Epileptic encephalopathy with electrical status epilepticus during slow sleep: evaluation of treatment response from a tertiary center.

BACKGROUND: This study aimed to evaluate the clinical, electrophysiological, etiological features, and treatment response in children with epileptic encephalopathy with electrical status epilepticus during slow sleep (ESES). METHODS: Clinical data, records of electroencephalograms (EEG), and brain magnetic resonance imaging (MRI) findings of 33 patients with ESES who were treated, and followed up for at least one year were retrospectively analyzed. RESULTS: Of all patients, 57.6% were male, and 42.4% were female. The mean age was 10.45 ± 2.88 years. At first admission, 90% of patients had seizures, and 10% had only school failure. Twelve patients had childhood focal epileptic syndrome. In etiology, asphyxia (n=6), hydrocephalus (n=2), polymicrogyria (n=1), and mesial temporal sclerosis (n=1) were determined. Neurological examination was abnormal in 27.2%, and brain MRI findings were pathological in 36.3% of the patients. During the ESES phase, the spike-wave index (SWI) on the non-rapid eye movement (NREM) sleep EEG was > 85% in 16 patients and 50-85% in 17 patients. Only one patient received one, and the others had at least two antiseizure medications. Benzodiazepines were found to be the most effective treatment. In the two-year follow-up, 24 patients (72.7%) were seizure-free, and nineteen patients (57.5%) had complete recovery of SWI on their NREM sleep EEG. There was a significant correlation with reduction of the SWI on the EEG and seizure control (p < 0.001). In addition, a significant correlation was found between neurocognitive and behavioral scores scored before and after treatment, seizure control, and EEG recovery. CONCLUSIONS: ESES is an epileptic encephalopathy that can be treated safely with antiseizure medications. Neurocognitive examinations and follow-up of EEG findings are valuable in terms of the treatment response. Benzodiazepines were found to be very effective in additional treatment.

EEG Characteristics in COVID-19 Survivors and Non-survivors With Seizures and Encephalopathy.

The objective of this study is to report EEG findings in both COVID-19 survivors and non-survivors who underwent EEG either due to seizure or encephalopathy. Out of total 1468 COVID-19-positive patients, 19 patients underwent EEG. Eight out of 19 patients had a history of seizure disorder and in the remaining 11 with no prior history of seizures, four had a clinical seizure during their hospital stay. Only one had new-onset complex focal status epilepticus on EEG. Amongst the survivors (13/19), the most common EEG findings were normal followed by mild diffuse slowing. Amongst the non-survivors (6/19), the most common EEG finding was moderate to severe slowing in 50% of the patients. It can be deduced that COVID-19 infection does not increase the propensity of epileptiform discharges on EEG. There is perhaps a trend towards increased risk of new-onset status epilepticus in patients with encephalopathy and focal lesions.

Evaluation and management of chimeric antigen receptor (CAR) T-cell-associated neurotoxicity.

Adoptive cell therapies are a group of cancer immunotherapies that involve the infusion of engineered immune cells targeting specific tumor antigens, with chimeric antigen receptor (CAR) T cells at the vanguard of this revolution in cancer therapy. Several CAR T-cell products have been approved for the treatment of leukemia and lymphoma and many more are currently undergoing evaluation in clinical trials for the treatment of other liquid and solid malignancies. Despite their remarkable effectiveness, as with other immunotherapies, CAR T cells are frequently associated with systemic and neurologic toxicity. There has been a major effort by many institutions to develop specific protocols to guide the management of treatment-associated toxicities (eg, cytokine release syndrome [CRS]). However, neurotoxic effects of CAR T-cell therapies are more difficult to evaluate and treat, not easily lending themselves to an algorithmic approach to diagnosis and management. Given the steadily expanding use of CAR T-cell therapies for various malignancies, it is of critical importance for neuro-oncologists to be familiar with the clinical presentation and management principles of CAR T-cell-associated neurotoxicity. Here, we present key principles for the evaluation and management of patients affected by CAR T-cell-associated neurotoxicity based on the most recent evidence.

EEG Findings in Posterior Reversible Encephalopathy Syndrome.

Introduction. Posterior reversible encephalopathy syndrome (PRES) is a relatively common cause of encephalopathy in the hospital setting, and the EEG findings have not been well described. The purpose of this study was to review the EEG findings in a series of patients with PRES. Methods. We retrospectively reviewed our electronic medical record database to identify patients who received a diagnosis of PRES at Tampa General Hospital from January 2016 to October 2017. The diagnosis of PRES was suspected on clinical presentation and confirmed by magnetic resonance imaging. We selected patients with PRES who had received at least 1 EEG. EEGs were interpreted by 2 board-certified electroencephalographers. Results. From January 2016 to October 2017, 19 patients were diagnosed with PRES at Tampa General Hospital. Of those, 10 received at least 1 EEG. Four patients were male, 6 were female. The ages ranged from 21 to 87 (mean was 47). The patients had the following clinical presentations: 5 with encephalopathy, 8 with seizures, 2 with vision changes, and 3 with headache (some patients had more than 1 symptom). EEGs findings were as follows: 3 were normal; 3 showed intermittent generalized slowing; 2 showed continuous generalized slowing; 3 showed background slowing; 1 showed background suppression; 1 showed generalized rhythmic delta activity (GRDA); 1 showed GRDA, plus spike/sharp-wave discharges; 1 showed generalized periodic discharges. The etiologies were as follows: 9 from hypertension, 1 secondary to eclampsia, 3 due to posttransplant immunosuppression, and 1 patient was undergoing chemotherapy (some were multifactorial). Conclusion. EEG findings in PRES are diverse, with no specific or even predominant pattern, based on this small sample size.

Specific EEG markers in POLG1 Alpers' syndrome.

OBJECTIVE: To examine whether rhythmic high-amplitude delta with superimposed (poly)spikes (RHADS) in EEG allow a reliable early diagnosis of Alpers-Huttenlocher syndrome (AHS) and contribute to recognition of this disease. METHODS: EEGs of nine patients with DNA-proven AHS and fifty age-matched patients with status epilepticus were retrospectively examined by experts for the presence of RHADS and for accompanying clinical signs and high-frequency ripples. Reproducibility of RHADS identification was tested in a blinded panel. RESULTS: Expert defined RHADS were found in at least one EEG of all AHS patients and none of the control group. RHADS were present at first status epilepticus in six AHS patients (67%). Sometimes they appeared 5-10 weeks later and disappeared over time. RHADS were symptomatic in three AHS patients and five AHS patients showed distinct ripples on the (poly)spikes of RHADS. Independent RHADS identification by the blinded panel resulted in a sensitivity of 87.5% (95% CI 47-100) and a specificity of 87.5% (95% CI 77-94) as compared to the experts' reporting. CONCLUSION: RHADS are a highly specific EEG phenomenon for diagnosis of AHS and can be reliably recognized. Clinical expression and EEG ripples suggest that they signify an epileptic phenomenon. SIGNIFICANCE: RHADS provide a specific tool for AHS diagnosis.