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Results 401 - 450 of 891
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Nordli Douglas R DR - - 2005
There are eight syndromes currently recognized by the International League Against Epilepsy (ILAE) that would fit the original operational definition of idiopathic generalized epilepsy (IGE) syndromes, including benign myoclonic epilepsy in infancy; generalized epilepsy with febrile seizures plus, an entity in evolution; epilepsy with myoclonic absences; epilepsy with myoclonic-astatic seizures; ...
Kagitani-Shimono Kuriko - - 2005
PURPOSE: We investigated the evolution of epilepsy, seizure types, and effective drugs in Wolf-Hirschhorn syndrome, which is a malformation syndrome often with refractory seizures and status epilepticus. METHODS: We reviewed 11 cases of Wolf-Hirschhorn syndrome (age range, 2-25 years; SD, 7.2 years) and who were treated in Osaka University or ...
Panayiotopoulos Chrysostomos P - - 2005
This chapter assesses probable epileptic syndromes within the idiopathic generalized epilepsies (IGE) that have not yet been recognized by the International League Against Epilepsy (ILAE). Jeavons syndrome, a purely reflex IGE that predominantly manifests with eyelid myoclonia and electroencephalogram (EEG) abnormalities on eye closure, is the most distinct and undisputed ...
Wolf Peter - - 2005
Early in these proceedings, the origin of the three terms in the title, "idiopathic generalized epilepsy," is discussed with respect to their significance over time, and typical misunderstandings. In the mid-20th century, a rather chaotic use of a multitude of often loosely defined terms had developed, which increasingly became an ...
Jallon Pierre - - 2005
Idiopathic generalized epilepsies (IGEs) are a relatively new category of disorders defined by strict clinical and electroencephalogram (EEG) features proposed by the International League Against Epilepsy (ILAE) classification of epileptic syndromes. IGEs are usually easy to diagnose when clinical and EEG data are collected, but epilepsy is not synonymous with ...
Hirsch E - - 2005
Childhood is a time of considerable importance for the onset of epilepsy syndromes. Selection of an appropriate antiepileptic drug (AED) is central to its successful management. Different AEDs have various effects depending on whether seizures are focal or generalized and this is often used as a rational basis for drug ...
Ferrie Colin D - - 2005
Classification of epileptic seizures and epilepsy syndromes as either focal or generalized is a fundamental and early part in the diagnostic process and is generally fairly easily accomplished. However, in patients with idiopathic generalized epilepsies, seizure and EEG features may suggest, particularly to the unwary, the occurrence of focal rather ...
Boscolo S - - 2005
The Landau-Kleffner syndrome, the continuous spikes-waves during slow sleep syndrome and the benign epilepsy of childhood with rolandic spikes are rare childhood epilepsies with unknown etiology. Improvement in patients treated with immunoglobulin suggests an involvement of the immune system. We provide immunohistochemical evidence of autoantibodies against rat brain auditory cortex, ...
Yagi Kazuichi - - 2004
Zonisamide is a new type of benzisoxazole derivative, first marketed in Japan in 1989. This study analyzed: (1) the drug's efficacy by seizure and epilepsy type in a total of 1008 patients treated during the development of zonisamide in Japan; (2) the effectiveness of zonisamide for 726 newly-diagnosed patients treated ...
Lux Andrew L - - 2004
PURPOSE: To reach a broad consensus on case definitions, outcomes, and outcome measures that will ease future study design and facilitate comparison of data from different studies of infantile spasms and West syndrome. METHODS: Persons who had recently presented or published first-author original research in this field were invited to ...
de la Torre Carlos - - 2004
Gianotti-Crosti syndrome (GCS), or papular acrodermatitis of childhood, is a distinctive self-limiting rash with symmetrically distributed papules on the face, buttocks, and extremities that has been related to several underlying viral diseases. We report a case of a boy exhibiting involutive lesions of milkers' nodules who subsequently developed a rash ...
Verloes Alain - - 2004
We report a female patient with a unique pattern of pre- and postnatal growth deficiency, tetralogy of Fallot, very long eyelashes (trichomegaly), progressive and generalized hypertrichosis lanuginosa, brain atrophy with epilepsy, and puffy hands and feet. This appears to be a "new" entity within the group of syndromal hypertrichoses, possibly ...
Suresh Poovathinal A - - 2004
Congenital suprabulbar palsy is clinically characterized by problems of feeding, swallowing, drooling, and dysarthria. Epilepsy, delayed motor, cognitive, and language development, as well as learning disabilities may co-exist. Aetiology of the syndrome is diverse, yet studies often attribute it to specific entities. We report on nine patients (seven males, two ...
Jain S - - 2004
OBJECTIVES: To investigate the contribution of hereditary factors in the causation of different epilepsy syndromes. MATERIAL AND METHODS: Occurrence of seizures among first- and second-degree relatives of 5628 Indian probands with epilepsy, and 3357 probands with non-epilepsy neurological disorders (who acted as control population) was documented. Syndromic concordance between epilepsy ...
Grosso Salvatore - - 2004
Although epilepsy and mental retardation are commonly observed in individuals with chromosomal aberrations, the identification of EEG/epileptic profiles in those with specific chromosome anomalies remains difficult. A few syndromes seem to show peculiar clinical and EEG associations. The authors report an electroclinical investigation on a group of patients carrying X ...
Ceulemans Berten - - 2004
Severe myoclonic epilepsy in infancy, or Dravet syndrome, is one of the catastrophic epilepsy syndromes. In the past, treatment was mainly based on valproate and phenobarbital. Recently, some of the new antiepilepsy drugs, such as topiramate and stiripentol, have been shown to be promising in the treatment of this epilepsy ...
Vigliano, Piernanda
We report the clinical, electroencephalo-graphic and neuroradiologic findings of a patient with supernumerary der(22) syndrome-partial 22 trisomy, derived from a (11;22) maternal translocation-and a wide spectrum of cerebral malformation. Magnetic resonance study evidenced a partial midline defect (hypoplasia of the corpus callosum, mild dilatation of the mid portion of the ...
Taylor Isabella - - 2004
Although epileptic photosensitivity is well known, its genetics and syndromic associations are incompletely understood. Seizures triggered by photic stimulation are usually a manifestation of the idiopathic generalized epilepsies, especially juvenile myoclonic epilepsy (JME), or of the occipital epilepsies. Idiopathic photosensitive occipital epilepsy (IPOE) is a focal epilepsy with colourful elementary ...
Dunn David W - - 2004
PURPOSE: A severity rating for each of the pediatric epilepsy syndromes can contribute to a more comprehensive understanding of seizure condition severity. We describe the development of the Epilepsy Syndrome Severity Scores-Child (ESSS-C). METHODS: The Delphi Technique was used to establish severity scores for pediatric epileptic syndromes as defined by ...
Wieser Heinz-Gregor - - 2004
Experts discussed the definition, natural history, pathologic features, pathogenesis, electroclinical, neurophysiological, neuropsychological, structural and functional imaging findings, as well as surgical outcome in mesial temporal lobe epilepsy with hippocampal sclerosis (MTLE-HS). After a long-lasting consensus process the ILAE Commission Neurosurgery of epilepsy accepted the resulting conclusions as state-of-the art report ...
Goldstein Joshua L - - 2004
Children who present either to the pediatrician's office or the emergency department with seizures should undergo a careful and detailed history and physical examination. This is done to distinguish between epileptic and nonepileptic events and to characterize among acute symptomatic, remote symptomatic, and idiopathic etiologies. An EEG is obtained both ...
Hommet C - - 2004
Research in brain-damaged patients has suggested that the right hemisphere plays a role in unilateral spatial neglect (USN), but provides only limited information for pinpointing the intraparietal localization of the lesions associated with this syndrome. We report a case of unilateral neglect in a patient who underwent a right inferior ...
Vadlamudi L - - 2004
OBJECTIVE: To classify the Lennox twin pairs according to modern epilepsy classifications, use the classic twin model to identify which epilepsy syndromes have an inherited component, search for evidence of syndrome-specific genes, and compare concordances from Lennox's series with a contemporary Australian series. METHODS: Following review of Lennox's original files ...
Ahnlide J A - - 2004
In the congenital bilateral perisylvian syndrome, pseudobulbar symptoms, cognitive deficits and cortical malformations in the perisylvian region are typical features. We report two initially magnetic resonance imaging (MRI) negative patients from our epilepsy surgery program that shared the same seizure and ictal SPECT characteristics suggesting seizure onset localized in the ...
Ganguly Sutapa - - 2004
Sialidosis type 1 or the cherry red spot-myoclonus syndrome (CRSM) is an autosomal recessive disorder with the onset in adolescence of myoclonus and gradual visual failure. Here, a case of CRSM in a 12-year-old Bengali Muslim girl with the history of myoclonic jerks of limbs and the body since last ...
Somjit Sriudomkajorn - - 2004
We report a 27 year-old man with intellectual disability and no prior history of seizures who presented with episodes of abdominal pain, head/eye version and unresponsiveness that were misdiagnosed and treated as partial seizures. Associated vomiting and haematemesis led to the correct diagnosis and treatment of reflux oesophagitis. The episodes ...
Pardal-Fernández J M - - 2004
OBJECTIVE: To describe a specific electroclinical type of epileptic seizure in a patient with Rett syndrome. PATIENT AND METHODS: We present the case of a girl with Rett syndrome and epilepsy, whose seizures followed two differentiated phases, namely (a) a brief hypertonic onset with tonic-axial predominance, followed by (b) a ...
Rae Caroline - - 2004
Gender differences in brain morphology have previously been reported in the temporal lobe and an 'X-chromosome dosage effect' has been described in Turner syndrome (45,X). To examine this further, we investigated temporal lobe morphology, metabolism and function in nine children with non-mosaic Turner syndrome using magnetic resonance imaging, (1)H magnetic ...
Besag Frank M C - - 2004
Apart from control of the seizures, two of the most important factors in determining how well a child with epilepsy progresses toward independence are cognition and behavior. The diagnosis of the correct epilepsy syndrome often provides information with regard to probability of good seizure control and intellectual outcome. However, relatively ...
Guerrini Renzo - - 2004
Seizures induced by photic (or visual) stimuli or photosensitive seizures can be observed in generalized or focal, idiopathic, or symptomatic epilepsies, in progressive neurodegenerative disorders, and even in the context of situation-related (acute symptomatic) seizures. In addition to this "transversal" presence of the photosensitive trait across various epilepsy types and ...
Klein Andrea - - 2004
Progressive encephalopathy with edema, hypsarrhythmia and optic atrophy (PEHO) syndrome is a rare neurodegenerative syndrome first reported in 1991. Most patients described are of Finnish descent and very few patients have been reported in other countries. We report the first Swiss patient who fulfils the criteria of the PEHO syndrome. ...
Rho Jong M - - 2004
The major catastrophic epileptic syndromes of childhood include infantile spasms, Lennox-Gastaut syndrome, and the progressive myoclonus epilepsies (PMEs). Although each of these syndromes manifests in an age-specific manner and is defined by distinct electroclinical features, they are all refractory to medical therapy and are invariably associated with psychomotor deficits, and ...
Yamashita Yushiro - - 2004
We report a 4-month-old female infant who developed West syndrome eleven days after administration of a histamine H1 antagonist, oxatomide, for atopic dermatitis. It has been reported that some histamine H1 antagonists induce seizures in epileptic patients. The age, the interval between oxatomide administration, and the onset of West syndrome ...
Oksanen Virpi E - - 2004
Kabuki syndrome is a rare dysmorphogenic disorder. The central nervous system is often involved, and epilepsy is a common symptom. The diagnosis is clinical, and no typical electroencephalographic findings have thus far been reported. We have documented temporo-occipital spikes in sleep electroencephalogram in all our three Kabuki patients. The location ...
Camfield Peter - - 2003
BACKGROUND: West syndrome consists of infantile spasms with hypsarrhythmia and is perceived as a disorder of infants. METHODS: We describe 10 patients with West syndrome with spasms that remitted, started again, and persisted (followed up for 8-25 years). RESULTS: In all, West syndrome developed at younger than 17 months (five ...
Sabaz M - - 2003
OBJECTIVE: There is increasing awareness of the importance of assessing physical, psychological, social and behavioural well-being in chronic disease. The aim of this study was to examine the health-related quality of life (HRQoL) of children with common epilepsy syndromes and to explore if there are HRQoL differences between those syndromes. ...
Powell H W R - - 2003
Kabuki syndrome is a dysmorphogenic syndrome which has been reported in over 300 patients since it was first described in Japan in 1981. In addition to its cardinal features (typical facies, mild-to-moderate learning disability, short stature, skeletal anomalies, and dermatoglyphic abnormalities with persistent foetal fingerpads), neurological anomalies are frequently reported, ...
Nabbout Rima - - 2003
Epileptic encephalopathies are conditions in which neurologic deterioration is attributable entirely or partly to epileptic activity. It can be due to very frequent or severe seizures and/or to subcontinuous paroxysmal interictal activity. The former mainly consists of Dravet syndrome, in which patients have seizures from the middle of the first ...
Praline Julien - - 2003
PURPOSE: The aim of this study was to determine the clinical, social, and/or professional and cognitive outcomes in adulthood of the continuous spike-waves during slow sleep (CSWS) and Landau-Kleffner syndromes, which are two rare epileptic syndromes occurring in children. METHODS: We enrolled seven young adults, five who had a CSWS ...
Ohtahara Shunsuke - - 2003
Early infantile epileptic encephalopathy with suppression-burst, or Ohtahara syndrome (OS), and early myoclonic encephalopathy (EME) are epileptic encephalopathies with onset of frequent seizures in the neonatal and early infancy period and with a characteristic EEG pattern, namely, suppression-burst, in which higher-voltage bursts of slow waves mixed with multifocal spikes alternate ...
Markand Omkar N - - 2003
Lennox-Gastaut syndrome (LGS) is a childhood epileptic encephalopathy characterized by an electroclinical triad of generalized slow spike wave (SSW) activity in the EEG, multiple types of epileptic seizures, and slow mental development. It is usually subdivided into symptomatic and cryptogenic types, the latter accounting for at least one fourth of ...
Guerrini Renzo - - 2003
Myoclonic attacks are not characteristic of a specific syndrome. In infancy and early childhood, they are often observed in the context of syndromes that are associated with other types of seizures and with cognitive impairment but no obvious brain lesion. Characterization of the associated seizures and age of expression allows ...
Mewasingh L D - - 2003
In children, non-convulsive status epilepticus (NCSE) is rare and difficult to treat. Response to steroids and GABAergic medication is variable and often decreases with increasing duration of NCSE. We present our experience with oral ketamine, an NMDA-receptor antagonist, administered to five children with severe epilepsy (Lennox-Gastaut Syndrome, myoclonic-astatic epilepsy, progressive ...
Kossoff Eric H - - 2003
A 5-year-old girl with Landau-Kleffner syndrome is discussed. The child began having seizures at age 4 associated with language deterioration despite anticonvulsant therapy. With levetiracetam monotherapy to a dose of 60 mg/kg/day and discontinuation of carbamazepine and valproic acid, her language has improved and seizures are controlled. Levetiracetam should be ...
Cavazzuti G B - - 2003
Many cases of drug-resistant infantile epilepsy (West syndrome, Lennox-Gastaut syndrome, Landau-Kleffner syndrome, severe myoclonic epilepsy, and Rasmussen's encephalitis) were found to respond in some measure to treatments with intravenous immunoglobulins (IVIG), but relapses were common. However, as the side effects of IVIG infusion are insignificant, it is better not to ...
Rosser Tena - - 2003
The astute observations of Aicardi and colleagues led to the first description of Aicardi syndrome as a triad of infantile spasms, absence of the corpus callosum, and chorioretinal lacunae. Still diagnosed clinically, we now recognize an expanded version of this probable X-linked dominant disorder that predominantly affects females. In addition ...
Jacobs Dina A - - 2003
Although seizures can be a manifestation of paraneoplastic disorders, there are few descriptions of the association between the anti-Hu paraneoplastic syndrome and epilepsia partialis continua. A new case of refractory complex partial status epilepticus in a patient with a paraneoplastic syndrome associated with a poorly differentiated mediastinal tumor that expressed ...
Stone Jon - - 2003
Parry-Romberg syndrome (PRS) is a rare neurocutaneous disorder characterized by progressive facial hemiatrophy. In this study, 205 patients with PRS were surveyed using the Internet. Estimates of the frequency of limb involvement (19%), epilepsy (11%), and other clinical and etiologic features were obtained. There was a wide range of age ...
Maher Cormac O - - 2003
Linear sebaceous nevus syndrome (LSNS) is a rare neurocutaneous syndrome that is associated with seizures and mental retardation. The cortical abnormalities that are associated with this syndrome include focal cortical dysplasia, partial hemimegalencephaly and holohemispheric hemimegalencephaly. Few reports have addressed the utility of cortical resections for epilepsy treatment in the ...
Shah J R - - 2003
Parry-Romberg syndrome is a rare disorder associated with unilateral facial atrophy involving skin, subcutaneous tissue, skeletal muscle, and bone. Occasionally, there is CNS involvement with epilepsy being the most common CNS manifestation. The authors report a child with Parry-Romberg syndrome with a course strongly suggestive of Rasmussen encephalitis. The boy ...
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