Current developments in the diagnosis and treatment of giant cell arteritis

Giant cell arteritis is the most common vasculitis in adults above 50 years old. The disease is characterized by granulomatous inflammation of medium and large arteries, particularly the temporal artery, and is associated acutely with headache, claudication, and visual disturbances. Diagnosis of the disease is often complicated by its protean presentation and lack of consistently reliable testing. The utility of color doppler ultrasound at the point-of-care and FDG-PET in longitudinal evaluation remain under continued investigation. Novel techniques for risk assessment with Halo scoring and stratification through axillary vessel ultrasound are becoming commonplace. Moreover, the recent introduction of the biologic tocilizumab marks a paradigm shift toward using glucocorticoid-sparing strategies as the primary treatment modality. Notwithstanding these developments, patients continue to have substantial rates of relapse and biologic agents have their own side effect profile. Trials are underway to answer questions about optimal diagnostic modality, regiment choice, and duration

Opinion article: Neurosurgical treatment for neuro-ophthalmologic conditions

A significant role of the neuro-ophthalmologist is to counsel patients on appropriate management and anticipated visual prognosis for conditions affecting the afferent and efferent visual systems, including those requiring neurosurgical treatment. However, the literature regarding anticipated neuro- ophthalmologic prognosis after neurosurgical intervention for cerebral aneurysms, sellar lesions, optic pathway tumors, and elevated intracranial pressure is limited with many key questions unanswered. For example, if a cerebral aneurysm is equally amenable to clipping or endovascular coiling, is there a preferred approach in terms of visual prognosis based on aneurysm location? Is dural venous sinus stenting (VSS) for idiopathic intracranial hypertension (IIH) superior, equivalent or inferior to shunting in terms of visual recovery and safety profile? Landmark studies on pituitary tumors using pre-operative optical coherence tomography (OCT) imaging of the optic nerve head to predict visual recovery after surgical decompression of the optic chiasm have changed neuro-ophthalmologic practice and enabled patients to be better informed regarding expected visual outcomes. 1,2 In order to optimize an interdisciplinary team approach to patient care, further studies of visual outcomes for neuro- ophthalmologic conditions requiring neurosurgical intervention are needed

Orbital Inflammation in Boniva (Ibandronate)

Orbital Inflammatory Syndrome (OIS) is a benign, inflammatory process that is typically confined to the orbit although can involve extra-ocular structures. The pathogenesis of orbital inflammation is controversial and unclear, mainly due to the variety of presentation schema. It can be due to immune, infectious, or inflammatory causes

Biallelic Optic Atrophy 1 (OPA1) Related Disorder—Case Report and Literature Review

Dominant optic atrophy (DOA), MIM # 605290, is the most common hereditary optic neuropathy inherited in an autosomal dominant pattern. Clinically, it presents a progressive decrease in vision, central visual field defects, and retinal ganglion cell loss. A biallelic mode of inheritance causes syndromic DOA or Behr phenotype, MIM # 605290. This case report details a family with Biallelic Optic Atrophy 1 (OPA1). The proband is a child with a severe phenotype and two variants in the OPA1 gene. He presented with congenital nystagmus, progressive vision loss, and optic atrophy, as well as progressive ataxia, and was found to have two likely pathogenic variants in his OPA1 gene: c.2287del (p.Ser763Valfs*15) maternally inherited and c.1311A>G (p.lIle437Met) paternally inherited. The first variant is predicted to be pathogenic and likely to cause DOA. In contrast, the second is considered asymptomatic by itself but has been reported in patients with DOA phenotype and is presumed to act as a phenotypic modifier. On follow-up, he developed profound vision impairment, intractable seizures, and metabolic strokes. A literature review of reported biallelic OPA1-related Behr syndrome was performed. Twenty-one cases have been previously reported. All share an early-onset, severe ocular phenotype and systemic features, which seem to be the hallmark of the disease

Chiasmitis Secondary to Neuromyelitis Optica 25 Years After Initial Optic Neuritis

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Accidental ophthalmic artery Onyx embolisation for meningioma-Vision lost in an attempt to preserve

Presurgical embolization of vascular tumors is relatively common. Embolization via the ophthalmic artery carries risk of blindness from iatrogenic ophthalmic or retinal arterial occlusion. We report a case of visual loss following embolization to devascularize a meningioma via the ophthalmic artery; describe key clinical and radiographic features; and review considerations for prevention of potential visual complications of the procedure

Familial Cavernous Hemangioma

A 30-year-old woman presented with diplopia after resection of an intracranial cavernous malformation. Fundus examination showed an asymptomatic intraocular cavernous hemangioma of the retina. Clinicians should be aware of the potential coexistence of intraocular and intracranial cavernous malformations; the presence of both should suggest familial etiology. As with other intracranial and intraocular vascular lesions (e.g., arteriovenous malformation in Wyburn-Mason syndrome, retinal hemangioblastoma in von Hippel Lindau disease, and choroidal hemangioma in Sturge-Weber syndrome), the presence of a vascular lesion in either location should prompt evaluation for additional pathology

Bilateral Scalp Necrosis in Giant Cell Arteritis

Giant cell arteritis (GCA) is a medium-to-large vessel vasculitis of the elderly. Common constitutional clinical features include headache, scalp tenderness, and jaw claudication. Severe unilateral or bilateral visual loss is the most feared ophthalmic complication of GCA. Scalp necrosis is a known ischemic complication of GCA with approximately 100 cases reported in the literature to date. We report a case of scalp pain and an erythematous cutaneous lesion in the distribution of ophthalmic division of the trigeminal nerve that mimicked herpes zoster ophthalmicus. A temporal artery biopsy was positive for GCA, and small vessel arteritis was seen at the time of simultaneous skin biopsy. To the best of our knowledge, this is the first such report in the English language ophthalmic literature

Acute Vision Loss After Ophthalmic Artery Embolization of Meningioma

A 40-year-old man presented with acute-onset painless visual loss after preoperative embolization for a large skull base meningioma through the ophthalmic artery. We describe the clinical presentation of an isolated ipsilateral afferent and efferent pupillary defect in this patient who had radiographic documentation of embolization material in the intraorbital ophthalmic artery. Although ophthalmic and retinal artery occlusions have been described previously after endovascular embolization, our patient had a unique clinicoradiologic presentation