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Epilepsy : the ultimate teen guide
An updated overview of epilepsy that specifically addresses how this condition can affect teens. Epilepsy: The Ultimate Teen Guide, Second Edition gives positive, factual information and explains how young people can take control of their situations by understanding, managing, treating, and living normal lives with epilepsy. This edition includes updated chapters, resource lists, and statistics.
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The Antiepileptic Effect of the Glycolytic Inhibitor 2-Deoxy-d-Glucose is Mediated by Upregulation of K sub(ATP) Channel Subunits Kir6.1 and Kir6.2
Metabolic modulation of neuronal excitability is becoming increasingly important as an antiepileptic therapy. It was reported that the glycolytic inhibitor 2-deoxy-d-glucose (2-DG) and the activation of the ATP-sensitive potassium ion channel (K sub(ATP) channel) had an antiepileptic effect in models of epilepsy. To explore whether 2-DG exerts an antiepileptic effect through upregulation of the K sub(ATP) channel subunits Kir6.1 and Kir6.2, the expression of these subunits in hippocampus of five groups of mice with pilocarpine-induced status epilepticus (SE) was evaluated. A seizure group with pilocarpine-kindling convulsions (EP) was compared to similar groups treated with high, medium, and low 2-DG concentrations (100-500 mg/kg) and a normal control group (Con). Kir6.1 and Kir6.2 mRNAs and proteins were analyzed at 4 h, 1 days (acute period), 7 days (latent period), 30, and 60 days (chronic period) following SE. In the seizure group (compared to the Con group), hippocampal expression of Kir6.1 and Kir6.2 increased dramatically at 1, 7, and 30 days, and was further increased after treatment with medium and high dose 2-DG (all P < 0.05). Our results suggest that 2-DG may exert an antiepileptic effect through up-regulation of mRNAs and protein levels of Kir6.1 and Kir6.2, which may therefore be used as molecular targets in the treatment of epilepsy with 2-DG.
Journal Article
58 Toclizumab for RSE in those with or without pre-existing epilepsy – time for a trial?
Refractory status epilepticus (RSE) is defined as prolonged or repeated seizures without recovery despite the initiation of at least two anti-seizure medications (ASMs). It is a neurological emergency, with potentially devasting short term consequences and significant risk of longer-term morbidity.Tocilizumab is a humanized monoclonal antibody against the IL-6 receptor, used primarily for rheumatological conditions and cytokine-release syndrome. It is thought to promote an anti-seizure effect for patients with status epilepticus via downregulation of IL-6, a component of the acute phase response. Tocilizumab has been successful in treating new onset refractory status epilepticus (NORSE) that has been refractory to other treatments, including rituximab, though has been associated with significant adverse events.We present five cases of RSE, including two with a pre-existing history of seizures. Toclizumab administered within 18 days of presentation (8mg/kg, second dose after 4 weeks) terminated status in four cases, longer term outcomes varied depending on the underlying aetiology.All patients were received steroids and a minimum of five ASMs in the context of sedation pre-Toclizumab. Plasma exchange had been given in four cases.There is a potential role for Toclizumab in the early treatment of RSE.asha.patel1@nhs.net
Journal Article
88 Exploding head syndrome complicating diagnosis and management in a patient with epilepsy
BackgroundExploding Head Syndrome (EHS) is a benign parasomnia characterized by sudden, loud noises or explosive sensations occurring during sleep-wake transitions. Although it is distinct, EHS can be misinterpreted as seizures, complicating diagnosis and management.Case PresentationA 41-year-old Caucasian male with a history of well-controlled combined generalized and focal epilepsy, diagnosed 28 years ago, presented with episodes of sudden perception of loud noises and a pressure-like sensation, associated with an inability to move from the chest up during sleep. These episodes have worsened with an increase of nocturnal carbamazepine dose. The episodes occurred independently of seizures, and he remained fully oriented through them. Magnetic resonance imaging showed focal encephalomalacia in the left frontal operculum and in the superior insular region compatible with chronic ischemic changes. The electroencephalogram was unremarkable. This presentation was compatible with EHS.The patient was reassured, referred for cognitive behavioural therapy, and started on Melatonin.ConclusionThis case highlights the importance of differentiating EHS from seizures, as it can mimic a worsening of epilepsy despite being completely benign. Accurate diagnosis prevents unnecessary changes in antiepileptic treatment. Further studies are required to explore the relationship of the individual antiepileptics in epilepsy patients having EHS.sathyajith.ambawatte@gmail.com
Journal Article
175 Hypophosphataemia as a potential biological marker in seizure diagnosis: a retrospective analysis
IntroductionDiagnosing epileptic seizures is challenging due to overlapping symptoms with non-epileptic events and limited clinical descriptions. Anecdotal case series highlights hypophosphatemia as an indicator of an epileptic seizure.ObjectivesThis study examines acute hypophosphatemia prevalence in patients at first seizure clinic and explores its association with different sub-groups and clinical variables. A statistical model that includes hypophosphatemia to predict the likelihood of an ictal-event will be explored.MethodologyA cross-sectional cohort study of patients seen at our First Seizure Clinic over a year period. Patients will be grouped into 2 major groups – ictal-event vs non-ictal event, based on expert clinician assessment. Investigation results, including detailed blood work-up, done within 24 hours of the event will be collected.ResultsPreliminary results (n=83) include 50 ictal events (60.2%) and 33 non-ictal events (39.8%). Mean age: 38.1 years (range 17–88), with 45 (54.2%) male. Hypophosphatemia occurred in 26.5% of cases, significantly higher in ictal (36%) vs. non-ictal (12.1%) events (p < 0.05). Blood samples within 2 hours showed a stronger association with hypophosphatemia in ictal events.ConclusionHypophosphataemia shows promising potential as a diagnostic marker for epileptic seizures. With further research, it could become a useful tool in improving diagnostic accuracy.ammar.abdelaziz1@nhs.net
Journal Article
237 A novel form of geographic specific reflex epilepsy
We present a case of a ‘Location specific reflex epilepsy’ of temporal lobe semiology aggravated by a particular geographical location and visual scene which has been captured in ambulatory EEG in a 34yr women. Patient started having stereotypical complex partial seizures initiating with a particular sequence of nausea resulting in dejavu further evolving from focal to bilateral tonic-clonic seizures. Seizures were mainly triggered in a specific geographical region as episodes occurred when she is driving up the road to her house in Omagh. In addition she has history of nocturnal seizures triggered when she had a dream of driving along the same lane from her home town going up towards family house. Brain MRI showed no epileptogenic lesion and inter-ictal EEG showed bilateral independent epileptiform discharges over temporal regions, with a left-sided emphasis. Clinical seizure was captured on ambulatory EEG whilst travelling along the same lane in Omagh. The fact that her seizures occur in a specific situation is exceptionally rare and to this date only one other case has ever been reported in the literature similar to ours. Patient responded well to Lamotrigine. Pathophysiology of reflex epilepsies postulates that simple triggers activate specific cortical regions in a circumscribed manner but complex stimuli may activate wider functional circuits where hyper-excitable areas could overlap with areas activated by specific stimuli. Further study of aberrant network dysfunction is required in such individuals.dhruvock27@yahoo.com
Journal Article
128 Think about whipple’s…no, not that one
A 58-year-old male presented with two generalised tonic-clonic seizures with post-ictal paranoia. Three months prior, he described an episode of being ‘hyper’ - characterised by running up and down the stairs. Investigations demonstrated mild hippocampal asymmetry on MRI, and temporal slowing (L>R) on EEG. He was initially diagnosed with focal onset epilepsy and discharged on levetiracetam. Over the following four months he continued to have episodes of abnormal behaviour, although without further tonic-clonic seizures. On further questioning it became evident that similar episodes had been occurring for the previous 3 years. During these episodes, various behaviours were described; laughing, running around, jumping on beds, doing handstands, pulling faces, opening and closing curtains - all of which self-terminated after a few hours. Consciousness was never impaired. The episodes frequently occurred on waking, were associated with diaphoresis, and improved with sugary foods. Reviewing ambulance records to previous callouts, hypoglycaemia was noted on initial assessment (blood glucose 1.2 - 2.3 mmol/L). A 72 hour fast was subsequently arranged, demonstrating fasting hypoglyaemia and inappropriate endogenous insulin secretion. Pancreatic imaging confirmed the presence of an insulinoma. Following resection, the patient is now symptom-free and off all medication.alexander.wallace2@wales.nhs.uk
Journal Article
8216 Paradoxical relief in the diagnosis of functional seizures
BackgroundDifferentiating between epileptic seizures (ES) and functional seizures (FS) without video evidence is a common yet challenging presentation in several healthcare environments. Often, the absence of a witnessed collateral history can make this diagnosis even more difficult.Post-ictally, ES classically lead to prolonged lethargy, confusion and headache whilst FS tend to have a much shorter recovery however in practice this may not always be clear.Case reports have anecdotally reported that individuals with FS can sometimes report a sense of relief after their seizures.1 However, this has never been assessed prospectively in a large group of individuals with FS or compared to those with ES.ObjectiveWe aimed to explore the phenomenon of a sense of relief after seizures (paradoxical relief) as a diagnostic tool for FS. We hypothesised that patients with FS may be more likely to feel a sense of relief after seizures than patients with ES.MethodWe prospectively asked 167 patients admitted to a tertiary video telemetry unit if they experienced paradoxical relief. The patients then underwent video telemetry, the gold standard for diagnosis of epileptic and functional seizures.ResultWithin this cohort of 167 patients, 82 patients had events whilst on EEG. 56 patients had FS, and 26 patients had ES. 43 out of the 56 patients with FS described paradoxical relief whereas 2 out of the 26 patients with ES described paradoxical relief. This gives the presence of paradoxical relief in FS a sensitivity of 0.77 and specificity of 0.92. The positive predictive value of presence of paradoxical relief in patients with FS was 0.96. The negative predictive value was 0.65.Some comments made by patients included:It is as if I feel earthed or fused again … whole again.’‘Sometimes it feels like it needed to happen to get my energy back’.‘This strange build up and strange taste has gone ... I am kind of glad it has happened.’ConclusionIn conclusion, paradoxical relief is present in most individuals with FS but not in patients with ES. The single question of presence of paradoxical relief may be valuable in the diagnosis of FS, especially where further investigation or collateral history is not available. The presence of paradoxical relief in patients with FS may be useful to further our understanding of the mechanisms underlying FS.ReferenceStone J, Carson AJ. The unbearable lightheadedness of seizing: wilful submission to dissociative (non-epileptic) seizures. J Neurol Neurosurg Psychiatry 2013;84(7):822–4. doi: 10.1136/jnnp-2012-304842. Epub 2013 Mar 28. PMID: 23538071.
Journal Article
205 Adjuvant cenobamate in adult lennox-gastaut syndrome patients post-cannabidiol
ObjectivesTo report efficacy of adjuvant cenobamate in adults with Lennox-Gastaut syndrome (LGS) following cannabidiol.MethodologyA retrospective record review was conducted of patients with LGS and ongoing seizures commenced on adjuvant cenobamate after cannabidiol, with changes in seizure frequency and concomitant anti-seizure medications (ASMs) reviewed.ResultsFifteen patients (7 male) started on cenobamate of whom fourteen were still on cannabidiol as they fulfilled NICE criteria for continuing prescribing. Of these, 8 had a Vagus Nerve Stimulator (VNS) in situ while an additional 5 were VNS non-responsive. Three had previous surgery (two corpus callosotomy; one left occipital tumour resection). Four had previously tried a ketogenic diet. Eleven had severe intellectual disability. All 15 were on ≥3 ASMs.Cenobamate dose range was 50-400 mg (median 200mg). Three were still titrating. In eight seizure improvement was >50% (including 2 >75%). Two stopped due to side effects and lack of efficacy. Concomitant ASM were simplified in 11 whereby 2 were able to withdraw at least one ASM and 9 were able to reduce doses of more than 2 ASMs.ConclusionAdjuvant cenobamate in adult LGS patients following cannabidiol offers further seizure control and reduces ASM burden as demonstrated in this drug resistant cohort.pyae.aung2@nhs.net
Journal Article
103 Outcome of seizure video MDT in a regional epilepsy service
BackgroundPatient generated videos can aid diagnosis of seizure disorders but interpretation can be difficult. We set up a seizure video MDT to standardise decision making from patient videos.MethodsWe conducted a service evaluation on the Seizure Video MDT. Between June 2022 and June 2024, a total of 94 patients were referred to the MDT.ResultsA clear diagnosis based on the video was obtained in 69 patients (73%), of which 28 were diagnosed with functional seizures, 30 with epilepsy and 1 with FND. Of the remaining, 10 patients were felt to have a disorder that was not seizure related, in 6 patients the video was not diagnostic due to quality and in 17 patients the diagnosis remained unclear.An outcome was documented in the patient recorded for 85 patients. A change in management occurred in 50 patients (58%). This included referral to NEAD service (12), review of medication (5) clarification of diagnosis for both clinician/patient (6), review of syndrome (2), referral to specialist clinic (2), further investigations (11), referral to VT (9) or advice for further history/videos (3).ConclusionsSeizure video MDT enables diagnosis to be made in most cases without the need for inpatient video telemetry.robyn.terry@nca.nhs.uk
Journal Article