Neuromyelitis Optica (Devic’s Syndrome): an Appraisal

Abstract

Neuromyelitis optica (NMO) and neuromyelitis optica spectrum disorders (NMOSD), previously known as Devic's syndrome, are a group of inflammatory disorders of the central nervous system (CNS) characterized by severe, immune-mediated demyelination and axonal damage, predominantly targeting optic nerves and the spinal cord typically associated with a disease-specific serum NMO-IgG antibody that selectively binds aquaporin-4 (AQP4). The classic and best-defined features of NMOSD include acute attacks of bilateral or rapidly sequential optic neuritis (leading to visual loss) or transverse myelitis (often causing limb weakness and bladder dysfunction) or both with a typically relapsing course. The diagnosis of NMO/NMOSD requires a consistent history and examination with typical clinical presentations, findings on spinal cord neuroimaging with MRI, cerebrospinal fluid analysis along with determination of AQP4-IgG serum autoantibody status, and exclusion of other disorders. Two major advances in this field has been the development of diagnostic criteria and treatment recommendations. Consensus diagnostic criteria have been established and were recently revised and published in 2015, enhancing the ability to make a diagnosis and appropriately evaluate these disorders. Expert recommendations and uncontrolled trials form the basis of treatment guidelines. All patients with suspected NMOSD should be treated for acute attacks as soon as possible with high-dose intravenous methylprednisolone -1 gram daily for three to five consecutive days and in some cases, plasma exchange should be used. It is recommended that every patient with NMOSD be started on an immunosuppressive agent, such as, azathioprine, methotrexate, or mycophenolate and in some cases, rituximab, soon after the acute attack and usually be treated for about 5 years after the attack. These advances have helped improve the prognosis and outcome in these disorders.

Neurological safety of fingolimod: An updated review

“…During acute attacks, high‐dose i.v. methylprednisolone (1 g/day for 3–5 days) or, in some cases, PLEX was used 92. All patients are started on an immunosuppressive agent at the same time.…”

Neuromyelitis optica spectrum disorder presenting in an octogenarian

“…Acute attacks of myelitis and optic neuritis are generally more severe and, if untreated, remission is poorer than in MS, which leads to more rapid permanent disability 12. Unlike MS, a primary progressive clinical course is very rare 12.…”

“…Unlike MS, a primary progressive clinical course is very rare 12. However, there is increasing recognition of the heterogeneity of the condition as clinical features reflect lesions in different areas of the CNS, particularly the optic nerve, spinal cord, area postrema, hypothalamus, brainstem and cerebral hemispheres.…”

Intractable Vomiting and Hiccups: An Atypical Presentation of Neuromyelitis Optica

“…A 33-year-old female patient with nil premorbid conditions came to our tertiary care centre with complaints of vomiting for the past 10 days, which was followed by intractable hiccups for eight days. The patient was having 10-20 episodes of vomiting per day, which was bilious and 1 1 small in quantity. It was not associated with fever, pain abdomen or loose stools.…”

“…It most commonly involves the demyelination of the optic nerves and the spinal cord, which is primarily immune-mediated. Neuromyelitis optica spectrum disorder (NMOSD) is a variant of NMO, which is usually associated with the presence of serum NMO-IgG antibody that selectively binds aquaporin-4 [1]. Optic neuritis and transverse myelitis are usually the most common presenting symptoms of the disease [2].…”