Childhood epileptic seizures imitating migraine and encephalitis

Introduction. Paroxismal events can resemble epileptic seizures, however, some epileptic seizures, especially benign occipital childhood epilepsies can imitate migraine, cycling vomiting or encephalitis. Objective. The aim of this study was evaluation of clinical and electroencephalographic (EEG) features and outcome in children with benign occipital childhood epilepsies. Methods. Investigation included 18 patients with benign occipital childhood epilepsies hospitalized in the period from 2007 to 2010. The diagnosis was based on clinical and EEG characteristics of seizures, while treatment included acute therapy for seizures and chronic antiepileptic drugs. Prognosis was analyzed in terms of neurological outcome and seizure recurrence rate. Results. Benign occipital childhood epilepsy with early onset was diagnosed in 15 children. Vegetative symptoms, mostly ictal vomiting (13), eye deviation and loss of consciousness (13) dominated in the clinical presentation. The most frequent EEG findings showed occipital epileptic discharges. Benign occipital childhood epilepsy with late onset was diagnosed in three cases. Seizures were manifested by visual hallucinations, headache and secondary generalized convulsions. All three patients were administered chronic antiepileptic drugs and had good outcome. Conclusion. In our patients, clinical manifestations of benign occipital epilepsies had some similarities with clinical features of migraine and encephalitis. It could explain misdiagnosis in some of them. Knowledge about main features and differences between each of these disorders is crucial for making appropriate diagnosis.

Download Full-text

Epilepsy in Down Syndrome: A Highly Prevalent Comorbidity

Journal of Clinical Medicine ◽

10.3390/jcm10132776 ◽

2021 ◽

Vol 10 (13) ◽

pp. 2776

Author(s):

Miren Altuna ◽

Sandra Giménez ◽

Juan Fortea

Keyword(s):

Down Syndrome ◽

Functional Outcomes ◽

Clinical Presentation ◽

Late Onset ◽

Current Knowledge ◽

Epileptic Seizures ◽

Myoclonic Epilepsy ◽

Increased Risk ◽

Number Of Individuals

Individuals with Down syndrome (DS) have an increased risk for epilepsy during the whole lifespan, but especially after age 40 years. The increase in the number of individuals with DS living into late middle age due to improved health care is resulting in an increase in epilepsy prevalence in this population. However, these epileptic seizures are probably underdiagnosed and inadequately treated. This late onset epilepsy is linked to the development of symptomatic Alzheimer’s disease (AD), which is the main comorbidity in adults with DS with a cumulative incidence of more than 90% of adults by the seventh decade. More than 50% of patients with DS and AD dementia will most likely develop epilepsy, which in this context has a specific clinical presentation in the form of generalized myoclonic epilepsy. This epilepsy, named late onset myoclonic epilepsy (LOMEDS) affects the quality of life, might be associated with worse cognitive and functional outcomes in patients with AD dementia and has an impact on mortality. This review aims to summarize the current knowledge about the clinical and electrophysiological characteristics, diagnosis and treatment of epileptic seizures in the DS population, with a special emphasis on LOMEDS. Raised awareness and a better understanding of epilepsy in DS from families, caregivers and clinicians could enable earlier diagnoses and better treatments for individuals with DS.

Download Full-text

Mouse Models Characterize GNAO1 Encephalopathy as a Neurodevelopmental Disorder Leading to Motor Anomalies: from a Severe G203R to a Milder C215Y Mutation

10.21203/rs.3.rs-1089935/v1 ◽

2021 ◽

Author(s):

Denis Silachev ◽

Alexey Koval ◽

Mikhail Savitsky ◽

Guru Padmasola ◽

Charles Quairiaux ◽

...

Keyword(s):

Mouse Models ◽

De Novo ◽

Late Onset ◽

Wide Spectrum ◽

Neurodevelopmental Disorder ◽

Point Mutations ◽

Clinical Manifestations ◽

Epileptic Seizures ◽

Motor Dysfunction ◽

Neuronal Precursor Cells

Abstract GNAO1 encephalopathy characterized by a wide spectrum of neurological deficiencies in pediatric patients originates from de novo heterozygous mutations in the gene encoding Gαo, the major neuronal G protein. Efficient treatments and even the proper understanding of the underlying etiology are currently lacking for this dominant disease. Adequate animal models of GNAO1 encephalopathy are urgently needed. Here we describe establishment and characterization of mouse models of the disease based on two point mutations in GNAO1 with different clinical manifestations. One of them is G203R leading to the early-onset epileptic seizures, motor dysfunction, developmental delay and intellectual disability. The other is C215Y producing much milder clinical outcomes, mostly – late-onset motor hyperactivity. The resultant mouse models show distinct phenotypes: severe neonatal lethality in GNAO1[G203R]/+ mice vs. normal vitality in GNAO1[C215Y]/+. The latter model further revealed strong hyperactivity and hyperlocomotion in a panel of behavioral assays, without signs of epilepsy, recapitulating the patients’ manifestations. Importantly, despite these differences the two models similarly revealed prenatal brain developmental anomalies, such as enlarged lateral ventricles and decreased numbers of neuronal precursor cells in the cortex. Thus, our work unveils GNAO1 encephalopathy as to a large extent neurodevelopmental malady. We expect that this understanding and the tools we established will be instrumental for future therapeutic developments.

Download Full-text

Secondary late-onset Lennox-Gastaut syndrome: a critical view

Arquivos de Neuro-Psiquiatria ◽

10.1590/s0004-282x1984000200001 ◽

1984 ◽

Vol 42 (2) ◽

pp. 95-104

Author(s):

Amilton Antunes Barreira ◽

Michel Pierre Lison

Keyword(s):

Late Onset ◽

Clinical Manifestations ◽

Epileptic Seizures ◽

Psychomotor Development ◽

Average Period ◽

Partial Seizures ◽

Simple Complex ◽

Early Form ◽

Eeg Abnormalities ◽

Interictal Eeg

From a group of 66 patients with the Lennox-Gastaut syndrome, 12 whose manifestations had started after the 6th year of life were selected for study. These patients were observed clinically and electroencephalographically for an average period of 2.5 years. We concluded that the late-onset syndrome can: occur after a long interval between diffuse encephalopathy and the first clinical manifestations, with or without previous alterations in psychomotor development; be associated from the onset with serious mental retardation; exhibit simple, complex and mixed seizures similar to those observed in the early form. These patients can also: suffer complex and mixed epileptic seizures previously unreported; paroxismal interictal EEG abnormalities that overlap those of the early form; and spike-slow wave complexes in the EEG that can be actived by hyperpnea. Our results demonstrate that the incidence of LGS after 6 years of age does not necessarily imply a lower frequency of organic antecedents, or beter neu-ropsychomotor development up to the onset of the syndrome or the presence of a higher rate of nonspecific seizures (generalized or partial seizures, and mainly those with elaborate symptomatolgy). The critical and encephalographic expression of the syndrome, which is secondary and starts after the 6th year of age, may depend at least in part on the age when diffuse encephalopathy started.

Download Full-text

Multiple Cavernous Malformations: A Case Report

Journal of Clinical Cases & Reports ◽

10.46619/joccr.2020.3-1053 ◽

2020 ◽

Vol 3 (1) ◽

pp. 5-13

Author(s):

Fernando Gutiérrez Rincón Oscar ◽

◽

Moreno García Santiago ◽

José Hoyos Bedoya Maria ◽

Juliana Builes Cerón Sarah ◽

...

Keyword(s):

Case Report ◽

Medical Management ◽

Clinical Case ◽

Autosomal Dominant ◽

Clinical Presentation ◽

Clinical Manifestations ◽

Epileptic Seizures ◽

Cavernous Malformations ◽

Arteries And Veins ◽

Main Clinical Manifestation

Cavernous malformations are alterations in the conformation of arteries and veins that can be found both intracranial and intraspinal; however, the variables are very important for the diagnosis and treatment of patients. The main clinical manifestation is epileptic seizures in cases of bleeding, but in many cases they are asymptomatic in the course of life and are found as findings related to neuroimaging studies for other reasons. It is more common to find unique lesions, but in cases of multiple lesions it is likely to find an autosomal dominant hereditary factor, which makes the person more likely to convulse due to sporadic bleeding. Medical management focuses on the clinical presentation and management of epileptic seizures, while surgical management takes into account the size, location and bleeding. Below is a clinical case that represents one of the different clinical manifestations and the approach that was given in said patient.

Download Full-text

Clinical Pharmacology of Current Antiepileptic Drugs

International Journal of Pharmaceutical Sciences and Nanotechnology ◽

10.37285/ijpsn.2014.7.1.1 ◽

2014 ◽

Vol 7 (1) ◽

pp. 2305-2319

Author(s):

D. Samba Reddy

Keyword(s):

Antiepileptic Drugs ◽

Enzyme Induction ◽

Neuronal Excitability ◽

Clinical Manifestations ◽

Epileptic Seizures ◽

Induction Treatment ◽

Drug Of Choice ◽

Voltage Gated Sodium Channels ◽

Intractable Seizures ◽

Spread Of Excitation

This article describes current antiepileptic drugs (AEDs) that are available for treatment of epilepsy. Epilepsy is characterized by repeated occurrence of seizures, which are clinical manifestations of abnormal electrical discharges in the brain. Epileptic seizures arise from a multiplicity of factors that regulate neuronal excitability and synchrony. Epilepsy is caused because of certain genetic defects or acquired due to brain injury. Epileptic seizures are classified into partial (simple partial and complex partial) and generalized (absence, tonic-clonic, myoclonic, and atonic seizures) types. Around two-dozen AEDs, classified as standard and newer agents, are available for treating epilepsy. AEDs act on diverse molecular targets to selectively modify the abnormal excitability of neurons by reducing the focal seizure discharges or preventing spread of excitation. AEDs suppress seizures by blocking the voltage-gated sodium channels (phenytoin, carbamazepine, valproate, lamotrigine, oxcarbazepine, topiramate), voltage-activated calcium channels (ethosuximide, gabapentin), potentiation of GABA inhibition (barbiturates, benzodiazepines, tiagabine), and reduction of glutamate excitation (felbamate). Carbamazepine, phenytoin, and valproate are the first-line agents for partial seizures and generalized tonic-clonic seizures. Ethosuximide is the drug of choice for absence seizures. Intravenous benzodiazepines diazepam or lorazepam are effective in terminating status epilepticus. AEDs are orally-active and show unique pharmacokinetic features. Some AEDs cause enzyme induction and hence produce drug interactions. Newer AEDs such as gabapentin, levetiracetam, tiagabine, zonisamide and pregabalin do not cause enzyme induction. Treatment in pregnancy must consider optimizing therapy while preventing teratotoxicity of AEDs. There are alternative options (ketogenic diet) for children. Despite many advances in epilepsy research, nearly 30% of people with epilepsy have “intractable seizures” that do not respond to drug therapy. Presently, there is no cure for epilepsy. Therefore, newer and better AEDs that can better prevent seizures and modify the disease are needed for curing epilepsy.

Download Full-text

Faculty Opinions recommendation of Individualised prediction model of seizure recurrence and long-term outcomes after withdrawal of antiepileptic drugs in seizure-free patients: a systematic review and individual participant data meta-analysis.

Faculty Opinions – Post-Publication Peer Review of the Biomedical Literature ◽

10.3410/f.727593462.793538870 ◽

2017 ◽

Author(s):

Richard FM Chin

Keyword(s):

Systematic Review ◽

Prediction Model ◽

Antiepileptic Drugs ◽

Meta Analysis ◽

Individual Participant Data ◽

Seizure Recurrence ◽

Long Term Outcomes ◽

Individual Participant

Download Full-text

Prolonged epileptic discharges predict seizure recurrence in JME: Insights from prolonged ambulatory EEG

Epilepsia ◽

10.1111/epi.16875 ◽

2021 ◽

Author(s):

Francesco Turco ◽

Enrica Bonanni ◽

Chiara Milano ◽

Chiara Pizzanelli ◽

Cecilia Steinwurzel ◽

...

Keyword(s):

Seizure Recurrence ◽

Epileptic Discharges

Download Full-text

A Case of Familial Creutzfeldt-Jakob Disease Presenting with Dry Cough

Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques ◽

10.1017/s0317167100005072 ◽

2006 ◽

Vol 33 (2) ◽

pp. 243-245 ◽

Cited By ~ 3

Author(s):

Sandrine Larue ◽

Steve Verreault ◽

Peter Gould ◽

Michael B. Coulthart ◽

Catherine Bergeron ◽

...

Keyword(s):

Clinical Presentation ◽

Mutation Screening ◽

Disease Onset ◽

Visual Hallucinations ◽

Progressive Dementia ◽

Gene Encoding ◽

Progressive Ataxia ◽

Persistent Cough ◽

Jakob Disease ◽

Classical Triad

ABSTRACT:Background:Clinical diagnosis of Creutzfeldt-Jakob disease (CJD) is based on the classical triad of rapidly progressive dementia, myoclonus and abnormal EEG. The 200k mutation within the gene encoding PrP, located on the short arm of chromosome 20, accounts for more than 70% of families with CJD worldwide.Case Report:Herein, we report a patient who developed persistent dry cough and classical signs of CJD, including severe cognitive decline, cerebellar signs, and myoclonic jerks, leading to death a few weeks after disease onset. Mutation screening showed that he had the 200k point mutation in the PRNP gene. His mother had died twenty years earlier with neuropathologically confirmed CJD. She had presented a rapidly progressive ataxia with myoclonus, dementia, visual hallucinations, and the same persistent dry cough.Conclusions:The clinical presentation of this familial CJD case with persistent dry cough is quite unusual. Therefore, a neurological etiology should be sought when confronted with an unexplained persistent cough.

Download Full-text

Overview of Lamotrigine and the New Antiepileptic Drugs: The Challenge

Journal of Child Neurology ◽

10.1177/0883073897012001111 ◽

1997 ◽

Vol 12 (1_suppl) ◽

pp. S48-S52 ◽

Cited By ~ 25

Author(s):

John M. Pellock

Keyword(s):

Adverse Effect ◽

Adverse Effects ◽

Antiepileptic Drugs ◽

Adjunctive Therapy ◽

Dose Titration ◽

Childhood Epilepsy ◽

Major Advance ◽

New Antiepileptic Drugs ◽

Life Threatening ◽

Titration Schedule

Lamotrigine, like all antiepileptic drugs, can be effective when used as monotherapy or adjunctive therapy. In general, adverse effects are reduced when monotherapy is employed. The most frequent adverse effect prompting withdrawal of lamotrigine is rash. This potentially life-threatening adverse effect occurs more frequently in children, is increased when a rapid dose titration schedule is employed, and is greater when lamotrigine is prescribed in combination with valproate. The availability of lamotrigine and other antiepileptic drugs represents a major advance for the treatment of childhood epilepsy. The challenge in using all of the new antiepileptic drugs, including lamotrigine, is to balance the expected improved efficacy with the potentially serious adverse effects. (J Child Neurol 1997;12(Suppl 1):S48-S52).

Download Full-text