Camille receives monthly infusions of IVIg to treat her immunodeficiency (hypogammaglobulinemia) and to help manage her seizures. Camille achieved seizure control with high dose IVIg treatment, though was recently diagnosed with Electrical Status Epilepticus in Sleep.
WHAT IS IVIg AND WHAT DISORDERS IS IT USED TO TREAT?
IVIg (Intravenous Immunoglobulins) is used for three purposes; for patients who do not make antibodies or gammaglobulin, for patients with specific infections, and for patients with autoimmune and inflammatory diseases.
Some individuals make no or too few antibodies and thus suffer from recurrent infections. Sometimes the antibody producing cells (B-cells) of individuals have an intrinsic inability to make antibodies; these are patients with Primary Immunodeficiency diseases, and many require life-long IVIg infusions. Examples include X-linked Agammaglobulinemia and Common Variable Immunodeficiency. Blood tests are available to determine if a patient has low antibodies and needs IVIg. Since this treatment is often life-long and very expensive, careful testing needs to be done prior to starting IVIG. Indeed, like other drugs, IVIg may have side effects, so its use is reserved for just a few patients.
A second use of IVIg is in the treatment of certain infections in which antibiotic or antiviral therapy is ineffective. Examples include severe parvovirus infection and chronic viral meningitis. These patients do not require life-long IVIg and it can be discontinued after the infection is controlled.
A final use of IVIg is in certain inflammatory and immunologic diseases, often of unknown causes. Examples include an acute fever-causing childhood disease termed Kawasaki disease, and a blood disease termed immune thrombocytopenia, where the blood platelet levels are low and the patient develops bruising or bleeding.
Can Immunotherapy control seizures?
Author: L Seiden and A Krumholz
The occurrence of seizures in systemic immune-mediated disorders demonstrates that epilepsy can be related to immunologic disorders. This raises the issues of whether immune mechanisms may be important in other, more common forms of epilepsy and whether immunotherapy merits further consideration.
Immunoglobulin therapy was first reported to be effective in epilepsy in 1977, when children with epilepsy were given intramuscular IgG to treat recurrent upper respiratory tract infections. Improvements in seizure frequency and behavior were noted. Subsequently, 16 children with Lennox-Gastaut syndrome were treated with intravenous IgG (IVIG), and significant improvement was seen in 8 of them. Since that time, several additional clinical trials have been published with similar results, reporting significant improvement in about half of cases.
In 1994, the first double-blind, placebo-controlled trial of IVIg for the treatment of epilepsy was published. Three different dosages were evaluated in 61 patients with epilepsy of various etiologies. Significant clinical improvement, defined as a reduction in seizures by at least 50% after 6 months, was reported in 52.5% of the IVIG group, compared to 27.8% of the placebo group, with a p value of .095. No dose relationship was identified.
There is emerging clinical evidence that IVIg may be of value in several refractory seizure conditions. The details regarding which patients and at what point of the illness it should be used need to be investigated further.