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The importance of early immunotherapy in patients with faciobrachial dystonic seizures

2017; Oxford University Press; Volume: 141; Issue: 2 Linguagem: Inglês

10.1093/brain/awx323

1460-2156

J. M. T. Thompson, Mian Bi, Andrew G. Murchison, Mateusz Makuch, Christian G. Bien, Kon Chu, Pue Farooque, Jeffrey M. Gelfand, Michael D. Geschwind, Lawrence J. Hirsch, Ernest Somerville, Bethan Lang, Angela Vincent, Maria Isabel Leite, Patrick Waters, Sarosh R. Irani, Müjgan Dogan-Onugoren, Alexander Rae‐Grant, Zsolt Illés, Mónika Szőts, Michael P. Malter, Guido Widman, Rainer Surges, Neil Archibald, John Reid, Callum Duncan, Anna Richardson, James B Lilleker, Raffaele Iorio, Morten Blaabjerg, Karin Abeler, Young-Hee Shin,

Peripheral Neuropathies and Disorders

Faciobrachial dystonic seizures and limbic encephalitis closely associate with antibodies to leucine-rich glioma-inactivated 1 (LGI1). Here, we describe 103 consecutive patients with faciobrachial dystonic seizures and LGI1 antibodies to understand clinical, therapeutic and serological differences between those with and without cognitive impairment, and to determine whether cessation of faciobrachial dystonic seizures can prevent cognitive impairment. The 22/103 patients without cognitive impairment typically had normal brain MRI, EEGs and serum sodium levels (P < 0.0001). Overall, cessation of faciobrachial dystonic seizures with antiepileptic drugs alone occurred in only 9/89 (10%) patients. By contrast, 51% showed cessation of faciobrachial dystonic seizures 30 days after addition of immunotherapy (P < 0.0001), with earlier cessation in cognitively normal patients (P = 0.038). Indeed, expedited immunotherapy (P = 0.031) and normal cognition (P = 0.0014) also predicted reduced disability at 24 months. Furthermore, of 80 patients with faciobrachial dystonic seizures as their initial feature, 56% developed cognitive impairment after 90 days of active faciobrachial dystonic seizures. Whereas only one patient developed cognitive impairment after cessation of faciobrachial dystonic seizures (P < 0.0001). All patients had IgG4-LGI1 antibodies, but those with cognitive impairment had higher proportions of complement-fixing IgG1 antibodies (P = 0.03). Both subclasses caused LGI1-ADAM22 complex internalization, a potential non-inflammatory epileptogenic mechanism. In summary, faciobrachial dystonic seizures show striking time-sensitive responses to immunotherapy, and their cessation can prevent the development of cognitive impairment.awx323media15681705685001.