TY - JOUR
T1 - Disability and Therapeutic Response in Paediatric Neuromyelitis Optica Spectrum Disorder
T2 - A Case Series from Iran
AU - Baghbanian, Seyed Mohammad
AU - Sahraian, Mohammad Ali
AU - Naser Moghadasi, Abdorreza
AU - Asgari, Nasrin
PY - 2019
Y1 - 2019
N2 - Objectives: The characteristics of paediatric neuromyelitis optica spectrum disorder (NMOSD) may indicate the degree of disability and identify factors that predict the response to treatment.Materials & Methods: Among 114 NMOSD patients in an acquired demyelinating syndromes registry at the Sina Hospital, in Tehran, Iran, 10 paediatric NMOSD patients with longitudinal follow-up from 2005 to 2016 were retrospectively identified. The median time between disease onset and diagnosis was 18 months (range 1-108 months).Results: All patients had a relapsing course, which resulted in disability in six with severe visual impairment and functional blindness in one and impaired ambulation in five patients during follow-up. Azathioprine (AZA) was first drug of choice for prophylaxis, but in five patients new attacks occurred and therapy was switched to rituximab (RTX) with no further relapses after median two years (range 1-3 y) follow-up.Conclusion: Paediatric onset of NMOSD was associated with severe attacks and poor response in 50 % of cases to AZA, RTX seemed to decrease the relapse rate.
AB - Objectives: The characteristics of paediatric neuromyelitis optica spectrum disorder (NMOSD) may indicate the degree of disability and identify factors that predict the response to treatment.Materials & Methods: Among 114 NMOSD patients in an acquired demyelinating syndromes registry at the Sina Hospital, in Tehran, Iran, 10 paediatric NMOSD patients with longitudinal follow-up from 2005 to 2016 were retrospectively identified. The median time between disease onset and diagnosis was 18 months (range 1-108 months).Results: All patients had a relapsing course, which resulted in disability in six with severe visual impairment and functional blindness in one and impaired ambulation in five patients during follow-up. Azathioprine (AZA) was first drug of choice for prophylaxis, but in five patients new attacks occurred and therapy was switched to rituximab (RTX) with no further relapses after median two years (range 1-3 y) follow-up.Conclusion: Paediatric onset of NMOSD was associated with severe attacks and poor response in 50 % of cases to AZA, RTX seemed to decrease the relapse rate.
KW - Disability
KW - Neuromyelitis optica spectrum disorder
KW - Paediatric
KW - Treatment
U2 - 10.22037/ijcn.v13i3.22715
DO - 10.22037/ijcn.v13i3.22715
M3 - Journal article
C2 - 31327974
SN - 1735-4668
VL - 13
SP - 99
EP - 104
JO - Iranian Journal of Child Neurology
JF - Iranian Journal of Child Neurology
IS - 3
ER -