Elsevier

Pediatric Neurology

Volume 26, Issue 4, April 2002, Pages 298-300
Pediatric Neurology

Original article
Efficacy of intravenous immunoglobulin in Landau-Kleffner syndrome

https://doi.org/10.1016/S0887-8994(01)00402-7Get rights and content

Abstract

We administered 2 gm/kg of intravenous gamma globulin (IVIG) to each of five consecutive patients with Landau-Kleffner syndrome, over 4 days. We compared the 1-month baseline to that following IVIG using a severity score assessing speech, comprehension, behavior, seizures, and electroencephalography. There was a significant drop in this score after IVIG (P = 0.025). Two patients had a dramatic response to IVIG, with complete resolution of symptoms. This finding suggests that IVIG has at least some efficacy for the therapy of Landau-Kleffner syndrome.

Introduction

Landau-Kleffner syndrome is a rare neurologic disorder characterized by loss of previously acquired language skills, auditory agnosia, and electroencephalographic (EEG) findings of spike and slow wave activity more prominent in sleep. Some patients, designated as LKS variants, also have behavioral features of autistic regression [1]. Antiepileptic medications, steroids, and surgery have had limited success in this disorder. Intravenous gamma globulin (IVIG) is used in multiple neurologic diseases, such as intractable epilepsy in children [2] and multiple sclerosis [3]. The use of IVIG has been described in three case reports, the first by us, to have encouraging results in Landau-Kleffner syndrome [4], [5], [6].

Section snippets

Methods

A prospective pilot study of IVIG therapy for Landau-Kleffner syndrome was undertaken. Children diagnosed with Landau-Kleffner syndrome based on the International League Against Epilepsy criteria [7] at the American University of Beirut Medical Center were recruited into the study. Inclusion criteria included the following: (1) onset of expressive aphasia and auditory agnosia with normal prior development of language skills; (2) EEG consistent with Landau-Kleffner syndrome; (3) willingness by

Results

Five consecutive patients satisfied the above criteria and all agreed to participate in the study (Table 1). Patient 1 had mumps parotitis approximately 5 weeks before the onset of her symptoms. Patient 3 had encephalitis of suspected varicella etiology a few months before presentation. Patient 4 had a perinatal stroke leading to right-sided hemiparesis. Patient 5 had a maternal history positive for anticardiolipin antibodies during pregnancy but no personal history of documented autoimmune

Discussion

Landau-Kleffner syndrome can be secondary to a number of etiologic factors, including structural lesions, such as brain tumors [8] and immune-mediated disorders, such as inflammatory demyelinating disease [9]. An autoimmune etiology is considered likely based on findings of autoantibodies to myelin in affected patients [10] and on the presence of IgG antibodies to brain endothelial cells in a significant number of children with LKS variant [1]. The results of our study support the hypothesis

References (11)

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    Timing of treatment initiation is important as NMDAR are vital for brain maturation and development, with variable expression of the different subunit throughout the developmental period (Henson et al., 2008). Although the response to both IVIG and steroids was previously described in various refractory epilepsies including LKS (Geva-Dayan et al., 2012; Mikati et al., 2002; Mikati and Saab, 2000; Mikati et al., 2010; Gallagher et al., 2006; Sinclair and Snyder, 2005), it is not reasonable to expect that patients with an underlying genetic etiology such as GRIN-related epilepsy would respond to this treatment. One of the possible explanations is the strongly evolving link between epilepsy and the immune system.

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