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Epilepsia partialis continua associated with the p.Arg403Cys variant of the DNM1L gene: an unusual clinical progression with two episodes of super-refractory status epilepticus with a 13-year remission interval Volume 24, issue 1, February 2022

Figure 1

T2-FLAIR MRI showing a low level of hyperintensity located in the left thalamus and in the posterior portion of the left putamen (asterisk) at first status epilepticus onset (2006 April) (A), disappearance of the hyperintensity two months later (2006 June) (B), normal MRI at 20 months after the status (2017 November) (C), contralateral right capsulo-thalamic hyperintensity (asterisk) during the second episode of status epilepticus (2019 December) (D), right lentiform nucleus hyperintensity (asterisk) 15 days later (2019 December) (E), and lateral ventricle enlargement and bilateral thalamic hyperintensity (asterisks) two months later (2020 February) (F).

Figure 2

Longitudinal bipolar EEG montage. EMG 1: right deltoid; EMG 2: left deltoid; EMG 3: right forearm EMG 4: left forearm. (A) Asymmetry of the background activity with continuous slow paroxysmal activity on left frontal regions (2006 April). (B) Focal clonic seizures, characterized by frontal high-amplitude fast activity with mixed rhythmic spike waves (2006 April). (C) 11-Hz background activity without asymmetry and without epileptiform discharges (2019 January). (D) Irregular background activity (8-9-Hz) with polyspike-wave discharges on the right frontal region; myoclonic phenomena associated with diffuse polyspike-wave discharges, prevalent on the right fronto-central region (2020 April). (E) Irregular background activity (8-9-Hz) with rhythmic theta-delta activity prevailing on the left central region; left arm myoclonic phenomena were inconstantly associated with diffuse polyspike-wave discharges (2020 July). (F) Irregular, low-amplitude background activity (6-7Hz) with small groups of diffuse polyspike-wave discharges inconstantly associated with myoclonic phenomena, mostly observed on the right arm (2020 November).