Advances in the diagnosis of giant cell arteritis Purpose of review To summarize recent advances in the diagnosis of giant cell arteritis (GCA). Recent findings Less common manifestations of GCA include corneal edema, proptosis from lacrimal gland ischemia and sensorineuronal hearing loss. Histology studies have suggested that temporal artery biopsies (TAB) with fixed specimen lengths of 15 mm may be adequate to prevent false negative biopsies. In centers with appropriate radiologic expertise, a European rheumatology consensus guideline has proposed Doppler ultrasound as a first-line confirmatory test for GCA in lieu of temporal artery biopsy. Finding extracranial large vessel disease can help to diagnose GCA. Statistical prediction rules can help risk stratify patients with suspected GCA. Age and platelet level when maintained as continuous variables are the strongest predictors for GCA. Summary GCA can present with diverse ophthalmic and systemic presentations and expedient recognition of same can avoid diagnostic delay and possible vision loss, among other complications. TAB remains the conventional diagnostic standard test for GCA. The use of statistical prediction models and increased expertise in noninvasive imaging techniques such as ultrasound may decrease reliance on TAB, especially in patients determined to be at low risk for GCA.

Clinical and radiologic approach to ‘typical’ versus antibody-related optic neuritis Purpose of review Optic neuritis is an autoimmune optic neuropathy that has been associated with multiple sclerosis (MS), neuromyelitis optica spectrum disorder (NMOSD), and more recently antimyelin oligodendrocyte glycoprotein (anti-MOG)-positive disorder. At initial presentation, it is often difficult to differentiate these entities given their significant overlap in clinical presentation and MRI findings. This review summarizes the distinguishing clinical and radiological features of MS, NMOSD, and anti-MOG disorders to help clinicians accurately diagnose and manage patients affected by these conditions. Recent findings Antiaquaporin-4 (AQP4) and more recently anti-MOG antibodies are both associated with central nervous system demyelinating diseases that often initially present with optic neuritis. Serologic testing now allows for a new classification of these overlapping conditions that can help to differentiate ‘typical’ optic neuritis that is often associated with MS from ‘atypical’ optic neuritis associated with NMOSD and anti-MOG-positive disorder. Summary Optic neuritis associated with MS, NMOSD, and anti-MOG-positive disease can have a similar clinical presentation. However, some clinical and radiologic findings can help clinicians to differentiate these entities so that they can be properly managed to optimize visual prognosis.

Update on pediatric optic neuritis Purpose of review To summarize recent developments in the classification, investigation and management of pediatric optic neuritis (PON). Recent findings A recent surge in interest surrounding antibodies to myelin oligodendrocyte glycoprotein antibody (MOG-Ab) has instigated a paradigm shift in our assessment of children with PON. This serological marker is associated with a broad spectrum of demyelinating syndromes that are clinically and radiologically distinct from multiple sclerosis (MS) and aquaporin-4 antibody positive neuromyelitis optica spectrum disorder (AQP4+NMOSD). Optic neuritis is the most common presenting phenotype of MOG-Ab positive-associated disease (MOG+AD). MOG-Ab seropositivity is much more common in the pediatric population and it predicts a better prognosis than MS or AQP4+NMOSD, except in the subset that exhibit a recurrent phenotype. Summary A better grasp of MOG+AD features and its natural history has facilitated more accurate risk stratification of children after a presenting episode of PON. Consequently, the initial investigation of PON has broadened to include serology, along with neuroimaging and cerebrospinal fluid analysis. Acute treatment of PON and chronic immunotherapy is also becoming better tailored to the suspected or confirmed diagnoses of MS, AQP4+NMOSD and MOG+AD.

Immune checkpoint inhibitors: what neuro-ophthalmologists need to know Purpose of review Immune checkpoint inhibitors are currently an exceedingly powerful tool in the management of hitherto incurable malignancies and their use in clinical practice is expected to increase in the near future. The purpose of this review is to discuss the current medical uses of checkpoint inhibitors with a focus on their neuro-ophthalmic side-effects. Recent findings Immune checkpoint inhibitors have emerged as a promising breakthrough in the treatment of several tumor types. However, these targeted therapies can induce a wide range of immune-related ophthalmic and neuro-ophthalmic toxicities. It is important for neuro-ophthamologists to promptly recognize and manage these adverse events that can potentially threaten vision. Summary There are currently seven FDA-approved immune checkpoint inhibitors and several ones are under investigation. In general, immunotherapy is considered a well tolerated, safe and efficacious treatment option for many cancer patients. Nevertheless, because of their unique mechanism of action, these molecules can alter the immune response and result in immune-related adverse effects in almost every organ with an estimated incidence of ophthalmic side effects in this patient population of less than 1%.

Neuro-ophthalmic manifestations of the phakomatoses Purpose of review The phakomatoses are a group of inherited disorders with variable clinical manifestations that are characterized by brain, cutaneous, ocular and other distinct lesions in multiple organs. Correctly recognizing the neuro-ophthalmic signs and symptoms can lead to early diagnosis and treatment. The group is composed of neurofibromatosis (type 1 and 2), tuberous sclerosis complex, von Hippel–Lindau, ataxia–telangiectasia and Sturge–Weber syndromes. However, more than 60 syndromes have been described in the medical literature. This review provides an update on the diagnosis and management of phakomatoses with a focus on their clinical neuro-ophthalmic manifestations. Recent findings Phakomatoses are a group of inherited syndromes with variable clinical manifestations that are characterized by brain, cutaneous, ocular and other distinct lesions in multiple organs. Recent advances in diagnostic and treatment options that have contributed to prompt recognition and management of these disorders are discussed with an emphasis on the beneficial effects on vision. Summary Phakomatoses, also known as neuro-oculo-cutaneous syndromes, are inherited disorders with characteristic lesions in multiple organs. Because of their frequent ocular involvement thorough ophthalmologic and neuro-ophthalmic evaluation is critical in this patient population in order to prevent vision loss and life-threatening complications that are often associated with these disorders.

Ocular motor manifestations of movement disorders Purpose of review Impaired eye movements are frequently seen in ophthalmic and neurologic clinical practice, especially in individuals with movement disorders. Identification of the abnormal movement can aid initial diagnosis and improve understanding of the underlying disease pathophysiology. The present article reviews the ocular motor manifestations and recent research on them in common movement disorders. Recent findings Ocular motor manifestations and their pathophysiologic correlates are being defined. In particular, study of eye movements can help clarify the changing clinicopathologic spectrum of atypical parkinsonian disorders. The pathophysiology and natural history of blepharospasm are being elucidated. Recent research focuses on high-resolution imaging and other technological advances to improve the sensitivity of the ocular motility exam. Eye movements are being studied as biomarkers for diagnosis and progression in clinical care and trials. Summary The current review summarizes ocular motor manifestations in common movement disorders, and presents recent research investigating their cause and treatment.

Visual and oculomotor outcomes in children with prenatal opioid exposure Purpose of review To summarize the visual and oculomotor outcomes in children with prenatal opioid exposure and review the effects of opioids on the developing central nervous system. Recent findings Animal models and imaging studies in children suggest that prenatal opioid exposure may affect neuronal survival and result in delayed maturation of white matter tracts and decreased volumes in certain brain areas. Visual evoked potential testing in children demonstrates delayed maturation of the afferent visual system in opioid-exposed groups compared with controls, though ‘catch-up’ development is seen with longitudinal follow-up. Strabismus and nystagmus are also more common in exposed children, and these findings appear to persist. Summary As rates of opioid dependence and prenatal opioid exposure continue to increase, it is important to evaluate the short-term and long-term effects of opioids on the developing visual system. An understanding of these risks is important when counseling the parents or guardians of opioid-exposed children, though larger studies with more long-term follow-up will improve our prognostic abilities.

Infectious ocular motor neuropathies Purpose of review Describe the range of infectious causes of ocular motor neuropathies, from common presentations to unusual manifestations of diseases less frequently seen in the developed world. Provide information on recent developments in diagnostic testing for pathogens that may cause ocular motor neuropathies. Recent findings Antigen detection in serum or CSF has improved the diagnosis of cryptococcal disease. Cartridge PCR testing for tuberculosis has increased diagnostic accuracy, though tuberculous meningitis remains difficult to diagnose. Rapid, multiplex PCR and unbiased sequencing allow for diagnosis of a wider range of organisms. Summary Infectious ocular motor neuropathies can occur anywhere along the length of cranial nerves III, IV, and VI. Characteristic clinical findings and imaging can be used to localize infections. Infectious causes may have characteristic clinical, laboratory, or imaging findings, but must still be carefully separated from inflammatory or neoplastic conditions.

Update: the Miller Fisher variants of Guillain–Barré syndrome Purpose of review This article will update and review the Miller Fisher variants (MFV) of Guillain–Barré syndrome (GBS) including the clinical presentation, diagnostic testing, and treatment. Recent findings Although the diagnosis of GBS and MFV can be made on clinical grounds, cerebrospinal fluid (CSF) analysis, nerve conduction studies, imaging (e.g. ultrasound and MRI), and serologic testing can help to confirm the diagnosis. Some patients may need immunotherapy with either intravenous immunoglobulin (IVIg) or plasma exchange, and recent studies suggest that complement inhibition combined with IVIg could be of benefit, but further studies are needed to prove efficacy. Summary GBS is characterized by an acute, ascending polyneuropathy, ataxia, areflexia, and CSF albuminocytologic dissociation. The MFV of GBS is associated with ophthalmoplegia. Clinicians should have high index of suspicion for MFV of GBS in patients with acute ophthalmoplegia in order to establish the diagnosis, perform appropriate evaluation, and start treatment. SDC video link: http://links.lww.com/COOP/A32