Junctional visual field loss: a reappraisal of nomenclatureHistorically, junctional visual field loss (JVFL) has been associated with a lesion at the junction of the optic nerve and chiasm, and the most common etiologies are neoplasm and aneurysm.1,2 We present 2 cases of JVFL and review the nomenclature on the various types of scotomas associated with lesions at the junction of the optic nerve and chiasm (e.g., junctional scotoma and junctional scotoma of Traquair). Based on our review of the English-language ophthalmic literature, we believe that our cases are unique and serve to demonstrate the need for expansion of the classification of JVFL.
Giant cell arteritis relapse presenting as idiopathic orbital inflammationGiant cell arteritis (GCA) is a systemic vasculitis that can cause permanent vision loss. Even after treatment, relapses have been reported in 40%–64% of patients, depending on how relapses are defined.1,2 Here, we present a rare case of a GCA relapse mimicking idiopathic orbital inflammation (IOI) on the side contralateral to the initial episode of the disease. Simultaneous, positive biopsies of the orbit and temporal arteries were obtained, offering histological evidence that the orbital inflammation and GCA flare were linked.
Chronic myelomonocytic leukemia-related vasculitis mimicking giant cell arteritisGiant cell arteritis (GCA) is a medium-to-large vessel vasculitis, commonly seen in the elderly, that can produce visual loss.1 Although the diagnosis of GCA is typically made via a temporal artery biopsy (TAB), other vasculitides have been diagnosed by TAB in patients with symptoms and signs initially suggestive of GCA. Chronic myelomonocytic leukemia (CMML) is a myeloid neoplasm characterized by persistent monocytosis, macrocytic or normocytic anemia, and thrombocytopenia.2 We report an unusual case of CMML-associated vasculitis mimicking GCA.
Primary central nervous system lymphoma of the optic chiasmPrimary CNS lymphoma (PCNSL) is a rare form of extranodal, high-grade, non-Hodgkin lymphoma (NHL), accounting for approximately 3% of newly diagnosed brain tumors and 2 to 3% of all NHL.1 PCNSL can also arise from posttransplant lymphoproliferative disorder (PTLD), a disorder in patients with secondary immunodeficiency after a solid organ or hematopoietic stem cell transplant.2 PCNSL is characterized by rapid growth usually confined to the CNS and can originate in the brain, leptomeninges, spinal cord, or eyes.
Elevated lipoprotein(a) levels as the cause of cryptogenic stroke in a young Ashkenazi Jewish femaleLipoprotein(a) is a cholesterol carrier molecule, structurally similar to low-density lipoprotein (LDL), but with distinct properties predisposing it to associations with increased risk of stroke, myocardial infarction (MI), and aortic stenosis.1–4 However, lipoprotein(a) levels are not routinely checked, although there is a known pattern of genetic inheritance.5 We present a case of a young patient with purported cryptogenic stroke and a family history of cardiovascular disease, which was attributed to elevated lipoprotein(a) levels.
Triple-hit lymphoma of the cavernous sinusDiffuse large B-cell lymphoma (DLBCL) is the most common non–Hodgkin-type lymphoma in adults, and the myriad of clinical presentations is well known to neuro-ophthalmologists.1 Traditionally, DLBCL was treated with standard chemotherapy, including R-CHOP (i.e., rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone). Newer cytogenetic, fluorescent in situ hybridization (FISH), and next-generation sequencing (NGS) techniques, however, have shown the presence of genomic abnormalities that have significant treatment and prognostic implications.
Vestibulocochlear symptoms as the initial presentation of giant cell arteritisGiant cell arteritis (GCA)—also known as temporal arteritis—is a systemic vasculitis that involves medium and large-caliber arteries such as the cranial arteries, great vessels, and aorta. Although the typical nonocular features of GCA are well known (e.g., new-onset headache, scalp tenderness, jaw claudication), vestibulocochlear presentations are rare and can mimic acute idiopathic sensorineural hearing loss (AISHL).1 Although steroids are given for both AISHL and GCA, the lower dose and shorter duration of steroid treatment in AISHL can obscure and delay the diagnosis of GCA.
Homonymous hemianopsia as the presenting sign of migrainous infarctionMigrainous infarction is a rare complication of migraine headaches that accounts for 0.2%−0.5% of ischemic strokes.1,2 This condition frequently occurs in the posterior cerebral circulation and typically affects a single vascular territory.1,2 The most common symptom preceding an acute migrainous infarction is a visual aura (82.3%) that resolves within minutes to hours.1 Visual field defects like partial or complete homonymous hemianopsia (HH) after a migrainous infarction have been reported, but the prevalence is unknown.