Search Results
Results 401 - 450 of 863
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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 ...
Zhang Wenbo - - 2003
Epidermal nevus syndrome is a kind of neurocutaneous syndrome that is associated with epidermal nevus and a variety of congenital CNS disorders. Clinical presentations include seizures, paresis, mental retardation, and developmental delay. We report three cases with MR imaging and magnetoencephalography findings; one patient underwent ictal and interictal single photon ...
Herrero Hernandez Elena - - 2003
Excess manganese (Mn) can cause several neurotoxic effects, however only a few studies have reported epileptic syndromes related to manganese intoxication. We describe an epileptic syndrome due to manganese intoxication in a 3 year old male child. His blood manganese was elevated, but no other abnormal values or toxic substances ...
Ogawa Atsushi - - 2003
Kabuki make-up syndrome is a mental retardation-malformation syndrome affecting multiple organ systems, with a broad spectrum of neuromuscular dysfunction and mental ability. The incidence of seizures associated with this syndrome ranges from 10 to 40%. However, details of the seizures in this syndrome have not been adequately reported or thoroughly ...
Riviello James J - - 2003
The management of seizures and epilepsy begins with forming a differential diagnosis, making the diagnosis, and then classifying seizure type and epileptic syndrome. Classification guides treatment, including ancillary testing, management, prognosis, and if needed, selection of the appropriate antiepileptic drug (AED). Many AEDs are available, and certain seizure types or ...
Caraballo Roberto Horacio - - 2003
Spasms are defined as epileptic seizures characterized by brief axial contraction, in flexion, extension or mixed, symmetric or asymmetric, lasting from a fraction of a second to 1-2s, and are associated with a slow-wave transient or sharp and slow-wave complex, followed or not by voltage attenuation. Epileptic spasms usually appear ...
Varon Daniel - - 2003
The characteristic features of Kluver-Bucy syndrome include hypersexuality, hyperorality, placidity, hypermetamorphosis, visual agnosia, changes in dietary habits, and memory impairment. Human cases have been reported with herpes simplex encephalitis, head injury, Pick's disease, transtentorial herniation, adrenoleukodystrophy, and Reye's syndrome, all involving bilateral temporal lobe pathology. We present the case of ...
Kjeldsen Marianne Juel - - 2003
The role of genetic factors in the occurrence of epilepsy syndromes was studied in twins recruited from the population-based Danish Twin Registry. A total of 34,076 twins were screened for epilepsy. Cases were confirmed and classified by two neurologists according to the classification systems of the International League Against Epilepsy ...
Grosso Salvatore - - 2003
Schinzel-Giedion syndrome (SGS) is a rare disorder with a likely autosomal recessive pattern of inheritance which is characterized by several facial dysmorphisms, midface hypoplasia, multiple skeletal anomalies including short and sclerotic skull base, short neck, and post-axial polydactyly. Cardiac and urogenital malformations are also present. Thirty-three cases have been described ...
Valente Kette D - - 2003
Wolf-Hirschhorn syndrome (WHS) is a genetic disorder caused by a deletion of the short arm of chromosome 4. Sgrò et al. described an electroclinical profile for WHS, but data regarding this issue are scarce. We report an 8-year-old girl presenting the classic phenotype for WHS, confirmed by FISH test. Epilepsy ...
M.B. Singh, J. Kalita, ...
Landau - Kleffner syndrome is a rare, functional, age-related epilepsy with aphasia and epileptiform discharges on EEG. The heterogenity of clinical presentations, course, longterm outcome and response to treatment suggests multiple underlying etiologies. Normal children abruptly develop deterioration of language functions along with spike and wave discharges on EEG. Clinical ...
G. Deda, H. Caksen
Two brothers (3 and 2 year old) with characteristic findings of atypical benign partial epilepsy of childhood (pseudo-Lennox syndrome) are reported, to emphasize the presence of a possibility of a genetic basis of this disorder and the importance of intravenous immune globulin (IVIG), vigabatrin (VGB) and lamotrigine (LTG) therapy. Sleep ...
Taylor Isabella - - 2003
Occipital epilepsies often elude diagnosis as they frequently masquerade as other seizure syndromes. Visual hallucinations are the key clinical symptoms indicating an occipital focus, but may be difficult to elicit on history, especially from children, and are not always present. When visual symptoms are not prominent, the seizure semiology and ...
Hirose Shinichi - - 2003
Mental retardation (MR) and epilepsy are both heterogeneous syndromes based on dysfunction in the brain and they are often closely associated. Hence, there should be some overlap in the underlying pathomechanisms, particularly when both syndromes result from genetic abnormalities, either polygenic or monogenic. Some 50 monogenic causes of MR have ...
Pies Norbert J - - 2003
The British surgeon William James West has not left a tremendous literary or scientific work as many of his contemporaries did. For this reason only a little has been known about him and the fate of his family for decades, even though the eponym was created in the 1960s. Only ...
Bennett B - - 2003
Neurologic complications of hemolytic uremic syndrome, including seizures, usually occur early during the acute phase of the illness. We report a3-year-old girl with classic diarrhea-associated hemolytic uremic syndrome who developed late onset seizures, hemiparesis and transient blindness on the 17th hospital day, at which time her recovery was characterized by ...
Kremer Stéphane - - 2003
We report the case of a 29-year-old patient, who suffered from drug resistant laughing seizures since childhood. The clinical examination was normal, except for sequelae of hand and feet surgery during infancy for post-axial polydactyly. Cerebral MRI showed a hypothalamic hamartoma. The association of complex limb abnormalities with hypothalamic hamartoma ...
Thomas Pierre - - 2003
A 15-year-old girl with a 3-year history of Rasmussen syndrome (RS) underwent left functional hemispherectomy by central disconnection. Clinical seizures then ceased. Five months postoperatively, ictal EEG discharges were associated with focal hyperperfusion on SPECT within the disconnected hypoperfused left hemisphere, suggesting that the basic mechanisms of RS may continue, ...
Satishchandra P - - 2003
Geographically specific epilepsy syndromes are of epidemiologic interest and may assist in understanding the pathogenic mechanism of epilepsy in general. Some may have a genetic basis with added environmental influence. Among these epilepsy syndromes in India is an interesting type of reflex seizure known as hot-water epilepsy (HWE) and single ...
Le Blanc Marc - - 2003
BACKGROUND: Over the last 40 years, numerous studies have proposed that various deviant behaviours are part of a latent construct now labelled 'general deviance' by criminologists or 'problem behaviour' by psychologists. During that period, many studies have documented the presence of specific forms of deviance. However, no study has tested ...
Faught Edward - - 2003
PURPOSE: To review the current state of knowledge of the treatment of primary (idiopathic) generalized epilepsy syndromes based on the efficacy results of clinical trials, to highlight obstacles in the design of these trials, and to suggest solutions for future research. METHODS: Published reports of controlled clinical trials, as well ...
Borgatti Renato - - 2003
It has recently been demonstrated that patients with Angelman's syndrome who exhibited a deletion on cytogenetic tests show more severe clinical pictures with drug-resistant epilepsy than patients with Angelman's syndrome not carrying the deletion. To verify if this difference in clinical severity can be attributed to genes for the three ...
Leppik Ilo E - - 2003
The myoclonic epilepsies are a collection of syndromes in which myoclonic seizures are a prominent feature. Proper classification of a patient's syndrome is critical for appropriate treatment and prognosis. However, classification of such syndromes is often difficult because the terminology used to describe seizures can be confusing and inconsistent. Myoclonic ...
Wheless James W - - 2003
Three of the seizure types (myoclonic, absence, and generalized tonic-clonic) and syndromes associated with idiopathic generalized or genetic epilepsies can present an acute status epilepticus picture that requires acute therapy. These are not the usual seizures observed in status epilepticus because most of these patients have secondary generalized or symptomatic ...
Gulati Sheffali - - 2002
Childhood epileptic syndrome characterized by early onset gelastic seizures, hypothalamic hamartoma and precocious puberty is well recognized though rare. We report association of agenesis of corpus callosum, Dandy-Walker complex and heterotopic gray matter with this childhood epileptic syndrome which is hitherto an unreported association. The child showed a satisfactory response ...
Ritaccio Anthony L. - - 2002
Cognition-induced epilepsy comprises a group of loosely tied syndromes characterized by seizures regularly precipitated by cognitive tasks. Linguistic operations (e.g., reading, writing) and decision making associated with visuospatial manipulation are the most frequent and best-characterized triggers. The syndromes reviewed have a high degree of overlap and clinical/EEG variability, suggesting that ...
Jones Jeffrey M - - 2002
Several factitious epileptic syndromes have been associated with famous literary characters. While these syndromes include symptoms other than pseudoseizures, and while pseudoseizures can occur in other syndromes, a review of these disorders provides insights into factitious seizures and epilepsy.
Berry-Kravis Elizabeth - - 2002
Epilepsy is reported to occur in 10 to 20% of individuals with fragile X syndrome (FXS). A frequent seizure/EEG pattern in FXS appears to resemble that of benign focal epilepsy of childhood (BFEC, benign rolandic epilepsy). To evaluate seizure frequency and type in a Chicago FXS cohort, data regarding potential ...
Tütüncüo─člu Sarenur - - 2002
Landau-Kleffner syndrome is marked by an acquired aphasia in children who have had normal language and motor development. A 3.5-year-old girl was referred to our clinic with stuttering. She was diagnosed as having benign myoclonic epilepsy of infancy at 3.5 months of age and treated with valproic acid. Her electroencephalogram ...
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