Treatment of new onset refractory status epilepticus/febrile infection-related epilepsy syndrome with tocilizumab in a child and a young adult.

New onset refractory status epilepticus (NORSE) is a rare and devastating condition occurring in a previously healthy patient. It is called febrile infection-related epilepsy syndrome (FIRES) when preceded by a febrile infection. It often leads to intensive care treatment, including antiseizure drugs in combination with anesthetic agents, and sometimes ketogenic diet. The mortality rate is high, and severe epileptic and neuropsychiatric sequelae are usually observed. Based on the possible role of neuroinflammation, intravenous immunoglobulin, corticosteroids, and immunomodulatory treatment (anti-IL1, IL6) can be added. We describe here a child and a young adult with FIRES, both treated with tocilizumab. We observed a rapid positive response on the status epilepticus and good tolerance, but different neurological outcomes for our two patients. Further prospective studies may be necessary both to confirm the efficacy and the safety of this promising treatment and to optimize the immunomodulatory strategy in FIRES/NORSE.

Cerebral perfusion using ASL in patients with COVID-19 and neurological manifestations: A retrospective multicenter observational study.

BACKGROUND AND PURPOSE: Cerebral hypoperfusion has been reported in patients with COVID-19 and neurological manifestations in small cohorts. We aimed to systematically assess changes in cerebral perfusion in a cohort of 59 of these patients, with or without abnormalities on morphological MRI sequences. METHODS: Patients with biologically-confirmed COVID-19 and neurological manifestations undergoing a brain MRI with technically adequate arterial spin labeling (ASL) perfusion were included in this retrospective multicenter study. ASL maps were jointly reviewed by two readers blinded to clinical data. They assessed abnormal perfusion in four regions of interest in each brain hemisphere: frontal lobe, parietal lobe, posterior temporal lobe, and temporal pole extended to the amygdalo-hippocampal complex. RESULTS: Fifty-nine patients (44 men (75%), mean age 61.2 years) were included. Most patients had a severe COVID-19, 57 (97%) needed oxygen therapy and 43 (73%) were hospitalized in intensive care unit at the time of MRI. Morphological brain MRI was abnormal in 44 (75%) patients. ASL perfusion was abnormal in 53 (90%) patients, and particularly in all patients with normal morphological MRI. Hypoperfusion occurred in 48 (81%) patients, mostly in temporal poles (52 (44%)) and frontal lobes (40 (34%)). Hyperperfusion occurred in 9 (15%) patients and was closely associated with post-contrast FLAIR leptomeningeal enhancement (100% [66.4%-100%] of hyperperfusion with enhancement versus 28.6% [16.6%-43.2%] without, p = 0.002). Studied clinical parameters (especially sedation) and other morphological MRI anomalies had no significant impact on perfusion anomalies. CONCLUSION: Brain ASL perfusion showed hypoperfusion in more than 80% of patients with severe COVID-19, with or without visible lesion on conventional MRI abnormalities.

Recommendations for the use of electroencephalography and evoked potentials in comatose patients.

Predicting the outcome of a comatose or poorly responsive patient is a major issue for intensive care unit teams, in order to give the most accurate information to the family and to choose the best therapeutic option. However, determining the level of cortical activity in patients with disorders of consciousness is a real challenge. Reliable criteria are required to help clinicians in the decision-making process, especially in the acute phase of coma. In this paper, we propose recommendations for recording and interpreting electroencephalography and evoked potentials in comatose patients based on the literature and the clinical experience of a group of neurophysiologists trained in the management of comatose patients. We propose methodological guidelines and discuss prognostic value of each test as well as the limitations concerning recording and interpretation. Recommendations for the strategy and timing of neurophysiological assessments are also proposed according to various clinical situations.

Cardioinhibition can be the unique manifestation of epilepsy-like syncope.

SUMMARY: Sudden death and syncope remain frequently unexplained despite numerous investigations. Here, we report the case of a pacemaker-implanted patient who presented during video-polysomnography recording a complete atrioventricular block simultaneously with an electrical seizure. Remarkably, the patient was completely asymptomatic. He had a history of recurrent syncope previously diagnosed as convulsive vasovagal syncope with cardioinhibition. This observation challenges the current belief that epilepsy-like syncope is a partial complex seizure systematically characterized by a stereotypical clinical course and ending suddenly with syncope. Physicians should know that syncope, followed by jerking movements, of cardiac origin is frequent and often misdiagnosed as epilepsy. Conversely, and although this is a rare condition, they should also be aware of the possibility of epilepsy-like syncope, even in the absence of any other principle symptoms evocative of epilepsy.

Emotional electrodermal response in coma and other low-responsive patients.

Emotional processing in coma remains an open question. Skin conductance responses to emotional and neutral auditory stimuli were recorded in 13 low-responsive patients (12 of whom were in coma). A differential response between emotional and neutral stimuli was found, which significantly correlated with the Glasgow Coma Scale and the Cook and Palma score. These correlations indicate that emotional processing can occur in coma patients with relatively high clinical scores of reactivity.

Cortical information processing in coma.

OBJECTIVES: To evaluate cortical information processing (particularly, semantic processing) in acute nontraumatic coma by means of event-related brain potentials (ERPs). METHODS: The tests included measures of obligatory auditory processing (N100), automatic (Mismatch Negativity) and controlled (P300) detection of stimulus deviance, and semantic processing (ERP effects in word pairs and sentences). The tests were presented to 20 healthy participants and 42 coma patients with Glasgow Coma Scale <9. RESULTS: Responders (ie, patients whose ERP data indicate that their brain was able to process the corresponding stimuli) were found in each ERP test, and their distribution was statistically different from that expected by chance. Particularly, 7 responders were found in the word pair paradigm and 3 responders in the sentence paradigm. The P300 responsiveness highly correlated with other ERP responses, with Glasgow Coma Scale and with the future development of coma (ie, P300 on day 4 was related to the clinical state on day 20). CONCLUSIONS: Results suggest a wide range of cortical information processing in coma, including semantic processing. The question is discussed of whether, and to what extent, these processing operations are related to conscious awareness of stimuli.