Search Results
Results 301 - 350 of 863
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Olmez Akgün - - 2006
Cyclic vomiting syndrome is a disorder characterized by recurrent attacks of vomiting and intervals of normal health between vomiting episodes averaging 2-4 weeks. It has been described by a variety of names such as abdominal migraine, abdominal epilepsy, and periodic syndrome but now has been classified in the subgroup of ...
Finsterer J - - 2006
The central nervous system (CNS) is, after the peripheral nervous system, the second most frequently affected organ in mitochondrial disorders (MCDs). CNS involvement in MCDs is clinically heterogeneous, manifesting as epilepsy, stroke-like episodes, migraine, ataxia, spasticity, extrapyramidal abnormalities, bulbar dysfunction, psychiatric abnormalities, neuropsychological deficits, or hypophysial abnormalities. CNS involvement is ...
Bachiocco Valeria - - 2006
BACKGROUND: The aim of this investigation was to verify whether brain hypoxia represented a risk factor for the occurrence and severity of opioid abstinence syndrome. METHODS: Three newborns who manifested seizure activity as a result of hypoxia, focal brain ischemia, and hypoxia and sepsis, respectively, were compared with 17 neonates ...
Engel Jerome J - - 2006
A Core Group of the Task Force on Classification and Terminology has evaluated the lists of epileptic seizure types and epilepsy syndromes approved by the General Assembly in Buenos Aires in 2001, and considered possible alternative systems of classification. No new classification has as yet been proposed. Because the 1981 ...
Keränen Tapani - - 2006
The neurogenic muscle weakness, ataxia and retinitis pigmentosa (NARP) syndrome is a maternally inherited disorder attributable to a heteroplasmic mtDNA point mutation. Catastrophic epilepsy may accompany severe, early onset forms of NARP, but seizures seem to be rare in cases with adolescent and adult onset. We describe a patient who ...
Di Bonaventura Carlo - - 2006
Fragile X syndrome (Fra-X) is a common cause of mental retardation that can be associated with partial epilepsy characterized by a variety of electro-clinical features. A wide spectrum of interictal activities are reported, although no data regarding ictal EEG activity have as yet been published. Drug-resistant seizures are uncommon, and ...
Chahine Lama M - - 2006
There is currently increasing interest in identifying and classifying pediatric benign epilepsy syndromes and recently several new syndromes have been recognized. Benign epilepsy syndromes, by definition, occur in children with normal developmental history, respond well to therapy, and remit without sequelae. The large majority of children with benign epilepsy syndromes ...
Kröll-Seger Judith - - 2006
We report on a 3-year-old boy with myoclonic-astatic epilepsy who developed myoclonic status epilepticus with continuous twitching of the face and unresponsiveness under monotherapy with levetiracetam. Recently, a nonconvulsive status epilepticus in an adult epilepsy patient has also been described. Our observation points to the possibility of a causal relationship ...
Covanis Athanasios - - 2006
Panayiotopoulos syndrome is a common idiopathic childhood-specific seizure disorder formally recognized by the International League Against Epilepsy. An expert consensus has defined Panayiotopoulos syndrome as "a benign age-related focal seizure disorder occurring in early and mid-childhood. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms, and by an ...
Zuberi Sameer M - - 2006
Benign myoclonic epilepsy in infancy is a rare syndrome with just over 100 cases reported since the first syndromic description by Dravet and Bureau [Dravet, C., Bureau, M., 1981. The benign myoclonic epilepsy of infancy. Rev. Elecroencephalogr. Neurophysiol. Clin. 11, 438-444]. This includes 23 infants with reflex myoclonic epilepsy whose ...
Kang Hoon-Chul - - 2006
Landau-Kleffner syndrome is characterized by epileptic aphasia associated with electrical status epilepticus of slow wave sleep. A 5-year-old female, who had manifested normal developmental progress, was referred with principal complaints of fluctuating sensory aphasia and bizarre behavior during the preceding 4 months. Landau-Kleffner syndrome was confirmed by clinical and electroencephalographic ...
Kwan Shang-Yeong - - 2006
PURPOSE: This retrospective study was designed to clarify the role of West syndrome in post-callosotomy seizure outcome in patients with Lennox-Gastaut syndrome. METHODS: From September 1989 to May 1999, 74 patients diagnosed with Lennox-Gastaut syndrome received anterior corpus callosotomy at Taipei Veterans General Hospital, Taiwan. All patients were followed for ...
Hong Seung Bong - - 2006
A 22 year-old woman suffered from recurrent episodes of hypersomnia, apathy, and hyperphagia. The symptoms occurred 3 to 4 times per year, and each attack lasted 2 to 3 weeks. 99mTc-ethylcysteinate dimer brain single photon emission computed tomography (SPECT) was performed during symptomatic and asymptomatic periods. To localize brain regions ...
Boylan L S - - 2006
The occurrence of an aura is often considered evidence of a partial rather than an idiopathic generalized epilepsy syndrome. The authors examined this hypothesis by prospectively recording reports of auras by patients being admitted for video-EEG monitoring. Auras were equally common (70%) among patients with idiopathic generalized epilepsy as they ...
Ohtahara Shunsuke - - 2006
Ohtahara syndrome (OS) is well known as a peculiar early onset epileptic syndrome with serious prognosis. The outline of OS, mainly in relation to the evolution with age, and differentiation from related conditions, particularly early myoclonic encephalopathy (EME) were mentioned. Etiologically, structural brain lesions are most probable in OS, and ...
Yamatogi Yasuko - - 2006
Markand and Blume first realized the specificity of the EEG pattern of multiple independent spike foci (MISF) in the late 1970s; its close relation with hypsarrhythmia and slow spike-waves, extensive bilateral brain lesion, and intractable seizures. But they did not recognize it as an epileptic syndrome. On the other hand, ...
Seino Masakazu - - 2006
Care must be exercised not to intermingle with classification of seizures and classification of epilepsies in an inconsistent fashion. Criteria for each class must be defined as clearly as possible, and these criteria must be those which are necessary for classifying any given case. The international classification first proposed by ...
Djukic Aleksandra - - 2006
Early myoclonic encephalopathy (EME) and the Ohtahara syndrome are currently listed as two separate syndromes in the classification of epilepsies. The most prominent differentiating points are the observations that patients with Ohtahara syndrome experience predominantly tonic seizures; their seizures evolve to infantile spasms and the prognosis is often worse than ...
Engel Jerome J - - 2006
The efforts of the International League against Epilepsy (ILAE) to devise classifications of the epilepsies has greatly improved communication among epileptologists and influenced both basic and clinical research. Several classifications have been proposed since 1970; the most recent classification of epilepsy syndromes and epilepsies was published in 1989. Since 1997, ...
Fernández-Torre José L JL Department of Clinical Neurophysiology, University Hospital Marques de Valdecilla, Santander, Cantabria, Spain. - - 2006
Subacute encephalopathy with seizures in alcoholics (SESA syndrome) is a rare neurologic disorder complicating the chronic abuse of alcohol. This acute or subacute condition is characterized by transient neurologic deficits, seizures and severe lateralized abnormalities on the electroencephalogram (EEG). Our case demonstrates that in SESA syndrome, convulsive (generalized tonic-clonic) and ...
Destina Yalçin A - - 2006
Eyelid myoclonia with absences (EMA) and juvenile myoclonic epilepsy (JME) are two separate epileptic syndromes included in the new classification of epilepsies and epileptic syndromes by ILAE in 2001. Both are idiopathic generalized epilepsies with their clinical onset in the first two decades. EMA is characterized by eyelid myoclonia associated ...
Van Bogaert P - - 2006
The authors propose to define the epileptic syndromes with continuous spikes and waves during slow sleep (CSWS) as a cognitive or behavioral impairment acquired during childhood, associated with a strong activation of the interictal epileptiform discharges during NREM sleep--whatever focal or generalized--and not related to another factor than the presence ...
Lee Céline - - 2006
L-2-hydroxyglutaric aciduria was diagnosed in a 9-month-old female infant after a complex febrile convulsion with subsequent transient left-sided hemiplegia. The symptoms were consistent with acute hemiconvulsion-hemiplegia-epilepsy syndrome. Magnetic resonance imaging (MRI) of the brain revealed distinct white-matter abnormalities in the bifrontal and bioccipital periventricular area and increased signal intensity in ...
Nechay Alla - - 2006
We describe epileptic seizures including status epilepticus provoked by recurrent obstructive apnea in a child with Cornelia de Lange syndrome. From the age of 10 months, this boy had recurrent respiratory infections with obstructive apnea leading to cyanosis and loss of consciousness. Approximately, 25% of apneas were followed by clonic ...
Murphy Declan G M DG Institute of Psychiatry, Department of Psychological Medicine, London, UK. - - 2006
The cause of autistic spectrum disorder (i.e., autism and Asperger's syndrome) is unknown. The serotonergic (5-HT) system may be especially implicated. However, cortical 5-HT2A receptor density in adults with the disorder has not been examined, to the authors' knowledge. The authors investigated cortical 5-HT2A receptor binding in eight adults with ...
Nieuwenhuis Lotte - - 2006
Epilepsy with continuous spike- and -waves during slow-wave sleep and Landau-Kleffner syndrome are two rare childhood epilepsy syndromes. The underlying pathophysiology remains unknown. The current opinions about epidemiologic risk factors, genetic predisposition, EEG and MEG findings, influence of anti-epileptic drugs, neuroradiology, positron emission tomopgraphy, evoked potentials, auto-immunity and subpial transection ...
Korff Christian M - - 2006
An increasing number of infantile epilepsy syndromes have been recognized. However, a significant number of infants (children aged 1-24 months) do not fit in any of the currently used subcategories. This article reviews the clinical presentation, electroencephalographic findings, evolution, and management of the following entities: early infantile epileptic encephalopathy, early ...
van Rijckevorsel K - - 2006
Neurocognitive impairment is frequent in epilepsy patients. Causes are multiple, and may be influenced by several factors including the epilepsy syndrome. Most cognitive complaints in adult patients are mental slowness, memory difficulties and attention deficits. In children, cognitive problems are more diffuse, responsible for language troubles, learning difficulties, poor academic ...
Chen Horng-An - - 2006
Middle lobe syndrome - recurrent atelectasis and/or bronchiectasis involving the right middle lobe and/or lingula - has, up to now, not been reported as the pulmonary manifestation of primary Sjögren's syndrome. We describe a patient in whom lymphocytic bronchiolitis in the atelectatic lobes was proved histologically from two separate transbronchial ...
Kikumoto Kenichi - - 2006
We report on a 4-year 8-month-old boy with Panayiotopoulos syndrome who showed atypical evolution with newly developed absence seizures and EEG exacerbation induced by carbamazepine. Soon after the introduction of carbamazepine, EEGs began to worsen, and finally absence seizures and myoclonic seizures appeared. Immediately after we discontinued carbamazepine, the seizures ...
Ferrie Colin - - 2006
The aim of this paper is to promote the correct classification of, and provide guidelines on, the diagnosis and management of Panayiotopoulos syndrome (PS). An international consortium of established researchers in the field collaborated to produce a consensus document. The resulting document defines PS, characterizes its electro-clinical features, considers its ...
Corda Davide - - 2006
Patients with the syndrome of mitochondrial encephalopathy, lactic acidosis, and strokelike episodes (MELAS) may rarely present with complex partial status epilepticus (CPSE) at clinical onset. We report on a 38-year-old woman with MELAS presenting with multifocal CPSE and periodic lateralized epileptiform discharges (PLEDs) on EEG during her first strokelike episode. ...
Goldman Alica M - - 2006
Smith-Magenis syndrome is a multiple congenital anomalies/mental retardation syndrome associated with a heterozygous deletion of chromosome 17p11.2. Seizures have not been formally studied in this population. Our objectives were to estimate the prevalence of seizures and electroencephalographic (EEG) epileptiform abnormalities in patients with Smith-Magenis syndrome with defined chromosomal rearrangements and ...
Sevgi Demirci Eser Basak - - 2006
"Eyelid myoclonia with and without absences" has been incorporated into the new ILAE diagnostic scheme as a type of epileptic seizure with etiologic, therapeutic, and prognostic implications. Eyelid myoclonia with absences (EMA) is characterized by eyelid myoclonia and absences provoked mainly by eye closure and photosensivity. EMA can be a ...
Jacob Saiju - - 2006
Juvenile myoclonic epilepsy constitutes 5-10% of idiopathic generalised epileptic syndromes. The clinical triad of absence seizures, myoclonic jerks and generalised tonic clonic seizures (GTCS) rarely begin after the second decade of life. We present the case of a 74-year-old lady presenting for the first time with myoclonic jerks and absences. ...
Besag Frank M C - - 2006
The educational and social progress of a child with epilepsy depends not only on seizure control but also on cognitive and behavioral factors. The various epilepsy syndromes of childhood and adolescence differ greatly in terms of cognitive and behavioral outcome. A high proportion of babies who have West syndrome and ...
Velez Alberto - - 2006
PURPOSE: A national study was performed in Colombia to determine the general and regional prevalence of epilepsy, clinical profiles, seizure types, and clinical syndromes. METHODS: Based on the National Epidemiological Study of Neurological Diseases (EPINEURO), we evaluated and followed up for 1 year all the subjects with epilepsy from the ...
Stephani Ulrich - - 2006
The purpose of this article is to present a short review of the natural history of myoclonic astatic epilepsy (MAE; Doose syndrome) and the Lennox-Gastaut syndrome (LGS). In the 1989 classification of the International League Against Epilepsy (ILAE, 1989), MAE and LGS were initially included in group 2.2: "Cryptogenic or ...
Welty Timothy E - - 2006
Juvenile myoclonic epilepsy (JME) is a common epilepsy syndrome that begins most frequently in the early teenage years. It is officially classified as a type of idiopathic generalized epilepsy and is often under-recognized or misdiagnosed. This syndrome has a strong genetic component with multiple gene mutations being associated with the ...
Gobbi Giuseppe - - 2006
Benign childhood epilepsy with centrotemporal spikes (BCECTS) is an idiopathic focal epilepsy syndrome, the benign characteristics of which are debated; in fact, stable or intermittent specific neuropsychological deficits are frequent and may affect scholastic or adaptive abilities. Cognitive dysfunction may be related to a marked increment of the interictal epileptic ...
Auvichayapat Narong - - 2006
Kluver-Bucy syndrome is a rare neurobehavioral condition characterized by visual agnosia, excessive oral tendencies, hypermetamorphosis, placidity, altered sexual behavior, and changes in dietary habits. This description of a 14-year-old boy presenting with complete Kluver-Bucy syndrome after Mycoplasma pneumoniae bronchitis is the first such case report. MRI revealed left temporal horn ...
Carney Patrick - - 2005
BACKGROUND: Understanding the common childhood epilepsy syndromes is valuable when approaching the diagnosis and management of a child presenting with seizures. OBJECTIVE: This article discusses the common epilepsy syndromes in children and provides a guide to appropriate investigation and management of these syndromes. DISCUSSION: A careful history and examination, supported ...
Amâncio Edson José - - 2005
Stendhal's syndrome occurs among travelers when they encounter a work of art of great beauty. It is characterized by an altered perception of reality, emotional disturbances, and crises of panic and anxiety with somatization. The patient profile described originally for this syndrome was of particularly sensitive individuals who were admirers ...
Bahi-Buisson Nadia - - 2005
Hemiconvulsions-hemiplegia-epilepsy syndrome (HH/HHE) is a rare epileptic syndrome consisting of a prolonged unilateral convulsion producing a persisting hemiplegia, sometimes followed by epilepsy. We report on a 13-month-old male who presented with febrile left-sided HH syndrome with right hemispheric unilateral cytotoxic oedema followed by hemispheric atrophy on magnetic resonance imaging (MRI). ...
Parisi Pasquale - - 2005
OBJECTIVE: To describe the ictal polysomnographic features of a patient with Panayiotopoulos syndrome, a peculiar epileptic syndrome characterized by infrequent, often single, prolonged, nocturnal, focal seizures comprising an unusual constellation of autonomic symptoms (malaise, nausea, pallor, tachycardia, vomiting) and unilateral deviation of the eyes at the onset of seizures. These ...
Vanagt W Y - - 2005
A girl with Angelman syndrome had recurrent episodes of ventricular asystole and syncope caused by severe vagal hypertonia during outbursts of laughing. After intravenous administration of atropine, laughing no longer induced asystole or syncope. The vast majority of patients with Angelman syndrome have seizures. Since hypoxia associated with asystole can ...
Paprocka Justyna - - 2006
INTRODUCTION: Parry-Romberg syndrome (progressive facial hemiatrophy) is a unilateral, slowly progressive atrophy affecting the skin, subcutaneous tissues, muscles, and bones. The relationship between Parry-Romberg syndrome and connective tissue disorders, especially scleroderma en coup de sabre, is still unclear. The neurological symptoms, including epilepsy, migraine, and brain lesion, on neuroimaging may ...
Olafsson Elias - - 2005
BACKGROUND: No population-based incidence studies of epilepsy have studied syndrome classification from the outset. We prospectively studied the incidence of a single unprovoked seizure and epilepsy in the population of Iceland, and applied the syndrome classification endorsed by the International League Against Epilepsy to this population. METHODS: We used a ...
Kurca Egon - - 2005
Allgrove syndrome is a rare autosomal recessive disease with achalasia, alacrima, adrenocortical insufficiency, autonomic neuropathy and other neurological disturbances. A case of two brothers with Addison s disease from early childhood is presented. The younger brother with Addison disease died at the age of 5. The older brother was treated ...
Vigre Håkan - - 2005
The clinical syndrome Postweaning Multisystemic Wasting Syndrome (PMWS) in pigs has emerged globally during the last decade. In October 2001, the first pig herd diagnosed with PMWS was reported in Denmark, and since then the number of herds diagnosed with PMWS has increased markedly. The etiology of PMWS is not ...
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