Neuromyelitis optica and disorders of its broader spectrum

Neuromyelitis optica and its broader spectrum disorders (NMOSD) are autoimmune diseases of the central nervous system. They typically manifest as inflammation of the optic nerves and spinal cord, less often the brainstem. In most patients, we find antibodies against aquaporin-4 (AQP4-IgG) in the serum, which are specific for this disease and play a crucial role in the pathogenesis of the disease in more than about 75% of patients. Common key clinical features include optic neuritis, myelitis, and area postrema syndrome. Despite significant improvements in the diagnostic process due to the availability of AQP4 antibody testing, there are still delays in establishing the diagnosis in AQP4-IgG seronegative patients, patients with partial spinal cord syndromes, older patients, etc. The most frequently misdiagnoses include multiple sclerosis, spinal cord tumors, ischemic optic nerve lesions, neuroinfections, and others. It is also important to differentiate AQP4-IgG positive patients from patients with positivity for antibodies against myelin oligodendrocyte glycoprotein. These antibodies also define a new group of CNS demyelinations, the so-called “ myelin oligodendrocyte glycoprotein antibody– associated disease – MOGADISHU.
This book provides an overview of the basic pathogenetic mechanisms of NMOSD and MOGAD, explains differential diagnostic procedures, and presents typical findings on magnetic resonance imaging and optical coherence tomography in optic neuritis. It then summarizes the options for treating relapses and long-term chronic treatment of patients with these autoimmune CNS diseases.
The book is of interest to neurologists, ophthalmologists, and radiologists.