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Neuromyelitis Optica spectrum disorder (NMOSD) is a relapsing autoimmune disease of the central nervous system (CNS) where aquaporin-4 water channels are the antigenic target of the disease. The spectrum of the disease involves regions of the CNS where the water channel is widely expressed including the spinal cord, the optic nerve, dorsal medulla, brainstem, and thalamus/hypothalamus. Management of NMOSD includes acute as well as long term treatment. Acute symptoms are typically treated with intravenous corticosteroids and/or plasma exchange while long-term treatment involves the use of immunosuppression/immune modulation. The year 2019 is thought to be the "year of the NMOSD" as three new medications became available for this devastating disease. In this review, FDA approved NMOSD medications are discussed.

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BACKGROUND: Tocilizumab (Actemra) is a humanized anti-interleukin-6 receptor antibody that has been used as a steroid-sparing agent in giant cell arteritis (GCA). Although the clinical effects are well described in GCA, the cost-effectiveness of the use of tocilizumab in GCA is ill defined. The purpose of this study was to determine the cost-effectiveness of tocilizumab in GCA compared with prednisone alone. METHODS: A retrospective study of 32 patients with biopsy-proven GCA comparing prednisone alone (16 patients) and prednisone with tocilizumab (16 patients) was performed. The cost for tocilizumab therapy for 26 weeks with mild and severe side effects (Groups 1 and 2, respectively) and for 52 weeks with mild and severe side effects (Group 3 and 4, respectively) was compared with estimated costs of mild and severe steroid-induced side effects (Groups 5 and 6, respectively). Statistical analysis between groups was conducted using independent sample t tests. RESULTS: Three out of the 4 group combinations of tocilizumab with prednisone demonstrated a statistically significant (P < 0.05) difference in cost compared with prednisone alone for GCA. Group 2 (26-week tocilizumab therapy with severe steroid-induced side effects), with no statically significant difference in price when compared with steroid therapy alone and far fewer side effects, demonstrated the potential use of tocilizumab in GCA therapy. As expected, longer treatment duration with tocilizumab was associated with greater cost. With respect to side effect severity, the number of side effects of steroid therapy was inversely associated with difference in cost between tocilizumab therapy and steroid side effect treatment. CONCLUSION: This study demonstrates that combination therapy of tocilizumab and prednisone is significantly more expensive than steroids alone with or without accounting for the cost of steroid-induced side effects in treated GCA. The difference in cost between the 2 therapy types is directly related to tocilizumab therapy duration and inversely related to the number or severity of steroid side effects. Patients with GCA who require a shorter duration of steroid therapy and are at risk for a high number of side effects from steroid use may be potential candidates for tocilizumab therapy, from an economic perspective.

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Giant cell arteritis (GCA) is a condition that can cause irreversible visual loss if untreated. While corticosteroids remain the mainstay of treatment to prevent visual loss, the type, dose, route, and duration of corticosteroid treatment of GCA remain controversial. Our study surveyed neuro-ophthalmologists to determine commonly prescribed dosages of corticosteroids for the treatment of GCA with or without visual loss. For patients with acute visual loss, 52% would use intravenous (IV), 46% would use IV or oral and 2% would use oral corticosteroids. Seventy-three per cent would use 500 to 1000 mg IV methylprednisolone in this group. For patients with GCA without acute visual loss, 67% would use the oral route, 30% would use IV or oral, and 3% indicated they would use IV route of treatment. Seventy-five per cent would use 1.0 to 1.5 mg/kg oral prednisone in this group. Our results suggest a majority but not a complete consensus for route and dose of corticosteroid treatment in GCA and confirm conventional recommendations for high dose IV corticosteroids for GCA with visual loss and lower dose oral regimens for GCA without visual loss.

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ABSTRACT: Shock-induced anterior ischemic optic neuropathy (SIAION) is a known type of optic neuropathy in patients who experienced shock related to different etiologies such as anemia and severe intradialytic hypotension like in our patient. Nonarteritic anterior ischemic optic neuropathy (NAION) is the most common acute unilateral known type of optic neuropathy in older patients with vasculopathic risk factors such as hypertension, diabetes mellitus, and obstructive sleep apnea. Although SIAION and NAION are similar optic neuropathies due to ischemia and, in particular, hypotension, they may have different pathogenic mechanisms (e.g., acute shock or intradialytic hypotension vs nocturnal hypotension), laterality (e.g., unilateral vs bilateral), and severity (e.g., light perception or worse vision). We presented a case with restricted diffusion on the apparent diffusion coefficient and the diffusion weighted imaging confined to the optic disc head in a patient with pallid edema after intradialytic hypotension. Although DWI of the optic nerve is neither 100% specific nor 100% sensitive for ischemia, we believe that restricted diffusion of the optic nerve head in our case is a clinico-radiologic correlate to pallid edema in SIAION.

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ABSTRACT: A 42-year-old Algerian man presented for binocular oblique diplopia, hypersomnolence with drop attacks, bilateral hearing loss, and thoracic pain. He had a right thalamomesencephalic hemorrhage due to an underlying cavernous malformation treated with subtotal surgical resection. On neuro-ophthalmic examination, the patient had a left relative afferent pupillary defect and a right oculosympathetic efferent pupillary defect (i.e., Horner syndrome) in addition to other thalamomesencephalic eye and neurologic signs (right fourth nerve palsy, hearing loss, hemiparesis, and thalamic pain). Clinicians should recognize the localizing value of this unique constellation of mesencephalic afferent and efferent pupillary defects.

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Acute, painful, transient, right-sided, monocular visual loss lasting 4 hours developed in a 46-year-old man. This was followed by headache and left-sided transient hemiparesis. The association of ipsilateral transient vision loss with transient contralateral hemiparesis implicates involvement of the ipsilateral internal carotid artery (i.e., a crossed symptom).

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BACKGROUND: To describe the various neuro-ophthalmic presentations, key exam features, and clinical findings associated with 5 common primary and secondary intracranial malignancies. EVIDENCE ACQUISITION: Retrospective PubMed search and review of published case reports, case series, observational studies, book chapters, and review articles examining the neuro-ophthalmic features of intracranial malignancies including primary glial neoplasms (e.g., glioblastoma multiforme), primary and secondary lymphoma, intracranial metastases, carcinomatous/lymphomatous meningitis, and intracranial germ cell tumors. The search strategy used to perform the retrospective review included the aforementioned tumor type (e.g., glioblastoma multiforme) and the following terms and Boolean operators: AND ("visual loss" OR "papilledema" OR "diplopia" OR "ophthalmoplegia" or "neuro-ophthalmology" OR "proptosis"). RESULTS: The rate of growth and the location of an intracranial tumor are essential factors in determining the neuro-ophthalmic presentation of certain intracranial malignancies. Primary malignant brain glial neoplasms commonly present with visual afferent complaints (e.g., unilateral or bilateral visual acuity or visual field defects, bitemporal or homonymous hemianopsia), pupil abnormalities (relative afferent pupillary defect), and optic atrophy or papilledema. Primary intraocular lymphoma (with or without central nervous system lymphoma) typically presents as a painless bilateral vitritis. Secondary intracranial malignancies have variable afferent and efferent visual pathway presentations. Carcinomatous/lymphomatous meningitis is associated with diplopia (e.g., multiple ocular motor cranial neuropathies with or without vision loss from papilledema or compressive/infiltrative optic neuropathy). Intracranial germ cell tumors can present with a chiasmal syndrome or dorsal midbrain syndrome. CONCLUSION: Intracranial malignancies can present with neuro-ophthalmic symptoms or signs depending on topographical localization. Specific neuro-ophthalmic presentations are associated with different malignant intracranial tumors. Clinicians should be aware of the common malignant intracranial tumors and their associated clinical presentations in neuro-ophthalmology.

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This case report highlights intermittent proptosis precipitated by exercise or Valsalva maneuvers. Conventional orbital computed tomography scan and catheter angiography did not disclose any orbital vascular lesion. Orbital B-scan ultrasonography, however, before and after a Valsalva maneuver demonstrated intermittent orbital vein dilation adjacent to the optic nerve of the left eye. Clinicians should be aware that anatomic venous variations and vascular malformations including orbital varices may produce a characteristic symptom of recurrent proptosis with Valsalva or head position. Conventional structural orbital imaging (e.g., computed tomography or magnetic resonance imaging) may not demonstrate a lesion.

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Simultanagnosia is a well-known neurologic symptom characterized by the inability to conceptualize the whole picture despite being able to see individual elements within a visual scene. The pathophysiology involves a lesion to the bilateral parieto-occipital lobe. We report two unusual cases of simultanagnosia and juxtaposed homonymous visual field loss involving aqueductal stenosis-related obstructive hydrocephalus and cardiac arrest due to Brugada syndrome. Clinicians should be aware that simultanagnosia can be the presenting symptom of neuro-ophthalmic disease.

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A 74-year-old man with vasculopathic risk factors presented to the emergency room with a chief complaint of peripheral vision loss resulting from an intracranial hemorrhage in his right parietal and occipital lobes. Urgent craniotomy and ventriculostomy led to a stable clinical condition with subsequent development of a crossed quadrant homonymous hemianopsia (checkerboard visual field) due to a new right parieto-occipital infarct superimposed on a prior left occipital infarct. This uncommon visual field defect represents juxtaposed homonymous quadrantanopias that produce a striking checkerboard appearance that is almost pathognomonic for bilateral occipital lesions.

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This article will review the best approaches to neuroimaging for specific ophthalmologic conditions and discuss characteristic radiographic findings. A review of the current literature was performed to find recommendations for the best approaches and characteristic radiographic findings for various ophthalmologic conditions. Options for imaging continue to grow with modern advances in technology, and ophthalmologists should stay current on the various radiographic techniques available to them, focusing on their strengths and weaknesses for different clinical scenarios.

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