Q: 35 year old male with no past medical history is admitted to ICU with an acute attack of bilateral vision loss associated with lower limb weakness, sensory loss, and bladder dysfunction. MRI done in Emergency Department (ED) read as probable Devic Disease. What is Devic disease?
Answer: Although it is popularly known as Devic Disease, actual nomenclature is Neuromyelitis Optica Spectrum Disorders (NMOSD) or simply neuromyelitis optical (NMO) explains it well. These are the inflammatory disorders of the central nervous system characterized by severe, immune-mediated demyelination and axonal damage predominantly targeting optic nerves and spinal cord. The learning point for this pearl is to highlight that NMOSD is not a variation of multiple sclerosis (MS). It is a distinct disease of its own. It has its own pathogenesis, radiological features, biomarkers, neuropathology, and treatment.
Attacks can be severe causing optic neuritis and/or myelitis. In contrast to MS, necrosis and cavitation involve both gray and white matter. It is mediated via a humoral immune system whereas MS is mostly a cell-mediated disorder.
#neurology
References:
1. Devic E. Myélite aiguë compliquée de névrite optique. Bull Med (Paris) 1894; 8:1033.
2. Wingerchuk DM, Lennon VA, Lucchinetti CF, et al. The spectrum of neuromyelitis optica. Lancet Neurol 2007; 6:805.
3. Ghezzi A, Bergamaschi R, Martinelli V, et al. Clinical characteristics, course and prognosis of relapsing Devic's Neuromyelitis Optica. J Neurol 2004; 251:47.
4. Kim SH, Kim W, Li XF, et al. Clinical spectrum of CNS aquaporin-4 autoimmunity. Neurology 2012; 78:1179.
No comments:
Post a Comment