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content:opsoclonus_myoclonus_ataxia_syndrome [2024/03/11 19:35] – [Table] biju.hameed@gmail.com | content:opsoclonus_myoclonus_ataxia_syndrome [2024/03/15 19:11] (current) – biju.hameed@gmail.com | ||
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- | ^ Recommendations for the Dosing and Monitoring of Immunotherapeutic Agents in the Treatment of OMAS: Steroid Treatment and Ivig ^|| | + | ====== Opsoclonus Myoclonus Ataxia Syndrome ====== |
- | | Treatment of OMS in children | + | {{page> |
- | | Prompt treatment is generally considered important and should be initiated when a diagnosis of OMS has been made. Brief delay for surgical removal of an associated neuroblastoma may be considered or initial doses of immunotherapy given before tumor resection if there is delay in surgery. | + | ^ Recommendations for the Dosing and Monitoring of Immunotherapeutic Agents in the Treatment of OMAS: Steroid Treatment and Ivig |
- | | There remains uncertainty as to the benefits of a regimen of escalation of treatment vs front-loading treatment. Some children will respond to steroid | + | | **Treatment of OMS in children** || |
- | | Steroid Treatment | + | | Prompt treatment is generally considered important and should be initiated when a diagnosis of OMS has been made. Brief delay for surgical removal of an associated neuroblastoma may be considered or initial doses of immunotherapy given before tumor resection if there is delay in surgery. |
- | | Regimen | + | | There remains uncertainty as to the benefits of a regimen of escalation of treatment vs front-loading treatment. Some children will respond to steroid |
- | | | Pulsed dexamethasone has been widely used as first-line steroid treatment. This may be given as: | + | | **Steroid Treatment** |
- | | | + | | Regimen |
- | | | + | | | Pulsed dexamethasone has been widely used as first-line steroid treatment. This may be given as: | |
- | | | The scheduled 12 pulses of dexamethasone should always be completed, even with earlier complete remission. | + | | |20 mg/m< |
- | | | Additional dexamethasone pulses may be given, or the interval between the scheduled dexamethasone pulses may be shortened | + | | |12 pulses at 3 to 4 weekly intervals |
- | | | in patients showing insufficient response or improvement after dexamethasone but worsening of symptoms before the scheduled date of the next dexamethasone pulse. | + | | | The scheduled 12 pulses of dexamethasone should always be completed, even with earlier complete remission. |
- | | | ACTH may be given as: | | | + | | | Additional dexamethasone pulses may be given, or the interval between the scheduled dexamethasone pulses may be shortened |
- | | | 75 iu/m2 intramuscularly twice daily for 1 wk, once daily for 1 week, alternate days for 2 wk, and then a gradual wean over 11 mo, but a slower titration from daily to alternate day treatment is often needed. | + | | | in patients showing insufficient response or improvement after dexamethasone but worsening of symptoms before the scheduled date of the next dexamethasone pulse. |
- | | | Alternative corticosteroid regimens include: | + | | |
- | | | + | | | 75 iu/m2 intramuscularly twice daily for 1 wk, once daily for 1 week, alternate days for 2 wk, and then a gradual wean over 11 mo, but a slower titration from daily to alternate day treatment is often needed. |
- | | | Pulse repeated monthly for 6-12 mo or followed by oral prednisone or prednisolone (starting dose 1-2 mg/ | + | | |
- | | | Weaning may be performed over 12 mo, which may be more rapid with steroid-sparing agent. Longer treatment may be needed. | + | | |IV pulse methylprednisolone(30 mg/kg/d for 3-5 d) | |
- | | Adverse effects | + | | | Pulse repeated monthly for 6-12 mo or followed by oral prednisone or prednisolone (starting dose 1-2 mg/ |
- | | | Potential side effects include irritability, | + | | | Weaning may be performed over 12 mo, which may be more rapid with steroid-sparing agent. Longer treatment may be needed. |
- | | Safety monitoring | + | | Adverse effects |
- | | | Blood pressure, blood or urine glucose, full blood cell count, and blood electrolytes should be monitored by standard local procedures as clinically indicated. | + | | | Potential side effects include irritability, |
- | | | Patients on chronic corticosteroids should receive adjunctive treatment for bone health (calcium and vitamin D) with consideration | + | | Safety monitoring |
- | | | of Dual-energy X-ray absorptiometry scan if on steroids for more than 6 mo, and Pneumocystis prophylaxis (trimethoprim-sulfamethoxazole), | + | | | Blood pressure, blood or urine glucose, full blood cell count, and blood electrolytes should be monitored by standard local procedures as clinically indicated. |
- | | IVIG | + | | | Patients on chronic corticosteroids should receive adjunctive treatment for bone health (calcium and vitamin D) with consideration |
- | | Regimen | + | | | of Dual-energy X-ray absorptiometry scan if on steroids for more than 6 mo, and Pneumocystis prophylaxis (trimethoprim-sulfamethoxazole), |
- | | Side effects | + | | IVIG |
+ | | Regimen | ||
+ | | Side effects |