Original articleEfficacy of intravenous immunoglobulin in 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
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