nerve ganglion in the ophthalmic branch of the trigeminal nerve. The tell-tale rash along one side of the nerve tract accompanied by pain, a burning sensation, and itching alerts health practitioners on the right path to diagnosis. Conversely, HZO can present with other rarer complications such as intraocular and extraocular manifestations. This case report deals with a seemingly healthy 45-year-old female who developed left abducens nerve palsy within one week of developing a vesicular rash on the same side. Curiously, those afflicted are usually of an advanced age or suffer from an immunocompromised state; this patient however suffered from no other comorbidities nor did she report having been in contact with anyone of a similar affliction. In this case, the classical treatment regime of antivirals and corticosteroids resulted in the complete resolution of the infection and the return of full ocular function. Being able to recognize and appreciate these typical and atypical signs and symptoms of HZO can aid in the further propagation of good outcomes and timely resolutions.

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

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

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

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

Painful Eyes in Neurology Clinic: A Guide for Neurologists.

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

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

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

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

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