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A case series of super-refractory status epilepticus successfully treated with electroconvulsive therapy

Tan HJ, Tee TY, Husin M, Khoo CS, Woon LS

Epileptic Disord, 2020 Dec 01;22(6):828-833.
PMID: 33337333 DOI: 10.1684/epd.2020.1233

Super-refractory status epilepticus (SRSE) is a neurocritical emergency, associated with significant morbidity and mortality. The precise pathophysiology is still not completely understood. The likelihood of spontaneous seizure termination reduces with time, and it is of paramount importance to abort status in order to prevent permanent long-term neurological sequelae and death. A few neuroprotective strategies, such as general anaesthesia, steroids, ketogenic diet and hypothermia, have been used to treat SRSE, however, the clinical outcome remains inconclusive. We herein present two cases of SRSE, which were successfully treated with electroconvulsive therapy (ECT) after failing all pharmacological measures.

Matched MeSH terms: Status Epilepticus/physiopathology; Status Epilepticus/therapy*
EpiNet study of incidence of status epilepticus in Auckland, New Zealand: Methods and preliminary results

Bergin PS, Brockington A, Jayabal J, Scott S, Litchfield R, Roberts L, et al.

Epilepsia, 2018 10;59 Suppl 2:144-149.
PMID: 30159885 DOI: 10.1111/epi.14478

The EpiNet project has been commenced to facilitate investigator-led collaborative research in epilepsy. A new Web-based data collection tool has been developed within EpiNet to record comprehensive data regarding status epilepticus and has been used for a study of status epilepticus in Auckland, New Zealand. All patients aged >4 weeks who presented to any of the five public hospitals and the major private hospital within Auckland city (population = 1.61 million) with an episode of status epilepticus between April 6, 2015 and April 5, 2016 were identified using multiple overlapping sources of information. For this study, status epilepticus was defined as any seizure exceeding 10 minutes in duration, or repeated seizures lasting >10 minutes without recovery between seizures. Patients who had either convulsive or nonconvulsive status epilepticus were included. Episodes of status epilepticus were classified according to the 2015 International League Against Epilepsy ILAE status epilepticus classification. A total of 477 episodes in 367 patients were considered as definite or probable status epilepticus; 285 episodes (62%) lasted >30 minutes, which is the duration that has previously been used for epidemiological studies of status epilepticus.

Matched MeSH terms: Status Epilepticus/diagnosis; Status Epilepticus/epidemiology*; Status Epilepticus/physiopathology*
MR imaging of cerebral and cerebellar hypervascularity associated with status epilepticus: case report

Mukhtar AA, Ibrahim LS, Khairil AO

Nepal Med Coll J, 2007 Dec;9(4):289-91.
PMID: 18298025

A 20 year old male presented to the emergency department with generalized tonic clonic convulsions and up rolling of eye balls. He had history of seizure disorder for three years and on regular medical treatment and is compliant to medication. A non-contrast CT scan of the head was only done on 14th day of admission which showed hypodense areas in the right and left cerebellar hemisphere. MR imaging was performed four days later revealed high signal intensity in the both cerebellar hemispheres, both external capsules, both occipital and right parietal regions on fluid-attenuated inversion recovery (FLAIR). The post contrast MR imaging revealed diffuse cerebral and cerebellar hypervascularity in the similar region. This change of diffuse hypervascularity of both cerebral and cerebellar associated with seizure activity on post-contrast magnetic resonance imaging (MRI) has not been reported in any literature.

Matched MeSH terms: Status Epilepticus/diagnosis*; Status Epilepticus/pathology
Fulltext G2677T polymorphism can predict treatment outcome of Malaysians with complex partial seizures being treated with Carbamazepine

Subenthiran S, Abdullah NR, Muniandy PK, Joseph JP, Cheong KC, Ismail Z, et al.

Genet. Mol. Res., 2013;12(4):5937-44.
PMID: 24338387 DOI: 10.4238/2013.November.26.3

Carbamazepine (CBZ) is used as the first line of treatment of complex partial seizures (CPS) in Malaysia. While this drug is known to be effective for the treatment of CPS, more than 30% of patients remain drug resistant to CBZ mono-therapy. We examined a possible relationship between patients' response to CBZ mono-therapy and the G2677T SNP of the ABCB1 gene. Three hundred and fourteen patients with CPS were recruited from the Neurology Department of the Kuala Lumpur Hospital, of whom 152 were responders and the other 162 were non-responders to CBZ mono-therapy. DNA was extracted from blood samples and real-time PCR was performed to detect the G2677T SNP of the ABCB1 gene. Results were described as genotype frequencies and compared by logistic regression analysis. Among the 152 responders, 74% had the GG genotype. However, among the 162 non-responders, 26.5% had the GT genotype and 39% had the TT genotype. There was a significant difference in genotype frequency (TT vs GG; odds ratio 4.70; 95% confidence interval, 2.70-8.20) between responders and non-responders. The presence of the T allele of the G2677T SNP appears to be a useful screening marker to determine if a patient is going to be resistant to CBZ as a single drug therapy in the treatment of CPS.

Matched MeSH terms: Status Epilepticus/drug therapy; Status Epilepticus/genetics*
Fulltext Acute disseminated encephalomyelitis: a report of six cases

Thomas GST, Hussain IHMI

Med J Malaysia, 2004 Aug;59(3):342-51.
PMID: 15727380 MyJurnal

Six children with Acute Disseminated Encephalomyelitis (ADEM) were seen at the Penang Hospital over a two year period (July 1999-June 2001). Diagnosis was based upon typical clinical features and characteristic findings on neuroimaging. Cerebrospinal fluid examination and other investigations were done, where appropriate, to rule out other causes of central nervous system disease. Three children had a prodromal illness. The most common presenting symptoms were fever, seizures, ataxia, focal neurological deficits and labile mood. Two children presented with status epilepticus. All children had an abnormal neurological examination. Brain magnetic resonance imaging revealed hyperintense signals on T2-weighted and FLAIR sequences in the subcortical and deep white matter regions of the frontal, parietal, and temporal lobes, as well as in the thalami, cerebellum and brainstem. One child had multiphasic disseminated encephalomyelitis (three episodes). The child with multiphasic disease had only one treated episode, and has suffered mild disability. Three children were treated with either methylprednisolone or immunoglobulins, and remain well. One child received both treatments but expired as a result of severe gastrointestinal bleeding from the use of methylprednisolone. The child who was not treated has severe disability.

Matched MeSH terms: Status Epilepticus/etiology; Status Epilepticus/therapy
Fulltext Subdural collections due to non-typhi Salmonella infections in two Malaysian children

Intan HI, Zubaidah CD, Norazah A, Norlijah O

Singapore Med J, 2008 Jul;49(7):e186-9.
PMID: 18695854

Subdural collections caused by Salmonella infection are rarely encountered in children. We present two cases caused by non-typhi Salmonella, one a four-and-a-half-month-old boy presenting with subdural effusion, and the other, a 16-month-old boy with empyema. The diagnosis was confirmed on blood and subdural pus cultures. One patient had status epilepticus following focal fit, and the other had prolonged fever without any localising signs of infection on admission. They responded well to prompt surgical drainage and prolonged systemic antibiotic therapy. Contrary to previous reports, both patients showed favourable outcome in terms of neurological sequelae.

Matched MeSH terms: Status Epilepticus/diagnosis; Status Epilepticus/microbiology
The evolution of the pilocarpine animal model of status epilepticus

Ahmed Juvale II, Che Has AT

Heliyon, 2020 Jul;6(7):e04557.
PMID: 32775726 DOI: 10.1016/j.heliyon.2020.e04557

The pilocarpine animal model of status epilepticus is a well-established, clinically translatable model that satisfies all of the criteria essential for an animal model of status epilepticus: a latency period followed by spontaneous recurrent seizures, replication of behavioural, electrographic, metabolic, and neuropathological changes, as well as, pharmacoresistance to anti-epileptic drugs similar to that observed in human status epilepticus. However, this model is also characterized by high mortality rates and studies in recent years have also seen difficulties in seizure induction due to pilocarpine resistant animals. This can be attributed to differences in rodent strains, species, gender, and the presence of the multi-transporter, P-glycoprotein at the blood brain barrier. The current paper highlights the various alterations made to the original pilocarpine model over the years to combat both the high mortality and low induction rates. These range from the initial lithium-pilocarpine model to the more recent Reduced Intensity Status Epilepticus (RISE) model, which finally brought the mortality rates down to 1%. These modifications are essential to improve animal welfare and future experimental outcomes.

Matched MeSH terms: Status Epilepticus
Fulltext Status epilepticus as the initial presentation of antibody-negative Goodpasture's syndrome

Ting IP, Abdul Halim S, Adnan A, Jaafar H

BMJ Case Rep, 2017 Aug 01;2017.
PMID: 28765179 DOI: 10.1136/bcr-2017-219628

Goodpasture's syndrome is a rare pulmonary-renal disease. It is characterised by presence of auto-antibodies directed against the glomerular basement membrane (GBM) antigen. These antibodies that bind to the GBM antigens cause rapidly progressive glomerulonephritis. The alveolar basement membrane also contains similar antigen, leading to pulmonary haemorrhage in active disease. We report a case of a young man who initially presented with status epilepticus and later was found to have rapidly progressive glomerulonephritis with pulmonary haemorrhage. Serum anti-GBM antibody was negative but the renal biopsy confirmed the diagnosis by showing typical linear IgG along the GBM on immunofluorescent study. He was treated with plasmapheresis and high-dose steroid in combination with oral cyclophosphamide. His renal function normalised after treatment.

Matched MeSH terms: Status Epilepticus/immunology; Status Epilepticus/pathology*; Status Epilepticus/therapy
Status epilepticus in Auckland, New Zealand: Incidence, etiology, and outcomes

Bergin PS, Brockington A, Jayabal J, Scott S, Litchfield R, Roberts L, et al.

Epilepsia, 2019 08;60(8):1552-1564.
PMID: 31260104 DOI: 10.1111/epi.16277

OBJECTIVE: To determine the incidence, etiology, and outcome of status epilepticus (SE) in Auckland, New Zealand, using the latest International League Against Epilepsy (ILAE) SE semiological classification.
METHODS: We prospectively identified patients presenting to the public or major private hospitals in Auckland (population = 1.61 million) between April 6, 2015 and April 5, 2016 with a seizure lasting 10 minutes or longer, with retrospective review to confirm completeness of data capture. Information was recorded in the EpiNet database.
RESULTS: A total of 477 episodes of SE occurred in 367 patients. Fifty-one percent of patients were aged <15 years. SE with prominent motor symptoms comprised 81% of episodes (387/477). Eighty-four episodes (18%) were nonconvulsive SE. Four hundred fifty episodes occurred in 345 patients who were resident in Auckland. The age-adjusted incidence of 10-minute SE episodes and patients was 29.25 (95% confidence interval [CI] = 27.34-31.27) and 22.22 (95% CI = 20.57-23.99)/100 000/year, respectively. SE lasted 30 minutes or longer in 250 (56%) episodes; age-adjusted incidence was 15.95 (95% CI = 14.56-17.45) SE episodes/100 000/year and 12.92 (95% CI = 11.67-14.27) patients/100 000/year. Age-adjusted incidence (10-minute SE) was 25.54 (95% CI = 23.06-28.24) patients/100 000/year for males and 19.07 (95% CI = 16.91-21.46) patients/100 000/year for females. The age-adjusted incidence of 10-minute SE was higher in Māori (29.31 [95% CI = 23.52-37.14]/100 000/year) and Pacific Islanders (26.55 [95% CI = 22.05-31.99]/100 000/year) than in patients of European (19.13 [95% CI = 17.09-21.37]/100 000/year) or Asian/other descent (17.76 [95% CI = 14.73-21.38]/100 000/year). Seventeen of 367 patients in the study died within 30 days of the episode of SE; 30-day mortality was 4.6%.
SIGNIFICANCE: In this population-based study, incidence and mortality of SE in Auckland lie in the lower range when compared to North America and Europe. For pragmatic reasons, we only included convulsive SE if episodes lasted 10 minutes or longer, although the 2015 ILAE SE classification was otherwise practical and easy to use.

Matched MeSH terms: Status Epilepticus/etiology; Status Epilepticus/mortality; Status Epilepticus/epidemiology*
Iatrogenic tracheal flap mimicking tracheal stenosis with resultant stridor

Tan KL, Chong AW, Amin MA, Raman R

J Laryngol Otol, 2012 Jul;126(7):751-5.
PMID: 22578299 DOI: 10.1017/S0022215112000795

To illustrate a case of an iatrogenic mucosal tear in the trachea which caused a one-way valve effect, obstructing the airway and manifesting as post-extubation stridor.

Matched MeSH terms: Status Epilepticus/complications; Status Epilepticus/therapy
Fulltext EEG is sensitive in early diagnosis of anti NMDAR encephalitis and useful in monitoring disease progress

Abdullah S, Lim KS, Wong WF, Tan HJ, Tan CT

Neurology Asia, 2015;20(2):167-175.
MyJurnal

Background& Objective: Investigation modalities, such as MRI and CSF examination, are neither sensitive nor specific in the early phase of anti-NMDAR encephalitis. Nuclear imaging may be useful to monitor the response to treatment but limited by the availability.We aimed to determine the role of EEG as a tool for early diagnosis as well as a tool to assess disease progression and response to treatment. Methods: A total of 99 EEGsdone in 16 patients diagnosed with anti-NMDAR encephalitis throughout the course of illness, were reviewed retrospectively. The EEG changes were correlated with the clinical presentations and response to treatment. Sixteen EEGs of patients with schizophrenia and mood disorder, and 10 EEGs of patients with infective encephalitis were included as control. Results: EEGs performed during the psychiatric and cognitive dysfunctionphase in patient with anti-NMDAR encephalitis, showed diffuse background slowing in the delta-theta range in all the patients. Serial EEGs showed that the dominant background frequency improved with improvement in cognitive status. Nine patients had complete recovery with normalisation of the EEG abnormalities. Eight patients had their typical clinical seizure recorded during EEG monitoring, but only 2 (25.0%) with EEG correlation. Ten patients had status epilepticus (62.5%), 5 had EEG recorded during their status epilepticus, of which only one with EEG correlation (20.0%). Eleven patients had asymmetric background (68.8%), but only 1 has correlation with focal changes in the MRI brain (9.1%). Even though the EEGs of patients with infective encephalitis also showed background slowing, their CSF analysis was supportive of an infective cause. EEGs of patients with established psychiatric disorder were within normal limits.
Conclusion: EEG abnormality has a good correlation with the degree of psychiatric and cognitive dysfunction in patient with anti-NMDAR encephalitis, and is useful in early diagnosis, monitoring the progress and the response to treatment. However, it has poor correlation with clinical seizures.

Matched MeSH terms: Status Epilepticus
Fulltext Lack of meaningful genotype-phenotype association in SCN1A-related infantile-onset epileptic encephalopathies

Siti Aishah Abdul Wahab, Yusnita Yakob, Khoo,Teik-Beng, Sangita Dharshini Terumalay, Vigneswari Ganesan, Teh,Chee-Ming, et al.

Neurology Asia, 2017;22(2):99-111.
MyJurnal

Background & Objective: SCN1A gene which encodes for sodium channel alpha 1 subunit has been
found to be the most common mutated gene in patients with epilepsy. This study aims to characterize the
SCN1A mutations as well as to describe genotype and phenotype association in children with SCN1Arelated
infantile-onset epileptic encephalopathies in Malaysia.

Methods: Children with infantile-onset
epileptic encephalopathy mostly suspected to have Dravet syndrome who had mutational analysis for
SCN1A gene from hospitals all over Malaysia were included in the study. Their epilepsy syndrome
diagnosis was classified into severe myoclonic epilepsy in infancy and its variants. Polymerase chain
reaction and bidirectional sequencing were used to identify SCN1A mutations.

Results: A total of 38
children with heterozygous mutations were analysed, 22 (57.9%) of which were novel mutations.
Truncated mutations were the most common mutation type (19, 50%). Other mutation types were
missense mutations (14, 36.8%), splice site mutations (4, 10.5%) and in-frame deletion (1, 2.6%). The
mean age of seizure onset was 4.7 months. Seizure following vaccination was observed in 26.3% of
the children. All of them had drug resistant epilepsy. There was no significant association between
the type of mutation with the syndromic diagnosis, age of seizure onset, tendency of the seizures to
cluster or having status epilepticus, mean age when developmental delay was observed and response
to various antiepileptic drugs.

Conclusion: This study expands the spectrum of SCN1A mutations and proves the importance of
SCN1A gene testing in diagnosing infantile-onset epileptic encephalopathies patients. Although, our
study does not support any clinically meaningful genotype-phenotype association for SCN1A-related
infantile-onset epileptic encephalopathies, the clinical characteristics of our cohort are similar to those
that have been described in previous studies.

Matched MeSH terms: Status Epilepticus
Fulltext Tualang Honey Reduced Neuroinflammation and Caspase-3 Activity in Rat Brain after Kainic Acid-Induced Status Epilepticus

Mohd Sairazi NS, Sirajudeen KNS, Muzaimi M, Mummedy S, Asari MA, Sulaiman SA

Evid Based Complement Alternat Med, 2018;2018:7287820.
PMID: 30108663 DOI: 10.1155/2018/7287820

The protective effect of tualang honey (TH) on neuroinflammation and caspase-3 activity in rat cerebral cortex, cerebellum, and brainstem after kainic acid- (KA-) induced status epilepticus was investigated. Male Sprague-Dawley rats were pretreated orally with TH (1.0 g/kg body weight) five times at 12 h intervals. KA (15 mg/kg body weight) was injected subcutaneously 30 min after last oral treatment. Rats were sacrificed at 2 h, 24 h, and 48 h after KA administration. Neuroinflammation markers and caspase-3 activity were analyzed in different brain regions 2 h, 24 h, and 48 h after KA administration. Administration of KA induced epileptic seizures. KA caused significant (p < 0.05) increase in the level of tumor necrosis factor alpha (TNF-α), interleukin 1 beta (IL-1β), glial fibrillary acidic protein (GFAP), allograft inflammatory factor 1 (AIF-1), and cyclooxygenase-2 (COX-2) and increase in the caspase-3 activity in the rat cerebral cortex, cerebellum, and brainstem at multiple time points. Pretreatment with TH significantly (p < 0.05) reduced the elevation of TNF-α, IL-1β, GFAP, AIF-1, and COX-2 level in those brain regions at multiple time points and attenuated the increased caspase-3 activity in the cerebral cortex. In conclusion, TH reduced neuroinflammation and caspase-3 activity after kainic acid- (KA-) induced status epilepticus.

Matched MeSH terms: Status Epilepticus
Fulltext Role of Pre-Synaptic NMDA Receptors in the Modulation of Inhibitory Synaptic Transmission in Sensory-Motor and Visual Cortical Pyramidal Neurons in Brain Slices of Young Epileptic Mice

Lah MHC, Reza F, Begum T, Abdullah JM

Malays J Med Sci, 2018 May;25(3):27-39.
PMID: 30899185 MyJurnal DOI: 10.21315/mjms2018.25.3.4

Background: Previous studies from animal models have shown that pre-synaptic NMDA receptors (preNMDARs) are present in the cortex, but the role of inhibition mediated by preNMDARs during epileptogenesis remains unclear. In this study, we wanted to observe the changes in GABAergic inhibition through preNMDARs in sensory-motor and visual cortical pyramidal neurons after pilocarpine-induced status epilepticus.
Methods: Using a pilocarpine-induced epileptic mouse model, sensory-motor and visual cortical slices were prepared, and the whole-cell patch clamp technique was used to record spontaneous inhibitory post-synaptic currents (sIPSCs).
Results: The primary finding was that the mean amplitude of sIPSC from the sensory-motor cortex increased significantly in epileptic mice when the recording pipette contained MK-801 compared to control mice, whereas the mean sIPSC frequency was not significantly different, indicating that post-synaptic mechanisms are involved. However, there was no significant pre-synaptic inhibition through preNMDARs in the acute brain slices from pilocarpine-induced epileptic mice.
Conclusion: In the acute case of epilepsy, a compensatory mechanism of post-synaptic inhibition, possibly from ambient GABA, was observed through changes in the amplitude without significant changes in the frequency of sIPSC compared to control mice. The role of preNMDAR-mediated inhibition in epileptogenesis during the chronic condition or in the juvenile stage warrants further investigation.

Matched MeSH terms: Status Epilepticus
Fulltext Clinical experience of medical students at University Sains Malaysia

Quah BS, Malik AS, Simpson H

Malays J Med Sci, 2000 Jan;7(1):27-32.
PMID: 22844212 MyJurnal

Experience of acute medical, surgical conditions, and clinical procedures of undergraduate students were assessed via a questionnaire survey during the final week of the 1993/1998 programme at the School of Medical Sciences, Univestiti Sains Malaysia. Individual performances were assessed by a scoring system. One hundred and twenty four students responded, (response rate 97%). More than 90% had seen myocardial infarction, cerebrovascular accident, pneumonia, respiratory distress, gastroenteritis, coma, and snake bite. Less than 33% had witnessed acute psychosis, diabetic ketoacidosis, acute hepatic failure, status epilepticus, near drowning, hypertensive encephalopathy, acute haemolysis or child abuse.Acute surgical/obstetrics cases, seen by >90% students, included fracture of long bones, head injury, acute abdominal pain, malpresentation and foetal distress. Less than 33% had observed epistaxis, sudden loss of vision, peritonitis or burns. Among operations only herniorrhaphy, Caesarian section, internal fixation of fracture and cataract extraction were seen by >80% students. The main deficits in clinical procedures are in rectal and vaginal examinations, urine collection and microscopic examinations. The performance of individual students, assessed by a scoring system, showed 15 students had unacceptably low scores (<149/230, 50%), 37 had good scores (>181.4/230, 70%) and 5 had superior scores (197.6/230, 80%).

Matched MeSH terms: Status Epilepticus

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