Opsoclonus Myoclonus Ataxia Syndrome [ICNApedia]

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====== Opsoclonus Myoclonus Ataxia Syndrome ======
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^ Recommendations for the Dosing and Monitoring of Immunotherapeutic Agents in the Treatment of OMAS: Steroid Treatment and Ivig                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                               ||
| **Treatment of OMS in children**                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                             ||
| 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  treatment alone and will therefore be overtreated by a front-loading regimen. However, delay in effective treatment may be associated with a poorer disease outcome. If an escalation regimen is used, aggressive escalation may be required in cases with a poor response to initial treatment.                                                                                                                                                                                                                     ||
| **Steroid Treatment**                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                        ||
| Regimen                                                                                                                                                                                                                                                                                                                                                                                                                                                   | Various regimens have been used including pulsed dexamethasone, adrenocorticoptropic hormone (ACTH), and methylprednisolone followed by prednisolone.                                                             |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           | Pulsed dexamethasone has been widely used as first-line steroid treatment. This may be given as:                                                                                                                  |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           |20 mg/m<sup>2</sup>/day in 2 divided doses on 3 consecutive days (3 d-treatment defined as 1 pulse)                                                                                                                        |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           |12 pulses at 3 to 4 weekly intervals                                                                                                                                                                            |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           | 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                                                                                         |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           | 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.  |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           | **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/kg/d)                                                                                                     |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           | Weaning may be performed over 12 mo, which may be more rapid with steroid-sparing agent. Longer treatment may be needed.                                                                                          |
| Adverse effects                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                              ||
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           | Potential side effects include irritability, hypertension, hyperglycemia, weight gain infections, and osteopenia, which may be lessened by using pulse rather than daily steroids.                                |
| Safety monitoring                                                                                                                                                                                                                                                                                                                                                                                                                                         | H2 blockers and/or antacids are recommended as prophylaxis for gastritis during steroid administration according to standard local procedures.                                                                    |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           | Blood pressure, blood or urine glucose, full blood cell count, and blood electrolytes should be monitored by standard local procedures as clinically indicated.                                                   |
|                                                                                                                                                                                                                                                                                                                                                                                                                                                           | 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), particularly if also had received other immunotherapy.                  |
| IVIG                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                                         ||
| Regimen                                                                                                                                                                                                                                                                                                                                                                                                                                                   | May be given as: 2 g/kg over 2-5 d followed by 1-2 g/kg every 4 wk for up to 12 mo                                                                                                                                |
| Side effects                                                                                                                                                                                                                                                                                                                                                                                                                                              | Potential side effects of IV1g include allergic reactions, thrombosis, headaches, and aseptic meningitis.                                                                                                         |