No evidence of neuronal/glial autoantibodies in febrile infection-related epilepsy syndrome (FIRES): a prospective clinic-serologic analysis

被引:3
作者
Wenglein, Janina Soler [1 ]
Kluger, Gerhard [2 ,3 ]
Leypoldt, Frank [4 ,5 ]
Wandinger, Klaus-Peter [5 ]
van Baalen, Andreas [1 ]
机构
[1] Kiel Univ CAU, Univ Med Ctr Schleswig Holstein, Dept Neuropediat, Kiel, Germany
[2] Schon Clin Vogtareuth, Clin Neuropediat & Neurorehabil, Epilepsy Ctr Children & Adolescents, Vogtareuth, Germany
[3] Paracelsus Med Univ Salzburg, Res Inst Rehabil Transit & Palliat, Salzburg, Austria
[4] Kiel Univ CAU, Univ Med Ctr Schleswig Holstein, Dept Neurol, Kiel, Germany
[5] Univ Med Ctr Schleswig Holstein, Inst Clin Chem, Neuroimmunol Sect, Kiel, Germany
关键词
FIRES; NORSE; status epilepticus; encephalopathy; autoimmune; inflammation; AE; autoantibodies; AUTOIMMUNE ENCEPHALITIS; DIFFERENTIAL-DIAGNOSIS; CASE SERIES; ANTIBODIES;
D O I
10.3389/fnins.2023.1221761
中图分类号
Q189 [神经科学];
学科分类号
071006 ;
摘要
The pediatric febrile infection-related epilepsy syndrome (FIRES) manifests with encephalopathy with super-refractory status epilepticus (SE) a few days after or accompanying a febrile illness. It often results in refractory epilepsy and cognitive dysfunction in previously healthy children and adolescents. The underlying pathomechanism is unknown, which is why causative neuronal and/or synaptic antibodies have been discussed. We report a prospective consecutive cohort of 14 children (10 male, four female) diagnosed with FIRES in the acute phase, whose serum and CSF were comprehensively screened for underlying synaptic/neuronal autoantibodies. The median age at onset was 6 years (range 4-9 years). None of the children had a medical history of epilepsy. Duration of SE varied from less than 1 week to 2.5 months (Median: 1 month, range < 1 week-2.5 months). Clinical response to treatment with antiseizure medications was poor as well as the outcome: one child died in the acute phase of SE, and two died in the long term. All surviving children showed neuropsychological impairments. No underlying synaptic or neuronal autoantibodies were identified in 13 of 14 children's sera or CSF. One child had currently uncharacterized neuronal autoantibodies in CSF, yet clinical presentation was atypical for FIRES. Based on our findings, the child was later diagnosed with autoimmune encephalitis (AE). We conclude that FIRES is not an autoantibody-mediated disease. However, a comprehensive screening for known and yet unknown antineuronal antibodies in serum and CSF is warranted to rule out AE mimicking FIRES.
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页数:7
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