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. 2021 Aug;28(8):2694-2699.
doi: 10.1111/ene.14891. Epub 2021 May 20.

Status epilepticus with prominent motor symptoms clusters into distinct electroclinical phenotypes

Simona Lattanzi  1 , Giada Giovannini  2   3 , Francesco Brigo  4   5 , Niccolò Orlandi  2   6 , Eugen Trinka  7   8   9 , Stefano Meletti  2   6
Affiliations

Status epilepticus with prominent motor symptoms clusters into distinct electroclinical phenotypes

Simona Lattanzi et al. Eur J Neurol. 2021 Aug.

Abstract

Background and purpose: Status epilepticus (SE) is a heterogeneous condition and considerable variability exists in its etiology, semiology, electroencephalographic correlates, and response to treatment. The aim of the present study was to explore whether distinct phenotypes may be identified within SE with prominent motor symptoms.

Methods: Consecutive episodes of SE with prominent motor symptoms in patients aged ≥14 years were included. Etiology of SE was defined as symptomatic (acute, remote, progressive) or unknown. Electroencephalogram (EEG) recordings were searched for lateralized periodic discharges (LPDs), generalized sharply and/or triphasic periodic potentials (GPDs), and spontaneous burst suppression (BS). According to treatment response, SE was classified into responsive, refractory and super-refractory. Average linkage hierarchical cluster analysis was performed with Pearson's correlation as a similarity measure.

Results: A total of 240 episodes of SE were identified. Three major clusters were found. The first cluster linked focal motor SE evolving into non-convulsive SE (NCSE), presence of LPDs/GPDs on EEG, unknown etiology and treatment refractoriness. The second cluster linked convulsive and myoclonic SE evolving into NCSE, presence of spontaneous BS on EEG, progressive symptomatic etiology and super-refractoriness. The third cluster linked convulsive and myoclonic SE not evolving into other semiologies, absence of LPDs/GPDs/spontaneous BS on EEG, acute symptomatic etiology and treatment responsiveness.

Conclusions: Distinct electroclinical phenotypes characterized by different response to pharmacological intervention can be identified within the heterogeneity of SE with prominent motor phenomena.

Keywords: hierarchical cluster analysis; phenotypes; status epilepticus.

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Conflict of interest statement

Simona Lattanzi has received speaker's or consultancy fees from Eisai, GW Pharmaceuticals and UCB Pharma, and has served on advisory boards for Arvelle Therapeutics, BIAL and GW Pharmaceuticals. Francesco Brigo has acted as a consultant for Eisai. Eugen Trinka has received speaker's honoraria from UCB, Biogen, Gerot‐Lannach, Bial, Eisai, Takeda, Newbridge, Sunovion Pharmaceuticals Inc., LivaNova and Novartis, consultancy funds from UCB, Biogen, Gerot‐Lannach, Bial, Eisai, Takeda, Newbridge, GW Pharmaceuticals, Sunovion Pharmaceuticals Inc. and Novartis, and directorship funds from Neuroconsult GmbH. E. Trinka's institution received grants from Biogen, Red Bull, Merck, UCB, the European Union, the FWF Österreichischer Fond zur Wissenschaftsförderung and the Bundesministerium für Wissenschaft und Forschung. Stefano Meletti received research grant support from the Ministry of Health, from the nonprofit organization "Fondazione Cassa di Risparmio di Modena ‐ FCRM", and has received personal compensation as a scientific advisory board member for UCB and EISAI. The remaining authors have no conflicts of interest.

Figures

FIGURE 1
FIGURE 1
Dendrogram of clinical features in status epilepticus (SE). Dendrogram based on the hierarchical cluster analysis of clinical data from 240 episodes of SE with prominent motor symptoms. The horizontal axis denotes the linkage distance. Distance is calculated and rescaled from 0 to 25 according to the measure of similarity (Pearson's correlation) and the cluster algorithm (average linkage). The dendrogram shows three major clusters: (i) focal motor SE evolving into non‐convulsive SE (NCSE), presence of lateralized periodic discharges (LPDs)/generalized sharply and/or triphasic periodic discharges (GPDs) on electroencephalogram (EEG), unknown etiology, treatment refractoriness; (ii) myoclonic/convulsive SE evolving into NCSE, presence of spontaneous burst suppression (BS) on EEG, progressive symptomatic etiology, treatment super‐refractoriness; (iii) convulsive only/myoclonic only SE, no LPDs/GPDs/spontaneous BS on EEG, acute symptomatic etiology, treatment responsiveness. Remote symptomatic etiology resulted associated with focal motor SE not evolving into any other semiology. [Colour figure can be viewed at wileyonlinelibrary.com]
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