John Libbey Eurotext

Facial dystonic seizures-plus associated with anti-LGI1 antibody encephalitis Volume 21, issue 5, October 2019

Videos

  • Facial dystonic seizures-plus associated with anti-LGI1 antibody encephalitis
  • Facial dystonic seizures-plus associated with anti-LGI1 antibody encephalitis
  • Facial dystonic seizures-plus associated with anti-LGI1 antibody encephalitis

Figures

  • Figure 1

We present a 39-year-old, right-handed woman with anti-LGI1 antibody encephalitis (LGI1 encephalitis). Indirect immune fluorescence revealed anti-LGI1 antibody titres of 1:3.2 and 1:32 in the CSF and blood, respectively. The most common seizure type associated with LGI1 encephalitis is faciobrachial dystonic seizure (FBDS) (Irani et al., 2011, 2013) and FBDS-plus (Beimer and Selwa, 2017; Chen et al., 2017). Left facial dystonic seizures (FDS) in the patient progressed to focal seizures with shouting, oral automatisms, numbness of the left side of the face and left upper limb, and palpitation (video sequences 1-3). Electrographically, artefacts of FDS were followed by rhythmic theta activity with evolution, predominant on right temporal and anterior frontal regions, and increased heat rate. MRI revealed right mesial temporal lobe, insular lobe, and thalamus high-intensity signal on Flair-weighted images (figure 1A). Arterial spin-labelling showed that the blood flow increased in the right mesial temporal lobe, insular lobe, and bilateral thalamus (figure 1B). We propose the term “facial dystonic seizures-plus” (FDS-plus) to describe these episodes. Recognition of the broad range of seizure types associated with LGI1 encephalitis is crucial for early diagnosis and definitive treatment.

 

Disclosures

None of the authors have any conflict of interest to declare.

Funding

The work was supported by the Young Scientists Fund of the National Natural Science Foundation of China (Grant No. 81501126, http://npd.nsfc.gov.cn/), the National Natural Science Foundation of China (Grant No. 81471324, http://npd.nsfc.gov.cn/), Science and Development Foundation of Nanjing Medical University (2014NJMU050), Young Medical Key Talents Foundation of Jiangsu Province (Grant No. QNRC2016053), and Training Project for Young Talents of Nanjing Brain Hospital.