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Nabbout Rima - - 2008
The aim of this article is to review new epilepsy syndromes, acquire a new understanding of older ones and emphasize the impact of this concept on basic research regarding aetiology and treatment. In addition to those included in the classification of the International League Against Epilepsy, new epilepsy syndromes comprise ...
Neubauer Bernd A - - 2008
More than 3% of the total population will suffer from epilepsy at some time during their lives. One quarter of newly diagnosed cases worldwide are children. With optimal treatment, around 70% will enter remission. In the long run, antiepileptic drugs can be discontinued in almost half of affected individuals. Most ...
Jea Andrew - - 2008
OBJECTS: Disconnection syndromes following corpus callosotomy represent complex and variably expressed groupings of signs and symptoms affecting motor control, spatial orientation, vision, hearing, and language. Little is known, however, about the functional topography of callosal fiber pathways. In addition, most published case reports and case series of corpus callosotomy seldom ...
Noureen Nuzhat - - 2008
Rasmussen syndrome or Rasmussen encephalitis is a rare, progressive gray matter disease of childhood. A case of 4-year-old boy is reported here, who presented with recurrent episodes of status epilepticus of simple partial fits along with progressive left sided hemiplegia. EEG showed focal discharges from right hemisphere. Serial MRI brain ...
Saito Naka - - 2008
Focal spike activities in Panayiotopoulos syndrome involve all brain regions in electroencephalography, and commonly reveal multiple foci, often through occipital predominance. To investigate correlations between developmental brain maturation and spike origin in Panayiotopoulos syndrome, we evaluated age-related or duration-related magnetoencephalographic spike localization in 25 patients with Panayiotopoulos syndrome. Regarding age ...
Jacob S - - 2008
Voltage-gated potassium channel antibody (VGKC-Ab)-associated limbic encephalitis (LE) is a recently described syndrome that broadens the spectrum of immunotherapy-responsive central nervous system disorders. Limbic encephalitis is typically characterised by a sub-acute onset of disorientation, amnesia and seizures, but the clinical spectrum is not yet fully defined and the syndrome could ...
Aina, O F; ;
Kluver-Bucy Syndrome, first described in monkeys as a result of bilateral ablation of their temporal lobes consists of hypermetamorphosis, visual agnosia, hyperarality and hyper-sexuality. In man, similar but not exact syndrome had been described and the symptom manifestations included placidity, apathy, bulimia, hyper-sexuality, agnosias, amnesia, seizure etc. We describe the ...
Bilgin Ozgür - - 2008
We report on a patient with right-sided Sturge-Weber syndrome (SWS), in whom earlier functional hemispherectomy failed. Subtraction of ictal and interictal single-photon-emission-computed-tomography (SPECT) superimposed on individual MRI showed a right fronto-orbital hyperperfusion, with a left-sided EEG seizure pattern. Ictal SPECT supported our assumption that right frontal originated seizure pattern propagated ...
Mastrangelo Mario - - 2009
The association of brain malformations and symptomatic epilepsy in the setting of neurofibromatosis type 1 (NF1) is rarely reported. When it occurs, patients can present clinically with infantile spasms, focal seizures, generalized tonic clonic seizures or atypical absences. We report on a 10-year-old (molecularly proven) NF1 girl manifesting a complex ...
Legriel Stephane - - 2008
INTRODUCTION: Takotsubo cardiomyopathy can complicate several conditions including neurological emergencies. A few recurrent cases associated with seizures have been reported, but none of the patients had status epilepticus. The pathophysiology of takotsubo syndrome, although debated, may involve stunning of the myocardium by a catecholamine storm triggered by stress. Patients with ...
Hoffmann Michael - - 2008
BACKGROUND: Right temporal lobe lesion syndrome elicitation presents a clinical challenge. Aside from occasional covert quadrantanopias, heralding elementary neurological deficits are absent. AIM: Isolated right and left temporal lobe stroke patients were analyzed for the panoply of known temporal and frontal cognitive and neuropsychiatric syndromes. METHODS: Temporal lobe stroke patients ...
Winston Ken R - - 2008
A male neonate with seizures, linear sebaceous nevus syndrome and hemimegalencephaly underwent hemispherectomy at 36 weeks' gestational age. He has had no clinical seizures in the 13 months since surgery but continues to have sharp wave activity over some parts of the intact hemisphere. He has moderate developmental delay and ...
Postiglione Alfredo - - 2008
Geschwind described a syndrome (Geschwind syndrome, GS) in patients with temporal lobe epilepsy, characterized by sexual behavioural disorders, hyper-religiosity, hypergraphia and viscosity. In this report we describe a patient affected by fronto-temporal dementia (FTD), who showed all the personality changes of GS without having epilepsy, and suggest that clinicians should ...
Bigler Erin D - - 2008
On the mild end of the acquired brain injury spectrum, the terms concussion and mild traumatic brain injury (mTBI) have been used interchangeably, where persistent post-concussive syndrome (PPCS) has been a label given when symptoms persist for more than three months post-concussion. Whereas a brief history of concussion research is ...
Genton Pierre - - 2008
PURPOSE: Absence status epilepticus (AS) is a prolonged, generalized, and nonconvulsive seizure that may occur in various circumstances. We report a series of patients in whom recurrent, unprovoked, typical AS was the main clinical feature. METHOD: We retrospectively reviewed consecutive patients referred to our epileptic centers, on the following criteria: ...
Hattori Junri - - 2008
PURPOSE: Our aim was to develop a screening test to predict Dravet syndrome before the first birthday based on the clinical characteristics of infants and the SCN1A mutation analysis. METHODS: Ninety-six patients who experienced febrile seizures before the age of one were enrolled. The patients were divided into two groups-the ...
Moriyama Yasushi - - 2007
A 68-year-old woman with schizophrenia after a cerebrovascular accident resulting in right medial temporal and occipital damage developed Frégoli syndrome. Neuropsychological testing revealed that she had impairment in facial recognition compatible with prosopagnosia. The Frégoli syndrome disappeared in three month when, remarkably, her prosopagnosia also disappeared. Our findings are consistent ...
Matsumoto Akiko - - 2007
The subjects were 106 SMIDS with epilepsy. They were classified into four epileptic syndromes: (1) SE-MISF (34.0%), (2) SGE (25.5%), (3) SLRE (20.7%), and (4) epileptic discharge-free patients (EDFP) (19.8%). Clinical electroencephalographic studies elucidated the following result: (1) The seizure disappearance rate was the highest in SLRE (54.5%), and it ...
Gümüş Hakan - - 2007
The combination of axial spasms in clusters, hypsarrhythmia, and psychomotor delay beginning in the first year of life defines West syndrome. Adrenocorticotrophic hormone remains the choice of treatment for many neurologists. Recent controlled studies support vigabatrin as first-line therapy, and open-label studies suggest that topiramate, lamotrigine, and zonisamide may be ...
Carinci Valeria - - 2007
The clinical distinction between cardiovascular and epileptic causes of loss of consciousness is sometimes difficult, but becomes challenging when a primary epileptic seizure secondarily causes an asystole. Epilepsy can be correlated to severe bradycardia or asystole. The syndrome is called the ictal bradycardia syndrome. Ictal bradycardia and asystole have been ...
MacAllister William S - - 2007
Seizure disorders are relatively common in childhood, and the International League Against Epilepsy (ILAE) provides a hierarchical classification system to define seizure types. At the final level of classification, specific epilepsy syndromes are defined that represent a complex of signs and symptoms unique to an epilepsy condition. The present review ...
Crespel Arielle - - 2007
Senile myoclonic epilepsy of Genton is a newer epileptic syndrome of the older patients with Down syndrome associated with an Alzheimer-type dementia. We report two observations in whom the clinical and electroencephalographic features are consistent with the description of this syndrome. Both experienced a progressive deterioration of cognitive functions few ...
Pelc Karine - - 2008
Angelman syndrome is a neurogenetic disorder caused by lack of UBE3A gene expression from the maternally inherited chromosome 15 due to various 15q11-q13 abnormalities. In addition to severe developmental delay, virtual absence of speech, motor impairment, a behavioural phenotype that includes happy demeanor, and distinctive rhythmic electroencephalographic features, over 90% ...
Berhouma Moncef - - 2007
A case of an acute life-threatening presentation of hemiconvulsion-hemiplegia-epilepsy (HHE) syndrome requiring an urgent decompressive hemicraniectomy is described. A 9 month-old baby had a status epilepticus following a sustained fever, leading to a comatose state and a right pupillary dilatation associated with a left hemiplegia. The MRI showed a swelling ...
Deng Yu Hong - - 2007
Focal seizures often secondarily generalize but the reverse is much less frequently documented. The idiopathic generalized epilepsies have the EEG signature of generalized, or bilaterally synchronous spike wave activity and although focal features can be seen, seizures are usually generalized once they commence. Although focal seizures can be seen in ...
Chen Jui-Cheng - - 2007
The mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a rare congenital disorder of mitochondrial DNA. Patients with this syndrome may present acute onset of sensorineural hearing loss, which is genetic in origin. An impression of the MELAS syndrome is favored because hearing loss is part of ...
NASIRIAN, A.
Early infantile epileptic encephalopathy or EIEE (Ohtahara syndrome; OS) is a kind of intractable seizure that begins in neonatal age with sudden onset of tonic spasms in series or single suppression-burst S-B in EEG.1 Imaging shows anatomic defects such as migration disorders and generalized atrophy2 with essentially normal metabolic tests. ...
Duarte Sofia - - 2008
Early-onset epileptic encephalopathies are devastating conditions. Little is known about pathophysiology and biological markers. We aimed to identify a relationship between the type and prognosis of epileptic encephalopathies starting in infancy and the cerebrospinal fluid profile of pterins and neurotransmitters. Cerebrospinal fluid samples of 23 infants with epileptic encephalopathies were ...
Hakimian Shahin - - 2007
Rufinamide (1-[2,6-difluorobenzyl]-1H-1,2,3-triazole-4-carboxamide) is a new anti-epileptic drug with a novel triazole derivative structure. The suspected mechanism of action is limitation of sodium-dependent action potentials, thought to result in a membrane stabilizing effect. Rufinamide is extensively metabolized in the liver by non-CYP450 enzymes with an elimination half-life of 8 - 12 ...
Unalp Aycan - - 2007
Wolf-Hirschhorn syndrome is defined by a collection of core characteristics that include mental retardation, epilepsy, growth delay, and craniofacial dysgenesis. The disorder is caused by subtelomeric deletions in the short arm of chromosome 4. The syndrome, as described in the literature, may have a progression to resistant seizures and status ...
Beretta L - - 2007
A newborn female born at term was admitted at 28 hours for seizures and generalized hypotonia. Cerebral ultrasound showed a right temporal echogenic lesion confirmed on MRI and thought to be secondary to thrombosis of the vein of Labbé. The EEG showed epileptic discharges over the right temporal region. Extensive ...
Larner Andrew J - - 2007
This paper presents a review of all patients with Down syndrome seen over a 5-year period by one consultant neurologist in general outpatient and specialist cognitive function clinics. It revealed only 7 cases in > 4500 general referrals (= 0.2%), all referred with suspected seizure disorders. The diagnosis of epilepsy ...
Simon Tony J - - 2007
This article presents the cognitive profile observed in children with one of several common genetic syndromes associated with "nonverbal learning disorders." It introduces the concept of a cognitive endophenotype to help explain the similar pattern of impairments across the syndromes. It explores the explanation of diverse impairments in higher-order visual, ...
Montenegro Maria Augusta - - 2007
BACKGROUND: Malformations of cortical development (MCD) usually manifest in childhood with epilepsy, developmental delay and focal neurological abnormalities. OBJECTIVE: To evaluate the presentation and severity of epilepsy in the different types of MCD. METHOD: We evaluated the first 100 consecutive patients with a neuroimaging diagnosis of MCD. They were identified ...
Verrotti A - - 2007
West syndrome is a generalized epilepsy syndrome composed of infantile spasms and onset is usually within the first year of life. Although West syndrome is well known clinical epileptic syndrome, there is no agreement about the first- and second-line treatments. In the last years a great progress in the development ...
Saneto Russell P - - 2007
Early infantile epileptic encephalopathy, or Ohtahara syndrome, is characterized by tonic spasms and a suppression-burst pattern on the electroencephalography (EEG). The EEG demonstrates a suppression-burst pattern during waking and sleeping states that often evolves into hypsarrhythmia and followed later by a diffuse slow spike-wave pattern. In other patients, the EEG ...
Carreño M - - 2007
We present one patient with Parry Romberg syndrome and another with linear scleroderma in coup de sabre, with focal neurologic deficits and intractable seizures arising from the hemisphere ipsilateral to the cutaneous lesion. Brain MRI showed progressive hemispheric atrophy. Pathology after functional hemispherectomy showed chronic inflammatory features suggestive of Rasmussen ...
Jian Le - - 2007
Rett syndrome is a neurodevelopmental disorder mainly affecting females. It is principally caused by mutations in the MECP2 gene. Seizures occur in about 80% of subjects but there has been little research into the factors contributing to their frequency. To investigate seizure frequency in Rett syndrome and its relationship with ...
Sethi N K - - 2007
Myoclonic epilepsies comprise a heterogeneous group of epileptic syndromes characterized by myoclonic jerks, generalized tonic-clonic seizures (GTCSs), and sometimes absence seizures (multiplicity of seizure types). JME (Juvenile Myoclonic Epilepsy) is relatively common and usually responds well to treatment with appropriate anticonvulsants. Others like the progressive myoclonic epilepsy syndromes are relentlessly ...
Parisi P - - 2007
Panayiotopoulos syndrome is a relatively frequent and benign epileptic syndrome, characterised by predominantly autonomic symptoms and/or simple motor focal seizures followed or not by impairment of consciousness. Interictal EEG shows occipital spikes although multifocal spikes with high amplitude sharp-slow wave complexes at various locations can be present. This syndrome can ...
Blum A - - 2007
A 39-year-old man, who presented at age 312 with Landau-Kleffner syndrome, had persisting oral and written language deficits into adulthood. Seizures were easily controlled in childhood, but reemerged in adulthood as medication-refractory complex partial seizures. Abnormal T2 signal hyperintensity was seen in the left mesial temporal area on brain MRI. ...
Warwick Tanya C - - 2007
Seizures are a common comorbidity of autism and occur in as many as 30% of patients. This case report describes a 23-year-old man diagnosed with both Asperger syndrome and bitemporal epilepsy. The patient had behavioral regression that correlated with worsening of his intractable seizures. He subsequently underwent implantation of a ...
Korff Christian - - 2007
To report the authors' experience with diagnosis and management of Dravet syndrome, or severe myoclonic epilepsy in infancy, in the era of commercially available genetic testing, the authors performed a retrospective study of 16 patients diagnosed with Dravet syndrome at a tertiary care pediatric epilepsy center. They analyzed their clinical ...
Benninger D H - - 2007
Ictal transient opercular syndrome is rarely observed in benign epilepsy with centro-temporal spikes in children, and even more rarely in epilepsia partialis continua and symptomatic focal status epilepticus in adults. Here we report the ictal and interictal neuroimaging and electrophysiological findings in an adult female suffering from discontinuous focal status ...
van Woerden Geeske M - - 2007
Angelman syndrome (AS) is a severe neurological disorder characterized by mental retardation, motor dysfunction and epilepsy. We show that the molecular and cellular deficits of an AS mouse model can be rescued by introducing an additional mutation at the inhibitory phosphorylation site of alphaCaMKII. Moreover, these double mutants no longer ...
Brown L M - - 2007
Lemierre's syndrome is a rare and sometimes life threatening condition that requires prompt management. A case is reported of a previously healthy young male with Lemierre's syndrome. He developed internal jugular vein and cavernous sinus thrombosis, metastatic abscesses in the temporal lobe and lungs, temporal lobe venous infarction and severe ...
Naudi Antoniella Busuttil - - 2007
18p- syndrome is caused by the deletion of all or a portion of genetic material on the short (p) arm of chromosome 18. It was first described in 1963 and since then there have been a variety of clinical characteristics associated with this syndrome. The patient described presented with learning ...
Abutalebi J - - 2007
Anosognosia for cortical blindness, also called Anton's syndrome, is a rare neurological disorder usually following bilateral lesions to occipital cortices. Neuropsychological, morphological and functional neuroimaging (SPECT and fMRI) findings are reported in a patient who incurred Anton's syndrome after an ischaemic lesion confined to the left occipital lobe involving the ...
Castro Luiz H - - 2007
PURPOSE: Hypothalamic hamartoma (HH) related epilepsy presents with gelastic seizures (GS), other seizure types and cognitive deterioration. Although seizure origin in GS has been well established, non-GS are poorly characterized. Their relationship with the HH and cognitive deterioration remains poorly understood. We analyzed seizure type, spread pattern in non-GS and ...
Chahine Lama M - - 2006
By definition, benign epilepsy syndromes occur in patients with no significant prenatal, perinatal, or postnatal complications, normal psychomotor development and negative laboratory and neuroimaging work-up, respond well to therapy, and remit without sequeale. The benign localization-related epilepsy syndromes of childhood include benign childhood epilepsy with centrotemporal spikes, Panayiotopoulos syndrome and ...
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