Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0038220 (status epilepticus)
 7,272 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report the first patient to die from refractory convulsive status epilepticus (SE) after the removal of a stimulator. The removal occurred after a two-year period of successful control of super-refractory convulsive SE with deep brain stimulation of the bilateral anterior nucleus of the thalamus (ANT-DBS). The female patient, born in 1990, suffered from high fever and seizures, and was diagnosed with viral encephalitis in 2005. After four weeks of medical treatment, she recovered with no neurological disabilities, but suffered from monthly seizures. Ten years later, the patient presented with convulsive SE, while four months pregnant in February of 2015. Her SE remained super-refractory to drugs despite the termination of pregnancy. Therefore, ANT-DBS was performed in March of 2015. The patient became SE-free following activation of an ANT-DBS stimulator. However, the stimulation treatment was terminated according to the family's request when a tremor developed two years after the treatment had begun. Subsequently, four SE episodes occurred and the tremor did not improve. The stimulator and electrodes were removed in August of 2017. The patient died of an uncontrolled SE two months later. This case demonstrates the effectiveness of ANT-DBS for emergency super-refractory convulsive SE with both positive and negative outcomes.
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PMID:Deep brain stimulation of the anterior nucleus of the thalamus in a patient with super-refractory convulsive status epilepticus. 3140 65

Febrile infection-related epilepsy syndrome (FIRES) is a severe epilepsy disorder that affects previously healthy children. It carries high likelihood of unfavourable outcome and putative aetiology relates to an auto-inflammatory process. Standard antiepileptic drug therapies including intravenous anaesthetic agents are largely ineffective in controlling status epilepticus in FIRES. Deep brain stimulation of the centromedian thalamic nuclei (CMN-DBS) has been previously used in refractory status epilepticus in only a few cases. The use of Anakinra (a recombinant version of the human interleukin-1 receptor antagonist) has been reported in one case with FIRES with good outcome. Here we describe two male paediatric patients with FIRES unresponsive to multiple anti-epileptic drugs, first-line immune modulation, ketogenic diet and cannabidiol. They both received Anakinra and underwent CMN-DBS. The primary aim for CMN-DBS therapy was to reduce generalized seizures. CMN-DBS abolished generalized seizures in both cases and Anakinra had a positive effect in one. This patient had a favourable outcome whereas the other did not. These are the first reported cases of FIRES where CMN-DBS has been used.
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PMID:Centromedian thalamic nuclei deep brain stimulation and Anakinra treatment for FIRES - Two different outcomes. 3153 Apr 49