Neuro-ophthalmic challenges and multi-morbidity in vasculitis among the older adults.

INTRODUCTION: Vasculitides are a heterogeneous group of disorders producing inflammation of blood vessels (e.g. arteries or veins). All major vasculitides potentially have ophthalmological symptoms and signs including visual loss. Co-morbidity, multimorbidity, polypharmacy, and geriatric syndromes all play important roles in patient outcomes for these rheumatic conditions in the elderly. This monograph reviews the NCBI PubMed database (Feb 2023) literature on the neuro-ophthalmic and geriatric considerations in vasculitis. AREAS COVERED: Cogan Syndrome, Granulomatosis with Polyangiitis, Giant Cell Arteritis, Polyarteritis Nodosa, Takayasu Arteritis, Vasculitis epidemiology, and neuro-ophthalmological symptoms. EXPERT OPINION: Geriatric patient care for vasculitis with neuro-ophthalmological manifestations can be complicated by the interplay of multiple co-morbidities, polypharmacy, and specific geriatric syndromes. The valuation and treatment of vasculitis and the complications associated with the disease can negatively impact patient care. Advances in noninvasive imaging and updates in diagnostic criteria have enabled increased identification of patients at earlier stages with less severe disease burden. Novel therapeutic agents can be glucocorticoid sparing and might reduce the adverse effects of chronic steroid use. Holistic care models like the 5 M geriatric care model (mind, mobility, medications, multicomplexity, and matters most) allow patients' needs to be in the forefront with biopsychosocial aspects of a patient being addressed.

Monoclonal antibodies in neuro-ophthalmology.

Neuro-ophthalmologic diseases include a broad range of disorders affecting the afferent and efferent visual pathways. Recently, monoclonal antibody (mAb) therapies have emerged as a promising targeted approach in the management of several of these complex conditions. Here, we describe the mechanism-specific applications and advancements in neuro-ophthalmologic mAb therapies. The application of mAbs in neuro-ophthalmologic diseases highlights our increasing understanding of disease-specific mechanisms in autoimmune conditions such as neuromyelitis optica, thyroid eye disease, and myasthenia gravis. Due to the specificity of mAb therapies, applications in neuro-ophthalmologic diseases have yielded exceptional clinical outcomes, including both reduced rate of relapse and progression to disability, visual function preservation, and quality of life improvement. These advancements have not only expanded the range of treatable neuro-ophthalmologic diseases but also reduced adverse events and increased the response rate to treatment. Further research into neuro-ophthalmologic disease mechanisms will provide accurate and specific targeting of important disease mediators through applications of future mAbs. As our understanding of these diseases and the relevant therapeutic targets evolve, we will continue to build on our understanding of how mAbs interfere with disease pathogenesis, and how these changes improve clinical outcomes and quality of life for patients.

Stress-Induced Oculogyric Crisis in Septo-Optic Dysplasia: Case Report.

Introduction: Oculogyric crisis (OGC) is a dystonic movement disorder of varying durations that manifests as bilateral paroxysmal upward eye deviation accompanied by involuntary blinking, tongue protrusion, and autonomic symptoms. Separately, septo-optic dysplasia (SOD) is a congenital disorder involving hypoplasia of the optic nerve as well as hypothalamic and pituitary abnormalities. In the presented case, we report a case of OGC in the setting of SOD with proposed pathogenesis. Case Presentation: A 27-year-old female presented with a history of SOD (optic nerve hypoplasia and hypopituitarism) with acute, recurrent, painless, bilateral, intermittent, simultaneous tonic conjugate upward eye deviation (i.e., OGC) and dystonic body posturing. She experienced her first episode upon meeting her biological sister for the first time at a loud, crowded public restaurant with continued episodes of OGC increasing in frequency and duration over the subsequent months. She later responded well to treatment with carbidopa/levodopa. Conclusion: Based on our current understanding of OGC, we hypothesize that acute stressful life events in the setting of prior hypothalamic-pituitary axis dysfunction secondary to SOD could lower the threshold for developing OGC. Although most cases of OGC are idiopathic, various etiologies including medications, stress, and hormonal imbalance have been postulated as possible pathogenic mechanisms. We describe a case of SOD with OGC, and based upon our review of the English language ophthalmic literature, we believe that our case is novel.

Transient Monocular Visual Loss (Amaurosis Fugax): How Does Age Impact Diagnosis?

Transient monocular visual loss (TMVL), also known as transient monocular blindness or amaurosis fugax ("fleeting blindness"), is a temporary loss of vision often due to ischemia to the retina. While acute TMVL should be considered an emergency that further requires exhaustive investigation, there are some cases in which TMVL arises secondary to benign causes. Age has a major impact in the diagnosis of ischemia and although the differential diagnosis of TMVL can be broad, timely and appropriate history, examination, diagnostic testing, and treatment can be vision- or life-saving. We review the causes of TMVL and the impact of age on the differential diagnoses and management.

Bilateral medial medullary stroke: "The heart sign".

Bilateral medial medullary stroke is a very rare condition. It is usually associated with severe motor deficits, dysarthria, sensory symptoms, nystagmus, and respiratory disturbances. The most common etiology is atherosclerotic disease of the vertebral and anterior spinal artery or its branches. We present the case and brain imaging of a 48-year-old man with a bilateral medial medullary stroke and its classic "Heart Sign" in the magnetic resonance imaging. This case highlights the anatomy of the rostral medulla, clinical presentation, etiology, and characteristic radiologic findings of this uncommon type of stroke.

Neuro-ophthalmic manifestations of Ehlers-Danlos syndrome.

PURPOSE OF REVIEW: To review the neuro-ophthalmic manifestations of Ehlers-Danlos syndrome (EDS). RECENT FINDINGS: Ehlers-Danlos syndrome (EDS) is a rare genetic disorder with an estimated prevalence of 1 in 5000 individuals, but its true prevalence may be underestimated because of variable clinical presentations and limited awareness among healthcare professionals. The neuro-ophthalmic features of EDS may be difficult to recognize in context but new molecular genetic testing is now available for identification of specific subtypes of EDS. SUMMARY: Ophthalmic manifestations of EDS include loss of vision and double vision (strabismus), high myopia, retinal detachment, and blue sclera. The vascular subtype of EDS can present as a carotid-cavernous fistula, intracranial aneurysm, or arterial dissection.

Paracentral Bitemporal Hemianopsia and Acquired Supranuclear Ocular Motor Paresis Following Cardiac Surgery.

Acquired supranuclear ocular motor paresis (ASOMP) is a rare complication following cardiac surgery, characterized by limited voluntary eye motility. The exact cause and development of ASOMP after cardiac surgery remain unclear. We report a case of ASOMP with paracentral bitemporal hemianopsia with optic atrophy after cardiac surgery, which, to our knowledge, is novel. The patient demonstrated bilateral ophthalmoplegia, with gradual improvement in voluntary smooth pursuit but persistent impairment in saccadic eye movements. Interestingly, the patient showed improved proprioceptive-driven pursuit of their own hand compared to pursuit or saccades following an examiner's hand. The visual field examination revealed a bilateral paracentral temporal hemianopic field defect. The underlying mechanisms of ASOMP and potential chiasmal ischemia in this case remain unknown. Clinicians should be aware of the possibility of ASOMP following cardiac surgery, with potential slow improvement over time.

Lymphocytic Panhypophysitis Mimicking Glaucoma: Case Report.

Lymphocytic hypophysitis (LH) is a primary inflammatory disorder of the pituitary gland and infundibulum that commonly manifests in both mass effect and endocrinologic symptoms. Although the exact pathophysiology remains unclear, it has been increasingly linked to an autoimmune process. Complications arise by two separate mechanisms. Inflammation in the sella can lead to headaches and visual field defects. Pituitary inflammation and, chronically, fibrosis interfere with the gland's hormone-secreting capacity, often resulting in various endocrinopathies such as polyuria, polydipsia, amenorrhea, and others. While final histologic classification requires pathologic evaluation, diagnosis can often be made clinically with appropriate imaging. Treatment often consists of conservative management but can also include glucocorticoids or surgical resection. We present a case of biopsy-proven LH involving the entire pituitary, dubbed lymphocytic panhypophysitis (LPH) that was misdiagnosed for years as glaucoma due to the lack of endocrinopathy as well as delay in magnetic resonance imaging. After imaging revealed the sellar mass, the patient responded symptomatically to surgical resection and glucocorticoid treatment. LPH may present without endocrinologic symptoms and therefore mimic alternate diagnoses such as glaucoma. Clinicians should be suspicious of a diagnosis of glaucoma in the setting of a temporal field defect and lack of response to traditional therapy. A personal or family history of autoimmune disease in such patients should prompt further imaging and investigation. Therefore, endocrinopathy is supportive but not present in every case of LPH.