1,698 results match your criteria Focal Status Epilepticus


Cortical inhibitory dysfunction in epilepsia partialis continua: A high frequency oscillation somatosensory evoked potential study.

Clin Neurophysiol 2019 Jan 25;130(4):439-444. Epub 2019 Jan 25.

Neurology, Neurophysiology and Neurobiology Unit, Department of Medicine, Università Campus Bio-Medico di Roma, Rome, Italy. Electronic address:

Objective: The pathophysiology of epilepsia partialis continua (EPC) is still unclear, a thalamo-cortical circuit dysfunction has been hypothesized. The aim of present study is the functional evaluation of the thalamo-cortical network in EPC by means of the study of low- and high-frequency somatosensory evoked potentials (LF-SEP and HF-SEP).

Methods: Median LF-SEP and HF-SEP were recorded in 3 patients with EPC and in 2 patients with rolandic lesions without EPC (non-EPC). Read More

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http://dx.doi.org/10.1016/j.clinph.2019.01.005DOI Listing
January 2019

Rasmussen syndrome: absence seizures may be induced by oxcarbazepine.

Epileptic Disord 2019 Feb 15. Epub 2019 Feb 15.

Department of Neurology, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires.

A female patient with electroclinical and neuroradiological features compatible with Rasmussen syndrome developed a particular clinical and EEG pattern. As the seizures were refractory to valproate at 750 mg/kg/day, oxcarbazepine (OXC) at 30 mg/kg/day was added. Seizures became more frequent and on neurological examination, no hemiparesis was detected. Read More

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http://dx.doi.org/10.1684/epd.2019.1035DOI Listing
February 2019

Focal visual status epilepticus.

Epileptic Disord 2019 Feb 15. Epub 2019 Feb 15.

Department for Clinical Neurophysiology, University Medical Center Göttingen, Göttingen, Germany.

Epileptic visual auras are elementary to complex and sometimes occur as colourful visual phenomena located close to or within the central part of the contralateral hemi-field. They typically last from seconds to a few minutes, which discriminates them from the usually longer-lasting visual auras (5-30 minutes) of patients suffering from migraine. We present an adult patient with occipital lobe epilepsy whose visual aura under epilepsy monitoring lasted for more than 30 minutes with almost no propagation, demonstrating a rare, but remarkable, sustained local epileptic network activity associated with resection of an occipital arterio-venous malformation. Read More

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http://dx.doi.org/10.1684/epd.2019.1034DOI Listing
February 2019

A Rasmussen encephalitis, autoimmune encephalitis, and mitochondrial disease mimicker: expanding the DNM1L-associated intractable epilepsy and encephalopathy phenotype.

Epileptic Disord 2019 Feb 15. Epub 2019 Feb 15.

Department of Pediatric Epilepsy, Beaumont Health, Royal Oak, MI 48073.

Dynamin-1-like protein (DNM1L) gene variants have been linked to childhood refractory epilepsy, developmental delay, encephalopathy, microcephaly, and progressive diffuse cerebral atrophy. However, only a few cases have been reported in the literature and there is still a limited amount of information about the symptomatology and pathophysiology associated with pathogenic variants of DNM1L. We report a 10-year-old girl with a one-year history of mild learning disorder and absence seizures who presented with new-onset focal status epilepticus which progressed to severe encephalopathy and asymmetric hemispheric cerebral atrophy. Read More

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http://dx.doi.org/10.1684/epd.2019.1036DOI Listing
February 2019

Electrical cortical stimulation for refractory focal epilepsy: A long-term follow-up study.

Epilepsy Res 2019 Jan 9;151:24-30. Epub 2019 Jan 9.

Section of Epilepsy, Department of Neurology, Chang Gung Memorial Hospital Linkou Medical Center and Chang Gung University College of Medicine, Taoyuan, Taiwan; Department of Neurology, Xiamen Changgung Hospital, China. Electronic address:

Objective: To report the long-term seizure control and safety of open-loop electrical cortical stimulation in patients with refractory focal epilepsy of diverse etiologies.

Methods: Six patients who received a therapeutic trial of cortical stimulation were included retrospectively. The frequency of seizures was recorded before and after implantation. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S09201211183049
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http://dx.doi.org/10.1016/j.eplepsyres.2019.01.003DOI Listing
January 2019
8 Reads
2.015 Impact Factor

Use of Newer Anticonvulsants for the Treatment of Status Epilepticus.

Pharmacotherapy 2019 Feb 5. Epub 2019 Feb 5.

University of Illinois at Chicago, Department of Pharmacy Practice, Chicago, IL.

Status Epilepticus (SE) has a high mortality rate and is one of the most common neurological emergencies. Fast progression of this neurological emergency and lack of response to traditional antiepileptic drugs (AEDs) in most cases has challenged clinicians to use new agents. The objective of this paper was to evaluate the efficacy and safety of AEDs released to the market after 2000 for SE, refractory SE (RSE), and super refractory SE (SRSE). Read More

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http://dx.doi.org/10.1002/phar.2229DOI Listing
February 2019

Adult-onset Rasmussen's Syndrome with associated cortical dysplasia.

Epilepsy Behav Case Rep 2019 28;11:54-57. Epub 2018 Nov 28.

Department of Neurosurgery, UT Health San Antonio, San Antonio, TX, United States of America.

We describe a 23-year-old woman with previous right temporal lobe surgeries for underlying cortical dysplasia, presenting with right hemispheric seizures and epilepsia partialis continua (EPC). After anti-seizure medication adjustments, she developed focal status epilepticus with progressive EEG and neuroimaging changes. Cerebrospinal fluid and serum autoimmune panels were negative except for an elevated serum acetylcholine-receptor antibody titer, but she underwent immunosuppressive therapy. Read More

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http://dx.doi.org/10.1016/j.ebcr.2018.11.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6349012PMC
November 2018
4 Reads

Nonconvulsive status epilepticus secondary to acute porphyria crisis.

Epilepsy Behav Case Rep 2019 28;11:43-46. Epub 2018 Nov 28.

Department of Neurology, Mayo Clinic, 5777 East Mayo Boulevard, Phoenix, AZ 85054, USA.

Both variegate and acute intermittent porphyria can manifest with various neurological symptoms. Although acute symptomatic seizures have been previously described, they are typically tonic-clonic and focal impaired awareness seizures. Convulsive status epilepticus and epilepsia partialis continua are rare and have been described on a case report basis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22133232183012
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http://dx.doi.org/10.1016/j.ebcr.2018.11.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6327909PMC
November 2018
9 Reads

Sudden unexpected death in GEFS+ families with sodium channel pathogenic variants.

Epilepsy Res 2019 Feb 14;150:66-69. Epub 2019 Jan 14.

Research Institute of the McGill University Health Center, 2155 Guy Street, Montreal, PQ, H3H 2L9, Canada; Department of Pediatrics, Division of Child Neurology, Montreal Children's Hospital, McGill University Health Centre, 1001 Boulevard Décarie, Montreal, PQ, H4A 3J1, Canada.

We aimed to describe families with genetic epilepsy with febrile seizures plus (GEFS+) in which individuals suffered sudden unexpected death. The Epilepsy Pharmacogenomics Research Database was reviewed for GEFS + families in which at least one individual had suffered sudden death, and two families were identified. In Family A, five males had febrile seizures and one girl had febrile seizures plus. Read More

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http://dx.doi.org/10.1016/j.eplepsyres.2019.01.009DOI Listing
February 2019
3 Reads

EEG in WNV Neuroinvasive Disease.

J Clin Neurophysiol 2019 Jan 21. Epub 2019 Jan 21.

Department of Neurology, Mayo Clinic, Scottsdale, AZ, U.S.A.

Purpose: Neuroinvasive West Nile virus (WNV) is rare, occurring in less than 1% of those infected, and may manifest as meningitis, encephalitis, and/or acute flaccid paralysis. Patients may present initially with nonspecific symptoms including fevers. Although rare, neuroinvasive WNV is associated with significant morbidity and mortality. Read More

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http://Insights.ovid.com/crossref?an=00004691-900000000-9948
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http://dx.doi.org/10.1097/WNP.0000000000000558DOI Listing
January 2019
6 Reads

Follow-up of patients with epilepsy harboring antiglycine receptor antibodies.

Epilepsy Behav 2019 Jan 11;92:103-107. Epub 2019 Jan 11.

Department of Neuroscience, Istanbul University, Institute of Experimental Medicine, Istanbul, Turkey.

Objective: The long-term follow-up of patients with epilepsy harboring autoantibodies against the glycine receptor (also glycine receptor antibodies or GlyR-Ab) is not well-known. Our aim was to investigate the 5-year prognosis and treatment response of patients with epilepsy who were seropositive for GlyR-Ab.

Methods: Clinical features; electroencephalogram (EEG), neuroradiological, and neuropathological findings; and treatment responses of patients with epilepsy with GlyR-Ab seropositivity were investigated. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15255050183060
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http://dx.doi.org/10.1016/j.yebeh.2018.09.034DOI Listing
January 2019
9 Reads

Focal Status Epilepticus-Related Unilateral Brain Edema: Magnetic Resonance Imaging Study of Children in Southwest China.

Pediatr Neurol 2018 Nov 23. Epub 2018 Nov 23.

Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, PR China; China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing, PR China; Department of Neurology, Children's Hospital of Chongqing Medical University, Chongqing, PR China; Chongqing Key Laboratory of Translational Medical Research in Cognitive Development and Learning and Memory Disorders, Chongqing, PR China. Electronic address:

Background: Unilateral brain edema is a rare peri-ictal imaging abnormality related to focal status epilepticus. We present the largest series of these patients, describe their clinical features and magnetic resonance imaging (MRI) findings, and analyze the possible underlying pathophysiology.

Methods: We reviewed the medical records in Southwest China's largest tertiary's children's medical center from 2011 to 2017. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183049
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http://dx.doi.org/10.1016/j.pediatrneurol.2018.08.028DOI Listing
November 2018
5 Reads

Epilepsy in children with type 1 diabetes mellitus: Pathophysiological basis and clinical hallmarks.

Eur J Paediatr Neurol 2018 Dec 21. Epub 2018 Dec 21.

Pediatric Diabetology Unit, Department of Maternal and Infantile Health, Sapienza University of Rome, Italy.

We provide an overview on the current knowledge about the association between epilepsy and type 1 diabetes mellitus (T1DM). People with T1DM have a 2-6-fold higher risk of epilepsy than the general population. The onset of T1DM anticipates the onset of epilepsy by a mean period between 1,5 and 2,8 years. Read More

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http://dx.doi.org/10.1016/j.ejpn.2018.12.006DOI Listing
December 2018
1 Read

Valproic acid and epilepsy: from molecular mechanisms to clinical evidences.

Curr Neuropharmacol 2018 Dec 27. Epub 2018 Dec 27.

Neurology Clinic, University of Perugia - S. Maria della Misericordia Hospital, Perugia. Italy.

After more than a century from its discovery, valproic acid (VPA) still represents one of the most efficient antiepileptic drugs (AEDs). Pre and post-synaptic effects of VPA depend on a very broad spectrum of actions, including the regulation of ionic currents and the facilitation of GABAergic over glutamatergic transmission. As a result, VPA indirectly modulates neurotransmitter release and strengthens the threshold for seizure activity. Read More

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http://dx.doi.org/10.2174/1570159X17666181227165722DOI Listing
December 2018
11 Reads

Successful Surgical Treatment of Refractory Status Epilepticus in a 12-Day-Old Infant.

Pediatr Neurol 2018 Nov 23. Epub 2018 Nov 23.

Department of Neurosurgery, Wayne State University, Detroit, Michigan; Department of Pediatric Neurosurgery, Children's Hospital of Michigan, Detroit, Michigan.

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http://dx.doi.org/10.1016/j.pediatrneurol.2018.11.011DOI Listing
November 2018
1.504 Impact Factor

[Etiology and clinical features of epilepsia partialis continua: an analysis of six cases].

Zhongguo Dang Dai Er Ke Za Zhi 2018 Dec;20(12):1008-1014

Department of Emergency, Xiangya Hospital, Central South University, Changsha 410008, China.

Objective: To investigate the etiology and clinical features of epilepsia partialis continua (EPC) in children.

Methods: A retrospective analysis was performed for the clinical features, diagnosis and treatment of six children with EPC, and the clinical and laboratory features and prognosis were compared between the children with different etiologies.

Results: There were five girls and one boy, with an onset age ranging from one year and seven months to nine years. Read More

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December 2018
6 Reads

Predictors and outcome of status epilepticus in cerebral venous thrombosis.

J Neurol 2019 Feb 19;266(2):417-425. Epub 2018 Dec 19.

Department of Neurology, Neurology Sanjay Gandhi Post Graduate Institute of Medical Sciences, Raebareli Road, Lucknow, 226014, India.

We report the clinico-radiological characteristics of SE in the patients with cerebral venous thrombosis (CVT), and compare the relative risk of SE in determining death and disability compared with those patients with and without self-limiting seizures. Consecutive patients with magnetic resonance venography (MRV) confirmed CVT, admitted during 2006-2017, were included. Their clinical details, risk factors, and magnetic resonance imaging and MRV findings were noted. Read More

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http://link.springer.com/10.1007/s00415-018-9145-8
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http://dx.doi.org/10.1007/s00415-018-9145-8DOI Listing
February 2019
1 Read

Magnetoencephalographic Source Localization of the Eye Area of the Motor Homunculus.

Can J Neurol Sci 2018 Dec 17:1-7. Epub 2018 Dec 17.

1Division of Neurology,Krembil Brain Institute,University Health Network, Toronto Western Hospital,University of Toronto,Toronto, Ontario,Canada.

A patient with intractable epilepsy, previous right frontal resection, and active vagus nerve stimulation (VNS) developed new onset quasi-continuous twitching around the left eye. Electroencephalography showed no correlate to the orbicularis oculi twitches apart from myographic potentials at the left supraorbital and anterior frontal electrodes. Magnetoencephalography was performed using spatiotemporal signal space separation to suppress magnetic artifacts associated with the VNS apparatus. Read More

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https://www.cambridge.org/core/product/identifier/S031716711
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http://dx.doi.org/10.1017/cjn.2018.373DOI Listing
December 2018
2 Reads

Autoimmune encephalitis following alemtuzumab treatment of multiple sclerosis.

Mult Scler Relat Disord 2018 Dec 3;28:31-33. Epub 2018 Dec 3.

Department of Neurology, St Vincent's Hospital, 390 Victoria St, Darlinghurst, Sydney, NSW 2010, Australia.

Secondary autoimmune disorders are a recognised complication of alemtuzumab treatment for multiple sclerosis. We report a case of autoimmune encephalitis manifesting as a polymorphic epilepsia partialis continua / status epilepticus seven months after the second course of alemtuzumab in a patient with previous autoimmune hypothyroidism and immune thrombocytopenic purpura. An MRI revealed multifocal cortical abnormalities and neuronal loss was evident on biopsy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S22110348183053
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http://dx.doi.org/10.1016/j.msard.2018.12.004DOI Listing
December 2018
5 Reads

Discovery and validation of blood microRNAs as molecular biomarkers of epilepsy: Ways to close current knowledge gaps.

Epilepsia Open 2018 Dec 30;3(4):427-436. Epub 2018 Oct 30.

Department of Physiology & Medical Physics Royal College of Surgeons in Ireland (RCSI) Dublin Ireland.

There is a major unmet need for biomarkers of epilepsy. Biofluids such as blood offer a potential source of molecular biomarkers. MicroRNAs (miRNAs) fulfill several key requirements for a blood-based molecular biomarker being enriched in the brain and dysregulated in epileptic brain tissue, and manipulation of miRNAs can have seizure-suppressive and disease-modifying effects in preclinical models. Read More

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http://dx.doi.org/10.1002/epi4.12275DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6276772PMC
December 2018

New-Onset Geriatric Epilepsy in China: A Single-Center Study.

Chin Med J (Engl) 2018 Dec;131(24):2915-2920

Department of Neurology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing 100730, China.

Background: Few studies have been published on new-onset geriatric epilepsy especially in older Chinese people. This study was to have a comprehensive understanding of new-onset geriatric epilepsy and find a more reasonable diagnosis and management of epilepsy in older people.

Methods: One hundred and three patients with onset age 60 years and older were admitted between January 2008 and December 2016. Read More

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http://www.cmj.org/text.asp?2018/131/24/2915/247194
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http://dx.doi.org/10.4103/0366-6999.247194DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302641PMC
December 2018
11 Reads

Novel Use of Responsive Neurostimulation (RNS System) in the Treatment of Super Refractory Status Epilepticus.

J Clin Neurophysiol 2018 Dec 6. Epub 2018 Dec 6.

Departments of Neurology and.

There are very few randomized controlled trials studying treatment of super refractory status epilepticus (SE), despite estimated occurrence in about 15% of SE cases and its association with high morbidity and mortality rates. Small case series and case reports have described use of neurostimulation, including vagal nerve stimulation, transcranial magnetic stimulation, and deep brain stimulation, to treat super refractory SE when medical interventions have failed. To our knowledge, this is the first reported case of responsive neurostimulation being used to successfully treat a case of super refractory SE. Read More

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http://dx.doi.org/10.1097/WNP.0000000000000541DOI Listing
December 2018

Hemiconvulsion-Hemiplegia-Epilepsy in a girl with cobalamin C deficiency.

Epileptic Disord 2018 Dec;20(6):545-550

Division of Neurology, Department of Pediatrics, Montreal Children's Hospital, McGill University,, Department of Neurology and Neurosurgery, McGill University,, Research Institute of the McGill University Health Centre.

Hemiconvulsion-Hemiplegia-Epilepsy initially involves an infantile presentation of febrile focal motor status epilepticus, with subsequent hemiplegia of the initially affected side. Months to years later, affected children go on to develop a chronic epilepsy with recurrent focal seizures which are often refractory to treatment. This uncommon paediatric epilepsy syndrome is poorly understood, with only a very small minority of cases associated with an underlying genetic or metabolic abnormality. Read More

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http://www.john-libbey-eurotext.fr/medline.md?doi=10.1684/ep
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http://dx.doi.org/10.1684/epd.2018.1017DOI Listing
December 2018
7 Reads

KCNQ2 mutation in an infant with encephalopathy of infancy with migrating focal seizures.

Epileptic Disord 2018 Dec;20(6):541-544

Department of Pediatrics (Neurology), McMaster University, 1280 Main St., W. Hamilton, Ontario L8S 4K1, Canada.

A male neonate presented with seizures at 18 hours of life, characterized by tonic posturing with eye deviation to the right, apnoea, bradycardia, and oxygen desaturation. Initial structural, metabolic, and infectious work-up was unremarkable. He continued to have seizures refractory to a variety of antiepileptic medications. Read More

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http://dx.doi.org/10.1684/epd.2018.1011DOI Listing
December 2018
1 Read

Rasmussen syndrome: an atypical presentation in ten patients.

Epileptic Disord 2018 Dec;20(6):468-478

Department of Neurosurgery, Hospital de Pediatría "Prof Dr Juan P Garrahan", Buenos Aires, Argentina.

The aim of this study was to analyse the electroclinical and imaging findings and outcome of patients with Rasmussen syndrome (RS) with atypical manifestations. We conducted a retrospective, descriptive study of 10 of 44 consecutive patients with RS with atypical features, followed between 1999 and 2017. Six patients were boys and four were girls. Read More

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http://www.john-libbey-eurotext.fr/medline.md?doi=10.1684/ep
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http://dx.doi.org/10.1684/epd.2018.1007DOI Listing
December 2018
8 Reads

Creutzfeldt-Jakob Disease Presenting as Nonconvulsive Status Epilepticus.

Case Rep Neurol Med 2018 4;2018:3092018. Epub 2018 Nov 4.

Department of Neurology and Neurosurgery, University of Tartu, Estonia.

Creutzfeldt-Jakob disease is a rare, rapidly progressive spongiform encephalopathy in humans. EEG plays an important role in diagnosing this disease. In some patients, epileptic activity and encephalopathy from various aetiologies may share morphological features on EEG. Read More

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https://www.hindawi.com/journals/crinm/2018/3092018/
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http://dx.doi.org/10.1155/2018/3092018DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6241237PMC
November 2018
11 Reads

Progressive myoclonus epilepsy and ceroidolipofuscinosis 14: The multifaceted phenotypic spectrum of KCTD7-related disorders.

Eur J Med Genet 2018 Nov 27. Epub 2018 Nov 27.

Division of Child Neurology and Psychiatry, Department of Human Neurosciences, Sapienza University of Rome, Rome, Italy. Electronic address:

Background: Mutations in the KCTD7 gene have been associated with progressive myoclonus epilepsy and, in a single patient, with the so-called "Neuronal Ceroid Lipofuscinosis 14" (characterised by myoclonic seizures, cognitive regression, optic atrophy leading to visual loss, and progressive cortical and cerebellar atrophy).

Clinical Reports: We describe two new patients carrying two novel pathogenic mutations in the KCTD7 gene. Patient 1 (NM_153033. Read More

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http://dx.doi.org/10.1016/j.ejmg.2018.11.025DOI Listing
November 2018
8 Reads

New onset refractory status epilepticus (NORSE).

Seizure 2018 Sep 29. Epub 2018 Sep 29.

Département de Neurologie, Université Libre de Bruxelles, Hôpital Erasme, Brussels, Belgium; Comprehensive Epilepsy Center, Neurology Department, Yale University School of Medicine, New Haven, CT, USA.

Purpose: To summarize the clinical features, suggested work-up, treatment and prognosis of new-onset refractory status epilepticus (NORSE), a condition recently defined as the occurrence of refractory status epilepticus (RSE) in a patient without active epilepsy, and without a clear acute or active structural, toxic or metabolic cause; and of the related syndrome of febrile infection-related epilepsy syndrome (FIRES), also recently defined as a subgroup of NORSE preceded by a febrile illness between 2 weeks and 24 h prior to the onset of RSE.

Method: Narrative review of the medical literature about NORSE and FIRES.

Results: NORSE and FIRES mainly affect school-age children and young adults. Read More

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http://dx.doi.org/10.1016/j.seizure.2018.09.018DOI Listing
September 2018
12 Reads

Epidemiology of status epilepticus in adults: A population-based study on incidence, causes, and outcomes.

Epilepsia 2019 Jan 26;60(1):53-62. Epub 2018 Nov 26.

Department of Public Health, Health Services Research and Health Technology Assessment, UMIT - University for Health Sciences, Medical Informatics and Technology, Hall in Tirol, Austria.

Objective: In 2015, the International League Against Epilepsy (ILAE) proposed a new definition of status epilepticus (SE): 5 minutes of ongoing seizure activity to diagnose convulsive SE (CSE, ie, bilateral tonic-clonic SE) and 10 minutes for focal SE and absence SE, rather than the earlier criterion of 30 minutes. Based on semiology, several types of SE with prominent motor phenomena at any time (including CSE) were distinguished from those without (ie, nonconvulsive SE, NCSE). We present the first population-based incidence study applying the new 2015 ILAE definition and classification of SE and report the impact of the evolution of semiology and level of consciousness (LOC) on outcome. Read More

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http://dx.doi.org/10.1111/epi.14607DOI Listing
January 2019
2 Reads

Semiology, clustering, periodicity and natural history of seizures in an experimental occipital cortical epilepsy model.

Dis Model Mech 2018 12 14;11(12). Epub 2018 Dec 14.

Department of Clinical and Experimental Epilepsy, UCL Institute of Neurology, Queen Square, London WC1N 3BG, UK

Focal neocortical epilepsy is a common form of epilepsy and there is a need to develop animal models that allow the evaluation of novel therapeutic strategies to treat this type of epilepsy. Tetanus toxin (TeNT) injection into the rat visual cortex induces focal neocortical epilepsy without preceding status epilepticus. The latency to first seizure ranged from 3 to 7 days. Read More

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http://dx.doi.org/10.1242/dmm.036194DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307909PMC
December 2018
13 Reads

Defining the electroclinical phenotype and outcome of PCDH19-related epilepsy: A multicenter study.

Epilepsia 2018 Dec 19;59(12):2260-2271. Epub 2018 Nov 19.

Neurology Unit, Department of Neuroscience, Bambino Gesù Children's Hospital, IRCCS, Rome, Italy.

Objective: PCDH19-related epilepsy is an epileptic syndrome with infantile onset, characterized by clustered and fever-induced seizures, often associated with intellectual disability (ID) and autistic features. The aim of this study was to analyze a large cohort of patients with PCDH19-related epilepsy and better define the epileptic phenotype, genotype-phenotype correlations, and related outcome-predicting factors.

Methods: We retrospectively collected genetic, clinical, and electroencephalogram (EEG) data of 61 patients with PCDH19-related epilepsy followed at 15 epilepsy centers. Read More

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http://doi.wiley.com/10.1111/epi.14600
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http://dx.doi.org/10.1111/epi.14600DOI Listing
December 2018
22 Reads
4.571 Impact Factor

Electroencephalography in Pediatric Epilepsy.

Indian Pediatr 2018 Oct;55(10):893-901

Department of Pediatrics, BL Kapur Superspeciality Hospital, Delhi; India.

Surface electroencephalography (EEG) is a useful electrophysiological investigation for evaluating a paroxysmal event in children. It measures the electro potential difference between two points on the scalp. It is a non-invasive tool that analyzes neuronal maturation and abnormal cortical excitability. Read More

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October 2018
3 Reads

Recurrent focal seizures as a feature of status epilepticus presenting as a peri-ictal water drinking.

Epilepsy Behav Case Rep 2018 9;10:129-132. Epub 2018 Oct 9.

University Hospitals - Cleveland Medical Center, Department of Neurology, Case Western Reserve University School of Medicine, Cleveland, OH, USA.

We report a case of focal status epilepticus (SE) associated with peri-ictal water drinking (PIWD) behavior in a nine-year-old left-handed boy with epilepsy. We reviewed prior cases of epileptic peri-ictal water drinking. Only one adult patient with status epilepticus and PIWD has been reported previously. Read More

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http://dx.doi.org/10.1016/j.ebcr.2018.09.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6216106PMC
October 2018
1 Read

Post-operative nonketotic hyperglycemic induced focal motor status epilepticus related to treatment with corticosteroids following standard anterior temporal lobectomy.

Epilepsy Behav Case Rep 2018 2;10:124-128. Epub 2018 Oct 2.

Department of Neurosurgery, Henry Ford Health System, Detroit, MI, USA.

•Complications from standard ATL are uncommon and the use of post-operative corticosteroids may reduce complications.•Following standard ATL, FMSE was present after treatment with corticosteroids that resolved after blood sugar control.•After epilepsy surgery, corticosteroids should be used cautiously in people with comorbid diabetes mellitus. Read More

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http://dx.doi.org/10.1016/j.ebcr.2018.09.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6215994PMC
October 2018
1 Read

Neurologic phenotypes associated with / mutations: Expanding the spectrum of disease.

Neurology 2018 Nov 9;91(22):e2078-e2088. Epub 2018 Nov 9.

From the Department of Clinical and Experimental Epilepsy (S.Z., Z.M., L.H.-H., S.K., S. Balestrini, S.M.S.) and Division of Neuropathology (Z.M., M.T.), UCL Institute of Neurology, London, UK; Clinic of Neurology (S.Z.), Department of Experimental and Clinical Medicine, Marche Polytechnic University, Ancona, Italy; Department of Pediatric Neurology and Neurological Rehabilitation (C.S., T.H., P.W., G.J.K.) and Neurosurgery Clinic and Clinic for Epilepsy Surgery (M.K.), Schön Klinik Vogtareuth; Department of Pediatrics (C.S., M.S.), Children's Hospital Augsburg, Germany; UCL Great Ormond Street Institute of Child Health (J.R.N., K.V., S.M.V., J.H.C.), London, UK; Paediatric Neurology and Neurogenetics Unit and Laboratories (D.M., R.G.), A. Meyer Children's Hospital, University of Florence, Italy; Chalfont Centre for Epilepsy (Z.M., L.H.-H., S.K., S. Balestrini, S.M.S.), Chalfont-St-Peter, Buckinghamshire, UK; CeGaT-Center for Genomics and Transcriptomics (A.P., S. Biskup), Tübingen, Germany; Neurogenetics Unit (M.L.), Department of Medical Genetics, Hospital de São João, Porto, Portugal; Department of Pediatrics and Adolescent Medicine (J.G.), University Medical Center Göttingen; Hospital for Children and Adolescents (A.M.), University Clinic Leipzig, Germany; Freiburg Medical Laboratory (M.J.), Dubai; The Danish Epilepsy Centre (R.S.M., E.G.), Dianalund; Institute for Regional Health Services (R.S.M., E.G.), University of Southern Denmark, Odense; Department of Clinical Genetics (B.S.K.), Odense University Hospital; Hans Christian Andersen Children's Hospital (L.K.H.), Odense, Denmark; Pediatric Neurology and Muscular Diseases Unit (M.S.V., P.S.), Department of Neurosciences, Rehabilitation, Ophthalmology, Genetics, and Maternal and Child Health, University of Genoa "G. Gaslini" Institute, Italy; Division of Neurology (K.L.H.), Children's Hospital of Philadelphia, PA; Department of Neurology (S.D., C.L.S.-H.), Division of Neurogenetics, Kennedy Krieger Institute, Baltimore, MD; Center for Genomic Medicine (N.H.-F.), Tohoku University; Department of Pediatrics (N.H.-F.), Tohoku University School of Medicine, Sendai, Japan; Department of Pediatrics (T.T., R.L.) and Institute of Clinical Medicine (K.O.), University of Tartu; Children's Clinic (T.T., R.L.), Department of Radiology (P.I.), and Department of Clinical Genetics, United Laboratories (K.O.), Tartu University Hospital, Estonia; Ludwig-Maximilians-University Munich (I.K.); Department of Pediatric Neurology (A.H.), Clinic Traunstein; Children's Hospital (M.K.), Dr. Horst Schmidt Klinik, Wiesbaden; Altona Children's Hospital (J.H.), Hamburg; Department of Pediatrics (C. Makowski), Technische Universität München, Germany; Department of Clinical Genetics (S.G.), Royal North Shore Hospital, St Leonards; John Hunter Children's Hospital (G.M.S.), New Lambton Heights, New South Wales, Australia; Department of Neurology (R.T.), University Hospital of Wales; Institute of Psychological Medicine and Clinical Neurosciences (R.H.T.), Cardiff University; Division of Neuroradiology (C. Micallef), National Hospital for Neurology and Neurosurgery, London; Department of Brain Repair & Rehabilitation (D.J.W.), Stroke Research Centre, UCL Institute of Neurology, London, UK; Paracelsus Medical University (G.J.K.), Salzburg, Austria; and IRCCS Stella Maris Foundation (R.G.), Pisa, Italy.

Objective: To characterize the neurologic phenotypes associated with mutations and to seek genotype-phenotype correlation.

Methods: We analyzed clinical, EEG, and neuroimaging data of 44 new and 55 previously reported patients with mutations.

Results: Childhood-onset focal seizures, frequently complicated by status epilepticus and resistance to antiepileptic drugs, was the most common phenotype. Read More

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http://www.neurology.org/lookup/doi/10.1212/WNL.000000000000
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http://dx.doi.org/10.1212/WNL.0000000000006567DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6282239PMC
November 2018
13 Reads

Seizures in tuberculous meningitis.

Epilepsy Res 2018 12 25;148:90-95. Epub 2018 Oct 25.

Sanjay Gandhi Post Graduate Institute of Medical Sciences, Lucknow, India.

Objective: We report the frequency of seizure, its possible mechanisms and effect of seizure on the outcome of tuberculous meningitis (TBM).

Methods: Seventy-nine patients with TBM admitted during 2014-2017 were evaluated. The seizures were categorized as per International League Against Epilepsy as well as into early (within 1 month of meningitis) and late (>1 month) seizure. Read More

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http://dx.doi.org/10.1016/j.eplepsyres.2018.10.005DOI Listing
December 2018
5 Reads

Development of permanent brain damage after subacute encephalopathy with seizures in alcoholics.

J Neurol Sci 2019 Jan 25;396:12-17. Epub 2018 Oct 25.

Unit of Neurology and Clinical Neurophysiology, University hospital Bretonneau, Tours, France.

Purpose: To describe additional cases of subacute encephalopathy with seizures in alcoholics (SESA) syndrome, and to question the clinical and radiological course.

Methods: We retrospectively analyzed the clinical characteristics, electroencephalography (EEG), MRI studies at the admission and over the following 6 months of 5 cases of SESA syndrome visited our neurology department between 2010 and 2016.

Results: Five middle-aged males with history of chronic alcohol abuse were admitted for confusion, neurological deficit and seizures. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S0022510X183043
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http://dx.doi.org/10.1016/j.jns.2018.10.022DOI Listing
January 2019
35 Reads

No latency to dentate granule cell epileptogenesis in experimental temporal lobe epilepsy with hippocampal sclerosis.

Epilepsia 2018 Nov 19;59(11):2019-2034. Epub 2018 Oct 19.

Neuroscience Institute, Morehouse School of Medicine, Atlanta, Georgia.

Objective: To determine when spontaneous granule cell epileptiform discharges first occur after hippocampal injury, and to identify the postinjury "latent" period as either a "silent" gestational state of epileptogenesis or a subtle epileptic state in gradual transition to a more obvious epileptic state.

Methods: Nonconvulsive status epilepticus evoked by perforant path stimulation in urethane-sedated rats produced selective and extensive hippocampal injury and a "latent" period that preceded the onset of the first clinically obvious epileptic seizures. Continuous granule cell layer depth recording and video monitoring assessed the time course of granule cell hyperexcitability and the onset/offset times of spontaneous epileptiform discharges and behavioral seizures. Read More

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http://doi.wiley.com/10.1111/epi.14580
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http://dx.doi.org/10.1111/epi.14580DOI Listing
November 2018
3 Reads

Clinical and electrographic characteristics of seizures in LGI1-antibody encephalitis.

Epilepsy Behav 2018 Nov 15;88:277-282. Epub 2018 Oct 15.

Department of Neurology, First Affiliated Hospital of Zhengzhou University, China. Electronic address:

Purpose: The purpose of this study was to analyze the clinical and electrographic characteristics of seizures in LGI1-antibody encephalitis.

Methods: The methods utilized in this study were prospective analysis of the clinical manifestations, types of seizures, electroencephalogram (EEG), adjuvant examination, treatment and prognosis of 19 cases of LGI1-antibody encephalitis diagnosed from January 2017 to February 2018 in First Affiliated Hospital of Zhengzhou University, and reviewed related literatures.

Results: The 15/19 (79%) patients were male, and the average onset age was 58 years (23-82). Read More

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https://linkinghub.elsevier.com/retrieve/pii/S15255050183051
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http://dx.doi.org/10.1016/j.yebeh.2018.08.019DOI Listing
November 2018
15 Reads

Perfusion-CT imaging in epileptic seizures.

J Neurol 2018 Dec 16;265(12):2972-2979. Epub 2018 Oct 16.

Service of Neurology, Department of Clinical Neurosciences, Centre Hospitalier Universitaire Vaudois (CHUV), Lausanne, Switzerland.

Introduction: PCT is used in the diagnosis of acute neurological syndromes, particularly stroke. We aimed to evaluate PCT abnormalities in patients with acute epileptic seizures or status epilepticus (SE).

Methods: We collected patients undergoing acute PCT for the suspicion of acute ischemic stroke (AIS), who received a final diagnosis of focal seizures or generalised seizures with a post-ictal deficit, with or without concomitant AIS. Read More

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http://dx.doi.org/10.1007/s00415-018-9095-1DOI Listing
December 2018
10 Reads

Lethal NARS2-Related Disorder Associated With Rapidly Progressive Intractable Epilepsy and Global Brain Atrophy.

Pediatr Neurol 2018 Dec 4;89:26-30. Epub 2018 Aug 4.

Helen DeVos Children's Hospital, Grand Rapids, Michigan; College of Human Medicine Department of Pediatrics and Human Development, Michigan State University, Grand Rapids, Michigan.

Background: Infantile epileptic encephalopathy is a heterogeneous condition that has been associated with variants in more than 200 genes. The variability in findings and prognosis creates challenges to making the correct diagnosis and initiating the appropriate therapy. Biallelic variants in NARS2, a mitochondrial aminoacyl-tRNA synthetase gene, were recently associated with neurodegenerative disorders that include epilepsy. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S08878994183049
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http://dx.doi.org/10.1016/j.pediatrneurol.2018.07.014DOI Listing
December 2018
6 Reads
1.504 Impact Factor

Unusual cause of fever, vision loss and super refractory status epilepticus in association with simian virus 40 (SV40).

BMJ Case Rep 2018 Oct 12;2018. Epub 2018 Oct 12.

Pathology, all india institute of medical sciences, New Delhi, Delhi, India.

We present a case of a 23-year-old man with history of fever followed by painless complete vision loss, with subsequent new-onset refractory status epilepticus (NORSE). He initially developed bilateral retinitis. A few days later, he started having focal seizures, and subsequently developed super-refractory status epilepticus, requiring anaesthetic agents. Read More

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http://dx.doi.org/10.1136/bcr-2018-225539DOI Listing
October 2018
1 Read

Unusual Magnetic Resonance Imaging Abnormality in Nonketotic Hyperglycemia - related Epilepsia Partialis Continua.

Ann Indian Acad Neurol 2018 Jul-Sep;21(3):225-227

Department of Medical Imaging, Taipei Medical University Hospital, Taipei, Taiwan.

Epilepsia partialis continua (EPC) is a rare epileptic syndrome, presenting as continuous focal motor seizures for a period of minutes, hours, or days. EPC may develop in patients with cerebral cortical lesions and occasionally may develop in patients with metabolic disorders, such as nonketotic hyperglycemia (NKH). Here, we report a case of EPC following NKH, showing an unusual magnetic resonance imaging (MRI) finding of concurrent hypointensity on susceptibility-weighted image (SWI) and T2-weighted image (T2WI) with leptomeningeal and cortical enhancement, which have never been reported. Read More

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http://dx.doi.org/10.4103/aian.AIAN_386_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6137637PMC
September 2018

Epilepsia Partialis Continua of the Abdominal Musculature Caused by Acute Ischemic Stroke.

Can J Neurol Sci 2018 Nov 24;45(6):703-706. Epub 2018 Sep 24.

2Department of Neurology,Franz Tappeiner Hospital,Merano,Italy.

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http://dx.doi.org/10.1017/cjn.2018.330DOI Listing
November 2018
2 Reads

[Rasmussen syndrome: a clinicopathologic study of four cases].

Zhonghua Bing Li Xue Za Zhi 2018 Sep;47(9):676-681

Department of Pathology, Beijing Haidian Hospital/Haidian Section of Peking University Third Hospital, Beijing 100080, China.

To investigate the clinicopathologic features of Rasmussen syndrome (RS) and to raise awareness of this rare disease. Clinicopathologic data of 4 cases of RS were retrospectively analyzed at Beijing Haidian Hospital from 2008 to 2016. The clinical manifestations included epilepsia partialis continua and progressive neurologic deficits in all patients. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0529-5807.2018.09.005DOI Listing
September 2018
10 Reads

Electrographic Seizures in Patients with Acute Encephalitis.

Neurocrit Care 2019 Feb;30(1):207-215

Department of Neurology, Henry Ford Hospital, 2799 W Grand Blvd, Detroit, MI, 48202, USA.

Introduction: Clinical seizures and status epilepticus are frequent complications of encephalitis, can lead to depressed level of consciousness, and are associated with poor outcome. We sought to determine the frequency, risk factors, and clinical impact of electrographic seizures detected with continuing electroencephalography (cEEG) in patients with encephalitis and altered level of consciousness.

Methods: We retrospectively identified all patients with presumed or definite viral or autoimmune encephalitis who underwent cEEG monitoring at Henry Ford Hospital from January 2012 to October 2017. Read More

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http://link.springer.com/10.1007/s12028-018-0599-4
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http://dx.doi.org/10.1007/s12028-018-0599-4DOI Listing
February 2019
12 Reads

[Status epilepticus].

Medicina (B Aires) 2018;78 Suppl 2:12-17

Hospital de Pediatría Prof. Dr. Juan P. Garrahan, Buenos Aires, Argentina. E-mail:

Status epilepticus (SE) is one of the most common neurologic emergencies in pediatrics. It is a condition resulting either from the failure of the mechanisms responsible for seizure termination or from the initiation of mechanisms, which leads to abnormally, prolonged seizures. This definition provides a good guidance, when emergency treatment must be considered. Read More

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January 2018
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Fulminant Anti-GAD antibody encephalitis presenting with status epilepticus requiring aggressive immunosuppression.

J Neuroimmunol 2018 Oct 24;323:119-124. Epub 2018 Jul 24.

Department of Neurology, Sir Charles Gairdner Hospital, Nedlands, Australia. Electronic address:

Antibodies against glutamic acid decarboxylase (GAD) are reported in association with numerous neurological conditions including temporal lobe epilepsy and limbic encephalitis. We report a case of Anti-GAD-Antibody associated encephalitis presenting with epilepsia partialis continua (EPC) progressing to a fulminant encephalopathy preferentially affecting the frontal lobes associated with coma and refractory status epilepticus. The abnormalities identified on MRI included marked bilateral frontal lobe involvement which has not been reported in other auto-immune encephalitides and may be specific for Anti-GAD-Antibody associated encephalitis. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S01655728183022
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http://dx.doi.org/10.1016/j.jneuroim.2018.06.013DOI Listing
October 2018
3 Reads