Rituximab as a first-line treatment in pediatric neuromyelitis optica spectrum disorder
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Neuromyelitis optica (NMO) is an autoimmune astrocytopathy that involves the optic nerve and the spinal cord. The detection of immunoglobulin-G (IgG) autoantibodies specific for the aquaporin-4 (AQP4) water channel unifies a spectrum of neurological manifestation (NMO spectrum disorders, NMOSD) that includes NMO and its partial forms distinguishing them from other inflammatory diseases of the central nervous system (CNS) . Compelling evidence exists that AQP4-IgG has pathogenic potential.
Moreover, the detection of AQP4-IgG predicts optic neuritis and LETM relapses with cumulative attack-related neurological disability .
Here we describe the case of a young girl with a severe onset NMOSD, treated with Rituximab (MabThera©) as first-line therapy.
A 9-year-old girl was admitted to our Pediatric Intensive Care Unit for the onset of severe and increasing loss of strength, limb and joint pain, progressively increasing axial rigidity, neck stiffness and urinary...
KeywordsPediatric NMOSD Aquaporin-4 Rituximab First-line therapy
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Conflict of interest
The authors declare that they have no conflict of interest.