The Turkish Journal of Pediatrics 2015 , Vol 57 , Num 5
A case of hyperkinetic movement disorder associated with LGI1 antibodies
1Division of Neurology, Institute of Neurological Sciences, Uludag University Faculty of Medicine, Bursa, 2Division of Child Neurology, Department of Neurology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, 3Department of Neurosciences, Institute for Experimental Medical Research, Istanbul University, Istanbul, Turkey. E-mail: drerdem@yahoo.com Received: 30 October 2014, Revised: 28 November 2014, Accepted: 29 December 2014 Erer-Özbek S, Yapıcı Z, Tüzün E, Giriş M, Duran S, Taşkapılıoğlu Ö, Okan M. A case of hyperkinetic movement disorder associated with LGI1 antibodies. Turk J Pediatr 2015; 57: 514-517.

Encephalitis associated with leucine-rich glioma inactivated 1 (LGI1) antibodies is often encountered in elderly male patients and may infrequently present with isolated syndromes. A 6-year-old boy was admitted with acute onset severe oral and facial stereotypic and choreiform movements. On his neurologic examination, he had repetitive and rhythmic movements in orolingual muscles including tongue protrusion, limb chorea and minimal facial stereotypic movements. Anti-streptolysin O (ASO) titers were found severely elevated in several measurements. Well-characterized antibodies against ion channels and synapse proteins were negative whereas LGI1 antibody was positive in both serum and CSF. Marked clinical improvement was observed after immunotherapy. Here, we present the first pediatric case with LGI1 antibody associated hyperkinetic movement disorders and emphasize the importance of investigating neuronal autoantibodies in patients with isolated and treatment resistant movement disorders. Keywords : hyperkinetic movement, autoimmune encephalitis, LGI1 antibody, Sydenham’s chorea, immunoglobulin therapy.

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