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INTRODUCTION: Status Epilepticus (SE) stands as a prominent neurological emergency, showing a mortality rate of approximately 20%. Since February 2021, a worldwide vaccination campaign has been launched against the Coronavirus 19 disease (COVID-19) pandemic. Several possible vaccine-related adverse events have been identified, including neurological manifestations. SE is beginning to surface in literature as an emergent condition in COVID-19-vaccinated individuals, though defined reasons accounting for this correlation are still missing. METHODS: We report two cases of SE related to the SARS-CoV-2 vaccine. In addition, we performed a systematic search of the literature to identify the consistency of the association between the SARS-CoV-2 vaccine and the SE onset. The following databases were consulted: PubMed and Google Scholar. RESULTS: Two novel super-refractory status epilepticus (SRSE) cases associated with the BNT162b2 mRNA COVID-19 vaccine were identified. Both patients received the second dose of the vaccine about 14 days prior to SE onset. Patients showed a non-convulsive semiology and were treated with a combined anesthetic and immunomodulant therapy, leading to SE resolution in both cases. The literature review identified seven additional cases, primarily non-convulsive SE. Four patients received the Spikevax (ex-COVID-19 Moderna mRNA -1273 vaccine), 2 patients the BNT162b2 (Pfizer/Biotech), and 1 patient the ChAdOx1-s (AstraZeneca) vaccine. The first vaccine dose (5/7, 71.4%) emerged as the most frequently associated with SE onset, which manifested at an average of 4.5 days (± 3.4) following vaccination. Five patients presented RSE and required continuous intravenous anesthetic drug administration. Resolution of SE was achieved in all cases. CONCLUSIONS: Status Epilepticus is a rare complication associated with Sars-CoV-2 vaccines. Additional studies are needed to ascertain the potential association between Sars-CoV-2 vaccines and status epilepticus.
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INTRODUCTION: The late onset myoclonic epilepsy in Down Syndrome (LOMEDS) is a peculiar epilepsy type characterized by cortical myoclonus and generalized tonic-clonic seizures (GTCS), in people suffering from cognitive decline in Down syndrome (DS). In this review, we analyzed available data on the diagnostic and therapeutic management of individuals with LOMEDS. METHODS: We performed a systematic search of the literature to identify the diagnostic and therapeutic management of patients with LOMEDS. The following databases were used: PubMed, Google Scholar, EMBASE, CrossRef. The protocol was registered on PROSPERO (registration code: CRD42023390748). RESULTS: Data from 46 patients were included. DS was diagnosed according to the patient's clinical and genetic characteristics. Diagnosis of Alzheimer's dementia (AD) preceded the onset of epilepsy in all cases. Both myoclonic seizures (MS) and generalized tonic-clonic seizures (GTCS) were reported, the latter preceding the onset of MS in 28 cases. EEG was performed in 45 patients, showing diffuse theta/delta slowing with superimposed generalized spike-and-wave or polyspike-and-wave. A diffuse cortical atrophy was detected in 34 patients on neuroimaging. Twenty-seven patients were treated with antiseizure medication (ASM) monotherapy, with reduced seizure frequency in 17 patients. Levetiracetam and valproic acid were the most used ASMs. Up to 41% of patients were unresponsive to first-line treatment and needed adjunctive therapy for seizure control. CONCLUSIONS: AD-related pathological changes in the brain may play a role in LOMEDS onset, although the mechanism underlying this phenomenon is still unknown. EEG remains the most relevant investigation to be performed. A significant percentage of patients developed a first-line ASM refractory epilepsy. ASMs which modulate the glutamatergic system may represent a good therapeutic option.