**9. IVIg in therapy of drug resistant epilepsy (DRIE)**

Drug-resistant infantile epilepsy (DRIE) syndromes include a number of diseases such as Landau-Kleffner syndrome (LKS), West syndrome, Lennox-Gastaut syndrome, severe myoclonic epilepsy or RE that typically manifest in childhood or adolescence and are characterized by epilepsy and progressive neurological dysfunction.

Intravenous Immunoglobulins in Neurological Diseases: Established and Novel Clinical Applications 351

patients with AD. Autoantibody-decorated plaques were found frequently in patients with AD and patients with low antibody-levels were shown to harbour more diffuse plaques than patients with high levels. Autoantibodies against Ab may therefore be important for

IVIg has been shown to contain autoantibodies against many states of Ab peptide aggregation including monomers, oligomers and fibrils and may therefore have a distinct advantage over monoclonal anti-Ab until the precise pathogenic state(s) of the Ab peptide is

Recently, commercially available IVIg have been used in small pilot trials for the treatment of patients with AD, based on the hypothesis that IVIG contains naturally occurring autoantibodies (nAbs-Abeta) that specifically recognize and block the toxic effects of Abeta. Furthermore, these nAbs-Abeta are reduced in AD patients compared with healthy controls, supporting the notion of replacement with IVIg. Beyond the occurrence of nAbs-Abeta, evidence for several other mechanisms associated with IVIg in AD has been reported in preclinical experiments and clinical studies. In 2009, a phase III clinical trial involving more than 360 AD patients was initiated and may provide conclusive evidence for the effect of

Its efficacy has been proved in GBS, CIDP and MMN, where it is considered as the first-line treatment. However, questions remain regarding the dose, timing and duration of IVIg

It is also successfully used in acute exacerbations of MG and as a short-term treatment of severe MG. It is recommended in SPS, in some paraneoplastic syndromes and as a secondline treatment in combination with prednisone in dermatomyositis and a treatment option

In MS, IVIg is indicated mainly in reducing disease activity during pregnancy and

In addition to these major indications, IVIg is increasingly used even in such conditions where the strong evidence is currently lacking, like refractory epilepsy, narcolepsy, post

According to preliminary data, IVIg might be a promising candidate for the treatment of (AD). Large-scale randomized trials are under way, and the results of these studies are

When considering treatment options, it is important to notify that uncontrolled use may lead to high costs and limited availability of IVIg. Careful selection of patients who will

maintaining plaque homeostasis.

IVIg as a treatment option for AD in 2011(Dodel et al, 2010).

IVIg is used increasingly in neurological diseases.

known (Hughes et al, 2008).

**12. Conclusion** 

in polymyositis.

breastfeeding.

polio syndrome.

ABBREVIATIONS:

treatment in these disorders.

awaited eagerly worldwide.

AChR: acetylcholine receptor AD: Alzheimer's Disease

benefit from IVIg is extremely important.

AMAN: acute motor axonal neuropathy

ADEM: Acute-disseminated encephalomyelitis

AIDP: acute inflammatory demyelinating polyneuropathy

AMSAN: acute motor and sensory axonal neuropathy CIDP: chronic idiopathic demyelinating polyneuropathy

Standard treatment of RE consists of anti-epileptic drugs, high-dose steroids or PE. Surgical treatment also may be considered.

Case studies and small series have reported that some patients with RE respond in some measure to treatment with IVIG (class IV).

Approximately a hundred patients with West or Lennox-Gastaut syndromes have been treated with IVIg with widely varying results. The treatment has resulted in reduction in the number of seizures with improvement in the EEG in about half of the cases. The positive effects were noted few days to several weeks to months after treatment. Relapses have been common.

Successful use of IVIg as initial monotherapy in LKS has been reported in case studies and after initial therapy by steroids or antiepileptic drugs and steroids in only few patients. Case studies on the use of IVIg in RE have suggested that monthly IVIg therapy (0.4 g/kg for 5 days at 4-week interval followed by monthly maintenance IVIg) may ameliorate disease in patients who are refractory to antiepileptic drugs or steroids and PE (EFNS task force, 2008). Recommendation:

