1,860 results match your criteria Focal Status Epilepticus


Focal EEG changes indicating critical illness associated cerebral microbleeds in a Covid-19 patient.

Clin Neurophysiol Pract 2020 10;5:125-129. Epub 2020 Jun 10.

EEG and Epilepsy Unit, Division of Neurology, Geneva University Hospitals, Switzerland.

Objectives: We describe a patient suffering from Covid19-related acute respiratory distress syndrome (ARDS), highlighting the diagnostic role of the EEG in ICU.

History: A Covid-19 patient undergoing mechanical ventilation due to related acute respiratory distress syndrome (ARDS), presented altered mental status in the ICU. Video-EEG revealed a focal monomorphic theta slowing in bilateral frontal-central regions. Read More

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http://dx.doi.org/10.1016/j.cnp.2020.05.004DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7286251PMC

[Interictal psychosis of epilepsy].

Encephale 2020 Jun 25. Epub 2020 Jun 25.

Service de neurologie, CHRU Nancy, 54000 Nancy, France; Pôle universitaire de psychiatrie du grand Nancy, CPN, 54520 Laxou, France.

Interictal psychosis (IIP) refers to psychosis that occurs in clear consciousness in persons with epilepsy (PWE) with temporal onset not during or immediately following a seizure. The pooled prevalence estimate of psychosis in PWE is 5.6%. Read More

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http://dx.doi.org/10.1016/j.encep.2020.04.014DOI Listing

Characterization and Outcomes of Epileptic Seizures in Mexican Pediatric Patients With Anti-N-Methyl-D-Aspartate Receptor Encephalitis.

Cureus 2020 May 20;12(5):e8211. Epub 2020 May 20.

Pediatric Neurology, Instituto Nacional de Pediatría, Mexico CIty, MEX.

Introduction Anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis is one of the most common autoimmune encephalitides. The frequency of anti-NMDAR encephalitis is known to exceed the frequency of any individual viral encephalitis in young subjects. Epileptic seizures are a cardinal symptom in anti-NMDAR encephalitis; a significant amount of pediatric patients exhibit seizures as the first symptom of the disease, and most of them will develop them during the acute phase. Read More

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http://dx.doi.org/10.7759/cureus.8211DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7305580PMC

Seizures in the Emergency Department: Clinical and diagnostic data from a series of 153 patients.

Neurologia 2020 Jun 18. Epub 2020 Jun 18.

Servicio de Neurología, Hospital Clínico San Carlos, Madrid, España; Instituto de Investigación Sanitaria del Hospital Clínico San Carlos (IdISSC), Madrid, España; Unidad de Epilepsia, Servicio de Neurología, Hospital Clínico San Carlos, Madrid, España.

Introduction: Seizures are a frequent reason for admission to emergency departments and require early, precise diagnosis and treatment. The objective of this study was to describe the clinical and prognostic characteristics of a series of patients with seizures attended at our hospital's emergency department.

Methods: We performed a retrospective, observational study of all patients with seizures who were admitted to our hospital's emergency department and attended by the on-call neurology service between February and August 2017. Read More

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http://dx.doi.org/10.1016/j.nrl.2020.02.007DOI Listing

A multimodal diagnostic approach for lateralised rhythmic delta activity in the ictal-interictal continuum.

Epileptic Disord 2020 Jun;22(3):337-341

SOD Neurofisopatologia, Dipartimento Neuromuscoloscheletrico e Organi di Senso, AOU Careggi,, IRCCS Fondazione Don Carlo Gnocchi, Florence, Italy.

The ictal-interictal continuum represents a diagnostic challenge even for expert neurrophysiologists, often requiring an additional multimodal diagnostic workup to understand its clinical significance. Lateralised rhythmic delta activity (LRDA) is an ictal-interictal continuum pattern that has only recently been investigated and recognised as potentially ictogenic or sometimes even ictal. We describe a patient who presented with acute-onset aphasia, initially suspected of having a stroke; advanced brain imaging with CT-perfusion showed features suggesting regional left temporo-parietal hyperperfusion and an EEG revealed LRDA with fluctuations and intermixed sharp waves in the same areas. Read More

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http://dx.doi.org/10.1684/epd.2020.1169DOI Listing

Seizure Activity Across Scales From Neuronal Population Firing to Clonic Motor Semiology.

J Clin Neurophysiol 2020 May 26. Epub 2020 May 26.

Department of Neurology, Columbia University Medical Center, New York, New York, U.S.A.

The correlation of clinical semiology with neuronal firing in human seizures has not been well described. Similarly, the neuronal firing patterns underlying high-frequency oscillations during seizures remain controversial. Using implanted subdural electrodes and a microelectrode array in a patient with focal status epilepticus, in which 40 habitual focal motor seizures and 101 subclinical seizures were captured, the authors analyzed the association of EEG, high-frequency oscillations, and multiunit activity to facial motor semiology. Read More

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http://dx.doi.org/10.1097/WNP.0000000000000706DOI Listing

Dynamic oxygen changes during status epilepticus and subsequent endogenous kindling.

Epilepsia 2020 Jun 1. Epub 2020 Jun 1.

Hotchkiss Brain Institute, University of Calgary, Calgary, Alberta, Canada.

Objective: Brain tissue oxygen (partial oxygen pressure [pO ]) levels are tightly regulated to stay within the normoxic zone, with deviations on either side resulting in impaired brain function. Whereas pathological events such as ischemic attacks and brief seizures have previously been shown to result in pO levels well below the normoxic zone, oxygen levels during prolonged status epilepticus (SE) and the subsequent endogenous kindling period are unknown.

Methods: We utilized two models of acquired temporal lobe epilepsy in rats: intrahippocampal kainic acid infusion and prolonged perforant pathway stimulation. Read More

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http://dx.doi.org/10.1111/epi.16554DOI Listing

Top of Basilar Syndrome Presenting With Hyperekplexia Initially Diagnosed as a Convulsive Status Epilepticus.

J Emerg Med 2020 May 22. Epub 2020 May 22.

Department of Neurology University of Oklahoma Medical Center, Oklahoma City, Oklahoma.

Background: Hyperekplexia is a rare neurologic disorder characterized by pronounced startle responses to tactile or acoustic stimuli and increase tone. Acquired hyperekplexia is usually seen in brainstem pathologies and when it develops acutely it can be easily misdiagnosed as a convulsive seizure.

Case Report: A 38-year-old man presented with acute onset generalized brief involuntary jerky movements and a decreased level of consciousness. Read More

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http://dx.doi.org/10.1016/j.jemermed.2020.04.038DOI Listing
May 2020
1.175 Impact Factor

Prolonged prophylactic effects of gabapentin on status epilepticus-induced neocortical injury.

Neurobiol Dis 2020 May 19;142:104949. Epub 2020 May 19.

Department of Neurology and Neurological Sciences, Stanford University School of Medicine, Stanford, CA 94305, USA. Electronic address:

Long-term consequences of status epilepticus (SE) occur in a significant proportion of those who survive the acute episode. We developed an in vivo model of acute focal neocortical SE (FSE) to study long-term effects on local cortical structure and function and potential strategies to mitigate adverse consequences of SE. An acute 2 h episode of FSE was induced in anesthetized mice by epidural application of gabazine +4-aminopyridine over sensorimotor neocortex. Read More

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http://dx.doi.org/10.1016/j.nbd.2020.104949DOI Listing

Clinical and Electroencephalographic Features of the Seizures in Neuronal Surface Antibody-Associated Autoimmune Encephalitis.

Front Neurol 2020 5;11:280. Epub 2020 May 5.

Department of Neurology, The First Affiliated Hospital of Dalian Medical University, Dalian, China.

To investigate clinical and electroencephalographic features of the seizures in different types of neuronal surface antibody (NSAb)-associated autoimmune encephalitis (AE). The clinical data of the seizures were analyzed in 18 patients with NSAb-associated AEs diagnosed in the First Affiliated Hospital of Dalian Medical University. From May 2013 to April 2019, a total of 18 cases of NSAb-associated AE were diagnosed, including 9 cases of leucine-rich glioma-inactivated 1 protein (LGI1) antibody-associated encephalitis, 7 cases of anti--methyl-d-aspartate receptor (NMDAR) encephalitis, and 2 cases of anti-γ-aminobutyric acid B receptor (GABAR) encephalitis. Read More

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http://dx.doi.org/10.3389/fneur.2020.00280DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7214674PMC

Investigation of Cerebral O-(2-[F]Fluoroethyl)-L-Tyrosine Uptake in Rat Epilepsy Models.

Mol Imaging Biol 2020 May 14. Epub 2020 May 14.

Institute of Neuroscience and Medicine (INM-4; INM-5; INM-11), Forschungszentrum Jülich, 52425, Jülich, Germany.

Purpose: A recent study reported on high, longer lasting and finally reversible cerebral uptake of O-(2-[F]fluoroethyl)-L-tyrosine ([F]FET) induced by epileptic activity. Therefore, we examined cerebral [F]FET uptake in two chemically induced rat epilepsy models and in patients with focal epilepsy to further investigate whether this phenomenon represents a major pitfall in brain tumor diagnostics and whether [F]FET may be a potential marker to localize epileptic foci.

Procedures: Five rats underwent kainic acid titration to exhibit 3 to 3. Read More

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http://dx.doi.org/10.1007/s11307-020-01503-xDOI Listing

The Perirolandic Sign: A Unique Imaging Finding Observed in Association with Polymerase γ-Related Disorders.

AJNR Am J Neuroradiol 2020 05 7;41(5):917-922. Epub 2020 May 7.

From the Departments of Radiology and Division of Neuroradiology (F.G.G., B.H., C.A.P.F.A., S.R.T., J.S.M.-S., A.V., G.Z.).

Pathogenic variants in the () cause a diverse group of pathologies known as -related disorders. In this report, we describe brain MR imaging findings and electroencephalogram correlates of 13 children with -related disorders at diagnosis and follow-up. At diagnosis, all patients had seizures and 12 had abnormal MR imaging findings. Read More

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http://dx.doi.org/10.3174/ajnr.A6514DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7228159PMC

Sudden Onset Fluent Aphasia: Stroke or Seizure?

Neurohospitalist 2020 Apr 28;10(2):121-126. Epub 2019 Jul 28.

Department of Neurology, Emory University, Atlanta, GA, USA.

Conventional understanding of acute onset language deficits indicates that fluent aphasias are due to perisylvian lesions in the dominant hemisphere, most often in the setting of acute stroke. Case studies and retrospective analyses, however, suggest the need to keep ictal phenomena as an alternative diagnostic possibility. The following case illustrates an epileptic mechanism of sudden onset fluent aphasia mimicking an acute stroke presentation. Read More

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http://dx.doi.org/10.1177/1941874419864757DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7191664PMC

New-onset refractory status epilepticus and febrile infection-related epilepsy syndrome.

Dev Med Child Neurol 2020 Aug 5;62(8):897-905. Epub 2020 May 5.

Rare and Complex Epilepsy Unit, Department of Neuroscience and Neurorehabilitation, Bambino Gesù Children Hospital, Rome, Italy.

New-onset refractory status epilepticus (NORSE) and febrile infection-related epilepsy syndrome (FIRES) are relatively rare clinical presentations. They are characterized by de novo onset of refractory status epilepticus (RSE) without clearly identifiable acute or active cause (structural, toxic, or metabolic). We reviewed the literature using PubMed reports published between 2003 and 2019 and summarized the clinical, neurophysiological, imaging, and treatment findings. Read More

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http://dx.doi.org/10.1111/dmcn.14553DOI Listing

Clinical characterization of status epilepticus in childhood: a retrospective study in 124 patients.

Seizure 2020 May 17;78:127-133. Epub 2020 Apr 17.

Child Neurology and Psychiatry Unit, S Orsola Malpighi Hospital, University of Bologna, Bologna, Italy.

Purpose: The aim of this study is to describe demographic data, semiology and etiology in a pediatric population with status epilepticus (SE) and refractory SE (RSE).

Method: We retrospectively reviewed patients with the following inclusion criteria: i) age between two months and eighteen years; ii) SE diagnosis; iii) admission from January 2001 to December 2016; iv) available clinical data.

Results: We enrolled 124 patients. Read More

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http://dx.doi.org/10.1016/j.seizure.2020.03.019DOI Listing

Post-Traumatic Status Epilepticus Masquerading as Acute Ischemic Stroke: A Case Report and Literature Review.

Am J Case Rep 2020 May 4;21:e922679. Epub 2020 May 4.

Department of Neurosurgery, Pediatric Hospital of Athens, Agia Sophia, Athens, Greece.

BACKGROUND Advanced imaging is one of the main modalities utilized in the diagnostic investigation of a first-time epileptic ictus, as well as in the evaluation of a patient suspected of having an ischemic stroke. CASE REPORT We report the case of a 7-year-old boy who was admitted to our hospital because of a depressed skull fracture. Soon after its initial evaluation, he had an episode of generalized tonic-clonic seizures; therefore, a detailed diagnostic work up was scheduled, which raised the diagnostic dilemma of ischemic stroke versus imaging alterations related to status epilepticus. Read More

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http://dx.doi.org/10.12659/AJCR.922679DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7213816PMC

Pathogenic variants in gene identified by clinical whole-genome sequencing.

Cold Spring Harb Mol Case Stud 2020 Jun 12;6(3). Epub 2020 Jun 12.

Center for Pediatric Genomic Medicine, Children's Mercy Hospital, Kansas City, Missouri 64108, USA.

Status epilepticus is not rare in critically ill intensive care unit patients, but its diagnosis is often delayed or missed. The mortality for convulsive status epilepticus is dependent on the underlying aetiologies and the age of the patients and thus varies from study to study. In this context, effective molecular diagnosis in a pediatric patient with a genetically heterogeneous phenotype is essential. Read More

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http://dx.doi.org/10.1101/mcs.a003970DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7304362PMC

Focal status epilepticus as unique clinical feature of COVID-19: A case report.

Seizure 2020 May 21;78:109-112. Epub 2020 Apr 21.

Neurologia, Dipartimento di Neuroscienze, Università Cattolica del Sacro Cuore, Rome, Italy; Neurologia, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Rome, Italy.

SARS-CoV-2, a novel zoonotic coronavirus, is currently spreading all over the world, causing a pandemic disease defined coronavirus disease 2019 (COVID-19). The spectrum of COVID-19 ranges from asymptomatic or mild infection to rapidly progressive, acute respiratory distress syndrome and death [1].To the best of our knowledge, status epilepticus has never been described as initial presentation of COVID-19. Read More

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http://dx.doi.org/10.1016/j.seizure.2020.04.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7172719PMC

Status epilepticus during correction of hyponatremia in a patient with Alzheimer's disease: A case report.

SAGE Open Med Case Rep 2020 15;8:2050313X20915416. Epub 2020 Apr 15.

Division of Neurosurgery, National Hospital Organization Shibukawa Medical Center, Shibukawa, Japan.

An 83-year-old Japanese man with Alzheimer's disease was admitted to our hospital for treatment of hyponatremia resulting from water intoxication. During hospitalization, the patient developed focal impaired awareness seizures, focal to bilateral tonic-clonic seizures, and subsequent status epilepticus. Electroencephalogram during focal impaired awareness seizures showed rhythmic 5-9 Hz theta activity in the right frontotemporal region. Read More

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http://dx.doi.org/10.1177/2050313X20915416DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7160763PMC

Praxis-induced reflex seizures in two Japanese cases with ring chromosome 20 syndrome.

Epileptic Disord 2020 Apr;22(2):214-218

Department of Paediatrics, Jichi Medical University, Shimotsuke, Japan.

Ring chromosome 20 syndrome is an epileptic and neurodevelopmental encephalopathy that occurs in children, characterised by a triad of refractory frontal lobe seizures, recurrent non-convulsive status epilepticus and frontal lobe-dominant paroxysmal discharges. However, details of other clinical features associated with ring chromosome 20 syndrome remain unknown. Here, we report two patients with ring chromosome 20 syndrome who had praxis-induced reflex seizures. Read More

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http://dx.doi.org/10.1684/epd.2020.1146DOI Listing

Splenial Restricted Diffusion as MRI Correlate of Diaschisis in a Blind Infant With Unilateral Posterior Cerebral Artery Stroke.

J Neuroophthalmol 2020 Apr 6. Epub 2020 Apr 6.

Department of Ophthalmology and Visual Sciences (EAE, TPP, JDT), Kellogg Eye Center, University of Michigan, Ann Arbor, Michigan; Department of Radiology (Neuroradiology) (AS, TM), University of Michigan, Ann Arbor, Michigan; and Department of Neurology (JDT), University of Michigan, Ann Arbor, Michigan.

A 3-month-old male infant appeared on multiple clinical examinations to have acutely developed bilateral retrogeniculate blindness. Electroencephalography showed focal status epilepticus confined to the left posterior cerebral hemisphere. MRI demonstrated restricted diffusion in the domain of the left posterior cerebral artery consistent with acute stroke. Read More

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http://dx.doi.org/10.1097/WNO.0000000000000954DOI Listing

Anxiety as the only manifestation of focal non-convulsive status epilepticus: case report and review of the literature.

Neurocase 2020 04 31;26(2):98-102. Epub 2020 Mar 31.

Department of Human Neurosciences, Neurology Unit, 'Sapienza' University of Rome, Rome, Italy.

Psychiatric disorders represent common comorbidities in epileptic patients. Sometimes anxiety is part of the ictal semiology, especially during seizures arising from/involving frontal or temporal lobes. We describe a patient with focal epilepsy and recurrent hyperkinetic seizures who also presented prolonged episodes characterized by massive anxiety, alarm and fear. Read More

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http://dx.doi.org/10.1080/13554794.2020.1741647DOI Listing

"Negative T2 shine through" in patients with hyperglycemia and seizures: a frequently overlooked MRI pattern.

Neuroradiology 2020 Jul 26;62(7):895-899. Epub 2020 Mar 26.

Department of Neurology, University Medical Center Bonn, Bonn, Germany.

Epileptic seizures associated with hyperglycemia have a rare but characteristic MR imaging pattern which however is frequently missed. It consists of a T2 hypointensity and an apparent diffusion coefficient (ADC) decrease of the white matter underlying the epileptic cortex; the cortex itself may be DWI hyperintense and show a blood-brain barrier disruption. Prompt diagnosis is relevant since treatment of the hyperglycemic state rather than treatment with anti-epileptic drugs frequently interrupts the seizures. Read More

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http://dx.doi.org/10.1007/s00234-020-02408-wDOI Listing

A model for focal seizure onset, propagation, evolution, and progression.

Elife 2020 Mar 23;9. Epub 2020 Mar 23.

Department of Physiology and Cellular Biophysics, Columbia University, New York, United States.

We developed a neural network model that can account for major elements common to human focal seizures. These include the tonic-clonic transition, slow advance of clinical semiology and corresponding seizure territory expansion, widespread EEG synchronization, and slowing of the ictal rhythm as the seizure approaches termination. These were reproduced by incorporating usage-dependent exhaustion of inhibition in an adaptive neural network that receives global feedback inhibition in addition to local recurrent projections. Read More

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http://dx.doi.org/10.7554/eLife.50927DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7089769PMC

The ketogenic diet all grown up-Ketogenic diet therapies for adults.

Epilepsy Res 2020 May 10;162:106319. Epub 2020 Mar 10.

Comprehensive Epilepsy Center, Department of Neurology, Johns Hopkins University, Baltimore, MD, USA. Electronic address:

The use of ketogenic diet therapies (KDT) in adults has expanded in the last two decades and has been accompanied by a surge of new retrospective as well as prospective studies evaluating its efficacy in adults with epilepsy. In this review article, we will highlight the recent clinical trials and advances in the use of the ketogenic diet therapy (KDT) in adult patients with epilepsy. We will analyze the responder rate in regard to the epilepsy syndrome (focal vs generalized) to identify adults who are optimal to consider for KDT. Read More

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http://dx.doi.org/10.1016/j.eplepsyres.2020.106319DOI Listing

Hepatic encephalopathy: a rare cause of focal seizures in chronic liver disease.

BMJ Case Rep 2020 Mar 12;13(3). Epub 2020 Mar 12.

Department of Gastroenterology, Homerton University Hospital NHS Foundation Trust, London, UK.

Hepatic encephalopathy (HE) is an extremely rare cause of focal seizures and is usually a diagnosis of exclusion when more commoner causes such as infection, autoimmune and malignancy have been discounted. The literature reports patients with generalised cerebral oedema and rarely status epilepticus, but these are often in the context of acute liver failure as opposed to chronic liver disease. Here we discuss a case of HE leading to focal neurological deficits and seizures in a 48-year-old woman with a background of chronic alcoholic liver disease. Read More

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http://dx.doi.org/10.1136/bcr-2019-233046DOI Listing

Bilateral asymmetric auricular myoclonus as a manifestation of focal motor seizure: Phenomenology, potential lateralizing value, and insights into auricular motor control.

J Neurol Sci 2020 Jun 2;413:116762. Epub 2020 Mar 2.

Division of Neurology, Department of Medicine, Faculty of Medicine, Ramathibodi Hospital, Mahidol University, Bangkok, Thailand. Electronic address:

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http://dx.doi.org/10.1016/j.jns.2020.116762DOI Listing

Nonconvulsive status epilepticus in the elderly.

Authors:
S Dupont K Kinugawa

Rev Neurol (Paris) 2020 Mar 10. Epub 2020 Mar 10.

Functional Explorations Unit for the older patients, Pitié-Salpêtrière-Charles-Foix group, Charles-Foix Hospital, AP-HP, 94200 Ivry-sur-Seine, France; CNRS, UMR 8256 Biological Adaptation and Aging, Sorbonne Université, 75005 Paris, France.

There is a higher incidence of status epilepticus in the older adult population that commonly presents as nonconvulsive status epilepticus (NCSE). NCSE most often corresponds to prolonged focal seizures with impaired consciousness with three main clinical presentations: i) an unexplained acute confusional state, ii) subtle eye, motor or behavioral signs or mood changes and iii) typical temporal or frontal seizures with impaired consciousness. Focal seizures without impaired consciousness or de novo absence status of late onset may also be met. Read More

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http://dx.doi.org/10.1016/j.neurol.2019.12.007DOI Listing

Cefepime-Induced Neurotoxicity Presenting with Nonconvulsive Status Epilepticus Admitted as a Stroke Alert.

Am J Case Rep 2020 Mar 9;21:e921643. Epub 2020 Mar 9.

Division of Hospital Medicine, Department of Medicine, Denver Health Medical Center, Denver, CO, USA.

BACKGROUND Cefepime-induced neurotoxicity has been described in intensive care units (ICUs) and neuro ICU settings, occurring in patients started on cefepime for management of severe infections and sepsis. Most cases occur within 1 to 10 days after starting the drug. We publish a case that occurred on the general medical ward of a patient who had been on cefepime therapy for 4 weeks prior to admission. Read More

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http://dx.doi.org/10.12659/AJCR.921643DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7081951PMC

[Epilepsy in old age].

Nervenarzt 2020 Apr;91(4):353-361

Epilepsiezentrum Frankfurt Rhein-Main, Klinik für Neurologie, Zentrum der Neurologie und Neurochirurgie, Universitätsklinikum Frankfurt, Goethe-Universität Frankfurt, Schleusenweg 2-16 (Haus 95), 60528, Frankfurt am Main, Deutschland.

In an aging society, epilepsy in old age will become a more and more relevant disease. The diagnosis is often difficult because of the frequent occurrence of focal seizures in old age, which are easily overlooked. The diagnosis is often delayed, particularly in older patients who, for example also suffer from dementia. Read More

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http://dx.doi.org/10.1007/s00115-020-00888-yDOI Listing

Epilepsia Partialis Continua as an Early Sign of Anti-Myelin Oligodendrocyte Glycoprotein Antibody-positive Encephalitis.

Intern Med 2020 Jun 5;59(11):1445-1449. Epub 2020 Mar 5.

Department of Neurology, Japanese Red Cross Medical Center, Japan.

Anti-myelin oligodendrocyte glycoprotein (MOG) antibodies have been associated with steroid-responsive cortical encephalitis and comorbid generalized epilepsy. A 44-year-old woman developed repeated epilepsia partialis continua (EPC) without generalized seizures and was anti-MOG antibody-positive. Radiological abnormalities were detected in the bilateral medial frontoparietal cortices, but there were no cerebrospinal fluid abnormalities. Read More

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http://dx.doi.org/10.2169/internalmedicine.3076-19DOI Listing

A case of disseminated microscopic demyelination with multifocal dystrophic calcification.

Neuropathology 2020 Jun 3;40(3):308-314. Epub 2020 Mar 3.

Department of Laboratory Medicine and Pathobiology & Keenan Research Centre of the Li Ka Shing Knowledge Institute, St. Michael's Hospital, University of Toronto, Toronto, Ontario, Canada.

We present a 47-year-old woman with a 10-year disease course consisting of episodic confusion, aphasia, psychosis, depression, migrainous headaches and seizures. There was mild elevation of protein levels in the cerebrospinal fluid, progressive cerebral atrophy, and numerous small T1 hypointensities appearing as central "holes" in the corpus callosum on magnetic resonance imaging. She eventually expired due to status epilepticus and subsequent significant respiratory complications. Read More

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http://dx.doi.org/10.1111/neup.12642DOI Listing

Clinical conundrum: status epilepticus culminating into acute dystonia myoclonus.

BMJ Case Rep 2020 Feb 28;13(2). Epub 2020 Feb 28.

Neurology, King George Medical University, Lucknow, Uttar Pradesh, India

A 7-year-old child who suffered from symptomatic focal epilepsy as a sequel to perinatal hypoxia used to have frequent seizures. This time she developed prolonged status epilepticus lasting for over 5 hours. She received a treatment in the form of intravenous midazolam and reinitiation of sodium valproate and clobazam that were discontinued previously. Read More

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http://dx.doi.org/10.1136/bcr-2019-233397DOI Listing
February 2020

[Clinical phenotypes of epilepsy associated with GABRA1 gene variants].

Zhonghua Er Ke Za Zhi 2020 Feb;58(2):118-122

Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.

To summarize the clinical phenotypes of epilepsy in patients with GABRA1 gene variants. A total of 11 epileptic patients (4 boys and 7 girls) who were treated in the Department of Pediatrics, Peking University First Hospital from March 2016 to July 2019 and detected with GABRA1 gene heterozygous pathogenic variants by targeted next-generation sequencing were enrolled. The features of clinical manifestations, electroencephalogram (EEG), and neuroimaging were analyzed retrospectively. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2020.02.010DOI Listing
February 2020

Consensus-based statements for the management of mitochondrial stroke-like episodes.

Wellcome Open Res 2019 13;4:201. Epub 2019 Dec 13.

Wellcome Centre for Mitochondrial Research, Newcastle University, UK, Newcastle upon Tyne, Tyne and Wear, NE2 4HH, UK.

Focal-onset seizures and encephalopathy are prominent features of a stroke-like episode, which is a severe neurological manifestation associated with subtypes of mitochondrial disease. Despite more than 30 years of research, the acute treatment of stroke-like episodes remains controversial. We used the modified Delphi process to harness the clinical expertise of a group of mitochondrial disease specialists from five European countries to produce consensus guidance for the acute management of stroke-like episodes and commonly associated complications. Read More

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http://dx.doi.org/10.12688/wellcomeopenres.15599.1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7014928PMC
December 2019

Very late-onset mitochondrial cytopathy featuring epilepsia partialis continua and bilateral deafness: A case report.

Seizure 2020 Jan 28;76:153-155. Epub 2020 Jan 28.

Oxford Epilepsy Research Group, NIHR Biomedical Research Centre, Nuffield Department of Clinical Neuroscience, John Radcliffe Hospital, Oxford OX3 9DU, UK. Electronic address:

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http://dx.doi.org/10.1016/j.seizure.2020.01.020DOI Listing
January 2020

Focal cortical hypermetabolism in atypical benign rolandic epilepsy.

Epilepsy Res 2020 Mar 10;161:106288. Epub 2020 Feb 10.

Department of Neurology, The Royal Children's Hospital, 50 Flemington Road, Parkville, Victoria, 3052, Australia; Developmental Brain Imaging and Neuroscience Research Groups, Murdoch Children's Research Institute, 50 Flemington Road, Parkville, Victoria, 3052, Australia; Department of Pediatrics, The University of Melbourne, Grattan Street, Parkville, 3010, Australia. Electronic address:

Objective: Atypical benign rolandic epilepsy (BRE) is an underrecognized and poorly understood manifestation of a common epileptic syndrome. Most consider it a focal epileptic encephalopathy in which frequent, interictal, centrotemporal spikes lead to negative motor seizures and interfere with motor and sometimes speech and cognitive abilities. We observed focal cortical hypermetabolism on PET in three children with atypical BRE and investigated the spatial and temporal relationship with their centrotemporal spikes. Read More

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http://dx.doi.org/10.1016/j.eplepsyres.2020.106288DOI Listing

Effects of Non-invasive, Targeted, Neuronal Lesions on Seizures in a Mouse Model of Temporal Lobe Epilepsy.

Ultrasound Med Biol 2020 May 17;46(5):1224-1234. Epub 2020 Feb 17.

Neuroradiology Section, Department of Radiology, School of Medicine, Stanford University, California, USA. Electronic address:

Surgery to treat drug-resistant epilepsy can be quite effective but remains substantially underutilized. A pilot study was undertaken to test the feasibility of using a non-invasive, non-ablative, approach to produce focal neuronal loss to treat seizures in a rodent model of temporal lobe epilepsy. In this study, spontaneous, recurrent seizures were established in a mouse model of pilocarpine-induced status epilepticus. Read More

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http://dx.doi.org/10.1016/j.ultrasmedbio.2020.01.008DOI Listing
May 2020
2.214 Impact Factor

Surgical management of pediatric patients with encephalopathy due to electrical status epilepticus during sleep (ESES).

Epileptic Disord 2020 Feb;22(1):39-54

Division of Pediatric Neurology, Nicklaus Children's Hospital, Miami, USA.

ESES is a developmental epileptic disorder directly responsible for progressive encephalopathy and neurocognitive regression. The natural history, indications for surgical intervention, and predictors for favorable seizure and neuropsychological outcome remain unclear. We performed a retrospective review of children who underwent resective or disconnective surgery for ESES between January 2009 and July 2016 at a large tertiary pediatric center. Read More

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http://dx.doi.org/10.1684/epd.2020.1129DOI Listing
February 2020
0.899 Impact Factor

Spontaneous interhemispheric subdural hematoma presenting as epilepsia partialis continua.

Acta Neurol Belg 2020 Feb 10. Epub 2020 Feb 10.

Department of Rheumatology, Shin Ill Medical Clinic, Seoul, South Korea.

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http://dx.doi.org/10.1007/s13760-020-01294-6DOI Listing
February 2020
0.598 Impact Factor

Encephalopathy related to status epilepticus during slow sleep (ESES) as atypical evolution of Panayiotopoulos syndrome: an EEG and neuropsychological study.

Epileptic Disord 2020 Feb;22(1):67-72

Department of Pediatrics, Tokyo Women's Medical University, Tokyo.

We report two patients with Panayiotopoulos syndrome (PS) who developed encephalopathy related to status epilepticus during slow sleep (ESES) at the peak of their clinical course. Clinical charts and EEG data were reviewed. The patients exhibited nocturnal autonomic seizures and occipital EEG foci, the latter of which later evolved into multifocal EEG foci with synchronous frontopolar and occipital spikes (Fp-O EEG foci), and finally into continuous spikes-waves during sleep (CSWS; spike-wave index >85% based on whole-night sleep recording) at eight years and seven years of age, respectively. Read More

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http://dx.doi.org/10.1684/epd.2020.1128DOI Listing
February 2020

Genotypes and estimated prevalence of phosphomannomutase 2 deficiency in Turkey differ significantly from those in Europe.

Am J Med Genet A 2020 04 25;182(4):705-712. Epub 2020 Jan 25.

Faculty of Medicine, Department of Pediatrics, Division of Pediatric Metabolism, Hacettepe University, Ankara, Turkey.

Phosphomannomutase 2 deficiency (PMM2-CDG) is an autosomal recessive congenital disorder of glycosylation, characterized by multisystem phenotypes, mostly including neurological involvement. In Turkey, due to high rates of consanguinity, many patients with autosomal recessive disorders have homozygous variants and these diseases are more common, compared to Europe. However, published reports of PMM2-CDG from Turkey are scarce. Read More

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http://dx.doi.org/10.1002/ajmg.a.61488DOI Listing

Breach Rhythm May Be Suppressed as a Form of Todd's Paralysis.

J Clin Neurophysiol 2020 May;37(3):271-273

Klinik für Neurologie, Klinikum Kassel, Kassel, Germany.

This case report will provide further evidence for the fact that breach rhythm is not the effect of a bone abnormality only. We present the case of an 84-year-old woman, who had a craniotomy 14 month before admission to our emergency department with a focal inhibitory status epilepticus. Even after clinical recovery, electroencephalography revealed frequent subclinical seizure patterns. Read More

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http://dx.doi.org/10.1097/WNP.0000000000000683DOI Listing

The mitochondrial epilepsies.

Eur J Paediatr Neurol 2020 Jan 7;24:47-52. Epub 2020 Jan 7.

Wellcome Centre for Mitochondrial Research, Institute of Neuroscience, Newcastle University, Newcastle-Upon-Tyne, NE2 4HH, United Kingdom; Department of Neurology, Royal Victoria Infirmary, Queen Victoria Rd, Newcastle-Upon-Tyne, NE1 4LP, United Kingdom; Institute of Neuroscience, Henry Wellcome Building, Framlington Place, Newcastle University, Newcastle-Upon-Tyne, NE2 4HH, United Kingdom. Electronic address:

Mitochondria are vital organelles within cells that undertake many important metabolic roles, the most significant of which is to generate energy to support organ function. Dysfunction of the mitochondrion can lead to a wide range of clinical features, predominantly affecting organs with a high metabolic demand such as the brain. One of the main neurological manifestations of mitochondrial disease is metabolic epilepsies. Read More

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http://dx.doi.org/10.1016/j.ejpn.2019.12.021DOI Listing
January 2020

Patient with Epilepsy Caused by the Spontaneous Rupture of an Intracerebral Dermoid Cyst.

World Neurosurg 2020 Apr 16;136:140-145. Epub 2020 Jan 16.

Department of Neurosurgery, Tianjin Medical University General Hospital, Tianjin, R.P. China. Electronic address:

Background: This is a rare case of a patient presenting with epileptic seizures and headaches who was diagnosed with spontaneous intracerebral dermoid cyst rupture via radiographic imagery, and rupture was confirmed via a pathology report.

Case Description: We report the case of a woman aged 26 years who presented with a history of chronic headache for 9 years without other symptoms, and progressive worsening of her headache had occurred for 1 month prior to admission. Radiologic examination showed a large mass located in the left temporal fossa and a large amount of homogeneous matter in the subarachnoid space of the ipsilateral cerebral hemisphere, then the tumor was completely excised. Read More

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http://dx.doi.org/10.1016/j.wneu.2020.01.069DOI Listing

Prevalence and clinical correlates of non-convulsive status epilepticus in elderly patients with acute confusional state: A systematic literature review.

J Neurol Sci 2020 Mar 7;410:116674. Epub 2020 Jan 7.

Department of Neuropsychiatry, University of Birmingham and BSMHFT, Birmingham, UK; School of Life and Health Sciences, Aston Brain Centre, Aston University, Birmingham, UK; Sobell Department of Motor Neuroscience and Movement Disorders, Institute of Neurology and UCL, London, UK. Electronic address:

Non-convulsive status epilepticus (NCSE) is a potentially treatable condition that poses considerable diagnostic challenges. NCSE is thought to be more common in the elderly than in the general population, however additional diagnostic challenges complicate its recognition in older patients, because of the wide differential diagnosis with common underlying causes of acute confusional state in this age group. We set out to review the existing evidence on the clinical correlates of NCSE in the elderly population. Read More

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http://dx.doi.org/10.1016/j.jns.2020.116674DOI Listing

Seizure Characteristics, Outcome, and Risk of Epilepsy in Pediatric Anti-N-Methyl-d-Aspartate Receptor Encephalitis.

Pediatr Neurol 2020 04 30;105:35-40. Epub 2019 Nov 30.

Department of Neurology, Children's Hospital of Chongqing Medical University, Chongqing, China; Ministry of Education Key Laboratory of Child Development and Disorders, Chongqing, China; National Clinical Research Center for Child Health and Disorders, Chongqing, China; China International Science and Technology Cooperation Base of Child Development and Critical Disorders, Chongqing, China; Chongqing Key Laboratory of Pediatrics, Chongqing, China. Electronic address:

Background: We identified seizure characteristics, long-term outcome, and predictors of persistent seizures in children with anti-N-methyl-d-aspartate receptor (anti-NMDAR) encephalitis.

Method: Data were analyzed from patients with anti-NMDAR encephalitis who presented with seizures at our center between August 2012 and June 2018.

Results: Sixty-two of 86 patients with anti-NMDAR encephalitis experienced seizures. Read More

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http://dx.doi.org/10.1016/j.pediatrneurol.2019.11.011DOI Listing

[Genotype and phenotype of children with KCNA2 gene related developmental and epileptic encephalopathy].

Zhonghua Er Ke Za Zhi 2020 Jan;58(1):35-40

Department of Pediatrics, Peking University First Hospital, Beijing 100034, China.

To investigate the genotype and phenotype of children with KCNA2 gene related developmental and epileptic encephalopathy (DEE). Clinical data including the manifestations and electroencephalogram of 8 children with KCNA2 variants treated in the Department of Pediatrics, Peking University First Hospital from March 2017 to June 2019 were collected and analyzed retrospectively. Among the 8 epileptic patients with KCNA2 variants, 5 were males and 3 were females. Read More

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http://dx.doi.org/10.3760/cma.j.issn.0578-1310.2020.01.009DOI Listing
January 2020

Phenotypic and genetic spectrum of SCN8A-related disorders, treatment options, and outcomes.

Epilepsia 2019 12;60 Suppl 3:S77-S85

Danish Epilepsy Center, Dianalund, Denmark.

Pathogenic variants in SCN8A have originally been described in patients with developmental and epileptic encephalopathy (DEE). However, recent studies have shown that SCN8A variants can be associated with a broader phenotypic spectrum, including the following: (1) Patients with early onset, severe DEE, developing severe cognitive and motor regression, pyramidal/extrapyramidal signs, and cortical blindness. Severe SCN8A-DEE is characterized by intractable seizures beginning in the first months of life. Read More

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http://dx.doi.org/10.1111/epi.16319DOI Listing
December 2019

The role of CT perfusion in the evaluation of seizures, the post-ictal state, and status epilepticus.

Epilepsy Res 2020 Jan 12;159:106256. Epub 2019 Dec 12.

Department of Neurology: Johns Hopkins University School of Medicine, Baltimore, MD, USA.

Rapid identification of stroke mimics, such as seizures, is a critical part of the evaluation of patients presenting with acute focal neurological deficits. The primary role of CT perfusion is in identification of patients with acute ischemic stroke who may be candidates for reperfusion therapy, but also holds promise as a modality for identifying a seizure etiology. We review the literature on this topic and attempt to define characteristic CT perfusion findings at various points along the ictal-interictal (epileptic) continuum. Read More

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http://dx.doi.org/10.1016/j.eplepsyres.2019.106256DOI Listing
January 2020