Contributions
Abstract: P636
Type: Poster Sessions
Abstract Category: Therapy - Others
Objective: To determine safety and clinical effect of plasma exchange (PE) as rescue therapy in paediatric acquired inflammatory CNS syndromes.
Methods: Single-center retrospective review of patient records over the period 2003-2017, to assess demographic/diagnostic data, attack characteristics, and short-term remission status after PE. All patients admitted to our hospital with a steroid-refractory acute event presumed to be inflammatory and who required PE were included.
Results: A total of 582 PE procedures for 87 attacks in 74 paediatric patients (63.5% neuromyelitis optica spectrum disorders, 8% acute disseminated encephalomyelitis, 4% multiple sclerosis, 12% clinically isolated syndromes, 12% others) were assessed. Mean age at PE 10.4 years (2-18), 47% girls. Serostatus in 42 patients were 17% AQP4+, 31% MOG+, 52% double negative. Attack phenotypes were optic neuritis (ON) 42%, transverse myelitis (TM) 31%, ON + TM 15%, other combined syndromes 11%. Visual outcome scale, Bladder control scale, Hauser ambulation scale, and Expanded Disability Status Scale were assessed before and after PE (at discharge, 30 days, and at 6 months) in every patient.
Overall, moderate to marked neurological improvement was observed in 67% of attacks at discharge, increasing to 77% at 6 months. No marked differences were observed in response rate among attack phenotypes or serostatus. No improvement was seen in 15% of events, mainly in children with diagnoses other than inflammatory CNS syndromes (enterovirus D68-related myelitis, chiasm glioma, LHON-related optic involvement, sarcoidosis).
Adverse events occurring during or immediately following PE were observed in 23/87 (26%) treatments, graded as moderate in 20, and severe in 3, corresponding to catheter removal-related adverse events (pneumothorax 1, pulmonary air embolism 1, and pulmonary thromboembolism with death in 1).
Conclusions: PE is a highly specialized procedure available in few hospitals in our region. Our results show that PE is an effective treatment alternative to intravenous steroids, improving neurological outcome after severe attacks of inflammatory CNS disorders in paediatric patients. Serious adverse events may occur and should be considered.
Disclosure: S. Tenembaum, MD, serves as a non-remunerated editorial board member of Neurology: Neuroimmunology & Neuroinflammation. She has received speaker honoraria from Biogen-Idec Argentina, Merck Serono LATAM, Genzyme, Novartis, and Teva Neuroscience.
A. Savransky, MD, has received speaker honoraria from Genzyme.
M. Huaman Rios, MD, has nothing to disclose
S. Vergel, MD, has nothing to disclose
M. Castro, MD, has nothing to disclose
S. Perez, MD, has nothing to disclose
G.C. Marcarian, MD, has nothing to disclose
R. Alba, MD, has nothing to disclose
A. M. Pugliese, MD, has nothing to disclose
Abstract: P636
Type: Poster Sessions
Abstract Category: Therapy - Others
Objective: To determine safety and clinical effect of plasma exchange (PE) as rescue therapy in paediatric acquired inflammatory CNS syndromes.
Methods: Single-center retrospective review of patient records over the period 2003-2017, to assess demographic/diagnostic data, attack characteristics, and short-term remission status after PE. All patients admitted to our hospital with a steroid-refractory acute event presumed to be inflammatory and who required PE were included.
Results: A total of 582 PE procedures for 87 attacks in 74 paediatric patients (63.5% neuromyelitis optica spectrum disorders, 8% acute disseminated encephalomyelitis, 4% multiple sclerosis, 12% clinically isolated syndromes, 12% others) were assessed. Mean age at PE 10.4 years (2-18), 47% girls. Serostatus in 42 patients were 17% AQP4+, 31% MOG+, 52% double negative. Attack phenotypes were optic neuritis (ON) 42%, transverse myelitis (TM) 31%, ON + TM 15%, other combined syndromes 11%. Visual outcome scale, Bladder control scale, Hauser ambulation scale, and Expanded Disability Status Scale were assessed before and after PE (at discharge, 30 days, and at 6 months) in every patient.
Overall, moderate to marked neurological improvement was observed in 67% of attacks at discharge, increasing to 77% at 6 months. No marked differences were observed in response rate among attack phenotypes or serostatus. No improvement was seen in 15% of events, mainly in children with diagnoses other than inflammatory CNS syndromes (enterovirus D68-related myelitis, chiasm glioma, LHON-related optic involvement, sarcoidosis).
Adverse events occurring during or immediately following PE were observed in 23/87 (26%) treatments, graded as moderate in 20, and severe in 3, corresponding to catheter removal-related adverse events (pneumothorax 1, pulmonary air embolism 1, and pulmonary thromboembolism with death in 1).
Conclusions: PE is a highly specialized procedure available in few hospitals in our region. Our results show that PE is an effective treatment alternative to intravenous steroids, improving neurological outcome after severe attacks of inflammatory CNS disorders in paediatric patients. Serious adverse events may occur and should be considered.
Disclosure: S. Tenembaum, MD, serves as a non-remunerated editorial board member of Neurology: Neuroimmunology & Neuroinflammation. She has received speaker honoraria from Biogen-Idec Argentina, Merck Serono LATAM, Genzyme, Novartis, and Teva Neuroscience.
A. Savransky, MD, has received speaker honoraria from Genzyme.
M. Huaman Rios, MD, has nothing to disclose
S. Vergel, MD, has nothing to disclose
M. Castro, MD, has nothing to disclose
S. Perez, MD, has nothing to disclose
G.C. Marcarian, MD, has nothing to disclose
R. Alba, MD, has nothing to disclose
A. M. Pugliese, MD, has nothing to disclose