Status Epilepticus Manifested as Continuous Epileptic Spasms.

Jianxiang Liao,Jianxiang Liao,Li Chen,Li Chen,Jialun Wen,Jialun Wen,Yuhan Xiao,Yuhan Xiao,Feiqiu Wen,Feiqiu Wen,Tieshuan Huang,Yan Chen,Yan Chen,Lan Xiong,Lan Xiong,Bing Li,Bing Li,Sufang Lin,Sufang Lin,Yan Hu,Myriam Srour,Myriam Srour,Lina Men,Lina Men

doi:10.3389/fneur.2020.00065

Abstract

Objective: The etiology and outcome of status epilepticus with continuous epileptic spasms have not been fully understood; and only rare cases have been reported in the literature. Here, we described 11 children, who manifested continuous epileptic spasms with various etiologies and different outcomes.Methods: This is a case series study designed to systematically review the charts, video-electroencephalography (video-EEG), magnetic resonance images, and longitudinal follow-up of patients who presented continuous epileptic spasms lasting more than 30 min.Results: Median age at onset was 2 years old, ranging from 2 months to 5.6 years. The etiology of continuous epileptic spasms for these 11 cases consisted of not only some known electro-clinical epilepsy syndromes like West Syndrome and Ohtahara Syndrome, but also secondary symptomatic continuous epileptic spasms, caused by acute encephalitis or encephalopathy, which extends the etiological spectrum of continuous epileptic spasms. The most characteristic feature of these 11 cases was prolonged epileptic spasms, lasting for a median of 13.00 days (95% CI: 7.26–128.22 days). The interictal EEG findings typically manifested as hypsarrhythmia or its variants, including burst suppression. Hospital stays were much longer in acute symptomatic cases than in primary epileptic syndromic cases (59.67 ± 50.82 vs. 15.00 ± 1.41 days). However, the long-term outcomes were extremely poor in the patients with defined electro-clinical epilepsy syndromes, including severe motor and intellectual developmental deficits (follow-up of 4.94 ± 1.56 years), despite early diagnosis and treatment. Continuous epileptic spasms were refractory to corticosteroids, immuno-modulation or immunosuppressive therapies, and ketogenic diet.Conclusion: Continuous epileptic spasms were associated with severe brain impairments in patients with electro-clinical syndromes; and required long hospital stays in patients with acute symptomatic causes. We suggest to include continuous epileptic spasms in the international classification of status epilepticus, as a special form. Further investigations are required to better recognize this condition, better understand the etiology, as well as to explore more effective treatments to improve outcomes.

Highlights

Epileptic spasms were recognized as a type of seizure by the International League against Epilepsy (ILAE) seizure classification in 2001 [1]
We identified 11 cases with status epilepticus of continuous epileptic spasms, which lasted longer than 30 min
Their clinical characteristics included: [1] each epileptic spasm episode lasted longer than 30 min though the seizure semiology was similar to the classical epileptic spasms; [2] etiologies were diverse, including acute symptomatic causes such as encephalitis, encephalopathy, and FIRES; even in the cases with known electro-clinical epilepsy syndromes showing different genetic mutations; [3] the disease course and hospital-stay in acute symptomatic cases were much longer than in those with known electro-clinical epilepsy syndromes; [4] the cases with the established electro-clinical syndromes showed the worst outcome, despite earlier diagnosis and interventions

Summary

Introduction

Epileptic spasms were recognized as a type of seizure by the International League against Epilepsy (ILAE) seizure classification in 2001 [1]. Epileptic spasms can be the main seizure type of some primary epilepsy syndromes [7, 8], such as infantile spasms, Ohtahara Syndrome, and West Syndrome. Burst suppression on EEG is one of the features of Ohtahara Syndrome [12,13,14], and hypsarrhythmia and its variants are recognized as the characteristic interictal EEG patterns of West Syndrome [15,16,17], patients with epileptic spasms may not have classic hypsarrhythmia EEG patterns but rather show some periodic and focal patterns [9, 18,19,20], such as hemispheric hypsarrhythmia [21], or even a lack of hypsarrhythmia on EEG [19]. The prognosis of epileptic spasms is generally devastating and is associated with poor neurodevelopmental and behavioral outcomes, in some patients with an underlying genetic etiology such as in CDKL5 encephalopathy, a rare X-linked genetic disorder, and CDKL5 genetic changes or mutations have been found in children diagnosed with infantile spasms, Lennox-Gastaut Syndrome, Rett Syndrome, West Syndrome [12]

Methods

Results

Discussion

Conclusion