Search Results
Results 451 - 500 of 893
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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 ...
Scantlebury Morris H - - 2002
The factor V Leiden mutation is the most common hereditary cause of venous thrombosis in our population. In the pediatric population, it has been associated with cerebrovascular thrombosis, cerebral palsy, and prosencephaly in neonates. We present two children with hemiconvulsion, hemiplegia, and epilepsy syndrome in whom the cause is likely ...
Salim Ali - - 2002
Klüver-Bucy syndrome (KBS) has been described as a disconnection of the temporal lobes from the remainder of the brain. Its presence in minor head trauma has not been previously reported. We therefore report what we believe to be the first case of KBS due to mild head trauma and unilateral ...
Trinka Eugen - - 2002
To analyze the spectrum of epilepsy syndromes which follow childhood febrile convulsions (FC) and to examine whether retrospective analysis of clinical features of the FC enables discrimination of patients who develop temporal lobe epilepsy (TLE) from those who develop generalized epilepsy (GE). One hundred and thirteen patients with epilepsy and ...
Carai Mauro A M - - 2002
The present study investigated the effect of the GABA(B) receptor antagonist, SCH 50911 [(2S)(+)-5,5-dimethyl-2-morpholineacetic acid], on the occurrence of seizures in ethanol-dependent rats undergoing ethanol withdrawal syndrome. The acute administration of nonconvulsive doses of SCH 50911 (0, 100, 170 and 300 mg/kg, i.p.) resulted in a dramatic facilitation of spontaneous ...
Go Tohshin - - 2002
A 6-month-old girl developed West syndrome and it remitted in association with valproate-induced hepatic dysfunction. Plasma -alanine elevated after valproate hepatotoxicity, which seemed to be a possible cause of remission. The patient has been seizure-free with normal electroencephalogram for 12 months without any anticonvulsants. Her developmental milestones were within normal ...
Raybarman C - - 2002
A healthy 5 year old boy developed aphasia, attention disorder and hyperkinesia preceded by transient formed visual hallucinations and emotional outburst, immediately after a stressful event of forced separation from his father. EEG showed generalized epileptiform activity. He was diagnosed as Landau-Kleffner syndrome (LKS). CT and MRI of the brain ...
Seeck M - - 2002
We describe a 20-year-old woman suffering from right temporal epilepsy, behavioral disorder, and a complaint of paroxysmal palpitations accompanied by anxiety. Detailed cardiac evaluation revealed that the palpitations were due to episodes of marked sinus tachycardia secondary to a concomitant postural orthostatic tachycardia syndrome (POTS) and not of psychogenic origin ...
Post Robert M - - 2002
Kindling, in the classical sense, involves progressively increasing responsivity to the intermittent repetition of the same 1-s subthreshold electrical stimulation over time, with the amygdala being the area most frequently studied. Such repeated subthreshold stimulation is associated with: lowering of the after-discharge (AD) threshold; lengthening and spread of the AD; ...
Kinton Lucy - - 2002
The genetic analysis of simple Mendelian epilepsies remains a key strategy in advancing our understanding of epilepsy. In this article, we describe a new family epilepsy syndrome, partial epilepsy with pericentral spikes, which we map to chromosome 4p15. We distinguish it clinically, electrophysiologically, and genetically from previously described Mendelian epilepsies. ...
Wallace R H - - 2002
Generalized epilepsy with febrile seizures plus (GEFS(+)) is an important childhood genetic epilepsy syndrome with heterogeneous phenotypes, including febrile seizures (FS) and generalized epilepsies of variable severity. Forty unrelated GEFS(+) and FS patients were screened for mutations in the sodium channel beta-subunits SCN1B and SCN2B, and the second GEFS(+) family ...
Mikati Mohamad A - - 2002
We administered 2 gm/kg of intravenous gamma globulin (IVIG) to each of five consecutive patients with Landau-Kleffner syndrome, over 4 days. We compared the 1-month baseline to that following IVIG using a severity score assessing speech, comprehension, behavior, seizures, and electroencephalography. There was a significant drop in this score after ...
Bauer Jan - - 2002
The present review describes advances in Rasmussen's encephalitis (also known as Rasmussen's syndrome), an unihemispheric intractable epileptic disease with persistent inflammation. Specific attention is given to the recent recognition of cytotoxicity by CD8+/granzyme-B-positive T lymphocytes as a new pathogenic mechanism of neuronal damage.
Möller J C JC Klinik für Neurologie, Philipps-University Marburg, - - 2002
The aim of this paper is to report a patient with late-onset myoclonic epilepsy in Down's syndrome (LOMEDS) as a differential diagnosis of adult-onset progressive myoclonic epilepsies. A 55-year-old male with Down's syndrome (DS) is described who developed progressively frequent myoclonus and generalized myoclonic-tonic seizures (GMTSs) at the age of ...
Eirís-Puñal Jesús - - 2002
Blue rubber bleb nevus syndrome is an uncommon neurocutaneous disorder characterized by distinctive vascular malformations on the body surface. Vascular malformations of internal organs (typically the gastrointestinal tract) are also frequently present. However, malformations of the central nervous system have only rarely been described. We report a case of blue ...
Ferrie Colin D - - 2002
Panayiotopoulos syndrome is a relatively common condition with susceptibility to early onset benign childhood seizures, which manifests primarily with autonomic and mainly emetic symptoms. It predominantly affects children of 3-6 years of age (13% of those with one or more non-febrile seizures). EEG shows great variability, with occipital, extra-occipital spikes ...
Aina O F - - 2002
Epilepsy is one of the commonest disorders presenting the neurological clinics in developing countries. There are attendant complications especially in untreated or poorly treated cases. In this article, we report an uncommon complication known as Landau-Kleffner syndrome in a Nigerian girl. The syndrome, typically seen in children consists of an ...
Trevathan Edwin - - 2002
Infantile spasms and Lennox-Gastaut syndrome are rare but are important to child neurologists because of the intractable nature of the seizures and the serious neurologic comorbidities. New antiepileptic drugs offer more alternatives for treating both infantile spasms and Lennox-Gastaut syndrome. Selected children with infantile spasms are candidates for epilepsy surgery. ...
Crumrine Patricia K - - 2002
Lennox-Gastaut syndrome is a type of childhood epilepsy that has enormous detrimental effects on the patient's physical and developmental health and can also take a dramatic toll on the well-being of the patient's family. Lennox-Gastaut syndrome is characterized by variable etiology, multiple types of intractable seizures, and cognitive impairment in ...
Drury Ivo - - 2002
Characteristic appearances of epileptiform EEG activity in children aged between 2 and 10 years are described. Emphasis is placed on the value of careful analysis of background EEG and how the epileptiform activity itself may overlap between benign and malignant epileptic syndromes. Illustrations are used to underscore this emphasis. Irrespective ...
Niedermeyer Ernst - - 2002
The Lennox-Gastaut syndrome (LGS) is widely known as one of the most severe and prognostically unfavourable epileptic conditions. Its entity as a syndrome is based on the occurrence of certain characteristic types of seizure (particularly typical drop attacks and axial tonic seizures) and on EEG patterns such as the slow ...
Freeman Jeremy L - - 2002
We report three patients with hemiconvulsion-hemiplegia-epilepsy syndrome who presented acutely and were shown to have striking neuroimaging findings suggestive of diffuse cytotoxic edema confined to one hemisphere, including extensive diffusion-weighted imaging abnormalities in two cases. Two patients subsequently developed progressive and extensive atrophy of the involved hemisphere. These findings are ...
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