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Sadleir Lynette Grant LG Department of Paediatrics, Wellington School of Medicine, University of Otago, Wellington, New Zealand. - - 2009
Factors influencing the electroencephalography (EEG) features of absence seizures in newly presenting children with idiopathic generalized epilepsy (IGE) have not been rigorously studied. We examined how specific factors such as state, provocation, age, and epilepsy syndrome affect the EEG features of absence seizures. Children with untreated absence seizures were studied ...
Ozkara Ciğdem - - 2009
Panayiotopoulos syndrome is a common benign epilepsy affecting otherwise healthy children that present with autonomic seizures, in which nausea, retching, and vomiting are particularly common and prominent. Because of the unusual ictal symptoms and lengthy manifestations, misdiagnosis is a common major problem. We describe a young girl with intractable and ...
Deprez Liesbet - - 2009
BACKGROUND: Incidence rates of epilepsy in children are highest during the first year of life. Most frequently, epilepsy results from a metabolic or structural defect in the brain. However, some infants have clearly delineated epilepsy syndromes for which no underlying etiology can be identified except for a genetic predisposition. METHODS: ...
Helbig Ingo - - 2009
We identified 15q13.3 microdeletions encompassing the CHRNA7 gene in 12 of 1,223 individuals with idiopathic generalized epilepsy (IGE), which were not detected in 3,699 controls (joint P = 5.32 x 10(-8)). Most deletion carriers showed common IGE syndromes without other features previously associated with 15q13.3 microdeletions, such as intellectual disability, ...
Whittaker R G - - 2009
The hallmarks of the myoclonic epilepsy with ragged red fibers (MERRF) syndrome are myoclonic epilepsy, ataxia and ragged red fibres detected on muscle biopsy. We present a case of a 25-year-old male who first presented to his general practitioner at the age of 22 years with myoclonic jerks affecting the ...
Stafstrom Carl E - - 2009
This review summarizes the treatment of Lennox-Gastaut syndrome, an intractable epileptic encephalopathy of early childhood. In particular, the review focuses on rufinamide, a recently released anticonvulsant medication with reported effectiveness in this epilepsy syndrome. A systematic literature search (PubMed) was performed to review the existing literature pertaining to the treatment ...
Hancock Eleanor C - - 2009
The Lennox-Gastaut syndrome is an age-specific disorder, characterised by epileptic seizures, a characteristic electroencephalogram (EEG), psychomotor delay and behaviour disorders. It occurs more frequently in males and onset is usually before the age of eight, with a peak between three and five years. Late cases occurring in adolescence and early ...
Iannetti Paola - - 2009
Panayiotopoulos syndrome is a common and benign childhood autonomic epilepsy of debated localization. Although officially considered as occipital epilepsy, this is most likely of multifocal origin. Ictal electroencephalography is the gold standard of seizure localization, but in Panayiotopoulos syndrome, because patients have single or rare seizures, only 7 cases with ...
Hirano Yoshiko - - 2009
OBJECTIVE: We conducted a video-polygraphic study of myoclonic seizures (MS) in different epileptic syndromes to clarify semiologic and electroencephalography (EEG) differences among them. SUBJECTS AND METHODS: The subjects were 26 children with MS, including benign myoclonic epilepsy in infants (BME) in 10, severe myoclonic epilepsy in infants (SME) in 6, ...
Tuchman Roberto - - 2009
The autisms and epilepsies are heterogeneous disorders that have diverse etiologies and pathologies. The severity of impairment and of symptoms associated with autism or with particular epilepsy syndromes reflects focal or global, structurally abnormal or dysfunctional neuronal networks. The complex relationship between autism and epilepsy, as reflected in the autism-epilepsy ...
Narain Nigam Prakash - - 2008
Dyke Davidoff Masson syndrome (DDMS) is characterized by seizures, facial asymmetry, contralateral hemiplegia and mental retardation. The characteristic radiologic features are cerebral hemiatrophy with homolateral hypertrophy of the skull and sinuses. We report a case of DDMS in an 18-month-old girl who presented with right sided focal seizures, hemiparesis of ...
Hartman Adam L - - 2008
The ketogenic diet (KD) has been used successfully in a variety of epilepsy syndromes. This includes syndromes with multiple etiologies, including Lennox-Gastaut syndrome and infantile spasms; developmental syndromes of unknown etiology, such as Landau-Kleffner syndrome; and idiopathic epilepsies, such as myoclonic-astatic (Doose) epilepsy. It also includes syndromes where genetics play ...
Yang Zhixian - - 2008
The authors report a patient with Lennox-Gastaut syndrome who was a fraternal twin. The twins encountered myoclonic seizures at the age of 4 years, but the seizures in the other patient were controlled very quickly without intellectual development damage. With the disease evolving, other characteristic seizures of Lennox-Gastaut syndrome appeared ...
Jocic-Jakubi Bosanka - - 2008
This article reports on a female infant with Aicardi syndrome presenting with malignant migrating partial seizures from her first day of life. Initially, unilateral tonic seizures were seen with contralateral ictal electroencephalogram findings. Typically, these tonic seizures were accompanied by head and eye deviation and were followed by a tonic ...
Chou Chao-Liang - - 2008
Klüver-Bucy (KBS) syndrome is a rare and complicated neurobehavioral syndrome in humans resulting from damage of bilateral anterior temporal portion, especially the amygdala. It can be seen in association with a variety of etiologies. Stroke is a rarely reported. Here we present a 50-year-old right handed man who developed persistent ...
Addas Bassam - - 2008
Gelastic epilepsy (GE) associated with hypothalamic hamartomas (HHs) is now a well-characterized clinical syndrome consisting of gelastic seizures starting in infancy, medically refractory seizures with or without the development of multiple seizure types, and behavioral and cognitive decline. It has been postulated that the development of the HH-GE syndrome is ...
Durá-Travé Teodoro - - 2008
All incident cases of children living in Navarre, Spain, younger than 15 years of age with newly diagnosed epilepsy (2002-2005) were registered in a prospective study, with epidemiologic and clinical data and complementary study results collected. Based on International League Against Epilepsy criteria, 191 patients were diagnosed as having epilepsy. ...
Taylor Isabella - - 2008
The early and late benign occipital epilepsies of childhood (BOEC) are described as two discrete electro-clinical syndromes, eponymously known as Panayiotopoulos and Gastaut syndromes. Our aim was to explore the clinical features, classification and clinical genetics of these syndromes using twin and multiplex family studies to determine whether they are ...
Koutroumanidis Michael - - 2008
PURPOSE: To define the relationship between two syndromes of idiopathic generalized epilepsy (IGE) with apparently similar phenotypes: The form with generalized tonic-clonic seizures only (IGE-GTCS) and that with phantom absences (IGE-PA). METHODS: We compared the electroclinical features of 33 consecutive patients with GTCS and generalized spike wave (GSW); 18 had ...
Sadleir Lynette G LG Department of Pediatrics, Wellington School of Medicine, University of Otago, Wellington, New Zealand. - - 2008
The clinical features of absence seizures in idiopathic generalized epilepsy have been held to be syndrome-specific. This hypothesis is central to many aspects of epilepsy research yet has not been critically assessed. We examined whether specific factors such as epilepsy syndrome, age, and state determine the features of absence seizures. ...
Nabbout Rima - - 2008
A puzzling EEG pattern combining frontal slow bi-tri spikes followed or not by slow waves when awake and activated by sleep with 5-10s discharges of 8-9Hz spikes in a minority of adolescents with Dravet syndrome (DS) was recorded in the context of stable seizure and cognitive status, and unchanged antiepileptic ...
Gerstner Thorsten - - 2008
Valproic acid (VPA) is considered to be a drug of first choice for the therapy of generalized and focal epilepsies, including special epileptic syndromes. The drug is usually well tolerated, rare serious complications may occur in some patients, including hemorrhagic pancreatitis, coagulapathies, bone marrow suppression, VPA-induced hepatotoxicity and encephalopathy. We ...
Rosen Noah - - 2008
We report on 2 patients who have a mitochondrial myopathy, encephalopathy, lactic acidosis, and recurrent cerebral insults that resemble strokes (MELAS). These 2, and 9 other, reported patients share the following features: ragged red fibers evident on muscle biopsy, normal early development, short stature, seizures, and hemiparesis, hemianopia, or cortical ...
Nabbout Rima - - 2008
The aim of this article is to review new epilepsy syndromes, acquire a new understanding of older ones and emphasize the impact of this concept on basic research regarding aetiology and treatment. In addition to those included in the classification of the International League Against Epilepsy, new epilepsy syndromes comprise ...
Neubauer Bernd A - - 2008
More than 3% of the total population will suffer from epilepsy at some time during their lives. One quarter of newly diagnosed cases worldwide are children. With optimal treatment, around 70% will enter remission. In the long run, antiepileptic drugs can be discontinued in almost half of affected individuals. Most ...
Jea Andrew - - 2008
OBJECTS: Disconnection syndromes following corpus callosotomy represent complex and variably expressed groupings of signs and symptoms affecting motor control, spatial orientation, vision, hearing, and language. Little is known, however, about the functional topography of callosal fiber pathways. In addition, most published case reports and case series of corpus callosotomy seldom ...
Noureen Nuzhat - - 2008
Rasmussen syndrome or Rasmussen encephalitis is a rare, progressive gray matter disease of childhood. A case of 4-year-old boy is reported here, who presented with recurrent episodes of status epilepticus of simple partial fits along with progressive left sided hemiplegia. EEG showed focal discharges from right hemisphere. Serial MRI brain ...
Saito Naka - - 2008
Focal spike activities in Panayiotopoulos syndrome involve all brain regions in electroencephalography, and commonly reveal multiple foci, often through occipital predominance. To investigate correlations between developmental brain maturation and spike origin in Panayiotopoulos syndrome, we evaluated age-related or duration-related magnetoencephalographic spike localization in 25 patients with Panayiotopoulos syndrome. Regarding age ...
Jacob S - - 2008
Voltage-gated potassium channel antibody (VGKC-Ab)-associated limbic encephalitis (LE) is a recently described syndrome that broadens the spectrum of immunotherapy-responsive central nervous system disorders. Limbic encephalitis is typically characterised by a sub-acute onset of disorientation, amnesia and seizures, but the clinical spectrum is not yet fully defined and the syndrome could ...
Aina, O F; ;
Kluver-Bucy Syndrome, first described in monkeys as a result of bilateral ablation of their temporal lobes consists of hypermetamorphosis, visual agnosia, hyperarality and hyper-sexuality. In man, similar but not exact syndrome had been described and the symptom manifestations included placidity, apathy, bulimia, hyper-sexuality, agnosias, amnesia, seizure etc. We describe the ...
Bilgin Ozgür - - 2008
We report on a patient with right-sided Sturge-Weber syndrome (SWS), in whom earlier functional hemispherectomy failed. Subtraction of ictal and interictal single-photon-emission-computed-tomography (SPECT) superimposed on individual MRI showed a right fronto-orbital hyperperfusion, with a left-sided EEG seizure pattern. Ictal SPECT supported our assumption that right frontal originated seizure pattern propagated ...
Mastrangelo Mario - - 2009
The association of brain malformations and symptomatic epilepsy in the setting of neurofibromatosis type 1 (NF1) is rarely reported. When it occurs, patients can present clinically with infantile spasms, focal seizures, generalized tonic clonic seizures or atypical absences. We report on a 10-year-old (molecularly proven) NF1 girl manifesting a complex ...
Legriel Stephane - - 2008
INTRODUCTION: Takotsubo cardiomyopathy can complicate several conditions including neurological emergencies. A few recurrent cases associated with seizures have been reported, but none of the patients had status epilepticus. The pathophysiology of takotsubo syndrome, although debated, may involve stunning of the myocardium by a catecholamine storm triggered by stress. Patients with ...
Hoffmann Michael - - 2008
BACKGROUND: Right temporal lobe lesion syndrome elicitation presents a clinical challenge. Aside from occasional covert quadrantanopias, heralding elementary neurological deficits are absent. AIM: Isolated right and left temporal lobe stroke patients were analyzed for the panoply of known temporal and frontal cognitive and neuropsychiatric syndromes. METHODS: Temporal lobe stroke patients ...
Winston Ken R - - 2008
A male neonate with seizures, linear sebaceous nevus syndrome and hemimegalencephaly underwent hemispherectomy at 36 weeks' gestational age. He has had no clinical seizures in the 13 months since surgery but continues to have sharp wave activity over some parts of the intact hemisphere. He has moderate developmental delay and ...
Postiglione Alfredo - - 2008
Geschwind described a syndrome (Geschwind syndrome, GS) in patients with temporal lobe epilepsy, characterized by sexual behavioural disorders, hyper-religiosity, hypergraphia and viscosity. In this report we describe a patient affected by fronto-temporal dementia (FTD), who showed all the personality changes of GS without having epilepsy, and suggest that clinicians should ...
Bigler Erin D - - 2008
On the mild end of the acquired brain injury spectrum, the terms concussion and mild traumatic brain injury (mTBI) have been used interchangeably, where persistent post-concussive syndrome (PPCS) has been a label given when symptoms persist for more than three months post-concussion. Whereas a brief history of concussion research is ...
Genton Pierre - - 2008
PURPOSE: Absence status epilepticus (AS) is a prolonged, generalized, and nonconvulsive seizure that may occur in various circumstances. We report a series of patients in whom recurrent, unprovoked, typical AS was the main clinical feature. METHOD: We retrospectively reviewed consecutive patients referred to our epileptic centers, on the following criteria: ...
Hattori Junri - - 2008
PURPOSE: Our aim was to develop a screening test to predict Dravet syndrome before the first birthday based on the clinical characteristics of infants and the SCN1A mutation analysis. METHODS: Ninety-six patients who experienced febrile seizures before the age of one were enrolled. The patients were divided into two groups-the ...
Moriyama Yasushi - - 2007
A 68-year-old woman with schizophrenia after a cerebrovascular accident resulting in right medial temporal and occipital damage developed Frégoli syndrome. Neuropsychological testing revealed that she had impairment in facial recognition compatible with prosopagnosia. The Frégoli syndrome disappeared in three month when, remarkably, her prosopagnosia also disappeared. Our findings are consistent ...
Matsumoto Akiko - - 2007
The subjects were 106 SMIDS with epilepsy. They were classified into four epileptic syndromes: (1) SE-MISF (34.0%), (2) SGE (25.5%), (3) SLRE (20.7%), and (4) epileptic discharge-free patients (EDFP) (19.8%). Clinical electroencephalographic studies elucidated the following result: (1) The seizure disappearance rate was the highest in SLRE (54.5%), and it ...
Gümüş Hakan - - 2007
The combination of axial spasms in clusters, hypsarrhythmia, and psychomotor delay beginning in the first year of life defines West syndrome. Adrenocorticotrophic hormone remains the choice of treatment for many neurologists. Recent controlled studies support vigabatrin as first-line therapy, and open-label studies suggest that topiramate, lamotrigine, and zonisamide may be ...
Carinci Valeria - - 2007
The clinical distinction between cardiovascular and epileptic causes of loss of consciousness is sometimes difficult, but becomes challenging when a primary epileptic seizure secondarily causes an asystole. Epilepsy can be correlated to severe bradycardia or asystole. The syndrome is called the ictal bradycardia syndrome. Ictal bradycardia and asystole have been ...
MacAllister William S - - 2007
Seizure disorders are relatively common in childhood, and the International League Against Epilepsy (ILAE) provides a hierarchical classification system to define seizure types. At the final level of classification, specific epilepsy syndromes are defined that represent a complex of signs and symptoms unique to an epilepsy condition. The present review ...
Crespel Arielle - - 2007
Senile myoclonic epilepsy of Genton is a newer epileptic syndrome of the older patients with Down syndrome associated with an Alzheimer-type dementia. We report two observations in whom the clinical and electroencephalographic features are consistent with the description of this syndrome. Both experienced a progressive deterioration of cognitive functions few ...
Pelc Karine - - 2008
Angelman syndrome is a neurogenetic disorder caused by lack of UBE3A gene expression from the maternally inherited chromosome 15 due to various 15q11-q13 abnormalities. In addition to severe developmental delay, virtual absence of speech, motor impairment, a behavioural phenotype that includes happy demeanor, and distinctive rhythmic electroencephalographic features, over 90% ...
Berhouma Moncef - - 2007
A case of an acute life-threatening presentation of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome requiring an urgent decompressive hemicraniectomy is described. A 9 month-old baby had a status epilepticus following a sustained fever, leading to a comatose state and a right pupillary dilatation associated with a left hemiplegia. The MRI showed a swelling ...
Deng Yu Hong - - 2007
Focal seizures often secondarily generalize but the reverse is much less frequently documented. The idiopathic generalized epilepsies have the EEG signature of generalized, or bilaterally synchronous spike wave activity and although focal features can be seen, seizures are usually generalized once they commence. Although focal seizures can be seen in ...
Chen Jui-Cheng - - 2007
The mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a rare congenital disorder of mitochondrial DNA. Patients with this syndrome may present acute onset of sensorineural hearing loss, which is genetic in origin. An impression of the MELAS syndrome is favored because hearing loss is part of ...
NASIRIAN, A.
Early infantile epileptic encephalopathy or EIEE (Ohtahara syndrome; OS) is a kind of intractable seizure that begins in neonatal age with sudden onset of tonic spasms in series or single suppression-burst S-B in EEG.1 Imaging shows anatomic defects such as migration disorders and generalized atrophy2 with essentially normal metabolic tests. ...
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