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content:opsoclonus_myoclonus_ataxia_syndrome [2024/03/11 19:30] – created 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. |
+ | | There remains uncertainty as to the benefits of a regimen of escalation of treatment vs front-loading treatment. Some children will respond to steroid | ||
+ | | **Steroid Treatment** | ||
| Regimen | | Regimen | ||
| | Pulsed dexamethasone has been widely used as first-line steroid treatment. This may be given as: | | | | Pulsed dexamethasone has been widely used as first-line steroid treatment. This may be given as: | | ||
- | | | + | | |20 mg/m< |
- | | | + | | |12 pulses at 3 to 4 weekly intervals |
| | The scheduled 12 pulses of dexamethasone should always be completed, even with earlier complete remission. | | | The scheduled 12 pulses of dexamethasone should always be completed, even with earlier complete remission. | ||
| | Additional dexamethasone pulses may be given, or the interval between the scheduled dexamethasone pulses may be shortened | | | Additional dexamethasone pulses may be given, or the interval between the scheduled dexamethasone pulses may be shortened | ||
| | in patients showing insufficient response or improvement after dexamethasone but worsening of symptoms before the scheduled date of the next dexamethasone pulse. | | | in patients showing insufficient response or improvement after dexamethasone but worsening of symptoms before the scheduled date of the next dexamethasone pulse. | ||
- | | | ACTH may be given as: | + | | |
| | 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. | | | 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. | ||
- | | | Alternative corticosteroid regimens include: | + | | |
- | | | + | | |IV pulse methylprednisolone(30 mg/kg/d for 3-5 d) |
| | Pulse repeated monthly for 6-12 mo or followed by oral prednisone or prednisolone (starting dose 1-2 mg/ | | | 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. | | | Weaning may be performed over 12 mo, which may be more rapid with steroid-sparing agent. Longer treatment may be needed. | ||
- | | Adverrse | + | | Adverse |
| | Potential side effects include irritability, | | | Potential side effects include irritability, | ||
| Safety monitoring | | Safety monitoring | ||
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| | Patients on chronic corticosteroids should receive adjunctive treatment for bone health (calcium and vitamin D) with consideration | | | Patients on chronic corticosteroids should receive adjunctive treatment for bone health (calcium and vitamin D) with consideration | ||
| | of Dual-energy X-ray absorptiometry scan if on steroids for more than 6 mo, and Pneumocystis prophylaxis (trimethoprim-sulfamethoxazole), | | | of Dual-energy X-ray absorptiometry scan if on steroids for more than 6 mo, and Pneumocystis prophylaxis (trimethoprim-sulfamethoxazole), | ||
- | | IVIG | | + | | IVIG |
| Regimen | | Regimen | ||
| Side effects | | Side effects |