Chen, John J.

AbstractNeuromyelitis optica (NMO) is an antibody-mediated inflammatory disease of the central nervous system with a predilection for the optic nerves, spinal cord, and certain brain regions. While NMO was previously considered a variant of multiple sclerosis (MS), it is now known to have distinct clinical, pathological, and immunological features. The identification of AQP4-IgG, a pathogenic antibody against aquaporin-4 (AQP4), delineated NMO from MS and markedly advanced insights into the unique features of this disease. The specificity of this antibody has allowed an expanded view of the clinical presentations of NMO-spectrum disorders (NMOSD), without requiring all the clinical features that were previously essential to make a clinical diagnosis. Early, accurate diagnosis of patients with NMOSD permits treatment with appropriate acute and long-term immunosuppressive agents that are critical to mitigate the risk of disability associated with this disease. More recently, a subset of patients with the NMOSD phenotype have been found to have autoantibodies targeting myelin oligodendrocyte glycoprotein (MOG), which has a different pathogenesis and expected outcome. Better understanding of the distinct pathophysiology of these disorders has laid the foundation for targeted efforts to develop novel, disease-specific treatments. In this review, we discuss the revised diagnostic criteria for NMOSD, appraise the diagnostic significance of the AQP4-IgG and MOG-IgG tests, review evidence supporting the use of available treatments for acute episodes and long-term disease modification, and highlight key emerging immunotherapies.

Purpose of reviewTo review the clinical characteristics, radiological manifestations and treatment of myelin oligodendrocyte glycoprotein (MOG)-immunoglobulin G (IgG) optic neuritis.Recent findingsSerum antibodies to MOG have recently been found to be a biomarker of MOG-IgG-associated disorder (MOGAD), a demyelinating disease distinct from both multiple sclerosis (MS) and aquaporin-4-IgG neuromyelitis optica spectrum disorder (AQP4-IgG-positive NMOSD). The phenotype of MOGAD is broad and includes optic neuritis, transverse myelitis, and acute demyelinating encephalomyelitis (ADEM). Optic neuritis is the most common presentation in adults, whereas ADEM is the most common presentation in children. Clinical characteristics suggestive of MOG-IgG optic neuritis include recurrent optic neuritis, prominent disc edema, and perineural enhancement of the optic nerve on magnetic resonance imaging. Although the nadir of vision loss is severe with MOG-IgG optic neuritis, the recovery is typically better than AQP4-IgG optic neuritis and therefore has a favorable overall prognosis. Patients with relapsing disease will often need chronic immunotherapy. Rituximab, azathioprine, mycophenolate mofetil, and monthly intravenous immune globulin are the most commonly utilized treatments.SummaryMOGAD is a unique entity that is separate from both MS and AQP4-IgG-positive NMOSD. Recognition of the clinical and radiologic features allow for the correct diagnosis. Future randomized trials will determine the optimal treatment for MOGAD.

Purpose: To describe two patients with giant cell arteritis who presented with macular lesions mimicking persistent placoid maculopathy. Methods: Retrospective case reports. Patient: Two patients, a 74-year-old man and an 85-year-old woman both were diagnosed with biopsy-confirmed giant cell arteritis with vision loss in one eye due to arteritic ischemic optic neuropathy. Both presented many years later with acute vision loss in the remaining eye. Results: Both patients had bilateral retinal whitening in the parafovea, and the second also had sectoral optic disk swelling. On fluorescein angiography, these areas of retinal whitening in both patients were hypofluorescent early with late hyperfluorescence staining. On indocyanine green angiography, there were areas of foveal hypocyanescence bilaterally in both patients that persisted throughout the imaging and were more apparent late. These findings can be seen in patients with bilateral persistent placoid maculopathy. Conclusion: Giant cell arteritis should be included in the differential diagnosis of persistent placoid maculopathy.

Background Hemicrania continua is a primary headache disorder characterized by a continuous, unilateral headache associated with ipsilateral cranial autonomic features that responds to indomethacin. By definition, the symptoms are not referable to an underlying structural pathology. However, several cases of secondary hemicrania continua related to underlying structural lesions have been reported. Case We present a case of a 53‐year‐old male with a prolonged, right‐sided headache associated with intermittent right‐sided ptosis, conjunctival injection, tearing, and nasal congestion, suggestive of hemicrania continua, who was found to have an indirect carotid‐cavernous fistula, and who, after endovascular treatment of the fistula, had resolution of his symptoms. Conclusion Alternative, and perhaps less common, causes of headache should be considered when the clinical presentation is atypical or does not clearly fulfill diagnostic criteria for primary headache disorders. Carotid cavernous fistulas should be included within this differential, and represent a potentially treatable and reversible cause of otherwise refractory headache.