Childhood choreoathetosis secondary to hyper-IgM syndrome (CD40 ligand deficiency)

Ian C. Coulter; Han Yan; Carolina Gorodetsky; Melika Akhbari; Sara Breitbart; Suneil K. Kalia; Alfonso Fasano; George M. Ibrahim

doi:10.1212/NXI.0000000000000899

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Figure Illustrative radiological and immunohistological imaging
(A and B) show axial and coronal T2 preoperative images demonstrating supratentorial volume loss and bilateral T2 globus pallidus hypointensity. (C) Coronal T2 sequence illustrating deep brain stimulation electrodes. (D) Immunohistological image demonstrating CD3 positivity (brown) indicative of lymphocytosis.

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Demonstrating our patient's choreiform movements preoperatively and after the initiation of stimulation 5 days postoperatively.Download Supplementary Video 1 via http://dx.doi.org/10.1212/000899_Video_1

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Childhood choreoathetosis secondary to hyper-IgM syndrome (CD40 ligand deficiency)

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