| Specific Conditions |
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| Indication for Ig Use |
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| Level of Evidence | Evidence of probable benefit – more research needed (Category 2a) |
| Description and Diagnostic Criteria |
Epilepsy is a clinical syndrome of recurrent epileptic seizures and has multiple causes. Immune mediated mechanisms can result in epilepsy. Patients with epilepsy due to clear cut inflammatory syndromes such as autoimmune encephalitis, Rasmussen encephalitis or post encephalitic epilepsy are considered elsewhere. It is possible that immune mechanisms have a role in some cases of epilepsy, however defining these mechanisms is challenging. A few epileptic encephalopathy syndromes in infants and young children are responsive to steroids, and for this reason, an immune mechanism is possible. Intravenous immunoglobulin (IVIg) has been trialled in these patients with mixed success. A subgroup of patients with West syndrome, Landau Kleffner syndrome and Lennox Gaustaut syndrome have been observed to respond to steroids or IVIg and there is uncontrolled case report data that supports a possible improvement of symptoms with IVIg. |
| Justification for Evidence Category | The literature on intravenous immunoglobulin (IVIg) in epileptic encephalopathy syndromes such as West syndrome, Landau Kleffner, Lennox Gaustaut is limited to case reports and small case series and the quality of this literature is poor. It can be concluded that a proportion of patients with these epileptic syndromes may benefit from IVIg, particularly those patients with demonstrable immune abnormalities in blood, CSF or neuroimaging. |
| Diagnosis Requirements |
A diagnosis must be made by a Neurologist. |
| Qualifying Criteria for Ig Therapy |
Children with epileptic encephalopathy resistant to anti-epileptic medications and steroid therapy or steroid responsive but dependent
Please use the indication Relapse of epileptic encephalopathy following a trial of weaning from Ig therapy in a patient previously demonstrating response for responding patients who have relapsed after weaning from previous Ig therapy.
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Documentation of clinical effectiveness (such as maintaining a seizure diary) is necessary for continuation of IVIg therapy. Once patients are stable, a trial of weaning from Ig therapy should be considered every year to identify those in remission. Responding patients who relapse within three months of cessation of Ig therapy can reapply under the indication Relapse of epileptic encephalopathy following a trial of weaning from Ig therapy in a patient previously demonstrating response. Relapse of epileptic encephalopathy following a trial of weaning from Ig therapy in a patient previously demonstrating response
This indication is for responding patients who relapse within six months of cessation of Ig therapy. For patients who have never trialled off a request must be submitted using the indication children with epileptic encephalopathy resistant to anti-epileptic medications and steroid therapy or steroid responsive but dependent.
AND
Documentation of clinical effectiveness (such as maintaining a seizure diary) is necessary for continuation of IVIg therapy. Once patients are stable, a trial of weaning from Ig therapy should be considered every year to identify those in remission. |
| Exclusion Criteria |
Rasmussen encephalitis - see Rasmussen encephalitis Post encephalitic epilepsy - see Autoimmune encephalitis mediated by extracellular antibodies - see Antibody mediated autoimmune encephalitis (AMAE) |
| Review Criteria for Assessing the Effectiveness of Ig Use |
Children with epileptic encephalopathy resistant to anti-epileptic medications and steroid therapy or steroid responsive but dependent
Review by a neurologist is required after three months of treatment to determine whether the patient has responded, and annually thereafter. If there is no benefit after the first three months of treatment, IVIg therapy should be abandoned.
Documentation of clinical effectiveness (such as maintaining a seizure diary) is necessary for continuation of IVIg therapy. Once patients are stable, a trial of weaning from Ig therapy should be considered every year to identify those in remission. Responding patients who relapse within three months of cessation of Ig therapy can reapply under the indication Relapse of epileptic encephalopathy following a trial of weaning from Ig therapy in a patient previously demonstrating response. Clinical effectiveness of Ig therapy can be demonstrated by: On review of the initial authorisation period
On review of a continuing authorisation period
Relapse of epileptic encephalopathy following a trial of weaning from Ig therapy in a patient previously demonstrating response
IVIg should be used for up to three months before a review by a neurologist is required to determine whether the patient has responded. Annual review is required thereafter. If there is no improvement after the initial three month’s treatment, Ig therapy should be abandoned.
Documentation of clinical effectiveness (such as maintaining a seizure diary) is necessary for continuation of IVIg therapy. Once patients are stable, a trial of weaning from Ig therapy should be considered every year to identify those in remission. Clinical effectiveness of Ig therapy can be assessed by: On review of the initial authorisation period
On review of a continuing authorisation period
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| Dose |
Children with epileptic encephalopathy resistant to anti-epileptic medications and steroid therapy or steroid responsive but dependent
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.
Refer to the current product information sheet for further information on dose, administration and contraindications. Relapse of epileptic encephalopathy following a trial of weaning from Ig therapy in a patient previously demonstrating response
The aim should be to use the lowest dose possible that achieves the appropriate clinical outcome for each patient.
Refer to the current product information sheet for further information on dose, administration and contraindications. |
| Bibliography |
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| Geng J, Dong J, Li, Y et al 2011, ‘Intravenous immunoglobulin for epilepsy’, Cochrane database of systematic reviews (online) Version 2 DOI:10.1002/14651858.CD008557.pub2. Available from: http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD008557.pub2/abstract Geva-Dayan, K, Shorer, Z, Menascu S, et al 2012, ‘Immunoglobulin treatment for severe childhood epilepsy’, Pediatric Neurology, vol. 46, no. 6, pp 375-81. Mikati, MA, Kurdi, R, El-Khoury Z, et al 2010, ‘Intravenous immunoglobulin therapy in intractable childhood epilepsy: open-label study and review of literature’, Epilepsy & Behaviour, vol. 17, issue. 1, pp. 90-4. Available from: http://www.sciencedirect.com/science/article/pii/S1525505009006027 Ontario Regional Blood Coordinating Network 2016. Ontario Intravenous Immune Globulin (IVIG) Utilization Management Guidelines, Version 3.0. [online]. Available from: http://transfusionontario.org/en/ UK Department of Health 2011, ‘Clinical Guidelines for Immunoglobulin Use: Second Edition Update’. Available from: https://www.gov.uk/government/uploads/system/uploads/attachment_data/file/216671/dh_131107.pdf UK Department of Health 2011, ‘Clinical Guidelines for Immunoglobulin Use: Second Edition Update: Summary Poster’. Available from: http://igd.mdsas.com/clinical-info/ Van Rijckevorsel K 2008, ‘Treatment of Lennox-Gestaut syndrome:overview and recent findings’, Neuropsychistr Dis Treat, vol. 4, no. 6, pp. 1001-1019. https://www.ncbi.nlm.nih.gov/pmc/articles/PMC2646636/ Van Rijckevorsel-Harmant K, Delire M, Schmitz-Moorman W, et al 1994, ‘Treatment of refractory epilepsy with intravenous immunoglobulins. Results of the first double-blind/dose finding clinical study’, International Journal of Clinical and Laboratory Research, vol. 24, no. 3, pp. 162-6. https://www.ncbi.nlm.nih.gov/pubmed/7819596 Walker, L, Pirmohamed, M & Marson, AG 2013, ‘Immunomodulatory interventions (treatments that target the immune system) for focal epilepsy syndromes’, Cochrane database of systematic reviews, Issue 6, Art no.:CD009945 DOI: 10.1002/14651858.CD009945.pub2. http://onlinelibrary.wiley.com/doi/10.1002/14651858.CD009945.pub2/pdf |