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Results 201 - 250 of 891
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Baldini Mariella - - 2010
Posterior reversible encephalopathy syndrome (PRES) is known to occur after solid organ transplantation, and is caused by immunosuppressive agents such as tacrolimus. PRES onset usually occurs within the first 2months after liver transplantation. Clinical findings include seizures, headache, focal neurological deficits, visual disturbances, and altered mental status. These are associated ...
Karagol Belma Saygili - - 2010
Hypoxic-ischemic cerebral injury that occurs during the perinatal period is one of the most commonly recognized cause of long-term neurological deficit in children, often referred to as cerebral palsy. We describe a case with capillary leak syndrome and seizures to co-morbid status epilepticus related to perinatal hypoxic-ischemic encephalopathy in newborn ...
Doherty Michael J - - 2010
Mechanisms of sudden unexpected death in epilepsy (SUDEP) are incompletely understood in part because the overwhelming majority of patients are not undergoing video telemetry when they die. Described here is a patient with Tourette syndrome and epilepsy who displayed a dangerous compulsive tic resulting in carotid occlusions and seizures as ...
Zhang Hong-Liang - - 2010
Kabicek and colleagues described a case of nephritic-syndrome-associated posterior reversible encephalopathy syndrome (PRES) (Kabicek, et al. 2010). This adds to the accumulating evidence that PRES can be associated with disorders other than hypertension. However, we wonder how the authors would explain the neuroimaging findings unsuggestive of vasogenic oedema. PRES (also ...
Wheless James W - - 2010
The last 20 years have witnessed a tremendous explosion in the number of antiepileptic drugs (AEDs) as well as the introduction of AEDS developed for specific epilepsy syndromes. The study of the efficacy and side effect profile of AEDs for unique epilepsy syndromes has allowed neurologists to utilize evidence-based medicine ...
Kabicek Pavel - - 2010
We thank Drs. Zhang, Yang and Wu for their comments regarding our recent paper describing the case report of a 5-year-old patient with clinically and MRI diagnosed posterior reversible encephalopathy syndrome (PRES), alternatively referred as the reversible posterior leukoencephalopathy syndrome (Kabicek et al. 2010). On MRI this syndrome typically manifests ...
Haberlandt Edda - - 2010
For treatment of intractable epilepsies, there are no data comparing conventional adrenocorticotropic hormone and pulsatile corticoid therapy with dexamethasone. A retrospective comparison of efficacy was therefore conducted for both forms of application. Between 1989 and 2001, a series of 11 children with West syndrome and 3 with Lennox-Gastaut syndrome were ...
Gresham Jessica - - 2010
Lennox-Gastaut syndrome (LGS) is a rare but debilitating pediatric epileptic encephalopathy characterized by multiple intractable seizure types. Treatment of LGS is challenging because of the small number of antiepileptic drugs (AEDs) which are effective for this syndrome, as well as the need for polytherapy in the majority of patients. This ...
Westmark Cara J CJ Department of Pathology & Laboratory Medicine and Waisman Center for Developmental Disabilities, University of Wisconsin, Madison, WI 53705, USA. - - 2010
Amyloid-beta protein precursor (AbetaPP) is overexpressed in Alzheimer's disease (AD), Down syndrome (DS), autism, and fragile X syndrome. Seizures are a common phenotype in all of these neurological disorders, yet the underlying molecular mechanism(s) of seizure induction and propagation remain largely unknown. We demonstrate that AD (Tg2576) and DS (Ts65Dn) ...
Hsu Yu-Chuan - - 2012
Background: Mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome is a rare mitochondrial disorder with a wide range of multisystemic symptoms. Epileptic seizures are common features of both MELAS and meningoencephalitis and are typically treated with anticonvulsants. Objectives: To provide the reader with a better understanding of MELAS ...
Tsai Pei-Lin - - 2009
The chromosome 22q11 deletion syndrome, which is synonymous with DiGeorge syndrome, is a congenital anomaly characterized by abnormal facies, congenital heart defects, hypoparathyroidism with hypocalcemia, and immunodeficiency. Neurological manifestations of the chromosome 22q11 deletion syndrome are variable, and include mental deficiency, speech disturbances, learning difficulties, attention deficit hyperactivity disorder, and ...
Blaivas Allen J - - 2009
Middle lobe syndrome is a term that refers to a recurrent collapse of a lung segment, typically the right middle lobe. We discuss a case that middle lobe syndrome occurred in an unusual lung segment and related to an unusual cause.
Devinsky Julie - - 2010
A 51-year-old right-handed man developed hypersexuality after a second right temporal lobectomy to treat epilepsy. His hypersexuality started with increased marital intercourse and masturbation but he later downloaded child pornography. Hyperphagia and distractibility, other features of the Kluver-Bucy syndrome, also developed. Resection of the amygdala and/or temporal lobe neocortical areas ...
Andrade Danielle M - - 2010
Dravet syndrome is a genetically determined severe epilepsy associated with cognitive decline and ataxia. The many types of seizures seen in these patients are typically pharmacoresistant. Here we describe two adults with Dravet syndrome who were treated with thalamic deep brain stimulation (DBS) and followed for 10 years. One patient ...
Dan Bernard - - 2009
Angelman syndrome is a neurogenetic disorder characterized by developmental delay, severe intellectual disability, absent speech, exuberant behavior with happy demeanor, motor impairment, and epilepsy, due to deficient UBE3A gene expression that may be caused by various abnormalities of chromosome 15. Recent findings in animal models demonstrated altered dendritic spine formation ...
Alvarez-Silva Iria - - 2010
An analysis is carried out of a set of psychic phenomena appearing always in the same way: an experience suddenly invades the consciousness, unfolding automatically and with great intensity. This psychic automatism, of which the patient is a passive observer, is accompanied by an overwhelming feeling of strangeness. Our hypothesis ...
Wakamoto Hiroyuki - - 2010
Although hypothalamic hamartomas (HHs) have been shown to be intrinsically epileptogenic and to participate in the generation of gelastic seizures, no evidence has been reported regarding its contribution to the pathogenesis of infantile spasms. We describe a male infant with Pallister-Hall syndrome who had a large HH presenting with infantile ...
Cazorla María Rosario - - 2010
The authors report the case of an infant suffered from early infantile epileptic encephalopathy with suppression-burst, or Ohtahara syndrome, a severe form of epilepsy in early childhood. The patient was treated with vigabatrin causing a favourable response. This unusual outcome may be related with the normality of neuroimaging and metabolic ...
Cerminara Caterina - - 2010
Pallister-Killian syndrome is a rare syndrome of multiple congenital anomalies attributable to the presence of a mosaic supernumerary isochromosome (12p). Although the clinical manifestations of Pallister-Killian syndrome are variable, the most common anomalies include craniofacial dysmorphisms, limb deformities, progressive psychomotor development delay, severe hypotonia, and epilepsy. Standard karyotype is nearly ...
Hammers A A MRC Clinical Sciences Centre, Imperial College London, South Kensington, Hammersmith Hospital, United Kingdom. - - 2009
There is no one ligand for visualising "the epileptogenic zone". Several PET ligands, however, can help by noninvasively or minimally invasively refining hypotheses regarding its location. Their relative merits depend not only on local availability and expertise, but also on epilepsy syndrome and overall diagnostic category. FDG, flumazenil (FMZ), alpha-methyl-tryptophan ...
Kasper B S - - 2009
Psychic and psychotic symptoms can be part of seizure-related symptoms, especially within the postictal phase in partial epilepsies. Among the classic examples are dysmnestic phenomena, visual and acoustic hallucinations, and more rarely delusional syndromes. Here we report about the unique seizure symptom of transformation towards the opposite gender in a ...
Maillard Louis - - 2010
Idiopathic partial reading epilepsy (RE) is a rare syndrome. We report the clinical and electroencephalographic characteristics of two right-handed patients with the following: reading-induced independent bilateral temporal lobe seizures, accompanied by alexia in left (dominant) sided seizures recorded on video-EEG (electroencephalography); subclinical activation over left posterior temporal and occipital electrodes ...
Caporale Christina M - - 2009
A 57-year-old man, operated eight years before for a left frontal falx meningioma, presented with short lasting, stereotyped episodes of paresthesias ascending from the right foot to the hand. A diagnosis of somatosensory seizures with jacksonian march was made. The patient was given antiepilectics but 5 days later, a few ...
Forrest Katharine M L - - 2009
Angelman syndrome is often associated with an intractable seizure disorder. We describe 4 children who demonstrated an excellent response to corticosteroid therapy. The benefits included not only reduction in clinical seizures but also modification of the "typical'' Angelman electroencephalogram. In addition, there was improvement in the myoclonic jerks, sleep pattern, ...
Zupanc Mary L - - 2009
The developing brain is particularly susceptible to seizures. Diffuse central nervous system pathology or injury in early infancy, when the brain is most vulnerable, may lead to catastrophic epilepsies such as Ohtahara's epileptic encephalopathy and early myoclonic epileptic encephalopathy. These epileptic encephalopathies are difficult to treat and have poor prognoses. ...
Wheless James W - - 2009
Managing severe epilepsy syndromes of early childhood is challenging as the seizures are typically resistant to treatment and may cause disabling mental and behavioral problems in later life. A comprehensive treatment plan includes pharmacologic, nonpharmacologic, and surgical options. This article reviews clinical studies examining the efficacies of antiepileptic medications in ...
Gerschlager Willi W Krankenhaus Hietzing mit Neurologischem Zentrum Rosenhügel, Vienna, - - 2009
This review examines recent developments in the field of myoclonus. The range of clinical features in myoclonic dystonia has been extended and its underlying pathophysiology better defined. The diverse causes leading to jerky tremor and orthostatic myoclonus have been clarified and the need to consider drugs as potential causes highlighted. ...
Andrade Danielle M - - 2009
BACKGROUND: Myoclonus is a brief shock-like movement that has many different etiologies. The degree to which it disturbs quality of life is extremely variable, as is its response to treatment. OBJECTIVE: In this review, we focus on the treatment strategies for epileptic myoclonus in some common disorders, and in others ...
Costello Daniel J - - 2009
BACKGROUND: The progressive myoclonic epilepsies (PMEs) are a disparate group of syndromes whose common features include disabling myoclonus, progressive cognitive decline, and seizures, typically with a relentless deterioration over time. OBJECTIVE: To report a novel PME syndrome. DESIGN: Case report. SETTING: Epilepsy service in a tertiary care urban medical center. ...
El Hajj Taghrid - - 2009
We describe a patient with Kleine-Levin syndrome who was initially misdiagnosed as having epilepsy and who achieved complete remission on carbamazepine treatment. A drug effect was established when symptoms recurred after carbamazepine taper and disappeared after reintroduction of the drug. Carbamazepine, a safer drug than lithium, can be a highly ...
Kuchukhidze Giorgi - - 2009
Abnormalities of deep cerebellar nuclei in Joubert syndrome have been previously reported only in rare autopsy cases. Epilepsy in association with Joubert syndrome is also rarely reported. In two new cases of patients with Joubert syndrome, bilateral hypoplasia of deep cerebellar nuclei was detected in vivo by magnetic resonance imaging. ...
Steinlein Ortrud K - - 2009
The combination of aphasia and epileptic seizures is characteristic for Landau-Kleffner syndrome and childhood disintegrative disorder. These disorders affect young children with previously normal psychomotor development, causing profound regression with loss of receptive and expressive language capabilities. The etiologies of these childhood epilepsy-aphasia syndromes are largely unknown, and the long-term ...
Hirano Yoshiko - - 2009
Prognostic factors for frequent seizure recurrences were studied in patients with Panayiotopoulos syndrome. The subjects were 79 children fulfilling the criteria of Panayiotopoulos syndrome who were monitored for longer than 2 years. Medical records and electroencephalograms were analyzed retrospectively. The total number of seizures in each patient at the final ...
Korff Christian M - - 2009
Benign myoclonic epilepsy of infancy is recognized as a generalized and idiopathic epilepsy by the International League Against Epilepsy. Unprovoked and reflex seizures have been reported in these patients. We describe a child diagnosed with benign myoclonic epilepsy of infancy, whose strictly unilateral and localized reflex myoclonias broaden the clinical ...
Saadeldin Imad Yassin - - 2010
PURPOSE: Benign infantile seizures [BIS], familial and non-familial, represent a benign, age-related idiopathic syndrome of infancy. The aim of the current paper is to document the presence of the syndrome in Saudi Arabia and in Arab populations and to discuss the characteristic electroclinical features and the benign nature of this ...
Thibert Ronald L - - 2009
Angelman syndrome (AS) commonly presents with epilepsy (>80%). The goal of this study was to examine the natural history and various treatments of epilepsy in AS in a large population. A detailed electronic survey containing comprehensive questions regarding epilepsy in AS was conducted through the Angelman Syndrome Foundation. There were ...
Coppola Giangennaro - - 2009
The syndrome of malignant migrating partial seizures in infancy was first reported in 1995, and is now included among the childhood epilepsy syndromes in development in the proposal of the revision of the International League Against Epilepsy (ILAE) classification of the epilepsies and epilepsy syndromes. The main clinical features are ...
Striano Salvatore - - 2009
Hypothalamic hamartoma (HH) is the pathologic hallmark of a spectrum of epileptic conditions, ranging from a mild form of epilepsy, whose seizures are an urge to laugh without cognitive defects, to the fully developed syndrome of early onset gelastic seizures (GS) associated with precocious puberty and the evolution to a ...
Capovilla Giuseppe - - 2009
In its first description (1989), Panayiotopoulos syndrome was defined as an idiopathic epilepsy syndrome with an excellent prognosis, characterized by a clinical ictal triad of nocturnal seizures, tonic deviation of the eyes, and vomiting. The electroencephalographic and clinical features of this condition were highly suggestive of occipital lobe involvement. Subsequently, ...
Striano Pasquale - - 2009
Work on the classification of epileptic syndromes is ongoing, and many syndromes are still under discussion. In particular, special difficulty still persists in correctly classifying epilepsies with myoclonic seizures. The existence of special familial epileptic syndromes primarily showing myoclonic features has been recently suggested on the basis of a clear ...
Elia Maurizio - - 2009
Myoclonic status in nonprogressive encephalopathies (MSNE) is an epileptic syndrome in development, characterized by the early onset of continuous diffuse epileptiform abnormalities, accompanied by positive and/or negative phenomena correlated with transient and recurring motor, cognitive, or behavioral disturbances. Outcome of MSNE is poor and may determine progressive neurologic deterioration. MSNE ...
Gambardella Antonio - - 2009
Familial mesial temporal lobe epilepsy (FMTLE) was first described as a benign syndrome with prominent psychic and autonomic seizures and no association with hippocampal sclerosis (HS) or febrile seizures (FS). Better definition of the syndrome allowed identification of more heterogeneous phenotypes with mild to severe epileptic disorders, and a variable ...
Stafstrom Carl E - - 2009
Infantile spasms is a developmental epilepsy syndrome with unique clinical and EEG features, a specific pattern of pharmacological responsiveness, and poor outcome in terms of cognition and epilepsy. Despite the devastating nature of infantile spasms, little is known about its pathogenesis. Until recently, there has been no animal model available ...
Striano Salvatore - - 2009
Eyelid myoclonia with absences (EMA), or Jeavons syndrome, is a generalized epileptic condition clinically characterized by eyelid myoclonia (EM) with or without absences, eye closure-induced electroencephalography (EEG) paroxysms, and photosensitivity; in addition, rare tonic-clonic seizures may also occur. Although first described in 1977 and widely reported by several authors within ...
Cortez Miguel A - - 2009
Infantile spasms is a catastrophic childhood seizure disorder for which few animal models exist. Children with Down syndrome are highly susceptible to infantile spasms. The Ts65Dn mouse is a valid model for Down syndrome; therefore, we tested the hypothesis that the Ts65Dn mouse represents a substrate for an animal model ...
Beghi Ettore - - 2009
An epilepsy syndrome is a disorder characterized by a cluster of symptoms and signs customarily occurring in combination. A syndromic approach to the epilepsies would be of practical value for diagnostic, prognostic, and therapeutic purposes. However, despite considerable efforts by leaders in the field of epileptology and the improved knowledge ...
Rubboli Guido - - 2009
The classification of idiopathic generalized epilepsies (IGEs) is still controversial, with special reference to absence epilepsy syndromes. Strict, well-defined criteria for syndromic definitions are necessary to delineate homogeneous conditions; however, this approach may leave a considerable group of patients unclassified, leading to the effort to categorize them in possible distinct ...
Michael Michael - - 2010
Panayiotopoulos syndrome is a common multifocal autonomic childhood epileptic disorder with significant clinical, pathophysiological and management implications. It affects otherwise normal children with onset at around 3-6 years. It is characterized by seizures, often prolonged, with predominantly autonomic symptoms and mainly ictal vomiting. EEG shows shifting and/or multiple foci, often ...
Smigielska-Kuzia Joanna - - 2009
Epilepsy is rarely considered as a major component of Down syndrome. We evaluated the prevalence of epileptic seizures in 252 (97 girls and 155 boys) children and adolescents with Down syndrome evaluated at Department of Pediatric Neurology between 1994 and 2007. Results showed that 15 (6%) patients had epileptic seizures: ...
Saneto Russell P - - 2009
Lennox-Gaustaut syndrome is an electroclinical epilepsy syndrome characterized by the triad of electroencephalogram showing diffuse slow spike-and-wave discharges and paroxysmal fast activity, multiple intractable seizure types, and cognitive impairment. The intractability to seizure medications and cognitive impairment gives rise to eventual institutionalized patient care. Only a small subset of seizure ...
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