We studied four children with diagnosis of absence seizures (generalized primary epilepsy), and with a generalized delta activity on the EEG during clinical attacks provoked by hyperventilation. The lack of ictal generalized spike-and-wave discharges with a frequency of 3 Hz in our patients, makes this an atypical pattern. All children had complete control of their seizures and disappearance of the EEG changes with valproate. We concluded that generalized delta activity observed on EEG during the hyperventilation in children should not always be considered as a normal finding for age, since it could be an ictal event of an absence seizure. PMID:7487533
Silva, D F; Lima, M M; Anghinah, R; Zanoteli, E; Lima, J G
Treatment of Long Evans hooded rats during post-natal brain development with the cholesterol synthesis inhibitor, AY-9944 (AY) results in the occurrence of atypical absence seizures, which are frequent, recurrent, and life-long. AY induced slow spike-and-wave discharges (SSWD) are significantly more frequent and prolonged in female Long Evans rats than males. Three groups of experiments were performed in order to characterize further the AY model of atypical absence seizures, (1) a developmental study was performed to ascertain whether AY-induced seizures appear before or after the onset of puberty; (2) male/female differences in severity of response to AY was determined in order to answer the question whether the gender specificity was a pre- or postpubertal phenomenon; (3) a time course study was done to determine the minimum number of postnatal AY doses needed to induce the life-long atypical absence seizure state. The data indicate that AY-induced atypical absence seizures emerge before the onset of puberty. Further, we show that the gender difference in severity of AY-induced seizures also is a pre-pubertal phenomenon. Finally, a single dose of AY (7.5 mg/kg) administered on post-natal day (P) 5 was sufficient to induce SSWD on the electrocorticogram (ECoG). Our results suggest that sex hormones are important in the AY model, although the exact role of cholesterol derived steroid hormones in the regulation and maintenance of AY induced atypical absence seizures remains to be determined. PMID:11823115
Persad, Vasan; Cortez, Miguel A; Snead, O Carter
In this report, we review the pharmacological and non-pharmacological treatments of the different absence seizure types as recently recognized by the International League Against Epilepsy: typical absences, atypical absences, myoclonic absences, and eyelid myoclonia with absences. Overall, valproate and ethosuximide remain the principal anti-absence drugs. Typical absence seizures exhibit a specific electroclinical semiology, pathophysiology, and pharmacological response profile. A large-scale comparative study has recently confirmed the key role of ethosuximide in the treatment of childhood absence epilepsy, more than 50 years after its introduction. No new antiepileptic drug has proven major efficacy against typical absences. Of the medications under development, brivaracetam might be an efficacious anti-absence drug. Some experimental drugs also show efficacy in animal models of typical absence seizures. The treatment of other absence seizure types is not supported with a high level of evidence. Rufinamide appears to be the most promising new antiepileptic drug for atypical absences and possibly for myoclonic absences. The efficacy of vagal nerve stimulation should be further evaluated for atypical absences. Levetiracetam appears to display a particular efficacy in eyelid myoclonia with absences. Finally, it is important to remember that the majority of antiepileptic drugs, whether they be old or new, may aggravate typical and atypical absence seizures.
Amantadine has demonstrated efficacy in small series for absence and myoclonic type seizures. We examined the efficacy of amantadine for treating refractory absence seizures in a cohort of pediatric patients. We retrospectively reviewed medical records for patients with absence seizures treated with amantadine at Cook Children's Medical Center after January 2007. Abstracted data included sex, age at initiation, concomitant antiepileptic drugs, amantadine dosing, and seizure frequency. Outcomes at 3, 6, and 12 months after initiation were categorized as >90%, ?50%, or <50% reduction in seizure frequency. Of 13 patients included in the study, many were exposed to multiple antiepileptic drugs (median, 3; range, 1-6). Three were implanted with a vagus nerve stimulator. A response of at least 50% seizure reduction was reported in more than 50% of patients reviewed at 3, 6, and 12 months after initiating treatment. Among responders, a majority had >90% reduction in seizure frequency. Amantadine may constitute an efficacious alternative treatment for refractory absence seizures. PMID:22490771
Perry, M Scott; Bailey, Laurie J; Kotecha, Amy C; Malik, Saleem I; Hernandez, Angel W
Tremor rats (tremor homozygous rats) exhibit spontaneous absence-like seizure, which is characterized by a sudden immobility with staring and the appearance of 5- to 7-Hz spike and wave complexes in cortical and hippocampal electroencephalogram (EEG). In this study, we examined the development of the seizure and the mode of inheritance. All tremor homozygous and heterozygous rats exhibited the seizure by
Takafumi Higashiguchi; Tadoo Serikawa; Takahisa Yamada; Katsumi Kogishi; Akira Kondo; Junzo Yamada
A simultaneous recording of mean flow velocity in the right middle cerebral artery by transcranial Doppler ultrasonography and electroencephalographic activity was performed in 5 children with multiple daily typical absence seizures. Twenty-eight absence episodes were recorded. Mean flow velocity increased gradually a few seconds before the clinical and electroencephalographic manifestations of each seizure and reached the maximum values (range of
Roberto De Simone; Mauro Silvestrini; Maria Grazia Marciani; Paolo Curatolo
Benign occipital seizure syndromes are benign childhood epilepsy syndromes and are mainly of two types, Panayiotopoulos syndrome, an autonomic epilepsy and idiopathic childhood occipital epilepsy of Gastaut (ICOE-G) including the idiopathic photosensitive occipital lobe epilepsy. Although both these types are categorized as occipital seizures, they are distinct in presentation and management. They can also be tricky to diagnose as visual symptoms may not always be the presenting feature and it is also not very easy to elicit visual hallucinations during history taking. These seizures have a good response to treatment; however, there could be atypical evolution and refractoriness to treatment especially with ICOE-G. We describe three children who presented with visual and non-visual symptoms and the electroencephalography (EEG) in all the three cases showed occipital paroxysms. We have emphasized the clues in the clinical history and EEG leading to the diagnosis of these distinct epilepsy syndromes. We have also discussed the natural course of these epilepsy syndromes with some atypical evolution, which clinicians need to be aware of during treatment of these children.
Chary, Prithika; Rajendran, Bhuvaneshwari
Dr. Masur and colleagues(1) from the Childhood Absence Epilepsy Study Group tried to answer a few important questions regarding childhood absence epilepsy (CAE) in their article "Pretreatment cognitive deficits and treatment effects on attention in childhood absence epilepsy." First, they wanted to know whether children with absence epilepsy have any problems with thinking before starting medications. If the children have problems with thinking, the authors wanted to identify the specific types of thinking problems the children have. The authors also wanted to assess what effect seizure medications have on attention in these children. In principle, medications could improve or worsen attention. The possible effects of medication on attention may or may not depend on whether the medications control the seizures. PMID:24166970
Thio, Liu Lin
Leukoencephalopathy refers to any disease of the white matter including hereditary as well as acquired and toxic causes. Inherited leukodystrophies are diseases of myelin including abnormal myelin development, hypomyelination, or degeneration of myelin. We report a 6-year old female who presented with absence seizure at the age of 4 years. Cerebral magnetic resonance imaging (MRI) of the brain showed bilateral periventricular confluent high signal intensity. The seizure responded to anticonvulsant therapy, and the clinical course was characterized by normal development and neurological examination.
Dweikat, Imad Mohammad; Damsah, Nadera; Khalaf, Reham
An enhanced tonic GABA(A) inhibition in the thalamus plays a crucial role in experimental absence seizures and has been attributed, on the basis of indirect evidence, to a dysfunction of the astrocytic GABA transporter-1 (GAT-1). Here, the GABA transporter current was directly investigated in thalamic astrocytes from a well-established genetic model of absence seizures, the genetic absence epilepsy rats from Strasbourg (GAERS), and its non-epileptic control (NEC) strain. We also characterized the novel form of GABAergic and glutamatergic astrocyte-to-neuron signalling by recording slow outward currents (SOCs) and slow inward currents (SICs), respectively, in thalamocortical (TC) neurons of both strains. In patch-clamped astrocytes, the GABA transporter current was abolished by combined application of the selective GAT-1 and GAT-3 blocker, NO711 (30 ?m) and SNAP5114 (60 ?m), respectively, to GAERS and NEC thalamic slices. NO711 alone significantly reduced (41%) the transporter current in NEC, but had no effect in GAERS. SNAP5114 alone reduced by half the GABA transporter current in NEC, whilst it abolished it in GAERS. SIC properties did not differ between GAERS and NEC TC neurons, whilst moderate changes in SOC amplitude and kinetics were observed. These data provide the first direct demonstration of a malfunction of the astrocytic thalamic GAT-1 transporter in absence epilepsy and support an abnormal astrocytic modulation of thalamic ambient GABA levels. Moreover, while the glutamatergic astrocyte-neuron signalling is unaltered in the GAERS thalamus, the changes in some properties of the GABAergic astrocyte-neuron signalling in this epileptic strain may contribute to the generation of absence seizures. PMID:23090943
Pirttimaki, Tiina; Parri, H Rheinallt; Crunelli, Vincenzo
We studied clinical and EEG manifestations of absence seizures (AS) in children with Dravet syndrome (DS) to clarify their characteristic features and differences from those of typical AS (TAS). The subjects were 12 children with DS. We retrospectively analyzed electroclinical characteristics of AS. We analyzed a total of 102 ictal EEGs characterized by generalized spike-and-wave (GSW) and semiology of 78 video-taped AS. The mean age at the onset of AS and at the time of the study was 16.2±7.1 months and 40.3±22.1 months, respectively. Ictal EEG showed the focality of initial discharge in 49/102 (48%), a duration ranging from 2 to 180 s (mean: 10.2±22.6 s; median: 4.0 s), frequency ranging from 2 to 4 Hz (median=3.0 Hz), and irregular and disorganized GSW morphology in 66/102 (65%). AS manifested with eyelid-myoclonus and generalized myoclonus in 9/54 (17%) and 34/78 (44%), respectively. In conclusion, AS in DS were characterized by an early-onset age, a high incidence of irregular and disorganized 3 Hz GSW morphology, and the frequent association of generalized myoclonic movement as well as the absence of automatism as compared to TAS. The results should be appreciated in the differential diagnosis of early-onset AS, the treatment of AS in DS and also a further clinical and genetic study for DS. PMID:22824327
Tsuda, Yuko; Oguni, Hirokazu; Sakauchi, Masako; Osawa, Makiko
Febrile seizures occurring in the neonatal period, especially when prolonged, are thought to be involved in the later development of mesial temporal lobe epilepsy (mTLE) in children. The presence of an often undetected, underlying cortical malformation has also been reported to be implicated in the epileptogenesis process following febrile seizures. This paper highlights some of the various animal models of febrile seizures and of cortical malformation and portrays a two-hit model that efficiently mimics these two insults and leads to spontaneous recurrent seizures in adult rats. Potential mechanisms are further proposed to explain how these two insults may each, or together, contribute to network hyperexcitability and epileptogenesis. Finally the clinical relevance of the two-hit model is briefly discussed in light of a therapeutic and preventive approach to mTLE.
Sanon, Nathalie T.; Desgent, Sebastien; Carmant, Lionel
The thalamic relay neurons, reticular thalamic nucleus, and neocortical pyramidal cells form a circuit that sustains oscillatory burst firing, and is regarded as the underlying mechanism of absence seizures. T-type calcium channels play a key role in this circuit. Here, we review the role of T-type calcium channel genes in the development of absence seizures, and emphasize gain or loss of function mutations, and other variations that alter both quantity and quality of transcripts, and methylation status of isoforms of T-type calcium channel proteins might be of equal importance in understanding the pathological mechanism of absence seizures.
Chen, Yucai; Parker, William Davis; Wang, Keling
Immunoreactive- (IR-) somatostatin (SRIF), neuropeptide Y (NPY) and corticotropin-releasing factor (CRF) contents were investigated in the brain of tremor rats with absence-like seizure and spontaneously epileptic rats (SER), which is a genetically defined doublemutant (zi\\/zi, tm\\/tm) obtained by mating zitter homozygote (zi\\/zi) with tremor heterozygote (tm\\/+) and shows both absence-like seizure and tonic convulsions. Increased levels of IR-NPY and IR-CRF
Miyuki Sadamatsu; Hirohiko Kanai; Akira Masui; Tadao Serikawa; Junzo Yamada; Masashi Sasa; Nobumasa Kato
gamma-Hydroxybutyric acid (GHB) produces absence-like seizures when given to animals. One of the distinguishing characteristics of experimental generalized absence seizures is that they are exacerbated by GABAA agonists. Therefore, the hypothesis that GHB-induced absence seizures result from an interaction between GHB and the GABAA receptor complex was tested. The effect of GHB on the function of various components of the GABAA receptor complex in the cortex of the rat, was determined in a series of in vitro experiments. Similar studies were carried out at various times following systemic administration of the prodrug of GHB, gamma-butyrolactone (GBL) and changes in the GABAA receptor were correlated with electrographic and behavioral changes. gamma-Hydroxybutyric acid had no effect on the binding of [3H]muscimol, [3H]flunitrazepam and [35S]t-butylbicyclophosphorothionate (TBPS) or on the uptake of 36Cl- into synaptoneurosomes in the in vitro studies. Nor were changes observed after the administration of GBL before the onset of GHB-induced absence seizures. However, at the onset of GHB-induced spike wave discharge, there was a significant (P < 0.04) decrease in the binding of [35S]TBPS, associated with a significant decrease in muscimol-stimulated uptake of 36Cl- with no other biochemical change. One minute after onset of GHB-induced absence seizure, a significant (P < 0.05) increase in the binding of [3H]muscimol was noted. Ten minutes later the decrease in muscimol-stimulated uptake of 36Cl- had normalized, while the changes in binding of [3H]muscimol and [35S]TBPS persisted. Because GABAA function remained unchanged in the in vitro studies, as well as prior to the onset of GHB-induced absence seizures in the in vivo experiments, these studies do not support the hypothesis that GHB interacts directly with the GABAA receptor complex to produce absence-like seizures. PMID:8388552
Snead, O C; Liu, C C
?-Hydroxybutyric acid (GHB) is a naturally occurring compound which has the ability to induce generalized absence seizures when given to animals. This effect of GHB may be blocked by either GHB or GABAB receptor antagonists. We sought to test the hypothesis that pre-synaptic GHB- and GABAB-mediated mechanisms in thalamus and cortex are operative in the GHB model of generalized absence
O. C. Snead
In this study we used EEG-fMRI to investigate whether peri-ventricular nodular heterotopia (PNH) are connected to the seizure generating network in individuals initially diagnosed with absence seizures (AS) who were later found to have co-existent PNH. We performed event related EEG-fMRI of the patients typical events as well as performing functional connectivity (FC) seeded from the PNH to answer this question. Both subjects demonstrated event related BOLD change in the "core" absence network. Subject 1 also displayed event related BOLD increase in the nodules while FC analysis demonstrated connectivity between the nodules and the thalami and striatum bilaterally. The second subject did not display event related BOLD in the PNH but FC analysis demonstrated strong connections between the PNH and the parietal cortex. This study demonstrates that the peri-ventricular nodules can show connectivity to the absence network in individuals with AS and may be involved in seizure generation. PMID:23992952
Carney, Patrick W; Masterton, Richard A J; Gill, Deepak; Jackson, Graeme D
... wnl.0000436060.67147.25 2013;81;e138-e139 Neurology Liu Lin Thio Childhood absence epilepsy: Poor attention ... current as of October 28, 2013 http://www.neurology.org/content/81/18/e138.full.html located ...
The ubiquitously expressed iron storage protein ferritin plays a central role in maintaining cellular iron homeostasis. Cytosolic ferritins are composed of heavy (H) and light (L) subunits that co-assemble into a hollow spherical shell with an internal cavity where iron is stored. The ferroxidase activity of the ferritin H chain is critical to store iron in its Fe3+ oxidation state, while the L chain shows iron nucleation properties. We describe a unique case of a 23-yr-old female patient affected by a homozygous loss of function mutation in the L-ferritin gene, idiopathic generalized seizures, and atypical restless leg syndrome (RLS). We show that L chain ferritin is undetectable in primary fibroblasts from the patient, and thus ferritin consists only of H chains. Increased iron incorporation into the FtH homopolymer leads to reduced cellular iron availability, diminished levels of cytosolic catalase, SOD1 protein levels, enhanced ROS production and higher levels of oxidized proteins. Importantly, key phenotypic features observed in fibroblasts are also mirrored in reprogrammed neurons from the patient's fibroblasts. Our results demonstrate for the first time the pathophysiological consequences of L-ferritin deficiency in a human and help to define the concept for a new disease entity hallmarked by idiopathic generalized seizure and atypical RLS. PMID:23940258
Cozzi, Anna; Santambrogio, Paolo; Privitera, Daniela; Broccoli, Vania; Rotundo, Luisa Ida; Garavaglia, Barbara; Benz, Rudolf; Altamura, Sandro; Goede, Jeroen S; Muckenthaler, Martina U; Levi, Sonia
?-Hydroxybutyric acid (GHB) has the ability to induce absence seizures. The precise way in which GHB causes seizures remains unclear, but GABAB- and\\/or GHB-mediated presynaptic mechanisms within thalamocortical circuitry may play a role. In the present study, we determined the basal and K+-evoked release of GABA and glutamate in the superficial laminae of frontal cortex during GHB-induced absence seizures. Our
R. Q Hu; P. K Banerjee; O. C Snead III
Intracerebral microinjection is a commonly used technique for local delivery of biologically active agents. However, it is known that mechanical injury of the cortex can induce spreading depression (SD), a wave of transient cellular depolarization. We examined the effects of intracortical microinjections of a new selective I(h) channel antagonist ORG 34167 and of different control treatments (saline and sham microinjections) on spontaneously occurring spike-wave discharges (SWDs) in WAG/Rij rats, a valid genetic model of absence epilepsy. Electroencephalographic (EEG) recording in awake rats has shown that both the drug and control microinjections are followed by long-term (for more than an hour) suppression of SWDs. dc-EEG recording in WAG/Rij rats has revealed that sham microinjections induce SD in 65% (31/48) cases. Number of SWDs decreased substantially for at least 90 min after the sham injections which induced cortical SD but remained unchanged if SD was not triggered by microinjection. These findings suggest that SD induced by intracortical microinjection may contribute to long-term suppression of non-convulsive epileptic activity after this experimental procedure. PMID:23200788
Samotaeva, I S; Tillmanns, N; van Luijtelaar, G; Vinogradova, L V
The absence epilepsy typical electroencephalographic pattern of sharp spikes and slow waves (SWDs) is considered to be due to an interaction of an initiation site in the cortex and a resonant circuit in the thalamus. The hyperpolarization-activated cyclic nucleotide-gated cationic I h pacemaker channels (HCN) play an important role in the enhanced cortical excitability. The role of thalamic HCN in SWD occurrence is less clear. Absence epilepsy in the WAG/Rij strain is accompanied by deficiency of the activity of dopaminergic system, which weakens the formation of an emotional positive state, causes depression-like symptoms, and counteracts learning and memory processes. It also enhances GABAA receptor activity in the striatum, globus pallidus, and reticular thalamic nucleus, causing a rise of SWD activity in the cortico-thalamo-cortical networks. One of the reasons for the occurrence of absences is that several genes coding of GABAA receptors are mutated. The question arises: what the role of DA receptors is. Two mechanisms that cause an infringement of the function of DA receptors in this genetic absence epilepsy model are proposed. PMID:23738145
Bazyan, A S; van Luijtelaar, G
Absence seizures are characterized by bilateral spike-and-wave discharges (SWDs) in thalamo-cortical circuits. In view of clinical studies indicating a critical involvement of intralaminar thalamic nuclei, we thought it timely to characterize the specific role and activity patterns of the respective neurons. Electrocorticographic (ECoG), intracellular, and unit activity recordings were performed in vivo from intralaminar thalamic neurons of the centrolateral (CL) and the paracentral (PC) thalamic nucleus in an established genetic rat model of absence epilepsy (WAG/Rij). Neurons in PC are depolarized to produce tonic series of action potentials at seizure-free episodes, and are rhythmically silenced concomitant with SWDs in a spike-locked manner. Rebound from spike-locked inhibition is associated with a transient increase in action potential activity. Neurons in CL possess a relatively negative membrane potential with overall low electrogenic activity at seizure-free episodes and generate burst-like discharges during SWDs that are locked to the decaying phase of the spike component on the ECoG. The SWD-locked membrane responses reverse close to the presumed chloride equilibrium potential, indicating GABA(A) receptor-mediated inhibitory postsynaptic potentials (IPSPs), with cell-type specific differences in polarity. In PC neurons, hyperpolarizing IPSPs result in spike-locked silencing of tonic firing and rebound burst discharges, while in CL neurons, IPSPs are depolarizing and trigger low-threshold burst firing likely mediated by a t-type Ca(2+) conductance. These data show a unique pattern of rhythmic SWD-locked IPSPs in PC and CL associated with paroxysms apt to impose a transient dysfunctional state to thalamo-striato-prefrontocortical networks during absence seizures. PMID:21458572
Gorji, Ali; Mittag, Christoph; Shahabi, Parviz; Seidenbecher, Thomas; Pape, Hans-Christian
Veins of the head and neck exhibiting anatomical variations or malformations are clinically significant. Anatomical variation in the external jugular vein is very common. However, anatomical variation in the retromandibular vein is rare. In this paper, we report a rare case of complete absence of the retromandibular vein. In the absence of the retromandibular vein, the maxillary vein divided into anterior and posterior divisions. The posterior division joined the superficial temporal vein to form an atypical external jugular vein, and the anterior division joined the facial vein to form an anonymous vein. In clinical practice, radiologists and surgeons use the retromandibular vein as a guide to expose the branches of the facial nerve during superficial parotidectomy. Therefore, absence of the retromandibular vein is a hurdle during this procedure and may affect the venous drainage pattern from the head and neck.
Patil, Jyothsna; Swamy, Ravindra S.; D'Souza, Melanie R.; Guru, Anitha; Nayak, Satheesha B.
Epileptic seizures are thought to be generated and to evolve through an underlying anomaly of synchronization in the activity of groups of neuronal populations. The related dynamic scenario of state transitions is revealed by detecting changes in the dynamical properties of Electroencephalography (EEG) signals. The recruitment procedure ending with the crisis can be explored through a spatial-temporal plot from which to extract suitable descriptors that are able to monitor and quantify the evolving synchronization level from the EEG tracings. In this paper, a spatial-temporal analysis of EEG recordings based on the concept of permutation entropy (PE) is proposed. The performance of PE are tested on a database of 24 patients affected by absence (generalized) seizures. The results achieved are compared to the dynamical behavior of the EEG of 40 healthy subjects. Being PE a feature which is dependent on two parameters, an extensive study of the sensitivity of the performance of PE with respect to the parameters' setting was carried out on scalp EEG. Once the optimal PE configuration was determined, its ability to detect the different brain states was evaluated. According to the results here presented, it seems that the widely accepted model of "jump" transition to absence seizure should be in some cases coupled (or substituted) by a gradual transition model characteristic of self-organizing networks. Indeed, it appears that the transition to the epileptic status is heralded before the preictal state, ever since the interictal stages. As a matter of fact, within the limits of the analyzed database, the frontal-temporal scalp areas appear constantly associated to PE levels higher compared to the remaining electrodes, whereas the parieto-occipital areas appear associated to lower PE values. The EEG of healthy subjects neither shows any similar dynamic behavior nor exhibits any recurrent portrait in PE topography. PMID:23186273
Mammone, Nadia; Labate, Domenico; Lay-Ekuakille, Aime; Morabito, Francesco C
Cobalamin deficiency is associated with a wide spectrum of hematologic, neurologic, gastroenterologic and psychiatric disorders or symptoms. We report a case of a 50-year-old man with complex partial seizures with secondary generalization, mood oscillations and psychotic symptoms alternating with confusion and reversible dementia secondary to cobalamin deficiency in the absence of typical neurologic and/or hematologic symptoms and signs. Exclusion of epilepsy, acute, atrophic or expansive lesion of central nervous system and usual etiology associated with reversible dementia (infectious diseases, an endocrine etiology and deficiency of vitamins other than cobalamin); finding of cobalamin deficiency only and complete neuropsychiatric recovery after substitution, confirmed etiology. Typical and atypical psychiatric manifestations due to cobalamin deficiency that precede neurologic and/or hematologic signs and symptoms can recover completely after adequate replacement therapy. PMID:23697293
Vilibi?, Maja; Juki?, Vlado; Vidovi?, Andelko; Breci?, Petrana
A girl of borderline intelligence developed repeated syncopal attacks that were compulsively self-induced, apparently by forced expiration against a closed glottis (Valsalva's maneuver). In addition, she had typical absence seizures triggered by her apneic attacks, which could also be induced by hyperventilation. The absence seizures were suppressed by treatment with valproate sodium, which had no effect on the self-induced apneas. These were considerably improved with fenfluramine hydrochloride treatment, which was also associated with marked improvement of her previously abnormal behavior. PMID:3134879
Aicardi, J; Gastaut, H; Misès, J
The systemic injection of-hydroxybutyric acid (GHB) elicits spike and wave discharges (SWDs), the EEG hallmark of absence seizures, and represents a well established, widely used pharmacological model of this nonconvulsive epilepsy. Despite this experimental use of GHB, as well as its therapeutic use in narcolepsy and its increasing abuse, however, the precise cellular mechanisms underlying the different pharmacological actions of
Nicolas Gervasi; Zohreh Monnier; Pierre Vincent; Daniele Paupardin-Tritsch; Stuart W. Hughes; Vincenzo Crunelli; Nathalie Leresche
We have investigated whether the pathogenesis of spontaneous generalized non-convulsive seizures in rats with genetic absence epilepsy is due to an increase in the brain levels of gamma-hydroxybutyric acid (GHB) or in the rate of its synthesis. Concentrations of GHB or of its precursor gamma-butyrolactone (GBL) were measured with a new GC/MS technique which allows the simultaneous assessment of GHB and GBL. The rate of GHB synthesis was estimated from the increase in GHB levels after inhibition of its catabolism with valproate. The results of this study do not indicate significant differences in GHB or GBL levels, or in their rates of synthesis in rats showing spike-and-wave discharges (SWD) as compared to rats without SWD. Binding data indicate that GHB, but not GBL, has a selective, although weak affinity for GABAB receptors (IC50 = 150 microM). Similar IC50 values were observed in membranes prepared from rats showing SWD and from control rats. The average GHB brain levels of 2.12 +/- 0.23 nmol/g measured in the cortex and of 4.28 +/- 0.90 nmol/g in the thalamus are much lower than the concentrations necessary to occupy a major part of the GABAB receptors. It is unlikely that local accumulations of GHB reach concentrations 30-70-fold higher than the average brain levels. After injection of 3.5 mmol/kg GBL, a dose sufficient to induce SWD, brain concentrations reach 240 +/- 31 nmol/g (Snead, 1991) and GHB could thus stimulate the GABAB receptor. Like the selective and potent GABAB receptor agonist R(-)-baclofen, GHB causes a dose-related decrease in cerebellar cGMP. This decrease and the increase in SWD caused by R(-)-baclofen were completely blocked by the selective and potent GABAB receptor antagonist CGP 35348, whereas only the increase in the duration of SWD induced by GHB was totally antagonized by CGP 35348. The decrease in cerebellar cGMP levels elicited by GHB was only partially antagonized by CGP 35348. These findings suggest that all effects of R(-)-baclofen are mediated by the GABAB receptor, whereas only the induction of SWD by GHB is dependent on GABAB receptor mediation, the decrease in cGMP being only partially so. Taken together with the observations of Marescaux et al. (1992), these results indicate that GABAB receptors are of primary importance in experimental absence epilepsy and that GABAB receptor antagonists may represent a new class of anti-absence drugs. PMID:1324978
Bernasconi, R; Lauber, J; Marescaux, C; Vergnes, M; Martin, P; Rubio, V; Leonhardt, T; Reymann, N; Bittiger, H
Absence seizures are caused by brief periods of abnormal synchronized oscillations in the thalamocortical loops, resulting in widespread spike-and-wave discharges (SWDs) in the electroencephalogram (EEG). SWDs are concomitant with a complete or partial impairment of consciousness, notably expressed by an interruption of ongoing behaviour together with a lack of conscious perception of external stimuli. It is largely considered that the paroxysmal synchronizations during the epileptic episode transiently render the thalamocortical system incapable of transmitting primary sensory information to the cortex. Here, we examined in young patients and in the Genetic Absence Epilepsy Rats from Strasbourg (GAERS), a well-established genetic model of absence epilepsy, how sensory inputs are processed in the related cortical areas during SWDs. In epileptic patients, visual event-related potentials (ERPs) were still present in the occipital EEG when the stimuli were delivered during seizures, with a significant increase in amplitude compared to interictal periods and a decrease in latency compared to that measured from non-epileptic subjects. Using simultaneous in vivo EEG and intracellular recordings from the primary somatosensory cortex of GAERS and non-epileptic rats, we found that ERPs and firing responses of related pyramidal neurons to whisker deflection were not significantly modified during SWDs. However, the intracellular subthreshold synaptic responses in somatosensory cortical neurons during seizures had larger amplitude compared to quiescent situations. These convergent findings from human patients and a rodent genetic model show the persistence of cortical responses to sensory stimulations during SWDs, indicating that the brain can still process external stimuli during absence seizures. They also demonstrate that the disruption of conscious perception during absences is not due to an obliteration of information transfer in the thalamocortical system. The possible mechanisms rendering the cortical operation ineffective for conscious perception are discussed, but their definite elucidation will require further investigations.
Chipaux, Mathilde; Vercueil, Laurent; Kaminska, Anna
Absence seizures are caused by brief periods of abnormal synchronized oscillations in the thalamocortical loops, resulting in widespread spike-and-wave discharges (SWDs) in the electroencephalogram (EEG). SWDs are concomitant with a complete or partial impairment of consciousness, notably expressed by an interruption of ongoing behaviour together with a lack of conscious perception of external stimuli. It is largely considered that the paroxysmal synchronizations during the epileptic episode transiently render the thalamocortical system incapable of transmitting primary sensory information to the cortex. Here, we examined in young patients and in the Genetic Absence Epilepsy Rats from Strasbourg (GAERS), a well-established genetic model of absence epilepsy, how sensory inputs are processed in the related cortical areas during SWDs. In epileptic patients, visual event-related potentials (ERPs) were still present in the occipital EEG when the stimuli were delivered during seizures, with a significant increase in amplitude compared to interictal periods and a decrease in latency compared to that measured from non-epileptic subjects. Using simultaneous in vivo EEG and intracellular recordings from the primary somatosensory cortex of GAERS and non-epileptic rats, we found that ERPs and firing responses of related pyramidal neurons to whisker deflection were not significantly modified during SWDs. However, the intracellular subthreshold synaptic responses in somatosensory cortical neurons during seizures had larger amplitude compared to quiescent situations. These convergent findings from human patients and a rodent genetic model show the persistence of cortical responses to sensory stimulations during SWDs, indicating that the brain can still process external stimuli during absence seizures. They also demonstrate that the disruption of conscious perception during absences is not due to an obliteration of information transfer in the thalamocortical system. The possible mechanisms rendering the cortical operation ineffective for conscious perception are discussed, but their definite elucidation will require further investigations. PMID:23483991
Chipaux, Mathilde; Vercueil, Laurent; Kaminska, Anna; Mahon, Séverine; Charpier, Stéphane
We have conducted a course of magnetic laser therapy targeted on the vegetative structures at the neck of the children suffering absence seizures in an attempt to optimize the functioning of the non-specific brain structures. The study has demonstrated that such treatment promotes normalization of the components of the orientation response to sound almost to the level observed in the healthy children. The alpha-index returned to the normal value as well. PMID:24864485
Several signaling pathways are believed to be involved in the epileptogenic process that triggers the subsequent changes in the brain causing epilepsy. The mammalian target of rapamycin (mTOR) is a serine/threonine kinase that in the brain, regulates several important physiological functions such as neuronal development and synaptic plasticity, and also seems to be involved in many pathologies, including epilepsy and psychiatric disorders. Previous work in animal models of both genetic and acquired generalized convulsive epilepsies, has suggested that modulators of the mTOR signaling pathway may have beneficial neuroprotective and antiepileptogenic effects. Here, we investigated for the first time, the effect of some treatment schedules (i.e. early chronic, sub-chronic and acute) with the specific mTOR inhibitor rapamycin, on the development of absence seizures and seizure parameters as well as depressive-like behavior in WAG/Rij rats, a genetic model of absence epilepsy, epileptogenesis and mild-depression comorbidity. In addition, we studied the possible interaction between rapamycin treatment and the effects of bacterial lipopolysaccharide (LPS) endotoxin administration, which is known to aggravate absence seizures through generation of increased neuroinflammatory responses. We found that rapamycin (early chronic treatment for 17 weeks, starting at P45) exhibited clear antiepileptogenic properties also in this animal epilepsy model; however, this effect was accompanied by unexpected prodepressant effects. Both acute and sub-chronic (7 day) treatments also had anti-absence properties, but the sub-chronic treatment produced contrasting antidepressant properties in the WAG/Rij rats that were not seen in control Wistar rats. The rapamycin/LPS co-administration studies showed that rapamycin blocked or prevented the LPS-dependent increase in absence seizures, suggesting an anti-inflammatory-like protective action. In conclusion, we have demonstrated a novel antiepileptogenic effect of rapamycin in a well-established animal model of absence epilepsy, and we suggest that this effect may be mediated by the inhibition of inflammatory processes that are developed in the brain of these specific animals during epileptogenesis and during seizures. Our experiments here suggest new insights into this intriguing field, which deserves to be further explored. This article is part of the Special Issue entitled 'New Targets and Approaches to the Treatment of Epilepsy'. PMID:23092918
Russo, Emilio; Citraro, Rita; Donato, Giuseppe; Camastra, Caterina; Iuliano, Rodolfo; Cuzzocrea, Salvatore; Constanti, Andrew; De Sarro, Giovambattista
Atypical absence epilepsy (AAE) showing slow spike-and-wave discharges (SWD) is characterized by severely abnormal cognition and neurodevelopmental or neurological outcomes in humans. However, despite the severe behavioral outcomes in AAE, the relationship between AAE and social-behavioral dysfunctions has not defined well, either experimentally or in patients with AAE. Experimentally, AAE can be produced by administering AY-9944 (AY), a cholesterol biosynthesis inhibitor. In this study, we characterized social behavior in the AY mouse model of AAE. AAE in the mouse was induced by repeated postnatal administration of AY every 6 days from postnatal day (P) 2 to P20. AY-treated mice exhibited spontaneous, recurrent, and synchronous SWD (4-5 Hz) in electroencephalographic recordings. AY-treated mice performed tasks involving sociability/social novelty preference, social interaction with a juvenile conspecific, observational fear, and resident-intruder aggression. They showed behavioral dysfunction in social interactions with a juvenile conspecific and sociability/social novelty preference tasks. They also exhibited reduced social fear learning in observational fear conditioning. Interestingly, they showed increased levels of offensive behaviors in a resident-intruder task. However, AY-treated mice displayed normal levels of anxiety in light/dark transition and the elevated plus maze tasks, and showed slightly increased locomotor activity in an open-field task. These results demonstrate social dysfunction in the AY-induced AAE model. Our study of social behavior can also provide valuable information about Lennox-Gastaut syndrome, in which AAE is a component. Thus, our findings may help to understand behavioral pathogenesis or characteristics of patients with AAE. PMID:22944514
Jung, Seungmoon; Seo, Jin Soo; Kim, Byung Sun; Lee, Doheon; Jung, Keun-Hwa; Chu, Kon; Lee, Sang Kun; Jeon, Daejong
In the current study the link among the ?-hydroxybutyrate (GHB)/pentylenetetrazole (PTZ)-induced absence-like seizures and concomitant decreases in the core temperature, as well as electroencephalographic (EEG) activity during rewarming from deep hypothermia produced by a drug-free protocol were investigated. During the rewarming period after deep cooling, most Wistar rats suffered from bilaterally synchronous spike and waves with no or mild behavioral correlates. Spike and wave seizures were temperature-dependent and were initially registered when body temperature (Tb) reached 25-27°C, but mostly during the mild hypothermia of 0.3-1.3°C (Tb of 36.3-37.3°C). In chemical absence models, spike and wave discharges were also closely accompanied by mild systemic hypothermia, as both PTZ- and GHB-induced temperature decreases ranged from about 1-1.4°C respectively, together with EEG markers of absence activity. Thus, throughout the different experimental designs, the occurrence of spike and wave discharges was always related to a mild (0.3-1.4°C) decrease of Tb. Benzodiazepine diazepam as the GABAA-positive allosteric modulator and CGP 62349 as the selective antagonist of GABAB receptors were used to determine if their well-known anticonvulsant properties also affect hypothermia elicited by these drugs. Finally, during the course of spontaneous rewarming from deep hypothermia, another selective GABAB-blocking agent, CGP 35348, was used to elucidate if GABAB inhibitory system could be critically implicated in the generation of hypothermia-dependent spike and waves. Diazepam prevented both the PTZ-induced hypothermia and electrographic absence seizures, but these two beneficial effects did not occur in the GHB model. Even though diazepam delayed GHB-induced maximal temperature decrease, the GHB effects remained highly significant. The GABAB antagonist CGP 62349 completely prevented hypothermia as well as absence seizures in both chemical models. Likewise, spike and wave discharges, registered during the spontaneous rewarming from deep hypothermia, were completely prevented by CGP 35348. These findings show that systemic hypothermia should definitely be regarded as a marker of GABAB receptor activation. Moreover, the results of this study clearly show that initial mild temperature decrease should be considered as strong absence-provoking factor. Hypothermia-induced nonconvulsive seizures also highlight the importance of continuous EEG monitoring in children undergoing therapeutic hypothermia after cardiac arrest. Since every change in peripheral or systemic temperature ultimately must be perceived by preoptic region of the anterior hypothalamus as the primary thermoregulatory and sleep-inducing center, the preoptic thermosensitive neurons in general and warm-sensitive neurons in particular, simply have to be regarded as the most probable candidate for connected thermoregulatory and absence generating mechanisms. Therefore, additional studies are needed to confirm their potential role in the generation and propagation of absence seizures. PMID:23415784
Ostoji?, Z S; Ili?, T V; Veskovi?, S M; Andjus, P R
Falciparum malaria is notoriously known to produce life-threatening complications. Despite growing reports of chloroquine resistance and severe disease, vivax malaria continues to be viewed as a benign disease. We report a rare case of a 47-year-old healthy man from a malaria-endemic region, presenting with intracerebral bleed, right haemiparesis, aphasia and seizures following vivax malaria. This was successfully managed conservatively, without any neurosurgical intervention, with combination therapy of intravenous artesunate, oral hydroxychloquine and primaquine. In a country where Plasmodium vivax is responsible for majority of cases of malaria, it is high time the national malaria control programmes focus on the elimination of P. vivax in addition to its more dangerous counterpart, P. falciparum. PMID:24920515
Karanth, Suman S; Marupudi, Krishna Chaitanya; Gupta, Anurag
gamma-Hydroxybutyric acid (GHB), a naturally occurring compound which is synthesized from gamma-aminobutyric acid (GABA), induces bilaterally synchronous spike wave discharges, associated with behavioral changes, reminiscent of petit mal or generalized absence seizures in rats. In the present study, possible involvement of excitatory amino acids (EAAs) in GHB-induced spike wave discharges was investigated. The noncompetitive antagonist of NMDA receptors, MK-801, attenuated GHB-induced spike wave discharges at all doses tested (0.025-1.0 mg/kg) but dose-dependently induced suppression of EEG bursts in GHB-treated animals. The suppression of bursts was never observed with GHB in control experiments. N-Methyl-D-aspartate (NMDA) had a similar effect on GHB-induced spike wave discharges, when it was administered prior to GHB. This effect of NMDA was partially reversed by MK-801. The competitive antagonists of NMDA receptors, (+/-)CPP and CGP 43487 and the antagonist at the strychnine-insensitive glycine site, HA-966, also suppressed GHB-induced spike wave discharges with the EEG progressing to suppression of bursts but were weaker in this regard than MK-801 or NMDA. These data raise the possibility of involvement of excitatory amino acids in the GHB model of absence seizures. PMID:1436382
Banerjee, P K; Snead, O C
The mechanisms by which valproate, one of the most widely prescribed anti-epileptic drugs, suppresses seizures have not been fully elucidated but may involve up-regulation of neuropeptide Y (NPY). We investigated the effects of valproate treatment in Genetic Absence Epilepsy Rats from Strasbourg (GAERS) on brain NPY mRNA expression and seizure control. GAERS were administered either valproate (42 mg.kg?1 hr?1) or saline continuously for 5 days. Electroencephalograms were recorded for 24 hrs on treatment days 1, 3 and 5 and the percentage of time spent in seizure activity was analysed. NPY mRNA expression was measured in different brain regions using qPCR. Valproate treatment suppressed seizures by 80% in GAERS (p<0.05) and increased NPY mRNA expression in the thalamus (p<0.05) compared to saline treatment. These results demonstrate that long-term valproate treatment results in an upregulation of thalamic expression of NPY implicating this as a potential contributor to the mechanism by which valproate suppresses absence seizures.
van Raay, Leena; Dedeurwaerdere, Stefanie; O'Brien, Terence J.; Morris, Margaret J.
This study aimed to use ictal high-frequency oscillations (HFOs) ranging from 80Hz to 500Hz to locate seizure onset zones in childhood absence epilepsy (CAE) using non-invasive magnetoencephalography (MEG). Ten drug-naïve children with CAE were studied using a 275-channel MEG system. MEG data were digitized at a sampling rate of 6000Hz. HFO spectral power in real-time spectrograms was assessed using Morlet continuous wavelet transform. Magnetic sources were volumetrically localized through dynamic magnetic source imaging with a slide window. HFOs were identified in all patients. The total time of fast ripples (250-500Hz) was greater than that of ripples (80-250Hz) during absence seizures. The rate of fast ripples was associated with seizure frequency. HFO duration was significantly longer when co-occurring with spikes than when occurring independently, and the maximum frequency of HFOs co-occurring with spikes was higher than that of HFOs occurring independently. HFOs were predominantly localized in the medial prefrontal cortex (MPFC), whereas spikes were widespread to a variety of regions during the absence seizures. Compared with spikes, HFOs appeared to be more focal. The findings indicate that HFOs in the MPFC have a primary function in initializing epileptic activity in CAE. PMID:24582907
Miao, Ailiang; Xiang, Jing; Tang, Lu; Ge, Huaiting; Liu, Hongxing; Wu, Ting; Chen, Qiqi; Hu, Zheng; Lu, Xiaopeng; Wang, Xiaoshan
Neocortical networks play a major role in the genesis of generalized spike-and-wave (SW) discharges associated with absence seizures in humans and in animal models, including genetically predisposed WAG/Rij rats. Here, we tested the hypothesis that alterations in GABA(B) receptors contribute to neocortical hyperexcitability in these animals. By using Real-Time PCR we found that mRNA levels for most GABA(B(1)) subunits are diminished in epileptic WAG/Rij neocortex as compared with age-matched non-epileptic controls (NEC), whereas GABA(B(2)) mRNA is unchanged. Next, we investigated the cellular distribution of GABA(B(1)) and GABA(B(2)) subunits by confocal microscopy and discovered that GABA(B(1)) subunits fail to localize in the distal dendrites of WAG/Rij neocortical pyramidal cells. Intracellular recordings from neocortical cells in an in vitro slice preparation demonstrated reduced paired-pulse depression of pharmacologically isolated excitatory and inhibitory responses in epileptic WAG/Rij rats as compared with NECs; moreover, paired-pulse depression in NEC slices was diminished by a GABA(B) receptor antagonist to a greater extent than in WAG/Rij rats further suggesting GABA(B) receptor dysfunction. In conclusion, our data identify changes in GABA(B) receptor subunit expression and distribution along with decreased paired-pulse depression in epileptic WAG/Rij rat neocortex. We propose that these alterations may contribute to neocortical hyperexcitability and thus to SW generation in absence epilepsy. PMID:17207629
Merlo, D; Mollinari, C; Inaba, Y; Cardinale, A; Rinaldi, A M; D'Antuono, M; D'Arcangelo, G; Tancredi, V; Ragsdale, D; Avoli, M
Absence epilepsy, characterized by spike–wave discharges (SWD) in the electroencephalogram, arises from aberrations within the circuitry of the cerebral cortex and thalamus that regulates awareness. The inbred mouse strain C3H/HeJ is prone to absence seizures, with a major susceptibility locus, spkw1, accounting for most of the phenotype. Here we find that spkw1 is associated with a hypomorphic retroviral-like insertion mutation in the Gria4 gene, encoding one of the four amino-3-hydroxy-5-methyl-4isoxazolepropionic acid (AMPA) receptor subunits in the brain. Consistent with this, Gria4 knockout mice also have frequent SWD and do not complement spkw1. In contrast, null mutants for the related gene Gria3 do not have SWD, and Gria3 loss actually lowers SWD of spkw1 homozygotes. Gria3 and Gria4 encode the predominant AMPA receptor subunits in the reticular thalamus, which is thought to play a central role in seizure genesis by inhibiting thalamic relay cells and promoting rebound burst firing responses. In Gria4 mutants, synaptic excitation of inhibitory reticular thalamic neurons is enhanced, with increased duration of synaptic responses—consistent with what might be expected from reduction of the kinetically faster subunit of AMPA receptors encoded by Gria4. These results demonstrate for the first time an essential role for Gria4 in the brain, and suggest that abnormal AMPA receptor-dependent synaptic activity can be involved in the network hypersynchrony that underlies absence seizures.
Beyer, Barbara; Deleuze, Charlotte; Letts, Verity A.; Mahaffey, Connie L.; Boumil, Rebecca M.; Lew, Timothy A.; Huguenard, John R.; Frankel, Wayne N.
Angelman syndrome is a neurogenetic condition namely characterized by developmental delay, virtual absence of expressive verbal language, peculiar organization of movement, seizures and happy demeanor. This syndrome has been recognized since 1965, but it seems that Walt Disney presented an original depiction of it in his first full-length animated film, including myoclonic jerks and an apparently generalized tonic-clonic seizure. PMID:10452923
Dan, B; Christiaens, F
While temporal lobe epilepsy is often considered in the differential diagnosis of patients with anxiety or panic disorders, other types of epilepsy can confound the presentation or treatment of adults with panic disorders. The cases of two patients are presented who were initially thought to have temporal lobe epilepsy producing panic attacks, but who were subsequently found to have primary generalized seizures. The clinical implications are discussed.
McNamara, M E
Summary The distinction between typical absences and hypomotor seizures in patients having frontal lesions is difficult. In focal epilepsy, generalized-like interictal discharges can reflect either a coexistent generalized epileptic trait or a secondary bilateral synchrony. Using combined measures of the EEG and blood oxygenation level dependent (BOLD) activity, we studied a 50-year-old patient with both absence-like and symptomatic focal motor seizures. Focal activity induced activation in the lesional area and deactivation in the contralateral central cortex. Generalized spike-and-wave discharges (GSWDs) resulted also in perilesional activation, and multifocal symmetrical cortical and thalamic activations, and deactivation in associative cortical areas. Although the central cortex was involved during both types of epileptic activity, electroencephalography (EEG)–functional magnetic resonance imaging (fMRI) revealed distinct neuronal networks at the time of the focal or generalized discharges, allowing a clear-cut differentiation of the generators. Whether the patient had distinct epileptic syndromes or distinct electrographic patterns from the lesional trigger remains debatable.
Chassagnon, Serge; Hawko, Colin S.; Bernasconi, Andrea; Gotman, Jean; Dubeau, Francois
Seizure clusters, also known as repetitive or serial seizures, occur commonly in epilepsy. Clustering implies that the occurrence of one seizure may influence the probability of a subsequent seizure; thus, the investigation of the clustering phenomenon yields insights into both specific mechanisms of seizure clustering and more general concepts of seizure occurrence. Seizure clustering has been defined clinically as a
Sheryl R. Haut
Medically-refractory seizures cause inflammation and neurodegeneration. Seizure initiation thresholds have been linked in mice to the serine protease tissue plasminogen activator (tPA); mice lacking tPA exhibit resistance to seizure induction, and the ensuing inflammation and neurodegeneration are similarly suppressed. Seizure foci in humans can be examined using PET employing 2-deoxy-2[18F]fluoro-D-glucose (18FDG) as a tracer to visualize metabolic dysfunction. However, there currently exist no such methods in mice to correlate measures of brain activation with behaviour. Using a novel method for small animal PET data analysis, we examine patterns of 18FDG uptake in wild type and tPA-/- mice and find that they correlate with the severity of drug-induced seizure initiation. Furthermore, we report unexpected activations that may underlie the tPA modulation of seizure susceptibility. The methods described here should be applicable to other mouse models of human neurological disease.
Mirrione, Martine M.; Schiffer, Wynne K.; Fowler, Joanna S.; Alexoff, Dave L.; Dewey, Stephen L.; Tsirka, Stella E.
Epilepsy affects 0.5% to 1% of the population and can cause focal, partial, generalized, and absence seizures and several unusual types. Seizure disorders often begin in childhood and are treated with a variety of pharmacologic or surgical interventions for those refractory to medical therapy. Functional imaging, with both PET and single-photon emission CT (SPECT), has been highly useful in the
Andrew B. Newberg; Abass Alavi
Focal seizure - discharge; Jacksonian seizure - discharge; Seizure - partial (focal) - discharge; TLE - discharge; Seizure - temporal lobe - discharge; Seizure - tonic-clonic - discharge; Seizure - grand mal - discharge; Grand mal seizure - discharge; Seizure - generalized - discharge
The aim of the study was to assess the efficacy, tolerability, and safety of levetiracetam therapy in children and adolescents with absence epilepsy. Twenty-one participants (11 male, 10 female) with typical absence seizures were enrolled in this prospective study from seven centres in Italy. The mean age and age range at time of enrollment into the study were 8 years 9 months (SD 0.9) and 5 years 1 month to 13 years respectively. All patients were carefully evaluated at 6 months from baseline, and 12 patients were also re-evaluated at 12 months after the beginning of therapy with levetiracetam. At the 6-month evaluation, out of 21 patients studied, 11 were seizure free and one showed 'decreased' seizures (more than 50% reduction in seizures). A less than 50% reduction in seizures was observed in nine patients. At the 12-month evaluation, 10 patients were completely seizure free and two were seizure free with some anomalies in electroencephalograms. Two patients who had shown no improvement at 6 months had decreased seizures at the second follow-up. Our results suggest that monotherapy with levetiracetam could be effective and well tolerated in patients with childhood absence epilepsy and juvenile absence epilepsy. Prospective, large, long-term double-blind studies are needed to confirm these findings. PMID:18808424
Verrotti, Alberto; Cerminara, Caterina; Domizio, Sergio; Mohn, Angelika; Franzoni, Emilio; Coppola, Giangennaro; Zamponi, Nelia; Parisi, Pasquale; Iannetti, Paola; Curatolo, Paolo
Among nonsymptomatic epilepsies exhibiting several types of generalized seizures in children two syndromes were progressively identified: epilepsy with myoclonic-astatic seizures (MAE) and nonsymptomatic Lennox-Gastaut syndrome (LGS). Various approaches based on etiology, electroclinical semiology, and mathematical analysis have progressively helped to distinguish these two conditions. Both conditions preferentially affect boys. The course is stereotyped in MAE, characterized by progressive worsening of epilepsy, usual pharmacoresistance at onset and tonic-clonic seizures, myoclonus and frequent episodes of myoclonic status epilepticus. EEG shows 3Hz spike wave bursts characteristic of idiopathic generalized epilepsy together with slowing of the tracing. In LGS, major seizures are mainly atypical absences and tonic seizures with 0.5-2Hz slow spike-waves and eventually focal anomalies. Prognosis in both syndromes ranges from recovery without sequelae to pharmacoresistant epilepsy that has improved over the past 2 decades with the new generation antiepileptic compounds. Iatrogenic factors may contribute to the poor prognosis, mainly in MAE. Pathophysiology remains speculative for both syndromes: although both share factors of brain maturation, MAE is probably mainly related to genetic predisposition whereas LGS results from some unidentified cortical brain malformation. In unfavorable cases, there may therefore be a continuum between both syndromes. They need to be distinguished from other epilepsy syndromes and inborn errors of metabolism that begin in the same age range: atypical idiopathic benign epilepsy, frontal lobe epilepsy with secondary bisynchrony, ring chromosome 20, ceroid lipofuscinosis, and nonsymptomatic late-onset spasms. PMID:23622212
Kaminska, Anna; Oguni, Hirokazu
Neural mechanisms underlying convulsive events are believed to be distinctly different from those of absence seizures.1 Typical absence epilepsy has been suggested to be related to a predominance of inhibitory activity, in contrast to generalized\\u000a or focal convulsive seizures where an excess of excitatory activity is present.2 Likewise, drugs that exacerbate seizure activity, clinically and in animal models, support the
Filiz Yilmaz Onat; Esat E?kazan; Rezzan Aker
We report pilomotor seizures in two patients who had piloerection or gooseflesh spreading in a pattern like the 'Jacksonian march', confined ipsilaterally to the lesion side. The first patient presented this in conjunction with complex partial seizures. Left anterior temporal lobectomy abolished pilomotor and other seizures. Pathological study demonstrated hippocampal sclerosis. A meningioma near the left sphenoid region, incompletely removed, is the likely cause of pilomotor seizures in the second patient. Carbamazepine reduced these attacks. To our knowledge, hippocampal sclerosis and meningioma have never been linked to pilomotor seizures. PMID:9693227
Yu, H; Yen, D; Yiu, C; Chung, W; Lirng, J; Su, M
We showed previously that the number and time of spike-wave discharges (SWDs) were increased after intraperitoneal (i.p.) injection of lipopolysaccharide (LPS), an effect, which was completely abolished by cyclooxygenase-2 (COX-2) inhibitor indomethacin (IND) pretreatment in Wistar Albino Glaxo/Rijswijk (WAG/Rij) rats. These and other results suggest that injection of LPS to genetically absence epileptic animals, such as WAG/Rij rats, may allow us to investigate relationships between absence epilepsy and LPS evoked neuroinflammation processes. However, LPS may evoke different effects on absence epileptic activity in various animal strains. Thus, to extend our previous results, we injected two doses of LPS (50?g/kg and 350?g/kg i.p.) alone and in combination with IND (10mg/kg IND i.p. +50?g/kg LPS) into rats of two model animal strains (WAG/Rij rats; GAERS rats: Genetic Absence Epileptic Rats from Strasbourg) and into Long Evans rats. The effects of treatments on SWD number and SWD duration were examined. Both doses of LPS increased the SWD number and the total time of SWDs dose-dependently during the whole 4-h recording period, which was abolished by IND pretreatment in all three investigated strains. These results extend our previous results suggesting that our methods using LPS injection into freely moving absence epileptic rats is applicable not only in well-established animal models of absence epilepsy such as WAG/Rij rats and GAERS rats but also in Long Evans rats to investigate links between inflammation and absence epilepsy. PMID:24704320
Kovács, Zsolt; Dobolyi, Arpád; Juhász, Gábor; Kékesi, Katalin A
Poisoning with many xenobiotics may result in seizures, which are usually generalized. Seizures leading to unexplained disturbances of consciousness are usually generalized tonic-clonic, absence seizures and complex partial seizures Prolonged seizures without recovery of consciousness during interictal period are called status epilepticus. This paper presents the etiology, patomechanism, clinical characteristics, as well as diagnostic and therapeutic principles of toxic seizures. Additionally, the clinical description of nonepileptic psychogenic seizures is given, which often need to be differentiated with true seizures. PMID:24466702
Ciszowski, Krzysztof; Mietka-Ciszowska, Aneta
The aim of the study was to assess the efficacy, tolerability, and safety of levetiracetam therapy in children and adolescents with absence epilepsy. Twenty-one participants (11 male, 10 female) with typical absence seizures were enrolled in this prospective study from seven centres in Italy. The mean age and age range at time of enrolment into…
Verrotti, Alberto; Cerminara, Caterina; Domizio, Sergio; Mohn, Angelika; Franzoni, Emilio; Coppola, Giangennaro; Zamponi, Nelia; Parisi, Pasquale; Iannetti, Paola; Curatolo, Paolo
Atypical moles can be distinguished visually by clinical features of size greater than 6 mm in diameter, color variegation, indistinct borders, and textured surface. All patients who have atypical moles should be counselled about sun avoidance, screening of family members, and regular skin checks at least once per year. Total body photography and dermoscopy can aid in regular skin monitoring for changes in atypical moles and the emergence of new lesions. The presence of multiple atypical moles increases the risk of melanoma. The greatest risk of melanoma is in patients who have more than 50 atypical moles and two or more family members with melanoma (familial atypical mole and melanoma syndrome). Atypical moles should be removed when they have features suggestive of malignant transformation. Elliptical excision is the preferred removal technique. Removing all atypical moles is neither necessary nor cost effective. PMID:18819240
Cyr, Peggy R
Psychogenic seizures are nonepileptic events that are varied in their clinical presentations. These events can be psychiatric or physiologic in their origin and can occur concomitantly in patients with epileptic seizure disorders. The diagnosis often requires laboratory and electrophysiologic investigation; however, historical, clinical, and laboratory findings can aid in making the diagnosis. PMID:7956884
Generalised-onset absence seizures can be resistant to treatment with currently available antiepileptic drugs. Ezogabine (retigabine), a potassium channel opener, is approved for the treatment of focal-onset seizures. This is a case report of an adult with childhood absence epilepsy whose daily absence seizures ceased with adjunctive ezogabine. A 59-year-old woman, with a history of typical absence seizures since the age of 6 years, had multiple seizures daily despite trials of over 11 antiepileptic drugs. While taking lamotrigine and zonisamide, ezogabine at 50 mg daily was added. The dose was slowly increased and once a total dose of only 200 mg/day was reached, she became seizure-free for three months. After subsequently discontinuing zonisamide, absence seizures returned. Further increasing the ezogabine to 400 mg/day, in addition to lamotrigine, did not restore seizure freedom, but adding back zonisamide at half dose again reduced their frequency. Ezogabine at low dose, added to lamotrigine and zonisamide, led to sustained absence seizure freedom. The return of seizures after zonisamide discontinuation suggests that the seizure freedom may have been the result of the different mechanisms of action of the antiepileptic drugs. PMID:24659629
Vossler, David G; Yilmaz, Ugur
Psychogenic nonepileptic seizures (PNES) are clinical events resembling epileptic seizures but lacking abnormal cortical electrical discharges. They are involuntary manifestations of a psychological distress. PNES are less frequent in the pediatric population than in adults, they represent from 3.5 to 9% of patients admitted for prolonged video-EEG (PV-EEG). Diagnosis is rarely made on history only and PV-EEG is mandatory to obtain a definitive diagnosis. Children as young as 5 years can present with PNES. They are more frequent in girls except in school age children where boys are identically or more represented than girls. PNES can either present with subtle signs, even unresponsiveness, or prominent motor activity. Major differential diagnosis is absences, day dreaming, and complex partial seizures including hyperkinetic frontal seizures. PNES are usually rapidly registered during PV-EEG and provocative methods have not been thoroughly studied in children. Major risk factors are psychological stressors, such as school or family problems. Psychiatric conditions are less frequent than in adults though they should be looked for. Prognosis is better than in adults, and most children become PNES-free. There are no guidelines for treatment, however stressors should be addressed. In general, it should be clearly explained that PNES are not epileptic seizures. PMID:23622297
Hypoglycaemia can lead to acute disorders of cognition, consciousness, epilepsy, transient ischemia, psychosis and chronic disorders of dementia and neuropathy. Misdiagnosis and delay in treatment are common and prolonged hypoglycemia can lead to permanent neurological deficit or fatal coma. Hypoglycemia caused by an insulinoma is a readily treatable condition that should be considered in the differential diagnosis of intractable seizures. The following case report highlights the need for careful reassessment of all seizures that are atypical and refractory to medication.
The clinical manifestations of seizures change in a predictable fashion with advancing age. For focal seizures these changes can be summarized into domains similar to those used in developmental models. These include fine motor, communication, and gross motor manifestations. Instead of socialization the fourth domain for seizure semiology concerns synchronization. Focal seizures in the very young tend to be simpler with fewer fine motor manifestations. Auras are uncommon, even in young children with some linguistic skill and it is often difficult to discern alteration of consciousness. Infantile focal seizures can present with spasms or even diffuse tonic seizures. In terms of synchronization, orderly secondary generalization is rarely seen so that primary generalized clonic seizures are rarely recorded in infants. Amongst so-called "generalized" seizures spasms are most often seen in the first year of life. Absence seizures, myoclonic-astatic and generalized tonic-clonic seizures are all usually not seen until after age 2 years. A full description of the clinical details of seizures is probably the most important part of the epilepsy history. A detailed knowledge of seizure semiology can make the history more effective and also in the identification of the correct seizure classification. PMID:23622194
Nordli, Douglas R
Seizure clusters, also known as repetitive or serial seizures, occur commonly in epilepsy. Clustering implies that the occurrence of one seizure may influence the probability of a subsequent seizure; thus, the investigation of the clustering phenomenon yields insights into both specific mechanisms of seizure clustering and more general concepts of seizure occurrence. Seizure clustering has been defined clin- ically as
Sheryl R. Haut
\\u000a Working with children with epilepsy is like reading a good mystery. When learning or emotional problems occur, the neuropsychologist\\u000a 's job is to help identify “whodunit.” The list of likely suspects from the epilepsy perspective include whatever is atypical\\u000a about the brain that is the basis of the seizures, seizures themselves, abnormal electrical discharges between seizures (also\\u000a known as sub-clinical
Lynn Bennett Blackburn
In a cohort of 59 consecutive children referred for staring spells, we analysed clinical and electroencephalogram (EEG) characteristics in 23 children with both a history of absence seizures and a generalized spike and wave pattern during long-term video EEG monitoring. In 10 children, a frontal spike preceded the generalized spike and wave pattern. In the remaining 13 children, primary generalized
L Lagae; J Pauwels; B Verhelle; J Vervisch
This report describes a sibling pair (ages 21 and 18), both with tuberous sclerosis. One sibling has atypical autism (but no mental retardation or seizure disorder) and the other has a seizure disorder but no autism or mental retardation. Both siblings had multiple bilateral brain lesions. Clinical findings are discussed in relationship to the…
Williamson, David A.; Bolton, Patrick
We sought to determine whether patterns of ictal hyperfusion demonstrated using [99mTC]HMPAO (hexamethylpropylene amine oxime) single photon emission computed tomography (SPECT) predict outcome of temporal lobectomy; in particular, whether the more extensive patterns of ictal hyperperfusion are associated with poor outcome. We studied 63 patients who had ictal SPECT studies prior to temporal lobectomy. Hyperperfusion on ictal SPECT scans was lateralized, and classified into: (i) 'typical', (ii) 'typical with posterior extension', (iii) 'bilateral' and (iv) 'atypical' patterns. Outcome (minimum of 2 years follow-up) was classified as either seizure free, or not seizure free. Actuarial analysis was used to test the relationship of SPECT patterns with outcome. There were 35 cases with the typical ictal SPECT pattern, 13 posterior, nine bilateral and six atypical cases. The atypical pattern was associated with lack of pathology in the surgical specimen. Outcome was similar for the typical, posterior and bilateral with 60%, 69% and 67% seizure free, respectively. In contrast, the atypical group had a worse outcome with only 33% seizure free. Actuarial analysis showed a significant difference in outcome between patients with the typical pattern, and patients with the atypical pattern (P = 0.04). We conclude that extended patterns of ictal perfusion in temporal lobe epilepsy do not predict poor outcome, indicating that extended hyperperfusion probably represents seizure propagation pathways rather than intrinsically epileptogenic tissue. Atypical patterns of hyperperfusion are associated with poor outcome and may indicate diffuse or extra-temporal epileptogenicity. PMID:9397011
Ho, S S; Newton, M R; McIntosh, A M; Kalnins, R M; Fabinyi, G C; Brazenor, G A; McKay, W J; Bladin, P F; Berkovic, S F
This article describes how an implantable device could greatly improve the quality of life for people with epilepsy. Gabe Anderson was diagnosed with bilateral heterotopia, a congenital condition that can lead to the onset of complex partial seizures stemming from both hemispheres of the brain. In early 2004, Gabe became one of the first 35…
Brain carbonic anhydrases (CAs) are known to modulate neuronal signalling. Using a novel CA VII (Car7) knockout (KO) mouse as well as a CA II (Car2) KO and a CA II/VII double KO, we show that mature hippocampal pyramidal neurons are endowed with two cytosolic isoforms. CA VII is predominantly expressed by neurons starting around postnatal day 10 (P10). The ubiquitous isoform II is expressed in neurons at P20. Both isoforms enhance bicarbonate-driven GABAergic excitation during intense GABAA-receptor activation. P13-14 CA VII KO mice show behavioural manifestations atypical of experimental febrile seizures (eFS) and a complete absence of electrographic seizures. A low dose of diazepam promotes eFS in P13-P14 rat pups, whereas seizures are blocked at higher concentrations that suppress breathing. Thus, the respiratory alkalosis-dependent eFS are exacerbated by GABAergic excitation. We found that CA VII mRNA is expressed in the human cerebral cortex before the age when febrile seizures (FS) occur in children. Our data indicate that CA VII is a key molecule in age-dependent neuronal pH regulation with consequent effects on generation of FS. PMID:23881097
Ruusuvuori, Eva; Huebner, Antje K; Kirilkin, Ilya; Yukin, Alexey Y; Blaesse, Peter; Helmy, Mohamed; Kang, Hyo Jung; El Muayed, Malek; Hennings, J Christopher; Voipio, Juha; Šestan, Nenad; Hübner, Christian A; Kaila, Kai
Brain carbonic anhydrases (CAs) are known to modulate neuronal signalling. Using a novel CA VII (Car7) knockout (KO) mouse as well as a CA II (Car2) KO and a CA II/VII double KO, we show that mature hippocampal pyramidal neurons are endowed with two cytosolic isoforms. CA VII is predominantly expressed by neurons starting around postnatal day 10 (P10). The ubiquitous isoform II is expressed in neurons at P20. Both isoforms enhance bicarbonate-driven GABAergic excitation during intense GABAA-receptor activation. P13–14 CA VII KO mice show behavioural manifestations atypical of experimental febrile seizures (eFS) and a complete absence of electrographic seizures. A low dose of diazepam promotes eFS in P13–P14 rat pups, whereas seizures are blocked at higher concentrations that suppress breathing. Thus, the respiratory alkalosis-dependent eFS are exacerbated by GABAergic excitation. We found that CA VII mRNA is expressed in the human cerebral cortex before the age when febrile seizures (FS) occur in children. Our data indicate that CA VII is a key molecule in age-dependent neuronal pH regulation with consequent effects on generation of FS.
Ruusuvuori, Eva; Huebner, Antje K; Kirilkin, Ilya; Yukin, Alexey Y; Blaesse, Peter; Helmy, Mohamed; Jung Kang, Hyo; El Muayed, Malek; Christopher Hennings, J; Voipio, Juha; Sestan, Nenad; Hubner, Christian A; Kaila, Kai
In this creative challenge, Surrealism and one-point perspective combine to produce images that not only go "beyond the real" but also beyond the ubiquitous "imaginary city" assignment often used to teach one-point perspective. Perhaps the difference is that in the "atypical cities challenge," an understanding of one-point perspective is a means…
The rare forms of migraine should be studied so that they can be recognized, thereby avoiding diagnostic errors and unnecessary complementary investigations. These rare forms can be divided into three categories: 1) atypical auras, characterized by the following: their semiology (visual or sensory illusions or hallucinations); their manner of onset (sudden onset of aura, with development of migraine in under four minutes); their duration (aura prolonged for between 60 minutes and seven days); aura with absence of accompanying cephalgia. This form of migraine could be confused with a case of partial epilepsy or AIT and raises a problem of differential diagnosis requiring an etiological investigation to ensure correct diagnosis; 2) clinical forms which are for the most part recognized by the International Headache Society's (IHS) classification, i.e., basilar artery migraine, familial hemiplegic migraine, ophthalmoplegic migraine and the rare occurrence of retinal migraine. Confusional migraine, a disorder which is mainly encountered in childhood, has not been categorized by the IHS but may be part of the symptomatology of the above-described forms; and 3) secondary migraines, in which a triggering mechanism causes migraine attacks to appear in a subject who was not previously affected by this disorder. This category covers post-traumatic migraine, migraine of cervical origin, the co-morbidity of migraine and epileptic seizures, and rare cases of symptomatic migraine which are indicative of underlying general disease or an intracranial lesion, i.e., a tumor or arteriovenous malformation. PMID:11072645
Early-onset absence epilepsy refers to patients with absence seizures beginning before age 4 and comprises a heterogeneous group of epilepsies. Onset of absence seizures in the first year of life is very rare. We report a boy with absence seizures with onset at age 11 months, whose seizures increased in frequency after the introduction of valproic acid (VPA) treatment and substantially improved upon cessation of treatment. The mechanism of seizure worsening did not involve VPA toxicity, encephalopathy, Glut-1 deficiency or overdosage, and the reason for absence seizure aggravation remained unclear. The patient showed complete control of absence seizures with levetiracetam treatment and the course was benign, both in terms of seizure control and neuropsychological aspects. The similar overall electroclinical picture and outcome between children with early-onset absences and those with CAE support the view that these conditions are a continuum within the wide spectrum of IGE. [Published with video sequences]. PMID:24169439
Belcastro, Vincenzo; Caraballo, Roberto Horacio; Romeo, Antonino; Striano, Pasquale
Occasionally, but more often than has been reported, true epileptic seizures are triggered by non-epileptic syncopes. This combination of syncope and epileptic seizure has been called an anoxic-epileptic seizure. A few examples of such anoxic-epileptic seizures, including the induction of status epilepticus, have been reported in books and medical journals, but no video-recordings have been published. We show here home video recordings of the first three known examples of the transition from the triggering syncope and anoxic seizure, to the subsequent epileptic seizure. In the first two children, a neurally-mediated syncope, probably mediated by prolonged expiratory apnoea (so-called breath-holding spells), induces a long, clonic epileptic seizure with some features of myoclonic absence. In the third example, a compulsive Valsalva in an older autistic child provokes a vibratory tonic epileptic seizure. In addition, we show two further video clips of the most usual type of epileptic seizure induced by syncopes in very young children. In one, the video recording begins after the end of the triggering syncope and shows a rhythmic clonic seizure that includes repetitive vocalizations. The final recoding is of a spontaneous epileptic seizure with features of myoclonic absence: this child had both epilepsy and identical episodes induced by syncopes, that is, anoxic- epileptic seizures. Not only paediatricians and paediatric neurologists, but also adult neurologists and epileptologists in general, should be aware of the important clinical scenario of true epileptic seizures induced by syncopes. This phenomenon is not considered in any international classification. (Published with videosequences) PMID:15075063
Stephenson, John; Breningstall, Galen; Steer, Chris; Kirkpatrick, Martin; Horrocks, Iain; Nechay, Alla; Zuberi, Sameer
We analyzed the frequency and morphological characteristics of the initial EEG manifestations of spontaneous seizures recorded from depth and subdural electrodes in 26 patients for whom pathological analysis of the area of seizure onset was available after resective surgery. Pathological features considered to be positive findings included well-defined structural lesions (hamartoma, neoplasm) or strictly defined mesial temporal sclerosis. Seizure onset was characterized by the frequency of the rhythmic discharge greater than 2 Hz in the first second and by the presence or absence of periodic low-frequency spikes (less than 2 Hz) preceding this stable change in background frequency. These features were correlated with the presence or absence of pathologic abnormalities in temporal and extratemporal locations. Although all patterns and frequencies of seizure onset were recorded in both medial temporal and extratemporal locations, medial temporal seizure onset was significantly more likely to have high frequency (greater than 13 Hz, p less than 0.00001) and tended to show periodic spikes prior to the seizure when it was associated with medial temporal sclerosis compared to when it was not. Extratemporal seizure onset associated with abnormal pathological substrate was significantly more likely to have a lower frequency (less than 13 Hz, p less than 0.05) and no periodic spikes before seizure onset (p less than 0.00001) than extratemporal seizure onset recorded from areas without pathological findings. Variability of seizure onset frequency was a characteristic of temporal, but not extratemporal, seizures (p less than 0.01).(ABSTRACT TRUNCATED AT 250 WORDS) PMID:1592034
Spencer, S S; Guimaraes, P; Katz, A; Kim, J; Spencer, D
The authors used /sup 18/F-fluorodeoxyglucose (FDG) positron emission tomography (PET) to study nine patients with clinical absence or generalized seizures. One patient had only absence seizures, two had only generalized tonic-clonic seizures, and six had both seizure types. Interictal scans in eight failed to reveal focal or lateralized hypometabolism. No apparent abnormalities were noted. Two patients had PET scans after isotope injection during hyperventilation-induced generalized spike-wave discharges. Diffusely increased metabolic rates were found in one compared with an interictal scan, and in another compared with control values. Another patient had FDG injected during absence status: EEG showed generalized spike-wave discharges (during which she was unresponsive) intermixed with slow activity accompanied by confusion. Metabolic rates were decreased, compared with the interictal scan, throughout both cortical and subcortical structures. Interictal PET did not detect specific anatomic regions responsible for absence seizure onset in any patient, but the results of the ictal scans did suggest that pathophysiologic differences exist between absence status and single absence attacks.
Theodore, W.H.; Brooks, R.; Margolin, R.; Patronas, N.; Sato, S.; Porter, R.J.; Mansi, L.; Bairamian, D.; DiChiro, G.
Most parents are used to erratic sleep patterns and mood swings in their teenagers. When these occur in an adolescent with seizures, however, the parent may wonder if sleep and mood problems are related to seizures. Sorting out the cause and effects of sleep in an adolescent with seizures can be confusing. Since stress can be a contributor to both…
Shafer, Patricia Osborne
Knowledge concerning the relationship between multiple sclerosis and epilepsy is reviewed. Epidemiological studies have established\\u000a that epileptic seizures are more frequent in multiple sclerosis than predicted by chance. Partial epilepsies with focal seizures\\u000a often with atypical symptoms and with or without secondary generalisation are the usual pattern. In the survey special emphasis\\u000a is laid on the direct correlation between paroxysmal
Josef Spatt; Robert Chaix; Bruno Mamoli
The budding bacteria, Pirellula and Planctomyces , with atypical 16S rRNA and absence of peptidoglycan, show eubacterial phospholipids and uniquely high proportions of long chain beta-hydroxy fatty acids in the lipopolysaccharide lipid A
Fatty acids of twelve strains of budding bacteria (Planctomyces and Pirellula spp.), which have atypical 16S rRNA and do not contain peptidoglycan cell walls, were shown to contain typical diacyl polar lipids with no indication of isoprenoid ether lipids suggestive of a relationship with the archaebacteria. The major ester-linked fatty acids of the phospholipids were palmitic, palmitoleic and oleic acids,
B. D. Kerger; C. A. Mancuso; P. D. Nichols; D. C. White; T. Langworthy; M. Sittig; H. Schlesner; P. Hirsch
This paper examines the practice of search and seizure from a legal perspective. All issues concerning lawful or unlawful search and seizure, whether in a public school or otherwise, are predicated upon the Fourth Amendment to the United States Constitution. The terms "search,""seizure,""probable cause,""reasonable suspicion," and "exclusionary…
Murray, Kenneth T.
Most medical therapies for epilepsy consist of daily (or multiple-daily) dose, fixed-schedule, pharmacologic oral agents. Despite adherence, many patients continue to experience seizures. Various products have been discovered, designed, and marketed to serve as seizure-abortant therapies. These agents can be administered rapidly, as a "rescue" therapy, once a clinical seizure or cluster of seizures starts. Rescue medications are given as needed in an attempt to disrupt progression of a given seizure, and forestall what would otherwise be a more prolonged or more severe clinical event. Seizure-abortants also serve to aid in the management of seizure emergencies, such as prolonged, repetitive seizures, or status epilepticus. These compounds are not appropriate for all patients. Nevertheless, they do provide therapeutic benefit to several groups of patients: 1) those who perceive the onset of their seizures and have time to perform a self-intervention, 2) patients' caregivers who administer the therapy when they witness the onset of an ictal event, and 3) patients who are in the midst of an out-of-the-hospital seizure emergency (a seizure cluster or status epilepticus). In this article we will review currently available and future rescue therapies for seizures: US Food and Drug Administration (FDA) approved and FDA nonapproved drugs, nonpharmacologic behavioral treatments, the vagus nerve stimulator and the NeuroPace RNS® System (Mountain View, CA). PMID:21509498
Poukas, Valeriya S; Pollard, John R; Anderson, Christopher Todd
Fifteen patients who experienced epileptic seizures while playing video games are described together with a review of 20 cases in the English literature. Nine of the 15 cases and all but two of the reported cases experienced their first seizure while playing video games. Two thirds of patients had idiopathic generalised epilepsy and mainly reported generalised tonic clonic seizures, but
C D Ferrie; P De Marco; R A Grünewald; S Giannakodimos; C P Panayiotopoulos
The clinical differentiation between epileptic seizures (ES) and non-epileptic seizures (NES) is often difficult and mostly based on the presence or absence of widely recognized features of ES such as tongue biting, falling, incontinence or concomitant epileptic abnormalities in the electroencephalogram (EEG). We retrospectively analysed the records of all patients referred to our Epilepsy Centre for refractory epilepsy and finally
P de Timary; P Fouchet; M Sylin; J. P Indriets; T de Barsy; A Lefèbvre; K van Rijckevorsel
A 69-year-old man with alcohol dependence and mild cognitive impairment (MCI) suffered from repeated tonic-clonic seizures. The seizures typically occurred several hours after his last alcohol intake at home (early withdrawal seizure) and 22 days after his last intake of alcohol (14 days after the last dose of diazepam substituting for alcohol: late withdrawal seizure) on the ward. Psychiatrists in charge of this patient found it difficult to attribute his seizures to alcohol withdrawal syndrome (AWS) because of the atypical onset. The patient responded to diazepam resumption and valproate combination. This case highlights the need to always consider AWS as a possible cause of seizures and to gradually decrease diazepam as a substitute for alcohol. Moreover, in this patient, MCI may have induced vulnerability in the brain for AWS and the patients' older age might have decreased liver function leading to delayed onset of the seizures after diazepam withdrawal. PMID:24272988
Ishii, Nobuyoshi; Terao, Takeshi; Araki, Yasuo; Hatano, Koji
Seizures frequently affect the heart rate and rhythm. In most cases, seizure-related cardiac changes are transient and do not appear to cause clinically significant abnormalities for the patient. Great interest in this area of research has been generated because of a possible connection with sudden unexpected death in epilepsy (SUDEP). While there are clear, but rare complications from seizure-related cardiac arrhythmias, such as ictal asystole that causes syncope, the overall risk of seizures on cardiac status and any potential connection between seizures and SUDEP still remain uncertain.
SUMMARY Although often overshadowed by factors influencing seizure initiation, seizure termination is a critical step in the return to the interictal state. Understanding the mechanisms contributing to seizure termination could potentially identify novel targets for anticonvulsant drug development and may also highlight the pathophysiological processes contributing to seizure initiation. In this article, we review known physiological mechanisms contributing to seizure termination and discuss additional mechanisms that are likely to be relevant even though specific data are not yet available. This review is organized according to successively increasing “size scales”—from membranes to synapses to networks to circuits. We first discuss mechanisms of seizure termination acting at the shortest distances and affecting the excitable membranes of neurons in the seizure onset zone. Next we consider the contributions of ensembles of neurons and glia interacting at intermediate distances within the region of the seizure onset zone. Lastly, we consider the contribution of brain nuclei, such as the substantia nigra pars reticulata (SNR), that are capable of modulating seizures and exert their influence over the seizure onset zone (and neighboring areas) from a relatively great—in neuroanatomical terms—distance. It is our hope that the attention to the mechanisms contributing to seizure termination will stimulate novel avenues of epilepsy research and will contribute to improved patient care.
Lado, Fred A.; Moshe, Solomon L.
A 75 years old man is suffering from recurrent seizures since several years. The seizures start with vegetative symptoms including tingling sensations, followed by a deep feeling of eternity and happiness. In one third of the cases he looses consciousness completely. Several workups have failed to reveal a diagnosis. An ECG loop recorder was implanted which finally revealed an asystole of 15 seconds duration. It was thought that this caused cerebral hypoxia which was triggering focal seizures. A pacemaker was inserted. The attacks of unconsciousness disappeared; however, the spells of vegetative sensations persisted. After antiepileptic treatment was initiated the symptoms improved, however it was not possible to achieve complete control. Is this patient primarily suffering from recurrent asystole or from epilepsy which causes complex seizures with disturbance of cardiac rhythm? The latter phenomenon in its most extreme presentation is better known as sudden unexplained death in epilepsy (SUDEP). The diagnostic approach and therapy of both diseases are discussed. PMID:23188781
Stähli, C; Müller, F; Krause, M
Reorganization of seizure networks during epileptogenesis involves cortico-subcortical and interhemispheric interactions. In the audiogenic kindling (AK) model of generalized tonic-clonic seizures, upstream seizure propagation along ascending brainstem-to-forebrain pathways determines progressive intensification of repeated sound-induced convulsions. Full-blown audiogenic seizures are bilaterally symmetric and their repetition results in bisynchronous recruiting the cortex in secondary epileptogenesis. The present study describes lateral asymmetry of initial behavioral and EEG manifestations of audiogenic seizures and AK in Wistar and WAG/Rij rats with acoustic hypersensitivity. These rats exhibit consistent individual lateralization of running seizures (run directionality) induced by repeated binaural stimulation. Since this initial preconvulsive running reflects seizure onset in the auditory brainstem, the running asymmetry suggests non-symmetric early epileptic activation of brainstem substrates by sound in these rats. Repetition of the asymmetric brainstem seizures led to asynchronous recruiting the cortex into seizure network and lateralization of running seizures was predictive for asymmetry of early cortical seizure manifestations in Wistar and WAG/Rij rats. Both electrographic markers of AK, spreading depression (SD) and post-running afterdischarge, first appeared in the cortex ipsilateral to run direction, suggesting lateralized brainstem-to-forebrain seizure generalization during AK. At the population level, no bias in lateralization of running and SD was found in Wistar and WAG/Rij rats but incidence of secondary cortical seizures varied, depending on strain and run laterality. Among Wistar rats, cortical seizures developed more rarely in right-runners than in left-runners, suggesting enhanced resistance of the right hemisphere to epileptogenesis in rats of this strain. WAG/Rij rats with mixed (absence and audiogenic) epilepsy showed weak lateralization of early cortical seizures and no left-right difference in their incidence during AK. Present findings suggest (1) lateralized brainstem-to-forebrain seizure propagation and hemispheric difference in its facility in Wistar rats, (2) alterations of intra- and interhemispheric seizure propagation in WAG/Rij rats with genetic absence epilepsy. PMID:22525136
Vinogradova, L V; Shatskova, A B
The characterization of a seizure as generalized or focal onset depends on a basic knowledge of the underlying pathophysiology. Recently, an uncommon phenomenon in generalized epilepsy—evolution of seizures from generalized to focal followed by secondary generalization—was reported for the first time. We describe a 15-year-old boy, initially classified as having partial epilepsy, who had a typical absence seizure that became
Adriana Patricia M. Mayorga; David J. Anschel; Alvaro Moreno Avellán; Maricarmen F. González-Aragón; Andrew J. Cole
Neonatal seizures are a neurological emergency and prompt treatment is required. Seizure burden in neonates can be very high, status epilepticus a frequent occurrence, and the majority of seizures do not have any clinical correlate. Detection of neonatal seizures is only possible with continuous electroencephalogram (EEG) monitoring. EEG interpretation requires special expertise that is not available in most neonatal intensive care units (NICUs). As a result, a simplified method of EEG recording incorporating an easy-to-interpret compressed trend of the EEG output (amplitude integrated EEG) from one of the EEG output from one or two channels has emerged as a popular way to monitor neurological function in the NICU. This is not without limitations; short duration and low amplitude seizures can be missed, artefacts are problematic and may mimic seizure-like activity and only a restricted area of the brain is monitored. Continuous multichannel EEG is the gold standard for detecting seizures and monitoring response to therapy but expert interpretation of the EEG output is generally not available. Some centres have set up remote access for neurophysiologists to the cot-side EEG, but reliable interpretation is wholly dependent on the 24 h availability of experts, an expensive solution. A more practical solution for the NICU without such expertise is an automated seizure detection system. This review outlines the current state of the art regarding cot-side monitoring of neonatal seizures in the NICU. PMID:23707519
Boylan, Geraldine B; Stevenson, Nathan J; Vanhatalo, Sampsa
Assessment of 84 children diagnosed with controlled or uncontrolled complex partial or absence seizures but no documented learning or emotional disorders found no influence of seizure type on achievement test scores or behavioral ratings. A main effect was found for degree of control, with poorly controlled seizures correlating with lower reading…
Williams, Jane; And Others
Introduction The case of an atypical hepatic angiocavernoma is referred. The lesion, first described as a hypoechogenic area compared to the surrounding parenchyma, with anechogenic shoots inside, suggestive for vascular structures developed one year later into a totally asonic area with frayed margins. This change is very unusual and uncommon for this kind of lesions. Case presentation The case of a 74-year old caucasian male, complaining of slight dyspeptic symptoms (post-prandial fullness and bloating) is referred. The routine blood tests were all normal. Abdominal ultrasound showed a large, roughly round-shaped lesion (diameter 14 cm) in the VIII hepatic segment diagnosed as hepatic angiocavernoma, which turned unexpectedly in a cystic like lesion one year later. Conclusion The atypical angioma's degeneration could account for one of the causes of the patient's exitus. It could be related to blood seizure by the large hepatic angioma due to the intratumoural haemorrhage.
A provoked seizure may be due to structural damage (resulting from traumatic brain injury, brain tumor, stroke, tuberculosis, or neurocysticercosis) or due to metabolic abnormalities (such as alcohol withdrawal and renal or hepatic failure). This article is a part of the Guidelines for Epilepsy in India. This article reviews the problem of provoked seizure and its management and also provides recommendations based on currently available information. Seizure provoked by metabolic disturbances requires correction of the triggering factors. Benzodiazepines are recommended for treatment of seizure due to alcohol withdrawal; gabapentin for seizure seen in porphyria; and antiepileptic drugs (AED), that are not inducer of hepatic enzymes, in the seizures seen in hepatic dysfunction. In severe traumatic brain injury, with or without seizure, phenytoin (PHT) may be given for 7 days. In ischemic or hemorrhagic stroke one may individualize the AED therapy. In cerebral venous sinus thrombosis (CVST), AED may be prescribed if there is seizure or computed tomographic (CT) abnormalities or focal weakness; the treatment, in these cases, has to be continued for 1 year. Prophylactic AED is not recommended in cases of brain tumor and neurosurgical procedures and if patient is on an AED it can be stopped after 1 week.
Misra, Usha Kant; Kalita, Jayantee
The effects of topiramate, a novel antiepileptic drug, on tonic and absence-like seizures in spontaneously epileptic rats (SER; zi/zi, tm/tm) and on sound-induced seizures in DBA/2 mice were investigated. Topiramate (20 and 40 mg/kg i.p.) inhibited both tonic and absence-like seizures in a dose-dependent manner, whereas phenytoin (20 mg/kg i.p.) and zonisamide (40 mg/kg i.p.) inhibited only the tonic seizures. The inhibitory effects of topiramate on absence-like seizures were antagonized by pretreatment with haloperidol (0.5 mg/kg i.p.), but those on the tonic seizures remained unaffected. Topiramate inhibited sound-induced seizures in DBA/2 mice (ED50 = 8.6 mg/kg p.o.). These findings suggest that topiramate may be effective for treatment of both convulsive and absence seizures of human epilepsy. The inhibitory effect of topiramate on absence-like seizures in SER may be mediated through the central dopaminergic system. PMID:8206119
Nakamura, J; Tamura, S; Kanda, T; Ishii, A; Ishihara, K; Serikawa, T; Yamada, J; Sasa, M
Low-frequency repetitive transcranial magnetic stimulation (rTMS) is emerging as a therapeutic tool for patients with intractable epilepsy. Although seizures during treatment have been reported as adverse events in some patients, the nature and severity of seizures that may be provoked by low-frequency rTMS in patients with epilepsy have not been extensively studied. Accordingly, this article documents seizures in patients (n=5) with intractable epilepsy and average seizure frequency greater than one per day who underwent 1-Hz rTMS for seizure suppression. We report three observations in the present case series: (1) in each instance the in-session seizure was typical in semiology to the patient's habitual seizures, (2) the duration of each documented seizure was either the same as or shorter than the patients' baseline seizures, and (3) the overall neurological outcome on follow-up was not affected by the in-session seizures. More data will be required for valid conclusions with respect to safety and tolerability of low-frequency rTMS in patients with epilepsy, but it is noteworthy from our perspective that seizures during rTMS in this series were similar to the patients' habitual seizures, occurred in patients with epilepsy with baseline seizure frequency exceeding one per day, and did not correlate with a poor neurological outcome or with absence of clinical response to rTMS. PMID:19747883
Rotenberg, Alexander; Bae, Erica Hyunji; Muller, Paul A; Riviello, James J; Bourgeois, Blaise F; Blum, Andrew S; Pascual-Leone, Alvaro
Low-frequency repetitive transcranial magnetic stimulation (rTMS) is emerging as a therapeutic tool for patients with intractable epilepsy. Although seizures during treatment have been reported as adverse events in some patients, the nature and severity of seizures that may be provoked by low-frequency rTMS in patients with epilepsy have not been extensively studied. Accordingly, this article documents seizures in patients (n = 5) with intractable epilepsy and average seizure frequency greater than one per day who underwent 1-Hz rTMS for seizure suppression. We report three observations in the present case series: (1) in each instance the in-session seizure was typical in semiology to the patient’s habitual seizures, (2) the duration of each documented seizure was either the same as or shorter than the patients’ baseline seizures, and (3) the overall neurological outcome on follow-up was not affected by the in-session seizures. More data will be required for valid conclusions with respect to safety and tolerability of low-frequency rTMS in patients with epilepsy, but it is noteworthy from our perspective that seizures during rTMS in this series were similar to the patients’ habitual seizures, occurred in patients with epilepsy with baseline seizure frequency exceeding one per day, and did not correlate with a poor neurological outcome or with absence of clinical response to rTMS.
Rotenberg, Alexander; Bae, Erica Hyunji; Muller, Paul A.; Riviello, James J.; Bourgeois, Blaise F.; Blum, Andrew S.; Pascual-Leone, Alvaro
In previous studies we showed that autonomous absence seizure generation and termination can be explained by realistic neuronal models eliciting bi-stable dynamics. In these models epileptic seizures are triggered either by external stimuli (reflex epilepsies) or by internal fluctuations. This scenario predicts exponential distributions of the duration of the seizures and of the inter-ictal intervals. These predictions were validated in rat models of absence epilepsy, as well as in a few human cases. Nonetheless, deviations from the predictions with respect to seizure duration distributions remained unexplained. The objective of the present work is to implement a simple but realistic computational model of a neuronal network including synaptic plasticity and ionic current dynamics and to explore the dynamics of the model with special emphasis on the distributions of seizure and inter-ictal period durations. We use as a basis our lumped model of cortical neuronal circuits. Here we introduce 'activity dependent' parameters, namely post-synaptic voltage-dependent plasticity, as well as a voltage-dependent hyperpolarization-activated current driven by slow and fast activation conductances. We examine the distributions of the durations of the seizure-like model activity and the normal activity, described respectively by the limit cycle and the steady state in the dynamics. We use a parametric ?-distribution fit as a quantifier. Our results show that autonomous, activity-dependent membrane processes can account for experimentally obtained statistical distributions of seizure durations, which were not explainable using the previous model. The activity-dependent membrane processes that display the strongest effect in accounting for these distributions are the hyperpolarization-dependent cationic (Ih) current and the GABAa plastic dynamics. Plastic synapses (NMDA-type) in the interneuron population show only a minor effect. The inter-ictal statistics retain their consistency with the experimental data and the previous model.
Koppert, M. M. J.; Kalitzin, S.; Lopes da Silva, F. H.; Viergever, M. A.
The objective of this study was to delineate the clinical and video-electroencephalographic (EEG) manifestations of children with complex partial seizures with a predominant "hyperkinetic" presentation. Certain types of partial seizures can be difficult to differentiate from nonepileptic seizures because of their intense motor presentation and, at times, lack of alteration of consciousness. Based on a published semiologic seizure classification, this type of seizures can be described as "hyperkinetic," characterized by intense motor activity involving the extremities and trunk. We report five children diagnosed with hyperkinetic seizures by video-EEG monitoring. All patients were referred for video-EEG evaluation because of an initial suspicion of pseudoseizures. Presented in this study is a review of the patients' clinical data, including video-EEG evaluation. There were three boys and two girls; the mean age at presentation was 10 +/- 3 years. In four patients, there was a history of behavioral disorder, with two patients carrying a diagnosis of attention-deficit hyperactivity disorder (ADHD). One girl had significant developmental delay and an abnormal neurologic examination. Brain magnetic resonance imaging was normal in three patients and abnormal in two. The semiology of the seizures consisted of stereotypic intense motor activity, mainly upper extremity flailing and kicking. Screaming and shouting were noted in three cases, and intense fear was present in two patients. The hyperkinetic ictal activity progressed to tonic-clonic seizures in two patients. Seizures occurred out of sleep or on awakening in four patients. The interictal EEG activity was normal in one patient and revealed a continuous generalized slowing and slowing of the posterior dominant rhythm in two patients. One of the latter patients had interictal epileptiform activity in the frontal and midline regions. An intermittent rhythmic slow activity of the left hemisphere with superimposed bifrontal sharp waves was noted in the fifth patient. The ictal EEG revealed profuse superimposed electromyographic (EMG) activity in all patients, making some of the EEG interpretation difficult to analyze, particularly a longitudinal bipolar montage. However, with digital manipulation of the ictal EEG data, such as changes in EEG sensitivity, application of fast frequency filters, and use of different EEG montages, it was possible to discern an ictal EEG pattern or postictal slowing following the diffuse EMG artifact in all patients. On clinical follow-up, adequate seizure control was achieved in three patients. Based on the clinical history, one patient was diagnosed with autosomal dominant nocturnal frontal lobe epilepsy. Diagnosis of hyperkinetic seizures can be difficult because of the similarity of the clinical manifestations with nonepileptic events such as certain parasomnias and pseudoseizures. Video-EEG is the most effective way of diagnosing this type of seizure. PMID:13677576
Weinstock, Arie; Giglio, Pierre; Kerr, Susan L; Duffner, Patricia K; Cohen, Michael E
Over the last decade, the search for a method able to reliably predict seizures hours in advance has been largely replaced by a more realistic goal of very early detection of seizure onset which would allow therapeutic or warning devices to be triggered prior to the onset of disabling clinical symptoms. We explore in this article the steps along the pathway from data acquisition to closed loop applications that can and should be considered to design the most efficient early seizure detection. Microelectrodes, high-frequency oscillations, high sampling rate, high-density arrays, and modern analysis techniques are all elements of the recording and detection process that in combination with modeling studies can provide new insights into the dynamics of seizure onsets. Each of these step needs to be considered if one wants to implement improved detection devices that will favorably impact the quality of life of patients.
Jouny, Christophe C.; Franaszczuk, Piotr J.; Bergey, Gregory K.
Various psychic symptoms as ictal manifestation have been found in epileptic patients. They are classified as psychic seizures within simple partial seizures, and subclassified into affective, cognitive, dysmnesic seizures and so on, although the subclassification is not yet satisfactory and almost nothing is known about their relationships with normal brain functions. In this presentation, the speaker picked ictal fear, déjà vu and out-of-body experience (OBE) from them and suggested that studies on these symptoms could uniquely contribute to the progress of cognitive neuroscience, presenting some results from the research and case study that he had been engaged in. Psychic seizures are prone to be missed or misdiagnosed unless psychiatrists with sufficient knowledge and experience on epilepsy care would not treat them, because they are subjective symptoms that are diverse and subtle, while they have some characteristics as ictal symptoms. PMID:16637586
\\u000a Clinical evidence, in particular the wide use of theophylline as a bronchodilator, suggests that methylxanthines can cause\\u000a seizures in patients without known underlying epilepsy. Theophylline is also known to be an added risk factor for seizure\\u000a exacerbation in patients with epilepsy. The proconvulsant activity of methylxanthines can best be explained by their antagonizing\\u000a the brain’s own anticonvulsant adenosine. Recent evidence
Absence seizures are common within many different epilepsies and span all the ages. Even though absence seizures were described more than three centuries ago advances associated with its classification, pathophysiology, genetics, treatment, prognosis, and associated co-morbidities continue to be made.
Tenney, Jeffrey R.; Glauser, Tracy A.
We describe an atypical clinical and electroencephalographic (EEG) pattern observed during the course of subacute sclerosing panencephalitis in a 14 year-old boy. In this patient with a two weeks history of partial complex seizures, the atypical EEG pattern was characterized by an initial left temporal focus which evolved to periodic lateralized epileptiform discharges (PLEDs) and, only during the 3rd and 4th weeks the typical bilateral and generalized periodic complexes appeared. PMID:7487538
Silva, D F; Lima, M M; Anghinah, R; Zanoteli, E; Lima, J G
Febrile seizures (FSs) occur in children older than 1 month and without prior afebrile seizures in the absence of a central nervous system infection or acute electrolyte imbalance. Their pathogenesis is multifactorial. The most relevant familial studies evidence an occurrence rate ranging from 10% to 46% and median recurrence rate of 36% in children with positive familial history for FS. The main twin studies demonstrated a higher concordance rate in monozygotic twins with FS than in dizygotic ones. Linkage studies have proposed 11 chromosomal locations responsible to FS attributed to FEB1 to FEB11. Population-based association studies have shown at least one positive association for 14 of 41 investigated genes with FS. The proinflammatory cytokine interleukin 1? (IL-1?) was the most investigated and also gene associated with susceptibility to FS. A possible role in the overlapping of epilepsy and FS was found for 16 of 36 investigated genes. SCN1A, IL-1?, CHRNA4, and GABRG2 were the most commonly involved genes in this context. The genetic background of FS involves the regulation of different processes, including individual and familial susceptibility, modulation of immune response, and neuronal excitability and interactions with exogenous agents such as viruses. PMID:24399675
Saghazadeh, Amene; Mastrangelo, Mario; Rezaei, Nima
Objective: All patients who exhibit seizure-like behavior cannot be evaluated by video-electroencephalography if their routine EEGs are negative, as this would be impractical and cost-prohibitive. The present article reviews a decision-making process that can be used for determining if further neurological evaluation is needed, the differential diagnoses and potential comorbidities involved when making this determination, and an approach to conveying the psychogenic nonepileptic seizure diagnosis to the patient that may help reduce symptom frequency. Design: Literature review. Conclusion: Psychogenic seizures are not caused by abnormal brain electrical activity. The symptoms of psychogenic seizures usually reflect a psychological conflict or a psychiatric disorder. However, psychogenic seizures are not "purposely" produced by the patient, and the patient is not aware that the seizures are non-epileptic, so the patient may become very anxious over having these symptoms. The presentation of the differential diagnosis should be done early in the course of treatment for better patient acceptance, and treatment options should be presented early in the evaluation period. PMID:24563816
Marie Gillig, Paulette
Objective: All patients who exhibit seizure-like behavior cannot be evaluated by video-electroencephalography if their routine EEGs are negative, as this would be impractical and cost-prohibitive. The present article reviews a decision-making process that can be used for determining if further neurological evaluation is needed, the differential diagnoses and potential comorbidities involved when making this determination, and an approach to conveying the psychogenic nonepileptic seizure diagnosis to the patient that may help reduce symptom frequency. Design: Literature review. Conclusion: Psychogenic seizures are not caused by abnormal brain electrical activity. The symptoms of psychogenic seizures usually reflect a psychological conflict or a psychiatric disorder. However, psychogenic seizures are not “purposely” produced by the patient, and the patient is not aware that the seizures are non-epileptic, so the patient may become very anxious over having these symptoms. The presentation of the differential diagnosis should be done early in the course of treatment for better patient acceptance, and treatment options should be presented early in the evaluation period.
A 9-year-old boy presented with fever not responding to antibiotic therapy and elevated blood urea and serum creatinine levels. The patient developed microangiopathic hemolytic anemia and thrombocytopenia during the hospital stay. Kidney biopsy confirmed the diagnosis of atypical hemolytic uremic syndrome (HUS). The patient had sufficient urine output, normal blood pressure, and no evidence of peripheral edema during the whole course of his disease. Serum levels of anti-Epstein-Barr virus immunoglobulin M was elevated, indicating the possible role of Epstein-Barr virus infection in inducing atypical HUS in this patient. The patient underwent hemodialysis with dramatic response. He was discharged with normal kidney function after a few days. Kidney function and platelet count were normal 12 months after the initial presentation. This case report shows that atypical hemolytic uremic syndrome could have unusual presentations such as the absence of oliguria, hypertension, and edema, with rapid recovery and good prognosis. PMID:25001143
Fallahzadeh, Mohammad Amin; Fallahzadeh, Mohammad Kazem; Derakhshan, Ali; Shorafa, Eslam; Mojtahedi, Yusof; Geramizadeh, Bita; Fallahzadeh, Mohammad Hossein
Seizures and epilepsy in children are common. They are caused by a variety of causes ranging from genetic to neuro -infections. History and actual observation or/video are very important to differentiate true seizure from non-epileptic event. A correct classification of seizure and epilepsy helps to decide need to treat, choice of anti-epileptic drugs (AED) and prognostication. Except for few seizure types, in majority of seizure types regular AED are started after second confirmed seizure. Goal of treatment is seizure control with minimal side effects and ensuring quality of life. With appropriate mono - therapy, about 70 % patients become seizure free and can be easily treated in community. An orderly approach to classify seizure type, holistic management and timely referral of intractable epilepsy will help in improving care of children with epilepsy. PMID:24048876
Seizures induced by fever (febrile seizures) are the most common type of pathological brain activity in infants and children. These febrile seizures and their potential contribution to the mechanisms of limbic (temporal lobe) epilepsy have been a topic of major clinical and scientific interest. Key questions include the mechanisms by which fever generates seizures, the effects of long febrile seizures on neuronal function and the potential contribution of these seizures to epilepsy. This review builds on recent advances derived from animal models and summarizes our current knowledge of the mechanisms underlying febrile seizures and of changes in neuronal gene expression and function that facilitate the enduring effects of prolonged febrile seizures on neuronal and network excitability. The review also discusses the relevance of these findings to the general mechanisms of epileptogenesis during development and points out gaps in our knowledge, including the relationship of animal models to human febrile seizures and epilepsy.
Dube, Celine M.; Brewster, Amy L.; Richichi, Cristina; Zha, Qinqin; Baram, Tallie Z.
Clinical evidence, in particular the wide use of theophylline as bronchodilator, suggests that methylxanthines can cause seizures in patients without known underlying epilepsy. Theophylline is also known to be an added risk factor for seizure exacerbation in patients with epilepsy. The proconvulsant activity of methylxanthines can best be explained by antagonizing the brain’s own anticonvulsant adenosine. Recent evidence suggests that adenosine dysfunction is a pathological hallmark of epilepsy contributing to seizure generation and seizure spread. Conversely, adenosine augmentation therapies are effective in seizure suppression and prevention, whereas adenosine receptor antagonists such as methylxanthines generally exacerbate seizures. The impact of the methylxanthines caffeine and theophylline on seizures and excitotoxicity depends on timing, dose, and acute versus chronic use. New findings suggest a role of free radicals in theophylline-induced seizures and adenosine-independent mechanisms for seizure generation have been proposed.
ALDH7A1 and PNPO deficiencies are rare inborn errors of vitamin B6 metabolism causing perinatal seizure disorders. The phenotypic variability, however, is broad. To assess the frequency of these deficiencies in unexplained infantile epilepsy, we screened 113 patients for mutations in both genes. We identified 1 patient with an epilepsy phenotype resembling Dravet syndrome and likely pathogenic mutations in ALDH7A1. Presenting features were highly atypical of pyridoxine-dependent epilepsy, including febrile seizures, response to anticonvulsive drugs, and periods of seizure freedom without pyridoxine treatment. "Hidden" vitamin B6 deficiencies might be rare but treatable causes of unexplained epilepsy extending beyond the classical phenotypes. PMID:24114605
Baumgart, Anna; Spiczak, Sarah von; Verhoeven-Duif, Nanda M; Møller, Rikke S; Boor, Rainer; Muhle, Hiltrud; Jähn, Johanna A; Klitten, Laura L; Hjalgrim, Helle; Lindhout, Dick; Stephani, Ulrich; van Kempen, Marjan J A; Helbig, Ingo
We have studied 124 children with typical absence epilepsy. The onset of symptoms was in 12% under 4 years, in 51% between 4-8 years and in 37% above 8 years. The F:M ratio was 2:1 in children under 4 years versus 1:1 above 8 years. Absences alone occurred in 82% and absences followed or preceded by generalized tonic-clonic seizures (GTCS) in 6.5% and 11%, respectively. Simple absences were not seen in children under 4 years and were more frequent (14%) in the 4-8 years age group. Family history was positive for epilepsy in 20% and febrile convulsion in 7%. Sixteen percent had a positive past history of febrile convulsions. All patients showed bilateral, synchronous spike-wave discharges from 2.5 to 4 c/s. Lateralized spikes, spike-slow wave complexes were found in 27%. Photosensitivity was present in 18% and was marked in 12%. Monotherapy with sodium valproate or ethosuximide (91% SV) was successful in 85% of patients with absences alone and 68% of the absences with GTCS. Only 2% were not fully controlled either on monotherapy or polytherapy. Treatment was withdrawn in 41 patients and 13 relapsed. We have identified four factors associated with relapses: (a) poor initial response to treatment, (b) lateralized focal EEG abnormality and/or marked photosensitivity, (c) the evolution to myoclonic epilepsy, and (d) early withdrawal of AED (< 3 years). PMID:1344778
Covanis, A; Skiadas, K; Loli, N; Lada, C; Theodorou, V
Neocortical seizures are often poorly localized, explosive and widespread at onset, making them poorly amenable to epilepsy surgery in the absence of associated focal brain lesions. We describe, for the first time in an unselected group of patients with neocortical epilepsy, the finding that high-frequency (60-100 Hz) epileptiform oscillations are highly localized in the seizure onset zone, both before and temporally removed from seizure onset. These findings were observed in all six patients with neocortical epilepsy out of 23 consecutive patients implanted with intracranial electrodes for pre-surgical evaluation during the study period. The majority of seizures (62%) in these patients were anticipated by an increase in high-frequency activity in the 20 min prior to neocortical seizure onset. Contrary to observations in normal brain, high-frequency activity was strongly modulated by behavioural state, and was maximal during slow-wave sleep, which may explain the propensity for neocortical onset seizures to begin during sleep. These findings point to an important role for neuromodulatory circuits, probably involving the thalamus, in mechanisms underlying seizure generation in neocortical epilepsy. These findings demonstrate that high-frequency epileptiform oscillations may prove clinically useful in localizing the seizure onset zone in neocortical epilepsy, for identifying periods of increased probability of seizure onset, and in elucidating mechanisms underlying neocortical ictogenesis. Confirmation that prolonged bursts of high-frequency activity may predict focal onset neocortical seizures will require prospective validation on continuous, prolonged recordings in a larger number of patients. Importantly, the results show that the dynamic range utilized in current clinical practice for localization of epileptogenic brain largely ignores fundamental oscillations that are signatures of an epileptogenic brain. It may prove that many currently available clinical EEG systems and EEG analysis methods utilize a dynamic range that discards clinically important information. PMID:15155522
Worrell, Greg A; Parish, Landi; Cranstoun, Stephen D; Jonas, Rachel; Baltuch, Gordon; Litt, Brian
Despite the frequency of seizure disorders in the human population, the genetic and physiological basis for these defects has been difficult to resolve. Although many genetic contributions to seizure susceptibility have been identified, these involve disparate biological processes, many of which are not neural specific. The large number and heterogeneous nature of the genes involved makes it difficult to understand the complex factors underlying the etiology of seizure disorders. Examining the effect known genetic mutations have on seizure susceptibility is one approach that may prove fruitful. This approach may be helpful in both understanding how different physiological processes affect seizure susceptibility and identifying novel therapeutic treatments. We review here factors contributing to seizure susceptibility in Drosophila, a genetically tractable system that provides a model for human seizure disorders. Seizure-like neuronal activities and behaviors in the fruit fly are described, as well as a set of mutations that exhibit features resembling some human epilepsies and render the fly sensitive to seizures. Especially interesting are descriptions of a novel class of mutations that are second-site mutations that act as seizure suppressors. These mutations revert epilepsy phenotypes back to the wild-type range of seizure susceptibility. The genes responsible for seizure suppression are cloned with the goal of identifying targets for lead compounds that may be developed into new antiepileptic drugs.
Parker, Louise; Howlett, Iris C.; Rusan, Zeid M.; Tanouye, Mark A.
In adolescents, diagnosing seizures can be challenging and can lead to many pitfalls. Because seizures are episodic and unpredictable events, they usually do not occur in the doctor's office. Thus, a diagnosis of epilepsy is usually based on information presented by the person with seizures and their family. Together with results of diagnostic…
A-type K+ channels are crucial determinants of neuronal firing. For example, reducing the amplitude of A-type currents (IA) increases seizure susceptibility. We have therefore examined the functional and molecular properties of IA in dentate granule neurons following pilocarpine-induced status epilepticus (SE). We found that the levels of various A-type channel subunit mRNAs are unaltered following SE. Furthermore, current density and
C. Rüschenschmidt; J. Chen; A. Becker; V. Riazanski; H. Beck
A monitor has been designed to detect the onset of status epilepticus associated with complex partial seizures in children. A unique sensor technology was developed to detect the minor, barely perceptible tremors characteristic of partial seizures. A micr...
M. A. Johnson G. Kendall P. J. Cote L. V. Meisel
So-called typical' autoerotic fatalities are the result of asphyxia due to mechanical compression of the neck, chest, or abdomen, whereas atypical' autoeroticism involves sexual self-stimulation by other means. The authors present five atypical autoerotic fatalities that involved the use of dichlorodifluoromethane, nitrous oxide, isobutyl nitrite, cocaine, or compounds containing 1-1-1-trichloroethane. Mechanisms of death are discussed in each case and the
Gerald T. Gowitt; Randy L. Hanzlick
Iopamidol, a water-soluble contrast agent, has been rarely associated with seizures. We describe a case of generalized tonic-clonic seizure after cervical myelography with iopamidol in a previously healthy young man. In patients presenting with seizures, a history of recent myelography should be considered as an etiology. Iopamidol myelography may be associated with a risk of seizures. Clinicians need to be aware of this complication and inform their patients about such risk.
Singh, Sonal; Rajpal, Chitra; Nannapeneni, Srikanth; Venkatesh, Sundar
\\u000a Nonepileptic seizures (NES) are operationally defined as episodes of involuntary movement, altered responsiveness, or subjective\\u000a experience that resemble epileptic seizures (ES), but are not accompanied by the abnormal electrical discharges in the brain\\u000a that is a seizure. (Lesser, 1996; Reuber and Elger, 2003). When these episodes are caused by psychological processes, they\\u000a are termed psychogenic nonepileptic seizures (PNES). Other terms,
Daniel L. Drane; Erica L. Coady; David J. Williamson; John W. Miller; Selim Benbadis
We investigated the electroclinical features of 12 patients with childhood absence epilepsy (CAE), presenting with typical absence seizures associated with myoclonic manifestations of the face or neck. All patients underwent repeated and prolonged split-screen video-polygraphic EEG recordings. The polygraphic recordings and clinical correlations of the absence seizures were analysed. All patients presented with multi-quotidian, typical absence seizures. During the absences, the patients could show mild, rhythmic, myoclonic jerks involving facial areas (eyebrows, nostrils, perioral region, chin) or neck muscles (sternocleidomastoideus), with the same frequency as the spike-wave complexes. Polygraphic tracings demonstrated that the myoclonias were correlated to the spike component. Clinically, all patients showed a benign course, with complete seizure control under antiepileptic treatment. In the follow-up, 7 patients withdrew from treatment without relapse. We conclude that all our patients showed an electroclinical picture consistent with CAE. The occurrence of myoclonic manifestations of the face or neck associated with the absences did not influence the benign course of their disease. The electroclinical features observed in our group of patients differentiates our cases both from epilepsy with myoclonic absences and from absences with perioral myoclonia (with Video). PMID:11431166
Capovilla, G; Rubboli, G; Beccaria, F; Lorenzetti, M E; Montagnini, A; Resi, C; Gardella, E; Gambardella, A; Romeo, A; Tassinari, C A
The important differential diagnosis between epileptogenic versus non-epileptogenic attacks becomes increasingly difficult with elderly patients: 1) Vasovagal syncopes may occur abruptly, not infrequently with injuries caused by the sudden fall ("Blitz-Synkope"). Other generalized non-epileptic seizures include drop-attacks, amnesic episodes, prolonged syncopes, and seizures caused by faulty metabolism. 2) Focal non-epileptic seizures in advanced age are mainly TIA and prolonged TIA (PRIND). Complicated migraine is more typical for the younger age group. In this connection it must be kept in mind that 10% of TIA are caused by brain tumor, 20% can be traced to cardiac origin. 3) In connection with the non-epileptic seizures mentioned above there may appear singular irregular cloni without any rhythmical sequence. We have come to call this type of attacks "incidental convulsions". Especially in these cases differential diagnosis is of great importance with respect to basically different therapeutic measures. 4) First manifestations of epilepsy in advanced age are--regarding etiology--in the first rank symptoms of cerebral vascular disease or of intracranial tumors. 5) In the diagnostic approach it is necessary to keep in mind all the above-mentioned possibilities and to exploit every possible access to anamnestic exploration, with the patient as well as with his family, friends and colleagues. Essential auxiliary diagnostic methods include EEG, computed tomography, Doppler-sonography, occasionally long-time EEG or ECG, in some cases NMR. PMID:1891917
Barolin, G S
Epilepsy, one of the most common neurological diseases, affects over 50 million people worldwide. Epilepsy can have a broad spectrum of debilitating medical and social consequences. Although antiepileptic drugs have helped treat millions of patients, roughly a third of all patients have seizures that are refractory to pharmacological intervention. The evolution of our understanding of this dynamic disease leads to new treatment possibilities. There is great interest in the development of devices that incorporate algorithms capable of detecting early onset of seizures or even predicting them hours before they occur. The lead time provided by these new technologies will allow for new types of interventional treatment. In the near future, seizures may be detected and aborted before physical manifestations begin. In this chapter we discuss the algorithms that make these devices possible and how they have been implemented to date. We also compare and contrast these measures, and review their individual strengths and weaknesses. Finally, we illustrate how these techniques can be combined in a closed-loop seizure prevention system.
Carney, Paul R.; Myers, Stephen; Geyer, James D.
Hydroxybutyric acid (GHB) is a naturally occurring compound that has the ability to induce generalized absence seizures possibly by GABAB-receptor-mediated mechanisms. The object of these experiments was to examine the effectiveness of a range of specific GABAB-receptor agonists and antagonists of varying specificity, as well as the specific GHB-receptor antagonist NCS 382, in two experimental animal models of generalized absence seizures: one in which the seizures are induced by GHB and the other in which the seizures are induced by administration of low-dose (20-mg/kg) pentylenetetrazole. All specific GABAB-receptor antagonists as well as the specific GHB-receptor antagonist produced blockade of experimental absence seizures in both models; pretreatment with GABAB-receptor agonists resulted in generalized absence status epilepticus lasting for hours. These data confirm the concept that specific GABAB-receptor antagonist activity confers antiabsence seizure activity, suggest that the same holds for specific GHB-receptor antagonists, and raise the possibility that both GHB- and GABAB-antagonist drugs have the potential to be useful therapeutic agents in generalized absence seizures. PMID:8848463
Snead, O C
Abstract Objective To review the diagnosis of patients with atypical cystic fibrosis (CF). Sources of information A comprehensive search of MEDLINE (1950 to the third week of May 2009), MEDLINE In-Process and Other Non-Indexed Citations and Cases (1950 to the third week of May 2009), and EMBASE (1980 to the fourth week of March 2009). The Cystic Fibrosis Canada website was also reviewed and the most recent patient data registry report was consulted. Main message Atypical CF is a milder form of the CF disorder, which is associated with mutations of the cystic fibrosis transmembrane receptor gene. Instead of having classic symptoms, individuals with atypical CF might only have mild dysfunction in 1 organ system and might or might not have elevated sweat chloride levels. Atypical CF is a very diverse disorder affecting different organ systems to varying degrees. The symptoms patients experience can also fluctuate over time; however, certain clinical signs and symptoms affecting the respiratory, gastrointestinal, endocrine and metabolic, and genitourinary systems should alert physicians to the possibility of CF. Patients with atypical CF often have fewer hospitalizations during childhood than those with classic CF do, and the disorder can remain undiagnosed for many years, at times into adulthood. Conclusion Although patients diagnosed with atypical CF have longer life expectancies than individuals with classic CF, the long-term expected outcome for many individuals with atypical CF is unknown. It is important to counsel patients about the possibility of future illness. Education about CF can help patients understand their symptoms, modify their lifestyles to optimize health, reduce the incidence of complications, and receive family planning counseling when appropriate.
Schram, Carrie A.
Dravet syndrome is an epileptic encephalopathy characterized by multiple types of seizures. We report the first case of musicogenic reflex seizures in a 7-year-old male with a mutation in the SCN1A gene causing Dravet syndrome. Reflex seizures have been reported in patients with Dravet syndrome provoked by body temperature elevation, looking at visual patterns, or under intermittent photic stimulation. The case we report widens the spectrum of reflex seizures recorded in patients with Dravet syndrome. Cortical hyperexcitability of genetic origin could explain the tendency of these patients to experience reflex seizures. PMID:23517304
Sanchez-Carpintero, Rocio; Patiño-Garcia, Ana; Urrestarazu, Elena
Objective To assess the efficacy of Topiramate as an add-on drug in the treatment of seizures in children of age group 0–12 years.\\u000a \\u000a \\u000a \\u000a Methods Fifty children of age 0–12 years with seizures viz. partial seizures with or without secondary generalization, myoclonic jerks,\\u000a infantile spasms, generalized tonic–clonic seizures, absence or mixed seizures were chosen from the out-patient department.\\u000a Topiramate was added in small doses to
Priya Sreenivasan; P. A. Mohammed Kunju
Aims: To describe a large series of children with anoxic-epileptic seizures (AES)—that is, epileptic seizures induced by syncopes. Methods: Retrospective case-note review in a tertiary paediatric neurology unit. For all 27 children seen with a definite diagnosis of AES between 1972 and 2002, a review of clinical histories, videotapes, and EEG/ECG studies was undertaken. Main outcome measures were: age of onset, frequency and type of syncopes; age of onset and frequency of AES; type and duration of induced epileptic seizures; effect of treatment of syncopal and epileptic components. Results: Median age of onset of syncopes was 8 months (range 0.2–120), frequency 2 in total to 40/day, median total ?200. Syncopes were predominantly reflex asystolic (RAS), prolonged expiratory apnoea (cyanotic breath-holding spells), or of mixed or uncertain origin; there was one each of ear piercing and hair grooming vasovagal syncope and one of compulsive Valsalva. Median age of onset of AES was 17 months (range 7–120), frequency from total 1 to 3/day, median total 3. The epileptic component was almost always bilateral clonic; three had additional epilepsy, one each with complex partial seizures, myoclonic absences, and febrile seizures plus. Median duration of epileptic component was 5 minutes (range 0.5–40, mean 11). Cardiac pacing prevented RAS in two patients: most other anti-syncope therapies were ineffective. Diazepam terminated the epileptic component in 6/8. Valproate or carbamazepine abolished AES in 5/7 without influencing syncope frequency. Conclusions: Although uncommon compared with simple syncopes, syncope triggered epileptic seizures (AES) are an important treatable basis of status epilepticus.
Horrocks, I; Nechay, A; Stephenson, J; Zuberi, S
Clozapine is a widely used atypical antipsychotic with a unique effectiveness in treatment-resistant schizophrenia. An important adverse effect is seizures, which have been observed at all stages of clozapine treatment. Valproate has traditionally been considered the drug of choice for the prophylaxis of clozapine seizures, however it may not be the most suitable choice for all patients. There is disagreement as to the best point to prescribe valproate or a suitable antiepileptic: as seizure prophylaxis at a certain clozapine dose or level, or only as remedial treatment. In this review, we examine the relevant literature with an aim to evaluate the following relationships: clozapine dose and electroencephalogram (EEG) abnormalities, plasma levels and EEG abnormalities, dose and occurrence of seizures and plasma levels and occurrence of seizures. Weighted linear regression models were fitted to investigate these relationships. There was a strong relationship between clozapine dose and plasma level and occurrence of clozapine-induced EEG abnormalities. However, a statistically significant relationship between dose and occurrence of seizures was not found. A relationship between clozapine plasma level and occurrence of seizures was not established because of the scarcity of useful data although our review found three case reports which suggested that there is a very substantial risk of seizures with clozapine plasma levels exceeding 1300 ?g/l. Seizures are more common during the initiation phase of clozapine treatment, suggesting a slow titration to target plasma levels is desirable. An antiepileptic drug should be considered when the clozapine plasma level exceeds 500 ?g/l, if the EEG shows clear epileptiform discharges, if seizures, myoclonic jerks or speech difficulties occur and when there is concurrent use of epileptogenic medication. The antiepileptics of choice for the treatment and prophylaxis of clozapine-induced seizures are valproate (particularly where there is mood disturbance) and lamotrigine (where there is resistance to clozapine).
Varma, Seema; Bishara, Delia; Besag, Frank M. C.; Taylor, David
Summary Hemolytic uremic syndrome (HUS) is a triad of microangiopathic hemolytic anemia, thrombocytopenia, and acute renal failure. The atypical form of HUS is a disease characterized by complement overactivation. Inherited defects in complement genes and acquired autoantibodies against complement regulatory proteins have been described. Incomplete penetrance of mutations in all predisposing genes is reported, suggesting that a precipitating event or trigger is required to unmask the complement regulatory deficiency. The underlying genetic defect predicts the prognosis both in native kidneys and after renal transplantation. The successful trials of the complement inhibitor eculizumab in the treatment of atypical HUS will revolutionize disease management.
Kavanagh, David; Goodship, Tim H.; Richards, Anna
An unusual case of atypical mycobacterium infection following breast ptosis correction is described. This was successfully treated with a combination antibiotic regime. Breast surgery and atypical mycobacterium is discussed with reference to diagnosis management and suggested treatment regimes. PMID:7486746
Widgerow, A D; Brink, A J; Koornhof, H J
... of Tumors Astrocytoma Atypical Teratoid Rhaboid Tumor (ATRT) Chondrosarcoma Choroid Plexus Craniopharyngioma Cysts Ependymoma Germ Cell Tumor ... of Tumors Astrocytoma Atypical Teratoid Rhaboid Tumor (ATRT) Chondrosarcoma Choroid Plexus Craniopharyngioma Cysts Ependymoma Germ Cell Tumor ...
Focal epilepsy is commonly pharmacoresistant, and resective surgery is often contraindicated by proximity to eloquent cortex. Many patients have no effective treatment options. Gene therapy allows cell-type specific inhibition of neuronal excitability, but on-demand seizure suppression has only been achieved with optogenetics, which requires invasive light delivery. Here we test a combined chemical-genetic approach to achieve localized suppression of neuronal excitability in a seizure focus, using viral expression of the modified muscarinic receptor hM4Di. hM4Di has no effect in the absence of its selective, normally inactive and orally bioavailable agonist clozapine-N-oxide (CNO). Systemic administration of CNO suppresses focal seizures evoked by two different chemoconvulsants, pilocarpine and picrotoxin. CNO also has a robust anti-seizure effect in a chronic model of focal neocortical epilepsy. Chemical-genetic seizure attenuation holds promise as a novel approach to treat intractable focal epilepsy while minimizing disruption of normal circuit function in untransduced brain regions or in the absence of the specific ligand. PMID:24866701
Kätzel, Dennis; Nicholson, Elizabeth; Schorge, Stephanie; Walker, Matthew C; Kullmann, Dimitri M
Focal epilepsy is commonly pharmacoresistant, and resective surgery is often contraindicated by proximity to eloquent cortex. Many patients have no effective treatment options. Gene therapy allows cell-type specific inhibition of neuronal excitability, but on-demand seizure suppression has only been achieved with optogenetics, which requires invasive light delivery. Here we test a combined chemical–genetic approach to achieve localized suppression of neuronal excitability in a seizure focus, using viral expression of the modified muscarinic receptor hM4Di. hM4Di has no effect in the absence of its selective, normally inactive and orally bioavailable agonist clozapine-N-oxide (CNO). Systemic administration of CNO suppresses focal seizures evoked by two different chemoconvulsants, pilocarpine and picrotoxin. CNO also has a robust anti-seizure effect in a chronic model of focal neocortical epilepsy. Chemical–genetic seizure attenuation holds promise as a novel approach to treat intractable focal epilepsy while minimizing disruption of normal circuit function in untransduced brain regions or in the absence of the specific ligand.
Katzel, Dennis; Nicholson, Elizabeth; Schorge, Stephanie; Walker, Matthew C.; Kullmann, Dimitri M.
In 96 patients with congenital absence of the uterus and upper vagina, the Mayer-Rokitansky-KüsterHauser (MRKH) syndrome, it proved possible to distinguish between the typical and the atypical form using laparoscopy. The typical form was characterized by symmetrical nonfunctioning muscular buds (the Müllerian duct remnants) and normal fallopian tubes, and the atypical form by aplasia of one or both buds, one
Ernst H. Striibbe; J. Albert M. Lemmens; Cornelis J. P. Thijn; Wim N. P. Willemsen; Bert S. J. Toor
Introduction Reversible posterior leukoencephalopathy syndrome – a reversible subacute global encephalopathy clinically presenting with headache, altered mental status, visual symptoms such as hemianopsia or cortical blindness, motor symptoms, and focal or generalized seizures – is characterized by a subcortical vasogenic edema symmetrically affecting posterior brain regions. Complete reversibility of both clinical signs and magnetic resonance imaging lesions is regarded as a defining feature of reversible posterior leukoencephalopathy syndrome. Reversible posterior leukoencephalopathy syndrome is almost exclusively seen in the setting of a predisposing clinical condition, such as pre-eclampsia, systemic infections, sepsis and shock, certain autoimmune diseases, various malignancies and cytotoxic chemotherapy, transplantation and concomitant immunosuppression (especially with calcineurin inhibitors) as well as episodes of abrupt hypertension. We describe for the first time clinical, radiological and histological findings in a case of reversible posterior leukoencephalopathy syndrome with an irreversible and fatal outcome occurring in the absence of any of the known predisposing clinical conditions except for a hypertensive episode. Case presentation A 58-year-old Caucasian woman presented with a two-week history of subacute and progressive occipital headache, blurred vision and imbalance of gait and with no evidence for raised arterial blood pressure during the two weeks previous to admission. Her past medical history was unremarkable except for controlled arterial hypertension. Cerebral magnetic resonance imaging demonstrated cortical and subcortical lesions with combined vasogenic and cytotoxic edema atypical for both venous congestion and arterial infarction. Routine laboratory and cerebrospinal fluid parameters were normal. The diagnosis of reversible posterior leukoencephalopathy syndrome was established. Within hours after admission the patient showed a rapidly decreasing level of consciousness, extension and flexion synergisms, bilaterally extensor plantar responses and rapid cardiopulmonary decompensation requiring ventilatory and cardiocirculatory support. Follow-up cerebral imaging demonstrated widespread and confluent cytotoxic edematous lesions in different arterial territories, global cerebral swelling, and subsequent upper and lower brainstem herniation. Four days after admission, the patient was declared dead because of brain death. Conclusion This case demonstrates that fulminant and fatal reversible posterior leukoencephalopathy syndrome may occur spontaneously, that is, in the absence of any of the known predisposing systemic conditions.
Charles Bonnet syndrome (CBS) is not uncommon disorder. It may not present with all typical symptoms and intact insight. Here, a case of atypical CBS is reported where antipsychotics were not effective. Patient improved completely after restoration of vision. PMID:24379505
Arun, Priti; Jain, Rajan; Tripathi, Vaibhav
\\u000a Seizures are a nonspecific neurological manifestation of cerebral dysfunction and not indicative of any particular disease\\u000a processes or pathology. As such, the evaluation and treatment of seizures in transplant patients generally follow the same\\u000a clinical approach as for other patients. A seizure in a transplant patient is commonly unanticipated and entirely unexplained.\\u000a The effects can be substantial with aspiration, loss
Tarek Zakaria; Eelco F. M. Wijdicks; Greg A. Worrell
Seizures are a nonspecific neurological manifestation of cerebral dysfunction and are not indicative of any particular disease\\u000a processes or pathology. Thus, the evaluation and treatment of seizures in transplant patients generally follows the same clinical\\u000a approach used for other patients. A seizure in a transplant patient is commonly unanticipated and entirely unexplained. The\\u000a effects can be substantial, with aspiration, loss
Greg A. Worrell; Eelco F. M. Wijdicks
A 32-year-old man presented to the emergency department (ED) with seizures 6 hours after a lumbar myelogram with iopamidol (Isovue-M 200, Squibb, Princeton, NJ). Seizures are a rare complication after myelography with the current nonionic contrast agents and have not been previously reported in the emergency medicine literature. Emergency physicians should be aware of this potential complication because outpatient myelograms are frequently performed, and these patients may present to the ED after seizures. PMID:8179742
We studied six children (four girls and two boys) suffering from cryptogenic myoclonic absence seizures with early onset. The age at onset of the seizures ranged between 6 and 27.8 months (mean age ± SD: 18.5 ± 12.4 months). The neurologic evaluation was normal in all patients at the first hospital admission. After the diagnosis, we followed up all children
Alberto Verrotti; Rita Greco; Francesco Chiarelli; Sergio Domizio; Giuseppe Sabatino; Guido Morgese
Epilepsy is characterized by intermittent, paroxysmal, hypersynchronous electrical activity, that may remain localized and/or spread and severely disrupt the brain’s normal multi-task and multi-processing function. Epileptic seizures are the hallmarks of such activity and had been considered unpredictable. It is only recently that research on the dynamics of seizure generation by analysis of the brain’s electrographic activity (EEG) has shed ample light on the predictability of seizures, and illuminated the way to automatic, prospective, long-term prediction of seizures. The ability to issue warnings in real time of impending seizures (e.g., tens of minutes prior to seizure occurrence in the case of focal epilepsy), may lead to novel diagnostic tools and treatments for epilepsy. Applications may range from a simple warning to the patient, in order to avert seizure-associated injuries, to intervention by automatic timely administration of an appropriate stimulus, for example of a chemical nature like an anti-epileptic drug (AED), electromagnetic nature like vagus nerve stimulation (VNS), deep brain stimulation (DBS), transcranial direct current (TDC) or transcranial magnetic stimulation (TMS), and/or of another nature (e.g., ultrasonic, cryogenic, biofeedback operant conditioning). It is thus expected that seizure prediction could readily become an integral part of the treatment of epilepsy through neuromodulation, especially in the new generation of closed-loop seizure control systems.
Iasemidis, Leon D.
Generalised (genetic) epilepsy with febrile seizures plus (GEFS+) is a familial epilepsy syndrome with various phenotypes. The majority of individuals with GEFS+ have generalised seizure types, in addition to febrile seizures (FS) or febrile seizures plus (FS+), defined as either continued FS after 6 years of age or afebrile seizures following FS. A 27-year-old man with no history of FS/FS+ experienced intractable generalised convulsive seizures. The patient's father had a history of similar seizures during puberty and the patient's siblings had only FS. No individual in the family had both generalised seizures and FS/FS+, although GEFS+ might be considered to be present in the family. Analysis of SCN1A, a sodium channel gene, revealed a novel mutation (c.3250A>T [S1084C]) in the cytoplasmic loop 2 of SCN1A in both the patient and his father. Most previously reported SCN1A mutations in GEFS+ patients are located in the conserved homologous domains of SCN1A, whereas mutations in the cytoplasmic loops are very rare. SCN1A gene analysis is not commonly performed in subjects with generalised seizures without FS. SCN1A mutation may be a clinically-useful genetic marker in order to distinguish GEFS+ patients from those with classic idiopathic generalised epilepsy, even if they present an atypical clinical picture. PMID:24842605
Jingami, Naoto; Matsumoto, Riki; Ito, Hirotaka; Ishii, Atsushi; Ihara, Yukiko; Hirose, Shinichi; Ikeda, Akio; Takahashi, Ryosuke
Aripiprazole (APZ) is regarded as a first-line atypical antipsychotic used for the treatment of first and multiple episodes of schizophrenia to improve positive- and negative-symptoms. Its therapeutic indications were extended to acute manic and mixed episodes associated with bipolar disorder. In addition, APZ was approved as an adjunct therapy for major depressive disorder in 2007. Compared to other antipsychotic drugs, APZ has a unique pharmacological profile. It is a partial agonist at D? dopamine receptors and serotonin 5-HT(1A) and 5-HT? receptors, whereas it is an antagonist at serotonin 5-HT(2A) and 5-HT? receptors. Since epilepsy is often accompanied with neurological comorbidities such as depression, anxiety and cognitive deficits caused by both the disease and/or drug treatment, we wished to examine the effects of a sub-chronic treatment (>14 consecutive days) with APZ (0.3, 1 and 3 mg/kg; i.p.) on both absence seizures and WAG/Rij rat's behavior using different standard paradigms: Open field (OF) test, elevated plus maze (EPM) test, forced swimming (FS) test, sucrose consumption (SC) test and Morris water maze (MWM). WAG/Rij rats represent a validated genetic animal model of absence epilepsy with mild-depression comorbidity, also including other behavioral alterations. APZ treatment showed some anti-absence properties and regarding the behavioral comorbidity in this rat strain, we observed that APZ possesses clear antidepressant effects in the FS and SC tests also increasing memory/learning function in the Morris water maze test. In the two anxiety models used, APZ showed only minor effects. In conclusion, our results indicate that APZ might actually have a potential in treating absence seizures or as add-on therapy but more interestingly, these effect might be accompanied by positive modulatory actions on depression, anxiety and memory which might be also beneficial in other epileptic syndromes. This article is part of a Special Issue entitled 'Cognitive Enhancers'. PMID:22766393
Russo, Emilio; Citraro, Rita; Davoli, Alessandro; Gallelli, Luca; Di Paola, Eugenio Donato; De Sarro, Giovambattista
Most people learn about seizures from their doctors, but others know only what they have seen on television. Unfortunately, visits to doctor's office aren't long enough to learn all that is needed, and often times, doctors and nurses aren't available to teach this information. Seizures are often represented inaccurately and too dramatically on…
Shafer, Patricia O.; Schachter, Steven C.
Medications are the primary treatment for epilepsy, yet many teens and their families have problems managing seizure medicines. Fear of side effects, difficulties remembering to take medicines and figuring out how to take them are common challenges. Unfortunately, not taking medicine as prescribed can lead to breakthrough seizures, which in turn…
Shafer, Patricia Osborne; Israel, Beth
This is a case study presented by the University of Pittsburgh Department of Pathology in which a 24-day-old baby is failing to thrive and experiencing seizures. Visitors are given the microscopic description, with images, the results of the postmortem examination, and are given the opportunity to diagnose the patient. This is an excellent resource for students in the health sciences to familiarize themselves with using patient history and laboratory results to diagnose disease. It is also a helpful site for educators to use to introduce or test student learning in pediatric pathology.
Dickman, Paul S.; Lyons, Valerie
Objective In patients with spontaneous intracerebral hemorrhage (ICH), the risk factors for seizure and the effect of prophylactic anticonvulsants are not well known. This study aimed to determine the risk factor for seizures and the role for prophylactic anticonvulsants after spontaneous ICH. Methods Between 2005 and 2010, 263 consecutive patients with spontaneous ICH were retrospectively assessed with a mean follow-up of 19.5 months using medical records, updated clinical information and, when necessary, direct patient contact. The seizures were classified as early (within 1 week of ICH) or late (more than 1 week after ICH). The outcomes were measured with the Glasgow Outcome Scale at discharge and the modified Rankin Scale (mRS) at both 2 weeks and discharge. Results Twenty-two patients (8.4%; 9 patients with early seizures and 13 patients with late seizures) developed seizures after spontaneous ICH. Out of 263 patients, prophylactic anticonvulsants were administered in 216 patients. The prophylactic anticonvulsants were not associated with a reduced risk of early (p=0.094) or late seizures (p=0.326). Instead, the factors associated with early seizure were cortical involvement (p<0.001) and younger age (60 years or less) (p=0.046). The risk of late seizure was increased by cortical involvement (p<0.001) and communicating hydrocephalus (p=0.004). The prophylactic anticonvulsants were associated with a worse mRS at 2 weeks (p=0.024) and at last follow-up (p=0.034). Conclusion Cortical involvement may be a factor for provoked seizures. Although the incidence of early seizures tended to decrease in patients prescribed prophylactic anticonvulsants, no statistical difference was found.
Woo, Kwang-Moo; Cho, Keun-Tae
It is agreed that 1% of the general population is afflicted with epilepsy and close to 30% of epilepsy patients are intractable to medications. In spite of a recent increase in the number of new medications that are available on the market, many patients continue to have seizures or their seizures are controlled at the expense of intolerable side effects.
Basim M Uthman
EPILEPSY, or the propensity to have recurrent seizures, is the most common chronic neurological disorder in dogs, with an estimated prevalence of between 0·5 and 5·7 per cent. There are numerous primary intracranial diseases and extracranial (eg, metabolic or toxic) conditions that cause recurrent seizures, although idiopathic epilepsy is the most common in dogs. Metabolic and toxic disorders are much
Kate Chandler; Holger Volk
Current epileptic seizure "prediction" algorithms are generally based on the knowledge of seizure occurring time and analyze the electroencephalogram (EEG) recordings retrospectively. It is then obvious that, although these analyses provide evidence of brain activity changes prior to epileptic seizures, they cannot be applied to develop implantable devices for diagnostic and therapeutic purposes. In this paper, we describe an adaptive procedure to prospectively analyze continuous, long-term EEG recordings when only the occurring time of the first seizure is known. The algorithm is based on the convergence and divergence of short-term maximum Lyapunov exponents (STLmax) among critical electrode sites selected adaptively. A warning of an impending seizure is then issued. Global optimization techniques are applied for selecting the critical groups of electrode sites. The adaptive seizure prediction algorithm (ASPA) was tested in continuous 0.76 to 5.84 days intracranial EEG recordings from a group of five patients with refractory temporal lobe epilepsy. A fixed parameter setting applied to all cases predicted 82% of seizures with a false prediction rate of 0.16/h. Seizure warnings occurred an average of 71.7 min before ictal onset. Similar results were produced by dividing the available EEG recordings into half training and testing portions. Optimizing the parameters for individual patients improved sensitivity (84% overall) and reduced false prediction rate (0.12/h overall). These results indicate that ASPA can be applied to implantable devices for diagnostic and therapeutic purposes. PMID:12769437
Iasemidis, Leon D; Shiau, Deng-Shan; Chaovalitwongse, Wanpracha; Sackellares, J Chris; Pardalos, Panos M; Principe, Jose C; Carney, Paul R; Prasad, Awadhesh; Veeramani, Balaji; Tsakalis, Konstantinos
A case is presented of a patient with a long history of epilepsy who presents with recurrent seizures and develops a period of asystole. The case highlights the need to consider the potential arrhythmic complications of seizures and the clinical characteristics that may be present in those with epilepsy that may warrant evaluation for arrhythmias. PMID:22877730
Stokes, Michael B; Palmer, Sonny; Moneghetti, Kegan J; Mariani, Justin A; Wilson, Andrew M
The topic of alcohol withdrawal syndrome (AWS), including delirium tremens and especially seizures, is reviewed. From mice and rat studies, it is known that both N-methyl-d-aspartate (NMDA) and gamma-aminobutyric acid (GABA) receptors are involved in AWS. During alcohol intoxication chronic adaptations of NMDA and GABA receptors occur, and during alcohol withdrawal a hyperexcitable state develops. In studies on humans, during intoxication the NMDA receptors are activated and mediate tonic inhibition. In withdrawal, a rebound activation of these receptors occurs. Both GABA-A and GABA-B receptors, especially the alpha2 subunit of GABA-A receptors, are also likely involved. Homocysteine increases with active drinking, and in withdrawal, excitotoxicity likely is induced by a further increase in homocysteine, viewed as a risk factor for AWS and also as a screening tool. The dopamine transporter gene is also associated with AWS. Characteristics involves changes in the ECG, especially an increase in QT interval, and EEG changes, including abnormal quantified EEG, at times periodic lateralized epileptiform discharges, and especially seizures, usually occurring 6-48h after the cessation of drinking. Therapy has emphasized benzodiazepines, mainly diazepam and lorazepam, but more standard antiepileptic drugs, like carbamazepine and topiramate, are also effective and safe. PMID:19249388
Hughes, John R
The FDA approval for clozapine in 1990 under several hematologic surveillance conditions has reactualized the debate on the use of atypical neuroleptics for adults with schizophrenia. The use of conventional neuroleptics in children and adolescents has always been a subject of controversy due to their side effects and the absence of controlled studies. The pharmacological action of clozapine and risperidone is mainly on D2 and 5HT. Since 1992 several studies concerning children and adolescents show the efficiency and the tolerance of the clozapine and risperidone in various disorders, especially in very early onset schizophrenia (VEOS). Controlled trials are necessary to confirm the data obtained in open studies. PMID:9809243
Naja, W J; Reneric, J P; Bouvard, M P
Epilepsy is the second most common neurological disorder, affecting 0.6–0.8% of the world's population. In this neurological disorder, abnormal activity of the brain causes seizures, the nature of which tend to be sudden. Antiepileptic Drugs (AEDs) are used as long-term therapeutic solutions that control the condition. Of those treated with AEDs, 35% become resistant to medication. The unpredictable nature of seizures poses risks for the individual with epilepsy. It is clearly desirable to find more effective ways of preventing seizures for such patients. The automatic detection of oncoming seizures, before their actual onset, can facilitate timely intervention and hence minimize these risks. In addition, advance prediction of seizures can enrich our understanding of the epileptic brain. In this study, drawing on the body of work behind automatic seizure detection and prediction from digitised Invasive Electroencephalography (EEG) data, a prediction algorithm, ASPPR (Advance Seizure Prediction via Pre-ictal Relabeling), is described. ASPPR facilitates the learning of predictive models targeted at recognizing patterns in EEG activity that are in a specific time window in advance of a seizure. It then exploits advanced machine learning coupled with the design and selection of appropriate features from EEG signals. Results, from evaluating ASPPR independently on 21 different patients, suggest that seizures for many patients can be predicted up to 20 minutes in advance of their onset. Compared to benchmark performance represented by a mean S1-Score (harmonic mean of Sensitivity and Specificity) of 90.6% for predicting seizure onset between 0 and 5 minutes in advance, ASPPR achieves mean S1-Scores of: 96.30% for prediction between 1 and 6 minutes in advance, 96.13% for prediction between 8 and 13 minutes in advance, 94.5% for prediction between 14 and 19 minutes in advance, and 94.2% for prediction between 20 and 25 minutes in advance.
Moghim, Negin; Corne, David W.
Opinion statement Solid organ transplantation is frequently complicated by a spectrum of seizure types, including single partial-onset or generalized tonic-clonic seizures, acute repetitive seizures or status epilepticus, and sometimes the evolution of symptomatic epilepsy. There is currently no specific evidence involving the transplant patient population to guide the selection, administration, or duration of antiepileptic drug (AED) therapy, so familiarity with clinical AED pharmacology and application of sound judgment are necessary for successful patient outcomes. An initial detailed search for symptomatic seizure etiologies, including metabolic, infectious, cerebrovascular, and calcineurin inhibitor treatment-related neuro-toxic complications such as posterior reversible encephalopathy syndrome (PRES), is imperative, as underlying central nervous system disorders may impose additional serious risks to cerebral or general health if not promptly detected and appropriately treated. The mainstay for post-transplant seizure management is AED therapy directed toward the suspected seizure type. Unfavorable drug interactions could place the transplanted organ at risk, so choosing an AED with limited interaction potential is also crucial. When the transplanted organ is dysfunctional or vulnerable to rejection, AEDs without substantial hepatic metabolism are favored in post-liver transplant patients, whereas after renal transplantation, AEDs with predominantly renal elimination may require dosage adjustment to prevent adverse effects. Levetiracetam, gabapentin, pregabalin, and lacosamide are drugs of choice for treatment of partial-onset seizures in post-transplant patients given their efficacy spectrum, generally excellent tolerability, and lack of drug interaction potential. Levetiracetam is the drug of choice for primary generalized seizures in post-transplant patients. When intravenous drugs are necessary for acute seizure management, benzodiazepines and fosphenytoin are the traditional and best evidence-based options, although intravenous levetiracetam, valproate, and lacosamide are emerging options. Availability of several newer AEDs has greatly expanded the therapeutic armamentarium for safe and efficacious treatment of post-transplant seizures, but future prospective clinical trials and pharmacokinetic studies within this specific patient population are needed.
Shepard, Paul W.
This article reviews the most frequent causes of seizure disorders in young children and the classification of different seizure types. It discusses current therapies, including alternatives to medication. Emergency response to seizures is covered a well as non-epileptic episodes that may resemble seizures. Epilepsy's potential impact on the…
McBrien, Dianne M.; Bonthius, Daniel J.
We apply chaotic time series analysis (CTSA) to human electroencephalogram (EEG) data. Three epoches were examined: epileptic seizure, non-seizure, and transition from non-seizure to seizure. The CTSA tools were applied to four forms of these data: raw EE...
L. M. Hively N. E. Clapp C. S. Daw W. F. Lawkins M. L. Eisenstadt
Reports focusing on auras of ecstasy or pleasure have been limited largely to single case descriptions. We examined 11 consecutive patients with such ictal symptoms. Eight had sensory hallucinations, four had erotic sensations, five described "a religious/spiritual experience," and several had symptoms that were felt to have no counterpart in human experience. Ictal EEG recordings were performed in four patients; two had seizure onset in the right temporal lobe and two in the left. In seven the onset could not be definitely localized. The diagnosis of epilepsy was often delayed. Eight patients wished to experience seizures; self-induction was possible in five and four showed treatment noncompliance. In patients with insufficient drug intake, in whom good compliance should be expected, it is relevant to consider seizures with pleasant symptomatology. According to the literature, experiential and ecstatic seizures seem to have had a substantial impact on our cultural and religious history. PMID:14698700
Asheim Hansen, Bjørn; Brodtkorb, Eylert
Background—The purpose of our study was to define the incidence and mechanisms of atypical right atrial flutter. Methods and Results—A total of 28 (8%) of 372 consecutive patients with atrial flutter (AFL) had 36 episodes of sustained atypical right AFL. Among 24 (67%) of 36 episodes of lower loop reentry (LLR), 13 (54%) of 24 episodes had early breakthrough at
Yanfei Yang; Jie Cheng; Andy Bochoeyer
Several potential pathophysiologic phenomena, including “cerebral shutdown,” are postulated to be responsible for SUDEP. Since the evidence for a seizure-related mechanism is strong, a poor understanding of the physiology of human seizure termination is a major handicap. However, rather than a failure of a single homeostatic mechanism, such as postictal arousal, it may be a “perfect storm” created by the lining up of a several factors that lead to death.
Bozorgi, Alireza; Lhatoo, Samden D.
Tobacco smoking is considered the greatest risk factor for death caused by noncommunicable diseases. In contrast to extensive research on the association between tobacco smoking and diseases such as heart attack, stroke, and cancers, studies on the association between tobacco smoking and seizures or epilepsy are insufficient. The exact roles tobacco smoking and nicotine use play in seizures or epilepsy have not been well reviewed. We reviewed available literature and found that 1) there are vast differences between tobacco smoke and nicotine based on their components and their effects on seizures or epilepsy; 2) the seizure risk in acute active tobacco smokers, women who smoke during pregnancy, electronic cigarette smokers, and the role of smoking in sudden unexplained/unexpected death in epilepsy remain unclear; 3) seizure risks are higher in acute secondhand smokers, chronic active smokers, and babies whose mothers smoke; 4) tobacco smoke protects against seizures in animal models whereas nicotine exerts mixed effects in animals; and 5) tobacco smoking agents can be noneffective, proconvulsant, or anticonvulsant. Finally, the opportunities for future research on this topic is discussed. PMID:24441294
Rong, Lingling; Frontera, Alfred T; Benbadis, Selim R
Objective To report a rare case of atypical histiocytic tumor of the lung with a review of literature. Methods The clinical materials were noted. Literature related to this condition from the past 50 years was reviewed from the group of histiocytic tumors. Results and conclusions Clinical manifestations were non-specific. The imaging characteristics of our case were infiltrative lesions with multiple cysts in both lungs. Pathology showed nodular proliferation of atypical cells. Immunohistochemistry suggested a histiocytic origin of the infiltrating atypical cells. Because the pathological findings did not fall into any particular category of typical histiocytic tumors, the final diagnosis was atypical histiocytic tumor. The presentation of atypical histiocytic tumor of the lungs, only, with infiltrative lesions and multiple air cysts seems very rare, with pathological examination being “gold standard” for the diagnosis.
Cao, Weijun; Zhang, Rongxuan; Zhou, Ying; Xu, Jinfu; Garfield, David H.
As the Internet has become rapidly and widely integrated into society, Internet addiction has become a growing psychosocial problem. However, epileptic seizure, another out-of-the-ordinary health problem, is often neglected in this regard. Ten patients who experienced epileptic seizures while playing the newest genre of electronic games -- Massively Multiplayer Online Role-Playing Games (MMORPGs) -- were investigated. Patients were predominantly male young adults, and most of the events were generalized tonic-clonic seizures, myoclonic seizures, and absences. These patients should be categorized into idiopathic generalized epilepsies. Even though photosensitivity was an important factor, behavioral and higher mental activities also seemed to be significant seizure precipitants. Results demonstrated that MMORPG-induced seizures were not analogous to the ordinary video game-induced seizures. Significantly, an epileptic seizure warning did not always appear on the websites of MMORPGs and instructions for the software. While the prevalence of MMORPG-induced seizures remains unknown, it may exceed our expectations and impact our society. Not only for clinical neurologists but also for the primary physicians, educators, sociologists, and global online game publishers, there should be an awareness of this special form of reflex seizures in order to provide an appropriate health warning to MMORPG players. PMID:16901249
Background Central serous chorioretinopathy (CSC) has been traditionally treated with laser photocoagulation. We thought that transpupillary thermotherapy (TTT) utilizing a lower temperature than that of conventional laser photocoagulation might minimize permanent retinal and choroidal damage. Studies suggest that undesirable effects on vision due to TTT are minimal even if it is applied to foveal and/or parafoveal lesions when TTT requires a larger irradiation spot. The aim of this study was to evaluate the efficacy of TTT in the management of atypical CSC. Methods We defined atypical CSC as bullous retinal detachment with diffuse or several leakages, severe leakage with fibrin formation under serous retinal detachment, or leakage within a pigment epithelium detachment. Eight consecutive patients with atypical CSC underwent visual acuity testing, ophthalmic examination, color photography, fluorescein angiography, and optical coherence tomography to evaluate the results of transpupillary thermotherapy. Retreatment of atypical CSC was based on ophthalmic examination, optical coherence tomography, and fluorescein angiography. TTT was performed on the leaking spots shown in fluorescein angiography, with a power of 50–250 mW, spot size of 500–1200 ?m, and exposure time of 13–60 seconds to minimize retinal damage. Results In five of eight affected eyes, serous detachments completely resolved within 1 month after the initial TTT. One eye had persistent subretinal fluid and required a second TTT treatment. Two eyes showed no resolution of CSC and were treated by conventional photocoagulation. Initial best-corrected visual acuity (BCVA) ranged from 20/600 to 20/20 (mean, 20/40; median, 20/30). Final BCVA ranged from 20/200 to 20/20 (mean, 20/25; median, 20/20). BCVA improved in all cases. Only two eyes with persistent subretinal fibrin and existing retinal pigment epithelial alternations in macular area showed limited improvement of BCVA despite the absence of subretinal exudation. The presence of retinal attachment was confirmed by optical coherence tomography in six eyes (75%). Conclusions TTT seems to be effective for the treatment of atypical CSC in the short term. Additional studies are necessary to evaluate the long-term effectiveness and safety.
Kawamura, Ryosuke; Ideta, Hidenao; Hori, Hideyuki; Yuki, Kenya; Uno, Tsuyoshi; Tanabe, Tatsurou; Tsubota, Kazuo; Kawasaki, Tsutomu
Cholestatic liver disease consists of a variety of disorders. Primary sclerosing cholangitis and primary biliary cirrhosis are the most commonly recognized cholestatic liver disease in the adult population, while biliary atresia and Alagille syndrome are commonly recognized in the pediatric population. In infants, the causes are usually congenital or inherited. Even though jaundice is a hallmark of cholestasis, it is not always seen in adult patients with chronic liver disease. Patients can have “silent” progressive cholestatic liver disease for years prior to development of symptoms such as jaundice and pruritus. In this review, we will discuss some of the atypical causes of cholestatic liver disease such as benign recurrent intrahepatic cholestasis, progressive familial intrahepatic cholestasis, Alagille Syndrome, biliary atresia, total parenteral nutrition induced cholestasis and cholestasis secondary to drug induced liver injury.
Nguyen, Ken D; Sundaram, Vinay; Ayoub, Walid S
Some patients with eating disorders have neither anorexia nervosa (A.N.) nor bulimia. Cases which do not rigorously meet the DSM-III-R criteria for anorexia nervosa or for bulimia are usually defined as "eating disorders N.O.S." Among them are patients with pathological characteristics very closely related to the above-mentioned categories. Others, however, although affected by an eating disorder, present a quite different clinical picture from either A.N. or bulimia. In a study of 80 eating disorder cases, only 45 met the strict definition of A.N. or bulimia. The other 35 were diagnosed as atypical eating disorders and are the focus of this presentation. 29 were classified as Eating Disorders N.O.S. and 6 as obesity. Co-morbidity, gender and age data, and clinical vignettes are presented. PMID:1390797
Wilson's disease is a genetic disorder of copper metabolism with a hepatic or neurologic presentation. A hepatic presentation is more common in young children. Neurologic Wilson's disease often manifests as a movement disorder with dystonia, tremor, and dysarthria. Psychiatric or behavioral symptoms can also be a presenting feature of Wilson's disease. We describe an atypical neurologic presentation in a prepubertal child with minimal hepatic involvement; in which transient hemiparesis and encephalopathy dominated her initial neurologic presentation. Brain magnetic resonance imaging revealed extensive cortical and subcortical signal change, in addition to the classical basal ganglia signal abnormality observed in Wilson's disease. She was treated with oral tetrathiomolybdate anticopper therapy, followed by zinc maintenance. Her clinical status and brain imaging improved considerably at 1 year after treatment initiation. Neurologic Wilson's disease may have diverse presentations, and should be considered in children who present with cortical features and signal change on magnetic resonance imaging. PMID:14738953
Carlson, Martha D; Al-Mateen, Majeed; Brewer, George J
Summary Seizure prediction has proven to be difficult in clinically realistic environments. Is it possible that fluctuations in cortical firing could influence the onset of seizures in an ictal zone? To test this, we have now used neural network simulations in a computational model of cortex having a total of 65,536 neurons with intercellular wiring patterned after histological data. A spatially distributed Poisson driven background input representing the activity of neighboring cortex affected 1% of the neurons. Gamma distributions were fit to the interbursting phase intervals, a non-parametric test for randomness was applied, and a dynamical systems analysis was performed to search for period-1 orbits in the intervals. The non-parametric analysis suggests that intervals are being drawn at random from their underlying joint distribution and the dynamical systems analysis is consistent with a nondeterministic dynamical interpretation of the generation of bursting phases. These results imply that in a region of cortex with abnormal connectivity analogous to a seizure focus, it is possible to initiate seizure activity with fluctuations of input from the surrounding cortical regions. These findings suggest one possibility for ictal generation from abnormal focal epileptic networks. This mechanism additionally could help explain the difficulty in predicting partial seizures in some patients.
Azhar, Feraz; Kudela, Pawel; Bergey, Gregory K.; Franaszczuk, Piotr J.
In recent years, an increasing number of studies have investigated the effects of closed-loop anti-epileptic treatments. Most of the current research still is very labour intensive: real-time treatment is manually triggered and conclusions can only be drawn after multiple days of manual review and annotation of the electroencephalogram (EEG). In this paper we propose a technique based on reservoir computing (RC) to automatically and in real-time detect epileptic seizures in the intra-cranial EEG (iEEG) of epileptic rats in order to immediately trigger seizure treatment. The performance of the system is evaluated in two different seizure types: absence seizures from genetic absence epilepsy rats from Strasbourg (GAERS) and limbic seizures from post status epilepticus (PSE) rats. The dataset consists of 452 hours iEEG from 23 GAERS and 2083 hours iEEG from 22 PSE rats. In the default set-up the system detects 0.09 and 0.13 false positives per seizure and misses 0.07 and 0.005 events per seizure for GAERS and PSE rats respectively. It achieves an average detection delay below 1s in GAERS and less than 10s in the PSE data. This detection delay and the number of missed seizures can be further decreased when a higher false positive rate is allowed. Our method outperforms state-of-the-art detection techniques and only a few parameters require optimization on a limited training set. It is therefore suited for automatic seizure detection based on iEEG and may serve as a useful tool for epilepsy researchers. The technique avoids the time-consuming manual review and annotation of EEG and can be incorporated in a closed-loop treatment strategy. PMID:22854191
Buteneers, Pieter; Verstraeten, David; Nieuwenhuyse, Bregt Van; Stroobandt, Dirk; Raedt, Robrecht; Vonck, Kristl; Boon, Paul; Schrauwen, Benjamin
Martin Luther's diseases are well documented, because he used to discuss them freely in his letters. There is also a wealth of evidence through reports by his friends. Most of his diseases were common and well known to the contemporary physicians, who accordingly interpreted them correctly: bladder stones, chronic constipation, hemorrhoids. Luther's death obviously was due to a coronary thrombosis. During the last 19 years of his life, in addition to these "natural diseases", Luther also suffered from recurring attacks of a peculiar symptomatology. Luther himself and his friends considered these seizures to be no "natural disease", but Satan punching his flesh, and he compared them to St. Paul's disease (2. Cor. 12). The first of these attacks occurred on July 6, 1527, when Luther was 43 years of age. It began with a roaring tinnitus in his left ear, which increased dramatically and seemed to occupy the left half of his head. Then a state of sickness and collapse followed, however, consciousness was retained throughout the whole period. After a night's rest all the symptoms had subsided, except the tinnitus, which, from that day on, continued for all the following years in varying intensity. Similar attacks with increase of the tinnitus and vertigo as the leading symptoms, seized Luther at irregular intervals and distressed him extremely. Former investigators of Luther's diseases interpreted these attacks as manifestations of a psychiatric disorder and a chronic inflammatory disease of the middle ear. The present detailed study reveals that it was a typical case of Menière's disease of the left ear manifesting itself more than 330 years before Menière's classical observation. PMID:2529669
In this retrospective study, we collected clinical and radiographic data on children (age range, 1 month to 18 years) with symptoms and radiographic confirmation of seizure after ischemic stroke for the period of January 1996 to July 2006. Thirty-nine out of 94 children with ischemic stroke had poststroke seizures. Thirty-three out of 39 children with poststroke seizures had new onset seizures but only data of 28 were available. Infection was the most common etiology in the early poststroke seizure group (52.4%) but not in the late poststroke seizure group (0%). Infarction involving arterial ischemic stroke of anterior circulation were the most common in both the early poststroke seizure (61.9%) and the late poststroke seizure group (57.1%). Epilepsy was the most common sequelae in both the early poststroke seizure (38.1%) and late poststroke seizure group (100%). Children who had initial focal neurological sign (100% vs. 38.1%; P=0.007) or the focal cortical dysfunction on EEG (85.7% vs. 33.3%; P=0.029) were prone to develop late poststroke seizures. Late poststroke seizures had a high risk of developing poststroke epilepsy (100% vs. 38.1%; P=0.007). We conclude that seizures commonly occur in childhood ischemic stroke. Most poststroke seizures developed at an early stage. Infection was the most common etiology that caused early poststroke seizures in childhood ischemic stroke. Initial focal neurological signs and focal cortical dysfunction on EEG are risk factors for developing epilepsy. Poststroke seizures did not affect mortality, but there was a significant difference in normal outcome and epilepsy between those with or without poststroke seizures. PMID:18650041
Lee, Jiun-Chang; Lin, Kuang-Lin; Wang, Huei-Shyong; Chou, Min-Liang; Hung, Po-Cheng; Hsieh, Meng-Ying; Lee, Ying-Ying; Lin, Jainn-Jim; Wong, Alex Mun-Ching
Sulfolane dosages that alter seizure susceptibility were determined using audiogenic (AG), pentylenetrazol (PTZ) and hippocampal afterdischarge (AD) seizure models. The presence of AG seizures and potentiation of PTZ seizures were investigated in rats injected IP with 0, 200, 400...
In mammals, odorants are detected by a large family of receptors that are each expressed in just a small subset of olfactory sensory neurons (OSNs). Here we describe a strain of transgenic mice engineered to express an octanal receptor in almost all OSNs. Remarkably, octanal triggered a striking and involuntary phenotype in these animals, with passive exposure regularly inducing seizures. Octanal exposure invariably resulted in widespread activation of OSNs but interestingly seizures only occurred in 30–40% of trials. We hypothesized that this reflects the need for the olfactory system to filter strong but slowly-changing backgrounds from salient signals. Therefore we used an olfactometer to control octanal delivery and demonstrated suppression of responses whenever this odorant is delivered slowly. By contrast, rapid exposure of the mice to octanal induced seizure in every trial. Our results expose new details of olfactory processing and provide a robust and non-invasive platform for studying epilepsy.
Nguyen, Minh Q.; Ryba, Nicholas J. P.
Mutations in the CDKL5 gene (also known as STK9) have recently been shown to cause early onset epilepsy and severe mental retardation (ISSX or West syndrome). Patients with CDKL5 mutations sometimes also show features similar to those seen in Rett Syndrome (RTT). We have screened the CDKL5 gene in 94 patients with RTT or a RTT-like phenotype who had tested negative for MECP2 mutations (13 classical RTT female subjects, 25 atypical RTT female subjects, 40 RTT-like female and 16 RTT-like male subjects; 33 of the patients had early onset seizures). Novel pathogenic CDKL5 mutations were identified in three girls, two of whom had initially been diagnosed with the early onset seizure variant of RTT and the other with early onset seizures and some features of RTT. In addition, the 33 patients with early seizures were screened for the most common mutations in the ARX gene but none were found. Combining our three new cases with the previously published cases, 13/14 patients with CDKL5 mutations presented with seizures before the age of 3 months. PMID:16015284
Evans, Julie C; Archer, Hayley L; Colley, James P; Ravn, Kirstine; Nielsen, Jytte Bieber; Kerr, Alison; Williams, Elizabeth; Christodoulou, John; Gécz, Jozef; Jardine, Philip E; Wright, Michael J; Pilz, Daniela T; Lazarou, Lazarus; Cooper, David N; Sampson, Julian R; Butler, Rachel; Whatley, Sharon D; Clarke, Angus J
We reviewed case records of 40 in-patients (22 boys) with serologically confirmed dengue fever between 1st October and 30th November, 2013. Severe dengue was seen in 30, out of which 12 (30%) had compensated shock. Splenomegaly (6,15%) and encephalopathy (4,10%) were the commonest atypical features. Atypical manifestations of dengue fever were more common than that reported in the past. PMID:24986292
Pawaria, Arti; Mishra, Devendra; Juneja, Monica; Meena, Jagdish
Transexamic acid (TXA) is an antifibrinolytic that has been used successfully to prevent blood loss during major surgery. However, as its usage has increased, there have been growing reports of postsurgical seizure events in cardiac surgery patients. In this issue of the JCI, Lecker et al. explore this connection and suggest that TXA-mediated inhibition of glycine receptors may underlie the effect. This finding prompted the authors to explore the preclinical efficacy of common anesthetics that function by reducing the TXA-mediated inhibition to prevent or modify postsurgical seizures.
Schwinn, Debra A.; Mackensen, G. Burkhard; Brown, Emery N.
A 40-year-old Mauritanian man consulted for back pain. A computed tomography of the spine showed patchy sclerosis of the fifth and seventh thoracic vertebral bodies with normal neural arch of T5 and sclerosis and hypertrophy of the neural arch of T7, as well as diffuse sclerosis of the T11 vertebral body with a normal neural arch. At MRI, low signal-intensity on T1-weighted images and high signal-intensity on T2-weighted images involved the whole T5 and T7 vertebrae and the vertebral body of T11. Working diagnoses included metastatic disease and lymphoma, and a biopsy of T7 and then T11 was carried out. Both showed pathological findings very suggestive of Paget's disease. Since CT is usually the more specific radiological examination in vertebral Paget's disease, we thought it could be useful to report this atypical CT presentation (patchy sclerosis of the vertebral body without diffuse bone texture changes and isolated involvement of the vertebral body) of vertebral Paget's disease. PMID:24445956
Beaudouin, Constance; Dohan, Anthony; Nasrallah, Toufic; Parlier, Caroline; Touraine, Sébastien; Ea, Korng; Kaci, Rachid; Laredo, Jean-Denis
Febrile seizures are the most common seizure type in young children. Whether they induce death of hippocampal and amyg- dala neurons and consequent limbic (temporal lobe) epilepsy has remained controversial, with conflicting data from prospec- tive and retrospective studies. Using an appropriate-age rat model of febrile seizures, we investigated the acute and chronic effects of hyperthermic seizures on neuronal integrity
Zsolt Toth; Xiao-Xin Yan; Suzie Haftoglou; Charles E. Ribak; Tallie Z. Baram
Psychogenic non epileptic seizure (PNES) can be induced by several induction tests but their relative usefulness has not been evaluated. In this study, we report the sensitivity and specificity of various induction tests in the diagnosis of PNES and assess their discomfort level. The induction tests were: (a) compression of temple region (CTR), (b) verbal suggestion (VS), (c) tuning fork application (TFA), (d) moist swab application (MSA), (e) torch light stimulation (TLS) and (f) saline injection (SI). Up to 3 trials were done for each test except for normal saline injection which was given once. For comparison of these tests, patients with epileptic seizures were included as controls. The time to precipitate PNES was recorded and patients' discomfort levels were noted on a 0-10 scale. Video EEG was recorded in the PNES patients. 140 patients with PNES and 50 controls with epileptic seizures were included. The diagnostic yield of CTR was 65.7%, TFA 61.4%, MSA 60.7%, SI 55.6%, VS 54.3% and TLS 40.7%. These tests did not induce seizures in the controls. All these tests had 100% specificity and 100% positive predictive value in the diagnosis of PNES. The maximum discomfort was reported with SI and minimum with MSA. The similarity of efficacy and discomfort with CTR and TFA appear to be the most optimal induction techniques when compared with VS, AMS, TLS, and SI. PMID:24802296
Goyal, Gourav; Kalita, Jayantee; Misra, Usha K
With a goal of automating visual analysis of electroencephalogram (EEG) data and assessing the performance of various features in seizure recognition, we introduce a mathematical model capable of recognizing patient-specific epileptic seizures with high accuracy. We represent multi-channel scalp EEG using a set of features. These features expected to have distinct trends during seizure and non-seizure periods include features from
Evrim Acar; Canan Aykut Bingol; Haluk Bingol; Rasmus Bro; B. Yener
In this case series we report on eight neonates with refractory seizures who received intravenous levetiracetam when seizures did not respond to two or more conventional anticonvulsants. Six of the eight neonates had an excellent response with either cessation, or reduction in seizures by at least 80%. One neonate showed a partial response while one did not have any reduction in seizure frequency. We did not encounter any adverse effects that could be attributable to levetiracetam. PMID:23664133
Rakshasbhuvankar, Abhijeet; Rao, Shripada; Kohan, Rolland; Simmer, Karen; Nagarajan, Lakshmi
A fugue state is defined as an altered state of consciousness with varying degrees of motor activity and amnesia for the event. It may last for hours to days and may be psychogenic or organic in nature. Epileptic fugue states can be encountered in patients with absence or complex partial nonconvulsive status epilepticus or may occur as a postictal phenomenon in patients with generalized seizures. “absence status epilepticus” (AS) is rare and seen in only 2.6% of the cases with “childhood absence epilepsy” (CAE). The diagnosis of AS can be elusive, but sudden onset and termination of the fugue state, classical electroencephalogram (EEG) features, and response to a therapeutic trial of benzodiazepines helps in confirming the diagnosis and differentiating it from nonepileptic fugue states. We report a childhood onset case, with a 10 years history of recurrent episodes of prolonged fugue state lasting for up to 24 h, as the sole manifestation of epileptic seizures. The EEG features were suggestive of an AS, but there was no history of typical absences, myoclonus, or generalized tonic clonic seizures. This unusual and rare case cannot be categorized into one of the defined epilepsy syndromes like CAE but belongs to a recently identified syndrome of idiopathic generalized epilepsy known as “Absence status epilepsy” in which AS is the sole or the predominant seizure type.
Khwaja, Geeta A.; Duggal, Ashish; Kulkarni, Amit; Chaudhry, Neera; Gupta, Meena; Chowdhury, Debashish; Bohra, Vikram
neralpopulation. These disturbances (seizures) result in a variety of intermittent clinical phenomena including motor, sensory, affective,cognitive, autonomic and psychic symptomology. In human epilepsy of mesial temporal lobe origin, seizures begin in the hippocampusand are often propagated throughout the brain. A central feature of the epileptogenic hippocampus is the tendency to make abrupttransitions to well organized oscillations, characteristic of a seizure.
Leonidas D. Iasemidis; Konstantinos E. Pappas; Jose C. Principe; J. Chris Sackellares
Hemolytic uremic syndrome (HUS) is defined by the triad of mechanical hemolytic anemia, thrombocytopenia and renal impairment. Atypical HUS (aHUS) defines non Shiga-toxin-HUS and even if some authors include secondary aHUS due to Streptococcus pneumoniae or other causes, aHUS designates a primary disease due to a disorder in complement alternative pathway regulation. Atypical HUS represents 5 -10% of HUS in children, but the majority of HUS in adults. The incidence of complement-aHUS is not known precisely. However, more than 1000 aHUS patients investigated for complement abnormalities have been reported. Onset is from the neonatal period to the adult age. Most patients present with hemolytic anemia, thrombocytopenia and renal failure and 20% have extra renal manifestations. Two to 10% die and one third progress to end-stage renal failure at first episode. Half of patients have relapses. Mutations in the genes encoding complement regulatory proteins factor H, membrane cofactor protein (MCP), factor I or thrombomodulin have been demonstrated in 20-30%, 5-15%, 4-10% and 3-5% of patients respectively, and mutations in the genes of C3 convertase proteins, C3 and factor B, in 2-10% and 1-4%. In addition, 6-10% of patients have anti-factor H antibodies. Diagnosis of aHUS relies on 1) No associated disease 2) No criteria for Shigatoxin-HUS (stool culture and PCR for Shiga-toxins; serology for anti-lipopolysaccharides antibodies) 3) No criteria for thrombotic thrombocytopenic purpura (serum ADAMTS 13 activity > 10%). Investigation of the complement system is required (C3, C4, factor H and factor I plasma concentration, MCP expression on leukocytes and anti-factor H antibodies; genetic screening to identify risk factors). The disease is familial in approximately 20% of pedigrees, with an autosomal recessive or dominant mode of transmission. As penetrance of the disease is 50%, genetic counseling is difficult. Plasmatherapy has been first line treatment until presently, without unquestionable demonstration of efficiency. There is a high risk of post-transplant recurrence, except in MCP-HUS. Case reports and two phase II trials show an impressive efficacy of the complement C5 blocker eculizumab, suggesting it will be the next standard of care. Except for patients treated by intensive plasmatherapy or eculizumab, the worst prognosis is in factor H-HUS, as mortality can reach 20% and 50% of survivors do not recover renal function. Half of factor I-HUS progress to end-stage renal failure. Conversely, most patients with MCP-HUS have preserved renal function. Anti-factor H antibodies-HUS has favourable outcome if treated early.
Available from: Journal of Pediatric Psychology, Child Study Center, 1100 N.E. 13th Street, Oklahoma City, Oklahoma 73117. Compared in intelligence, perceptual motor performance, and academic achievement were 30 children (mean age=8.6 years) with seizure disorders and 30 children (mean age=9 years) with learning problems. (IM)
Black, F. William
Occult vascular malformations are frequently associated with intractable seizures and are a common cause of lesional epilepsy. MR imaging can identify and characterize these lesions with accuracy. The presurgical evaluation must be tailored to the individual patient's presentation and circumstances. Surgical results following lesionectomy plus corticectomy may be slightly superior to lesionectomy alone, but the overall prognosis is excellent. PMID:10419577
Cosgrove, G R
Study Objectives: To describe three cases of sleep related, idiopathic rhythmic movement disorder (RMD) with atypical headbanging, consisting of head punching and head slapping. Methods: Three consecutive patients (2 males [11 and 13 years old) and one female [22 years old]) presented with atypical headbanging of 6 years, 7 years, and 17 years duration. In 2 cases, typical rhythmic headbanging (with use of the head) shifted after 3-4 years to atypical headbanging, with frontal head punching that was quasi-rhythmic. In one case, atypical headbanging (head-slapping) was the initial and only RMD. There was no injury from the headbanging. Prenatal, perinatal, developmental, behavioral-psychological, medical-neurological, and family histories were negative. Clinical evaluations and nocturnal video-polysomnography with seizure montage were performed on all patients. Results: Atypical headbanging was documented in all 3 cases; episodes always emerged late in the sleep cycle: from N2 sleep in 11 episodes, from REM sleep in 4 episodes, and from N1 sleep in 1 episode. Epileptiform activity was not detected. Clonazepam therapy was substantially effective in 1 case but not effective in 2 cases. Conclusions: These 3 cases of idiopathic atypical headbanging expand the literature on this RMD variant, as to our knowledge only one previously documented case has been reported. Citation: Yeh SB; Schenck CH. Atypical headbanging presentation of idiopathic sleep related rhythmic movement disorder: three cases with video-polysomnographic documentation. J Clin Sleep Med 2012;8(4):403-411.
Yeh, Shih-Bin; Schenck, Carlos H.
Oxcarbazepine has been reported to precipitate myoclonic, generalised tonic-clonic, absence, and complex partial seizures, and carbamazepine to precipitate absences, myoclonic seizures and spasms. Here, we report a one-year, six-month-old girl with complex partial seizures who developed infantile spasms, developmental regression, and hypsarrhythmia during the two weeks directly following initiation of oxcarbazepine (14?mg/kg/day). All of these resolved within a few days after discontinuation of this medication. Although we cannot rule out that the above association may have been coincidental, or that the improvement may have been due to concurrent therapy, this case raises the possibility that oxcarbazepine, like carbamazepine, may precipitate infantile spasms and West syndrome. PMID:22425652
Veerapandiyan, Aravindhan; Singh, Piyush; Mikati, Mohamad A
The apolipoprotein ?4 allele is the strongest genetic risk factor for late-onset Alzheimer's disease (AD) and is associated with earlier age of onset. The incidence of spontaneous seizures has been reported to be increased in sporadic AD as well as in the early onset autosomal dominant forms of AD. We now report the emergence of a seizure phenotype in aged apolipoprotein E4 (apoE4) targeted replacement (TR) mice but not in age-matched apoE2 TR or apoE3 TR mice. Tonic-clonic seizures developed spontaneously after 5 months of age in apoE4 TR mice and are triggered by mild stress. Female mice had increased seizure penetrance compared to male mice, but had slightly reduced overall seizure severity. The majority of seizures were characterized by head and neck jerks, but 25% of aged apoE4 TR mice had more severe tonic-clonic seizures which occasionally progressed to tonic extension and death. Aged apoE4 TR mice progressed through pentylenetetrazol-induced seizure stages more rapidly than did apoE3 TR and apoE2 TR mice. Electroencephalographic (EEG) recordings revealed more frequent bursts of synchronous theta activity in the hippocampus of apoE4 TR mice than in apoE2 TR or apoE3 TR mice. Cortical EEG recordings also revealed sharp spikes and other abnormalities in apoE4 TR mice. Taken together, these findings demonstrate the emergence of an age-dependent seizure phenotype in old apoE4 TR mice in the absence of human amyloid-? peptide (A?) overexpression, suggesting increased central nervous system neural network excitability. PMID:22682924
Hunter, Jesse M; Cirrito, John R; Restivo, Jessica L; Kinley, Robert D; Sullivan, Patrick M; Holtzman, David M; Koger, Deanna; Delong, Cynthia; Lin, Suizhen; Zhao, Lingzhi; Liu, Feng; Bales, Kelly; Paul, Steven M
Summary Purpose To study the development of epilepsy following hypoxia-induced neonatal seizures in Long Evans rats and to establish the presence of spontaneous seizures in this model of early life seizures. Methods Long-Evans rat pups were subjected to hypoxia-induced neonatal seizures at postnatal day 10 (P10). Epidural cortical electroencephalography (EEG) and hippocampal depth electrodes were used to detect the presence of seizures in later adulthood (>P60). In addition, subdermal wire electrode recordings were used to monitor age at onset and progression of seizures in the juvenile period, at intervals between P10–P60. Timm staining was performed to evaluate mossy fiber sprouting in the hippocampi of P100 adult rats that had experienced neonatal seizures. Key Findings In recordings made from adult rats (P60–P180), the prevalence of epilepsy in cortical and hippocampal EEG recordings was 94.4% following early life hypoxic seizures. These spontaneous seizures were identified by characteristic spike and wave activity on EEG accompanied by behavioral arrest and facial automatisms (electroclinical seizures). Phenobarbital injection transiently abolished spontaneous seizures. EEG in the juvenile period (P10–60) showed that spontaneous seizures first occurred approximately 2 weeks after the initial episode of hypoxic seizures. Following this period, spontaneous seizure frequency and duration progressively increased with time. Furthermore, significantly increased sprouting of mossy fibers was observed in the CA3 pyramidal cell layer of the hippocampus in adult animals following hypoxia-induced neonatal seizures. Notably, Fluoro-Jade B staining confirmed that hypoxic seizures at P10 did not induce acute neuronal death. Significance The rodent model of hypoxia-induced neonatal seizures leads to the development of epilepsy in later life, accompanied by increased mossy fiber sprouting. In addition, this model appears to exhibit a seizure-free latent period, following which there is a progressive increase in the frequency of electroclinical seizures.
Rakhade, Sanjay N; Klein, Peter; Huynh, Thanthao; Hilario-Gomez, Cristina; Kosaras, Bela; Rotenberg, Alexander; Jensen, Frances E.
Objectives: We aimed to determine the incidence of electrographic seizures in children in the pediatric intensive care unit who underwent EEG monitoring, risk factors for electrographic seizures, and whether electrographic seizures were associated with increased odds of mortality. Methods: Eleven sites in North America retrospectively reviewed a total of 550 consecutive children in pediatric intensive care units who underwent EEG monitoring. We collected data on demographics, diagnoses, clinical seizures, mental status at EEG onset, EEG background, interictal epileptiform discharges, electrographic seizures, intensive care unit length of stay, and in-hospital mortality. Results: Electrographic seizures occurred in 162 of 550 subjects (30%), of which 61 subjects (38%) had electrographic status epilepticus. Electrographic seizures were exclusively subclinical in 59 of 162 subjects (36%). A multivariable logistic regression model showed that independent risk factors for electrographic seizures included younger age, clinical seizures prior to EEG monitoring, an abnormal initial EEG background, interictal epileptiform discharges, and a diagnosis of epilepsy. Subjects with electrographic status epilepticus had greater odds of in-hospital death, even after adjusting for EEG background and neurologic diagnosis category. Conclusions: Electrographic seizures are common among children in the pediatric intensive care unit, particularly those with specific risk factors. Electrographic status epilepticus occurs in more than one-third of children with electrographic seizures and is associated with higher in-hospital mortality.
Arndt, Daniel H.; Carpenter, Jessica L.; Chapman, Kevin E.; Cornett, Karen M.; Gallentine, William B.; Giza, Christopher C.; Goldstein, Joshua L.; Hahn, Cecil D.; Lerner, Jason T.; Loddenkemper, Tobias; Matsumoto, Joyce H.; McBain, Kristin; Nash, Kendall B.; Payne, Eric; Sanchez, Sarah M.; Fernandez, Ivan Sanchez; Shults, Justine; Williams, Korwyn; Yang, Amy; Dlugos, Dennis J.
Routine EEGs remain a cornerstone test in caring for people with epilepsy. Although rare, a self-limited seizure (clinical or electrographic only) may be observed during such brief EEGs. The implications of observing a seizure in this situation, especially with respect to inferring the underlying seizure frequency, are unclear. The issue is complicated by the inaccuracy of patient-reported estimations of seizure frequency. The treating clinician is often left to wonder whether the single seizure indicates very frequent seizures, or if it is of lesser significance. We applied standard concepts of probabilistic inference to a simple model of seizure incidence to provide some guidance for clinicians facing this situation. Our analysis establishes upper and lower bounds on the seizure rate implied by observing a single seizure during routine EEG. Not surprisingly, with additional information regarding the expected seizure rate, these bounds can be further constrained. This framework should aid the clinician in applying a more principled approach toward decision making in the setting of a single seizure on a routine EEG.
Westover, M. Brandon; Bianchi, Matt T.; Shafi, Mouhsin; Hoch, Daniel B.; Cole, Andrew J.; Chiappa, Keith; Cash, Sydney S.
Routine EEGs remain a cornerstone test in caring for people with epilepsy. Although rare, a self-limited seizure (clinical or electrographic only) may be observed during such brief EEGs. The implications of observing a seizure in this situation, especially with respect to inferring the underlying seizure frequency, are unclear. The issue is complicated by the inaccuracy of patient-reported estimations of seizure frequency. The treating clinician is often left to wonder whether the single seizure indicates very frequent seizures, or if it is of lesser significance. We applied standard concepts of probabilistic inference to a simple model of seizure incidence to provide some guidance for clinicians facing this situation. Our analysis establishes upper and lower bounds on the seizure rate implied by observing a single seizure during routine EEG. Not surprisingly, with additional information regarding the expected seizure rate, these bounds can be further constrained. This framework should aid the clinician in applying a more principled approach toward decision making in the setting of a single seizure on a routine EEG. PMID:23545768
Westover, M Brandon; Bianchi, Matt T; Shafi, Mouhsin; Hoch, Daniel B; Cole, Andrew J; Chiappa, Keith; Cash, Sydney S
Depression is a common psychiatric problem in epileptic patients. Thus, it is important that an antidepressant agent has anticonvulsant activity. This study was organized to investigate the effects of tianeptine, an atypical antidepressant, on pentylenetetrazole (PTZ)-induced seizure in mice. A possible contribution of adenosine receptors was also evaluated. Adult male Swiss–Webster mice (25–35 g) were subjects. PTZ (80 mg\\/kg, i.p.)
Tayfun I Uzbay; Hakan Kayir; Mert Ceyhan
Drugs that modulate the endocannabinoid system and endocannabinoids typically play an anticonvulsant role although some proconvulsant effects have been reported both in humans and animal models. Moreover, no evidence for a role of the cannabinoid system in human absence epilepsy has been found although limited evidence of efficacy in relevant experimental animal models has been documented. This study aims to characterize the role of cannabinoids in specific areas of the cortico-thalamic network involved in oscillations that underlie seizures in a genetic animal model of absence epilepsy, the WAG/Rij rat. We assessed the effects of focal injection of the endogenous cannabinoid, anandamide (AEA), a non-selective CB receptor agonist (WIN55,212) and a selective CB1 receptor antagonist/inverse agonist (SR141716A) into thalamic nuclei and primary somatosensory cortex (S1po) of the cortico-thalamic network. AEA and WIN both reduced absence seizures independently from the brain focal site of infusion while, conversely, rimonabant increased absence seizures but only when focally administered to the ventroposteromedial thalamic nucleus (VPM). These results, together with previous reports, support therapeutic potential for endocannabinoid system modulators in absence epilepsy and highlight that attenuated endocannabinergic function may contribute to the generation and maintenance of seizures. Furthermore, the entire cortico-thalamic network responds to cannabinoid treatment, indicating that in all areas considered, CB receptor activation inhibits the pathological synchronization that subserves absence seizures. In conclusion, our result might be useful for the identification of future drug therapies in absence epilepsy. PMID:23860329
Citraro, Rita; Russo, Emilio; Ngomba, Richard T; Nicoletti, Ferdinando; Scicchitano, Francesca; Whalley, Benjamin J; Calignano, Antonio; De Sarro, Giovambattista
Atypical facial pain is a pain in the head, neck and the face, without organic causes. It is treated at departments of physical medicine, such as dental, oral and maxillofacial surgery, otolaryngology, cerebral surgery, or head and neck surgery. In primary care, it is considered to be a medically unexplained symptom (MUS), or a somatoform disorder, such as somatization caused by a functional somatic syndrome (FSS) by psychiatrists. Usually, patients consult departments of physical medicine complaining of physical pain. Therefore physicians in these departments should examine the patients from the holistic perspective, and identify organic diseases. As atypical facial pain becomes chronic, other complications, including psychiatric complaints other than physical pain, such as depression may develop. Moreover, physical, psychological, and social factors affect the symptoms by interacting with one another. Therefore, in examining atypical facial pain, doctors specializing in dental, oral and maxillofacial medicine are required to provide psychosomatic treatment that is based on integrated knowledge. PMID:19768920
Ishida, Satoshi; Kimura, Hiroko
Analysis of naturally occurring mutations that cause seizures in rodents has advanced understanding of the molecular mechanisms underlying epilepsy. Abnormalities of Ih and h channel expression have been found in many animal models of absence epilepsy. We characterized a novel spontaneous mutant mouse, apathetic (ap/ap), and identified the ap mutation as a 4 base pair insertion within the coding region of Hcn2, the gene encoding the h channel subunit 2 (HCN2). We demonstrated that Hcn2ap mRNA is reduced by 90% compared to wild type, and the predicted truncated HCN2ap protein is absent from the brain tissue of mice carrying the ap allele. ap/ap mice exhibited ataxia, generalized spike-wave absence seizures, and rare generalized tonic-clonic seizures. ap/+ mice had a normal gait, occasional absence seizures and an increased severity of chemoconvulsant-induced seizures. These findings help elucidate basic mechanisms of absence epilepsy and suggest HCN2 may be a target for therapeutic intervention.
Chung, Wendy K.; Shin, Minyoung; Jaramillo, Thomas C.; Leibel, Rudolph L.; LeDuc, Charles A.; Fischer, Stuart G.; Tzilianos, Efthia; Gheith, Ayman A.; Lewis, Alan S.; Chetkovich, Dane M.
Childhood absence epilepsy (CAE) is defined by absence seizures in a normally developing child, with onset between 4 and 10 years of age.(1) Typical absence seizures consist of behavioral arrest with or without automatisms, usually last 30-60 seconds, and demonstrate a characteristic 3-Hz generalized spike-wave (GSW) pattern on EEG, often in response to hyperventilation.(2) Children may have hundreds of episodes daily, many of them subclinical, which can impair sustained attention and memory processing. There is a strong association between CAE and disorders of attention and executive function. This association persists even when seizures are well-controlled. An underlying structural or functional abnormality of the brain has been postulated to explain both observations.(3,4) This article by Dlugos et al.(5) is a post hoc analysis of a randomized, double-blinded trial(6) that seeks to characterize the relationship between EEG characteristics prior to treatment, measures of attention, and the outcome of initial antiepileptic treatment. PMID:24799520
Bernson-Leung, Miya E; Mazumdar, Maitreyi
Epilepsy in neuroepithelial tumors is highly prevalent. Neurogliomas (dysembryoplastic neuroepitheliomas [DNETs] and gangliogliomas) have a seizure incidence of 80-100%, low-grade gliomas of 60-85%, and glioblastoma of 30-60%. With each type, the appearance of seizures is usually the presenting clinical symptom, and with neuroglial tumors often the only clinical sign. Tumor locations in the temporal and insular cortex are associated with a higher risk of developing epilepsy in both neuroglial tumors and low-grade gliomas. Focal seizures with or without alteration of consciousness and/or secondary generalization are common. Focal seizures with altered consciousness are present in 50-70% of neuroglial tumors, and secondarily generalized seizures in 70% of low-grade gliomas. Surgical treatment, particularly gross tumor resection, contributes strongly to seizure freedom, especially in neuroglial tumors. Refractory epilepsy is more common in low-grade gliomas, occurring in 30-35%. Recurrence or worsening of seizures is often associated with tumor recurrence in glioblastomas. Translational studies have revealed a strong prevalence of IDH1 enzyme mutation together with the presence of seizures and long-term survival in low-grade gliomas. Disturbances of glutamate metabolism occur both in low-grade tumors and glioblastomas, and provide insight into mutual cellular pathway abnormalities contributing to both seizure development and tumor growth. Likewise, the recent clinical observations on antitumor activity of the anticonvulsant valproic acid in glioblastoma now provide promising outlooks on single therapies that target both seizures and gliomas. PMID:24328866
Kerkhof, Melissa; Vecht, Charles J
Clinicians typically breathe a sigh of relief when they make the diagnosis of childhood absence epilepsy. The history is classic-a normal young child with myriad brief periods of staring. The clinic visit is powerful when the phenomena are replicated by having the child hyperventilate. Finally, the EEG is definitive. The ability to demonstrate to a family the abrupt eruption and cessation of spike-wave activity provides a framework for them to understand what is happening to their child. Parents are usually reassured that there are very good therapies, that seizures are typically controlled, and that their child will "outgrow" it. However, data from the multicenter Childhood Absence Epilepsy Study Group are rewriting this narrative.(1-3.) PMID:24089389
Vining, Eileen P G; Thio, Liu Lin
Refractory status epilepticus (RSE) poses significant challenge, with a variety of novel therapeutics employed. Our goal was to evaluate the effectiveness of N-methyl D-aspartate (NMDA) receptor antagonists in the control of RSE. We performed a systematic review of all the literature, with all articles pulled from MEDLINE, BIOSIS, EMBASE, Global Health, HealthStar, Scopus, Cochrane Library, the International Clinical Trials Registry Platform (inception to September 2013), reference lists of relevant articles, and gray literature. Two reviewers independently identified all manuscripts pertaining to the administration of NMDA receptor antagonists in humans for the purpose of controlling refractory seizures. Secondary outcome of adverse NMDA antagonist effects and patient outcome was assessed. Two reviewers independently extracted data including population characteristics, treatment characteristics, and outcomes. The strength of evidence was adjudicated using both the Oxford and GRADE methodology. Our search strategy produced a total of 759 citations. Twenty-three articles, 16 manuscripts, and seven meeting proceedings, were considered for the review with all utilizing ketamine for seizure control. Only three studies were prospective studies. Fifteen and nine studies pertained to adults and pediatrics, respectively. Across all studies, of the 110 adult patients described, ketamine was attributed to electroencephalogram seizure response in 56.5 %, with a 63.5 % response in the 52 pediatric patients described. Adverse events related to ketamine were rare. Outcomes were poorly documented in the majority of the studies. There currently exists Oxford level 4, GRADE C evidence to support the use of ketamine for refractory seizures in the adult and pediatric populations. Further prospective study of early ketamine administration is warranted. PMID:24519081
Zeiler, F A; Teitelbaum, J; Gillman, L M; West, M
Background: In this study, we tried to identify predictors of acute poststroke seizures (aPSS) in stroke patients. Methods:We analyzed a large prospective hospital-based stroke registry in Germany. 58,874 patients with the diagnosis of transient ischemic attack (TIA), ischemic stroke (IS) or intracerebral hemorrhage (ICH) were admitted within 24 h after symptom onset. Predictors for aPSS were identified using multivariate regression
Karsten Krakow; Matthias Sitzer; Felix Rosenow; Helmuth Steinmetz; Christian Foerch
Ictal patterns of local cerebral metabolic rate have been studied in epileptic patients by positron computed tomography with /sup 18/F-labeled 2-fluoro-2-deoxy-D-glucose. Partial seizures were associated with activation of anatomic structures unique to each patient studied. Ictal increases and decreases in local cerebral metabolism were observed. Scans performed during generalized convulsions induced by electroshock demonstrated a diffuse ictal increase and postictal decrease in cerebral metabolism. Petit mal absences were associated with a diffuse increase in cerebral metabolic rate. The ictal fluorodeoxyglucose patterns obtained from patients do not resemble autoradiographic patterns obtained from common experimental animal models of epilepsy.
Engel, J. Jr.; Kuhl, D.E.; Phelps, M.E.
The present study tested the therapeutic potential for prototype anti-epilepsy drugs using an animal model of infantile febrile seizures. The model consisted of immersion of weanling rats (21 days old) in a 45 degrees C water bath for a maximum of 4 min (four exposures over a 2 week period) and observing for the progression to stage-5 seizures. All compounds were administered orally at the respective ED50 for prevention of seizures in the maximal electroshock (MES) test. Clonazepam effectively lowered the score for seizure grade, shortened the duration of seizures, as well as reduced the number of animals experiencing seizures during three of the four testing periods. MK801 reduced both the maximum seizure grade, and the number of animals experiencing seizures during sessions two and three. However, the dose of MK801 caused behavioral side effects. Valproate actively decreased seizure grade, while it modestly acted to attenuate seizure duration, extended the time to seizure onset, and reduced the number of animals experiencing seizures on testing day 1. Remacemide hydrochloride and phenobarbital were not effective. The method appears useful for evaluating the potential of agents to prevent acute febrile seizures. PMID:9657648
Palmer, G C; Borrelli, A R; Hudzik, T J; Sparber, S
Background Seizure as the initial manifestation of aortic dissection is rare. Case report An 88-year-old female experienced a first generalized tonic clonic seizure, which was terminated with midazolam. Acute cerebral magnetic resonance imaging and angiography were non-informative. After awaking she complained about cramping pain in the right upper extremity, which was accompanied by involuntary flexion movements of the right upper extremity. Blood pressure was initially normal. Blood gases revealed metabolic acidosis and blood chemical investigations a markedly increased D-dimer. Consecutively blood pressure declined and transthoracic echocardiography showed pericardial effusion. A computed tomography scan of the thorax revealed an aortic dissection type A. The patient died 16 hours after admission after cardiothoracic surgeons had refused surgical treatment. Conclusion This case shows that a generalized tonic-clonic seizure may be the initial manifestation of an aortic dissection type A in the absence of thoracic chest pain and that brachyalgia may not develop earlier than with progression of the dissection.
Finsterer, Josef; Frank, Marlies; Keller, Hans; Bastowanski, Adam
We report a case of de novo absence status associated with focal discharge and polydipsia-induced hyponatremia. Nonconvulsive status epilepticus (NCSE) is classified as absence status or complex partial status. Absence status is characterized by bilateral synchronized spike and wave complex bursts and a variety of conscious disturbances. Possible precipitating factors for NCSE include benzodiazepine withdrawal, excessive use of psychotropic drugs, and electrolyte imbalances. Hyponatremia is a rare precipitating factor. In this case, the patient was 59 years old and had suffered from primary insomnia but had no history of epilepsy. NCSE improved by means of saline infusion. However after recovery from NCSE EEG revealed some spikes in the left frontal area. Absence seizures can also show generalized spike and slow waves, and cases of focal lesion-associated absence seizures have been reported. Although absence seizures and absence status are two distinct conditions, they should not be considered together. We assumed that hyponatremia induced by polydipsia precipitated epileptogenicity in the left frontal area, and then focal activity secondarily generalized and resulted in absence status.
Azuma, Hideki; Akechi, Tatsuo; Furukawa, Toshi A
We studied six children (four girls and two boys) suffering from cryptogenic myoclonic absence seizures with early onset. The age at onset of the seizures ranged between 6 and 27.8 months (mean age +/- SD: 18.5+/-12.4 months). The neurologic evaluation was normal in all patients at the first hospital admission. After the diagnosis, we followed up all children for at least 5 years. At the end of follow-up, two of these patients (a girl and a boy) showed severe mental retardation, a high number (from one to three per day) of seizures, and persistent pathologic electroencephalograms. The other patients showed normal electroencephalograms: all of them were seizure free and without mental retardation. The two patients with mental retardation have been treated with polytherapy. In all other children we used valproate alone successfully. Our data suggest that myoclonic absence seizures with early onset can have a good long-term prognosis. Valproate is a useful anticonvulsant drug in these patients. Mental retardation is present only in patients with poor seizure control. PMID:10593554
Verrotti, A; Greco, R; Chiarelli, F; Domizio, S; Sabatino, G; Morgese, G
Background: Adverse drug reactions are important determinants of inpatient and outpatient morbidity. Thiocolchicoside is a semisynthetic derivate of naturally occurring colchicoside, which is largely used in humans as a centrally acting muscle relaxant. Epileptic seizures after thiocolchicoside intake have been reported in individuals with a history of epilepsy, acute brain injury or possible blood–brain barrier disruption. Case report: We report the case of a 66-year-old male patient presenting a sudden epileptic seizure temporally related to the intake of thiocolchicoside for muscle contracture and pain. The probably causes of the seizures were thiocolchicoside intake and cerebral microhemorrhages attributed to cerebral amyloid angiopathy. Discussion: Drugs only rarely cause focal seizures. Our case indicates that thiocolchicoside can precipitate seizures in predisposed patients, and that its use should be avoided in patients with brain diseases (and therefore lower seizure thresholds) or blood–brain barrier disruption. This information should be provided in the drug package insert.
Giavina-Bianchi, Pedro; Giavina-Bianchi, Mara; Tanno, Luciana Kase; Ensina, Luis Felipe Chiaverini; Motta, Antono Abilio; Kalil, Jorge
We report the case of a 43-year-old man with history of nonsurgical partial epilepsy who previously failed multiple trials of antiepileptic drugs. Medium-chain triglycerides (MCT) were added to his regular diet in the form of pure oil. Subsequently, his seizure frequency was markedly reduced from multiple daily seizures to one seizure every four days. His seizures recurred after transient discontinuation of MCT over a period of ten days. His seizure improvement was achieved at a dose of four tablespoons of MCT twice daily with no reported side effects. He developed significant diarrhea and flatulence at higher doses. We conclude that MCT oil supplementation to regular diet may provide better seizure control in some patients. MCT oil supplementation may be a more tolerable alternative to the standard ketogenic diet.
Azar, Nabil J.
Mice deficient for the gene encoding the RNA-binding protein CELF4 (CUGBP, ELAV-like family member 4) have a complex seizure phenotype that includes both convulsive and non-convulsive seizures, depending upon gene dosage and strain background, modeling genetically complex epilepsy. Invertebrate CELF is associated with translational control in fruit fly ovary epithelium and with neurogenesis and neuronal function in the nematode. Mammalian CELF4 is expressed widely during early development, but is restricted to the central nervous system in adult. To better understand the etiology of the seizure disorder of Celf4 deficient mice, we studied seizure incidence with spatial and temporal conditional knockout Celf4 alleles. For convulsive seizure phenotypes, it is sufficient to delete Celf4 in adulthood at seven weeks of age. This timing is in contrast to absence-like non-convulsive seizures, which require deletion before the end of the first postnatal week. Interestingly, selective deletion of Celf4 from cerebral cortex and hippocampus excitatory neurons, but not from inhibitory neurons, is sufficient to lower seizure threshold and to promote spontaneous convulsions. Correspondingly, Celf4 deficient mice have altered excitatory, but not inhibitory, neurotransmission as measured by patch-clamp recordings of cortical layer V pyramidal neurons. Finally, immunostaining in conjunction with an inhibitory neuron-specific reporter shows that CELF4 is expressed predominantly in excitatory neurons. Our results suggest that CELF4 plays a specific role in regulating excitatory neurotransmission. We posit that altered excitatory neurotransmission resulting from Celf4 deficiency underlies the complex seizure disorder in Celf4 mutant mice.
Wagnon, Jacy L.; Mahaffey, Connie L.; Sun, Wenzhi; Yang, Yan; Chao, Hsiao-Tuan; Frankel, Wayne N.
Gelastic seizures have been described in various epilepsies arising from the temporal or frontal lobes, although the most commonly encountered form is related to the presence of an hypothalamic hamartoma. We report a patient with gelastic seizures involving the left parietal lobe. Our patient, a 8-year-old girl, underwent video-EEG monitoring, interictal and MRI. The seizures consisted of brief staring followed
René Andrade Machado; Adriana Goicoechea Astencio
Gelastic seizures are often associated with hypothalamic hamartomas. However, focal cortical dysplasias can also cause "laughing seizures", and such cases can be difficult to localize with EEG. This case report presents a 29-year-old woman who was successfully rendered free of gelastic seizures after resection of a frontal cortical dysplasia, localized through MRI and SPECT imaging.[Published with video sequences]. PMID:18077234
Cheung, Christina S; Parrent, Andrew G; Burneo, Jorge G
Seizure after laminectomy for spinal procedure is very rare and has not been reported after lipomyelomeningocele surgery beforehand. Here, two cases of seizure following laminectomy for lipomyelomeningocele are reported. The exact etiology of the event is unknown but anesthetic material, pneumocephalus, intracranial hypotension subsequent to cerebrospinal fluid leakage after spinal procedures, spinal-induced seizure and the potential toxic effect of fat molecules could be considered.
Habibi, Zohreh; Nejat, Farideh; El Khashab, Mostafa
\\u000a The association between seizures and blood pressure elevation remains a common medical emergency encountered in an ICU setting.\\u000a Syndromes such as pre-eclampsia or eclampsia, hypertensive encephalopathy, and posterior leukoencephalopathy commonly present\\u000a with seizures. The primary treatment goal is to reduce the arterial blood pressure. In most cases seizure control is thus\\u000a achieved, but unique medications, such as magnesium sulfate, may
Errol Gordon; Michel T. Torbey
The association between seizures and blood pressure elevation remains a common medical emergency encountered in the setting\\u000a of an intensive care unit. Syndromes such as preeclampsia or eclampsia, hypertensive encephalopathy, and posterior leukoencephalopathy\\u000a commonly present with seizures. The primary treatment goal is to reduce the arterial blood pressure. In most cases, seizure\\u000a control is thus achieved, but unique medications, such
Errol Gordon; Michel T. Torbey
Febrile seizures are the most common form of childhood seizures, affecting 2–5% of all children and usually appearing between\\u000a 3 months and 5 years of age. Despite its predominantly benign nature, a febrile seizure (FS) is a terrifying experience for\\u000a most parents. The condition is perhaps one of the most prevalent causes of admittance to pediatric emergency wards worldwide.\\u000a FS, defined as
Asphyxial seizures occurred in 89 of 101,829 infants born alive at term (0.87\\/1000) in three large maternity hospitals from January 1980 to December 1984. These seizures were significantly associated with antenatal complications, primiparity, and prolonged pregnancy. Meconium staining of the amniotic fluid was also associated with asphyxial seizures, but there were high false positive (11%) and false negative (50%) rates.
P D Curtis; T G Matthews; T A Clarke; M Darling; P Crowley; E Griffin; P OConnell; W Gorman; N OBrien; C OHerlihy
The aim of the study was to assess whether post-ictal symptoms can help distinguish patients who have epileptic seizures from those with non-epileptic seizures (NES). We reviewed the spontaneous responses to the question ‘What symptoms do you have after a seizure?’ in 16 patients with epileptic seizures (predominantly focal with secondary generalization or generalized tonic–clonic) and 23 NES patients. Six
Most patients with epilepsy report that seizures are sometimes, or exclusively, provoked by general internal precipitants (such as stress, fatigue, fever, sleep, and menstrual cycle) and by external precipitants (such as excess alcohol, heat, bathing, eating, reading, and flashing lights). Some patients describe very exotic and precise triggers, like tooth brushing or listening to a particular melody. Nevertheless, the most commonly noticed seizure increasers by far are stress, lack of sleep, and fatigue. Recognized reflex seizure triggers are usually sensory and visual, such as television, discotheques, and video games. Visually evoked seizures comprise 5% of the total of 6% reflex seizures. The distinction between provocative and reflex factors and seizures seems artificial, and in many patients, maybe all, there is a combination of these. It seems plausible that all of the above-mentioned factors can misbalance the actual brain network; at times, accumulation of factors leads then to primary generalized, partial, or secondarily generalized seizures. If the provoking factors are too exotic, patients may be sent to the psychiatrist. Conversely, if the seizure-provoking fluctuating mechanisms include common habits and environmental factors, these may hardly be considered as provocative factors. Awareness of precipitating factors and its possible interactions might help us to unravel the pathophysiology of epilepsy and to change the notion that seizure occurrence is unpredictable. This article provides an overview of the epidemiology, classification, diagnosis, treatment, and especially similarities in the variety of provocative and reflex factors with resulting general hypotheses. PMID:22946728
Kasteleijn-Nolst Trenité, Dorothée G A
Intellectual disability occurs as an isolated X-linked trait and as a component of recognizable X-linked syndromes in the company of somatic, metabolic, neuromuscular, or behavioral abnormalities. Seizures accompany intellectual disability in almost half of these X-linked disorders. The spectrum of seizures found in the X-linked intellectual disability syndromes is broad, varying in time of onset, type of seizure, and response to anticonvulsant therapy. The majority of the genes associated with XLID and seizures have now been identified.
Stevenson, Roger E.; Holden, Kenton R.; Rogers, R. Curtis; Schwartz, Charles E.
Studies of febrile seizures have been driven by two major enigmas: first, how these most common of human seizures are generated by fever has not been known. Second, epidemiological studies have linked prolonged febrile seizures with the development of temporal lobe epilepsy, yet whether long or recurrent febrile seizures cause temporal lobe epilepsy has remained unresolved. To investigate these questions, a model of prolonged (complex) febrile seizures was developed in immature rats and mice, permitting mechanistic examination of the potential causal relationships of fever and seizures, and of febrile seizures and limbic epilepsy. Although the model relied on hyperthermia, it was discovered that the hyperthermia-induced secretion of endogenous fever mediators including interleukin-1?, which contributed to the generation of these ‘febrile’ seizures. In addition, prolonged experimental febrile seizures provoked epilepsy in a third of the animals. Investigations of the mechanisms of this epileptogenesis demonstrated that expression of specific ion (HCN) channels and of endocannabinoid signaling, may be involved. These may provide novel drug targets for intervention in the epileptogenic process.
Dube, Celine M.; Brewster, Amy L.; Baram, Tallie Z.
In this overview, we consider epilepsies as dynamical diseases of brain systems since they are manifestations of the property of neuronal networks to display multistable dynamics. To illustrate this concept we may assume that at least two states of the epileptic brain are possible: the interictal state characterized by a normal, apparently random, steady-state electroencephalography (EEG) ongoing activity, and the ictal state, that is characterized by paroxysmal occurrence of synchronous oscillations and is generally called, in neurology, a seizure. The transition between these two states can either occur: 1) as a continuous sequence of phases, like in some cases of mesial temporal lobe epilepsy (MTLE); or 2) as a sudden leap, like in most cases of absence seizures. In the mathematical terminology of nonlinear systems, we can say that in the first case the system's attractor gradually deforms from an interictal to an ictal attractor. The causes for such a deformation can be either endogenous or external. In this type of ictal transition, the seizure possibly may be anticipated in its early, preclinical phases. In the second case, where a sharp critical transition takes place, we can assume that the system has at least two simultaneous interictal and ictal attractors all the time. To which attractor the trajectories converge, depends on the initial conditions and the system's parameters. An essential question in this scenario is how the transition between the normal ongoing and the seizure activity takes place. Such a transition can occur either due to the influence of external or endogenous factors or due to a random perturbation and, thus, it will be unpredictable. These dynamical changes may not be detectable from the analysis of the ongoing EEG, but they may be observable only by measuring the system's response to externally administered stimuli. In the special cases of reflex epilepsy, the leap between the normal ongoing attractor and the ictal attractor is caused by a well-defined external perturbation. Examples from these different scenarios are presented and discussed. PMID:12769430
Lopes da Silva, Fernando H; Blanes, Wouter; Kalitzin, Stiliyan N; Parra, Jaime; Suffczynski, Piotr; Velis, Demetrios N
Epilepsy is a chronic disorder characterized by repeated seizures resulting from abnormal activation of neurons in the brain. Although mutations in genes related to Na+, K+, Ca2+ channels have been defined, few studies show intracellular protein changes. We have used proteomics to investigate the expression of soluble proteins in a genetic rat model of absence epilepsy “Genetic Absence Epilepsy Rats
Özkan Dan??; Serap Demir; Asl?han Günel; Rezzan Gülhan Aker; Medine Gülçebi; Filiz Onat; Ay?e Ogan
We evaluated the efficacy of vagus nerve stimulation (VNS) in Genetic Absence Epilepsy Rats from Strasbourg (GAERS), a validated model for absence epilepsy. In the first experiment, we investigated whether VNS applied at seizure onset can interrupt spike and wave discharges (SWD). In the second experiment, we investigated whether SWD are suppressed or shortened in duration when VNS is applied
Stefanie Dedeurwaerdere; Kristl Vonck; Pieter Claeys; Peter Van Hese; Michel D’Havé; Thierry Grisar; Dean Naritoku; Paul Boon
We have previously reported that dogs can be trained to recognize specific changes preceding an epileptic seizure in humans. Such dogs can provide an overt signal that acts as a useful warning to the human. Early observations suggested that seizure frequency might also be reduced. We report a prospective study of 10 consecutive referrals to our Seizure Alert Dogs ®service
Margaret Huyton; Helen Coyle
An infant carrying a heterozygous c.43_46delACTA and a heterozygous c.668 G>A mutation in the ALPL gene with hypophosphatasia in the absence of bone deformities presented with therapy-resistant seizures. Pyridoxal phosphate was extremely high in CSF and plasma. Pyridoxine treatment had only a transient effect and the severe encephalopathy was fatal. Repeated brain MRIs showed progressive cerebral damage. The precise metabolic cause of the seizures remains unknown and pyridoxine treatment apparently does not cure the epilepsy. PMID:24100244
de Roo, Marieke G A; Abeling, Nico G G M; Majoie, Charles B; Bosch, Annet M; Koelman, Johannes H T M; Cobben, Jan M; Duran, Marinus; Poll-The, Bwee Tien
Seizures are a common phenotype in many neurodevelopmental disorders including fragile X syndrome, Down syndrome and autism. We hypothesized that phytoestrogens in soy-based infant formula were contributing to lower seizure threshold in these disorders. Herein, we evaluated the dependence of seizure incidence on infant formula in a population of autistic children. Medical record data were obtained on 1,949 autistic children from the SFARI Simplex Collection. An autism diagnosis was determined by scores on the ADI-R and ADOS exams. The database included data on infant formula use, seizure incidence, the specific type of seizure exhibited and IQ. Soy-based formula was utilized in 17.5% of the study population. Females comprised 13.4% of the subjects. There was a 2.6-fold higher rate of febrile seizures [4.2% versus 1.6%, OR?=?2.6, 95% CI?=?1.3–5.3], a 2.1-fold higher rate of epilepsy comorbidity [3.6% versus 1.7%, OR?=?2.2, 95% CI?=?1.1–4.7] and a 4-fold higher rate of simple partial seizures [1.2% versus 0.3%, OR?=?4.8, 95% CI?=?1.0–23] in the autistic children fed soy-based formula. No statistically significant associations were found with other outcomes including: IQ, age of seizure onset, infantile spasms and atonic, generalized tonic clonic, absence and complex partial seizures. Limitations of the study included: infant formula and seizure data were based on parental recall, there were significantly less female subjects, and there was lack of data regarding critical confounders such as the reasons the subjects used soy formula, age at which soy formula was initiated and the length of time on soy formula. Despite these limitations, our results suggest that the use of soy-based infant formula may be associated with febrile seizures in both genders and with a diagnosis of epilepsy in males in autistic children. Given the lack of data on critical confounders and the retrospective nature of the study, a prospective study is required to confirm the association.
Westmark, Cara J.
Seizures are a common phenotype in many neurodevelopmental disorders including fragile X syndrome, Down syndrome and autism. We hypothesized that phytoestrogens in soy-based infant formula were contributing to lower seizure threshold in these disorders. Herein, we evaluated the dependence of seizure incidence on infant formula in a population of autistic children. Medical record data were obtained on 1,949 autistic children from the SFARI Simplex Collection. An autism diagnosis was determined by scores on the ADI-R and ADOS exams. The database included data on infant formula use, seizure incidence, the specific type of seizure exhibited and IQ. Soy-based formula was utilized in 17.5% of the study population. Females comprised 13.4% of the subjects. There was a 2.6-fold higher rate of febrile seizures [4.2% versus 1.6%, OR?=?2.6, 95% CI?=?1.3-5.3], a 2.1-fold higher rate of epilepsy comorbidity [3.6% versus 1.7%, OR?=?2.2, 95% CI?=?1.1-4.7] and a 4-fold higher rate of simple partial seizures [1.2% versus 0.3%, OR?=?4.8, 95% CI?=?1.0-23] in the autistic children fed soy-based formula. No statistically significant associations were found with other outcomes including: IQ, age of seizure onset, infantile spasms and atonic, generalized tonic clonic, absence and complex partial seizures. Limitations of the study included: infant formula and seizure data were based on parental recall, there were significantly less female subjects, and there was lack of data regarding critical confounders such as the reasons the subjects used soy formula, age at which soy formula was initiated and the length of time on soy formula. Despite these limitations, our results suggest that the use of soy-based infant formula may be associated with febrile seizures in both genders and with a diagnosis of epilepsy in males in autistic children. Given the lack of data on critical confounders and the retrospective nature of the study, a prospective study is required to confirm the association. PMID:24622158
Westmark, Cara J
In a population of 425 individuals with Down's syndrome, 5 persons (1.2%) were identified as having Tourette's disorder. The lack of interrelationship between Down's syndrome and Tourette's disorder argues against an atypical Tourette's disorder. Diagnoses of tardive Tourette's disorder were based on absence of family history of Tourette's, late…
Myers, Beverly; Pueschel, Siegfried M.
...SECURITY; DEPARTMENT OF THE TREASURY (CONTINUED) INSPECTION, SEARCH, AND SEIZURE Controlled Substances, Narcotics, and Marihuana Â§ 162.63 Arrests and seizures. Arrests and seizures under the Controlled Substances Act (84 Stat. 1242, 21...
... disorder catalog Conditions > Malignant migrating partial seizures of infancy On this page: Description Genetic changes Inheritance Diagnosis ... 2014 What is malignant migrating partial seizures of infancy? Malignant migrating partial seizures of infancy (MMPSI) is ...
Incontinentia pigmenti (IP) is a rare affection inherited as X-linked dominant disease. It is usually lethal in male infants. IP can affect ectodermal tissues such as the skin, teeth, eyes, bones, and the central nervous system. Skin lesions occur mostly during the neonatal period and are characterized by a classic progression in 4 stages leading to hyperpigmentation. We report on the case of a female neonate presenting on the 3rd day of life with seizures without obvious cause, in which the diagnosis of IP was made 1 week later when skin lesions appeared. PMID:21795026
Nouri-Merchaoui, S; Mahdhaoui, N; Methlouthi, J; Zakhama, R; Seboui, H
Seizurogenic chemicals include a variety of toxic agents, including chemical warfare agents, toxic industrial chemicals, and natural toxins. Chemical weapons such as sarin and VX, and pesticides such as parathion and carbaryl cause hyperstimulation of cholinergic receptors and an increase in excitatory neurotransmission. Glutamatergic hyperstimulation can occur after exposure to excitatory amino acid toxins such as the marine toxin domoic acid. Other pesticides such as lindane and strychnine do not affect excitatory neurotransmission directly, but rather, they block the inhibitory regulation of neurotransmission by antagonism of inhibitory GABA and glycine synapses. In this paper, chemicals that cause seizures by a variety of molecular mechanisms and pathways are discussed. PMID:23085523
Jett, David A
Medulloblastoma is common in children as a tumor of midline posterior fossa, which arises from vermis and appears as a homogenously enhancing hyperdense mass on computed tomography scan and is associated with the clinical picture of posterior fossa syndrome. This unique clinic-radiological pattern in considered “typical” medulloblastoma, but medulloblastomas does not follow the typical clinic-radiological pattern in a significant number of children and adult cases. We review here the previous reports of atypical or uncommon features of medulloblastoma and add our very rare atypical cases of medulloblastomas to this list. Medulloblastoma should be considered in all midline posterior fossa tumors, hemisphere and cerebellopontine angle despite having clinical and radiological features suggestive of other tumors. Definitive diagnosis requires histologic confirmation in all cases.
Meshkini, Ali; Vahedi, Amir; Meshkini, Mohammad; Alikhah, Hossein; Naghavi-Behzad, Mohammad
First described by Robert Debré in 1950, cat scratch disease, usually observed in children and young adults (80% of the case occur in subjects under 18), is the principal cause of chronic benign lymph node enlargement. The Centers for Disease Control at Atlanta currently recognize Afipia felis and Rochalimaea henselae as the causal agents of cat scratch disease. Cats transmit the disease to humans by skin scratches or by licking open wounds. The bacilli can also be transmitted by dogs, monkeys, squirels or inert objects. The contaminating cats are usually young. There is no interhuman transmission. There are several uncommon manifestations of cat scratch disease which may misguide diagnosis. In atypical forms, the positive diagnosis of this benign disease can only be confirmed by serology or molecular biology techniques. In order to reduce the duration of the disease, antibiotic treatment is recommended in forms with systemic involvement and in atypical forms although there has been no proof of efficacy. PMID:8685112
Bourrillon, A; Leclainche, L
Anecdotal evidence suggests that some dogs may be able to sense the onset of seizures and other medical conditions in humans, although this has never been explored scientifically. There is, however, evidence that dogs can be specially trained to recognize specific changes preceding a seizure and give an overt signal enabling the dog to warn his\\/her owner. The introduction of
V. Strong; S. W. Brown; R. Walker
Neurocysticercosis (NCC), a helminth infection of the brain, is a major cause of seizures. The mediators responsible for seizures in NCC are unknown, and their management remains controversial. Substance P (SP) is a neuropeptide produced by neurons, endothelial cells and immunocytes. The current studies examined the hypothesis that SP mediates seizures in NCC. We demonstrated by immunostaining that 5 of 5 brain biopsies from NCC patients contained substance P (SP)-positive (+) cells adjacent to but not distant from degenerating worms; no SP+ cells were detected in uninfected brains. In a rodent model of NCC, seizures were induced after intrahippocampal injection of SP alone or after injection of extracts of cysticercosis granuloma obtained from infected wild type (WT), but not from infected SP precursor-deficient mice. Seizure activity correlated with SP levels within WT granuloma extracts and was prevented by intrahippocampal pre-injection of SP receptor antagonist. Furthermore, extracts of granulomas from WT mice caused seizures when injected into the hippocampus of WT mice, but not when injected into SP receptor (NK1R) deficient mice. These findings indicate that SP causes seizures in NCC, and, suggests that seizures in NCC in humans may be prevented and/or treated with SP-receptor antagonists.
Robinson, Prema; Garza, Armandina; Weinstock, Joel; Serpa, Jose A.; Goodman, Jerry Clay; Eckols, Kristian T.; Firozgary, Bahrom; Tweardy, David J.
Alterations in electrolyte homeostasis associated with major surgery and critical illness are common but usually non-specific. We report an unusual case of hypomagnesaemia induced seizures in a 73-year-old woman who was recovering from ileal resection and limited right hemicolectomy for small bowel volvulus. Hypomagnesaemia as a de novo cause of seizures is rarely reported.
Pande, Shrikant D; Wee, Chee Keong; Maw, Nyein Nyein
This report describes studies of anticonvulsants for the organophosphorus (OP) nerve agent soman: a basic research effort to understand how different pharmacological classes of compounds influence the expression of seizure produced by soman in rats, and a drug screening effort to determine whether clinically useful antiepileptics can modulate soman-induced seizures in rats. Electroencephalographic (EEG) recordings were used in these studies.
Tsung-Ming Shih; John H. McDonough; Irwin Koplovitz
Electrophysiological recordings show intense neuronal firing during epileptic seizures leading to enhanced energy consumption. However, the relationship between oxygen metabolism and seizure patterns has not been well studied. Recent studies have developed fast and quantitative techniques to measure oxygen microdomain concentration during seizure events. In this article, we develop a biophysical model that accounts for these experimental observations. The model is an extension of the Hodgkin-Huxley formalism and includes the neuronal microenvironment dynamics of sodium, potassium, and oxygen concentrations. Our model accounts for metabolic energy consumption during and following seizure events. We can further account for the experimental observation that hypoxia can induce seizures, with seizures occurring only within a narrow range of tissue oxygen pressure. We also reproduce the interplay between excitatory and inhibitory neurons seen in experiments, accounting for the different oxygen levels observed during seizures in excitatory vs. inhibitory cell layers. Our findings offer a more comprehensive understanding of the complex interrelationship among seizures, ion dynamics, and energy metabolism. PMID:24671540
Wei, Yina; Ullah, Ghanim; Ingram, Justin; Schiff, Steven J
Abstract Objective: To assess the incidence of seizures induced by cycloplegic ophthalmic drops. Materials and methods: A survey among members of the American Association for Pediatric Ophthalmology and Strabismus yielded five patients who received cycloplegic eye drops between 1998 and 2010 and who consequently developed a seizure. Results: The median age of the patients was 5 years (range 3 months to 12 years). Cyclopentolate hydrochloride 1% was the only causative agent. The seizure happened on average 12?min after the instillation of dilating eye drops. Three were generalized convulsions, and two patients had a focal seizure. Past medical history was unremarkable in four cases. In total, 16 previous cases of seizures induced by cycloplegic drugs were identified in reports published between 1890 and 2004, implicating atropine in nine reports, tropicamide and phenylephrine eye drops in one and cyclopentolate in six. Discussion: A small amount of cyclopentolate drops could induce convulsions in young children after only minutes to less than an hour, while a larger dosage of atropine over the span of several hours could cause this rare and unpredictable complication. Predisposing factors were rare and those developing the seizures were healthy subjects. Generalized seizures were much more frequent than focal convulsions. Conclusions: Seizures after instillation of cycloplegic drops are extremely rare. PMID:23815170
Wygnanski-Jaffe, Tamara; Nucci, Paolo; Goldchmit, Mauro; Mezer, Eedy
A number of seizure events, typical of everyday clinical practice, were tested for possible chaos. After a careful elimination of spurious effects, evidence of chaos was found in two seizure events. This was confirmed by direct examination of exponential separation of initially nearby states in low-dimensional trajectory recoveries. The resulting Lyapunov exponent calculation provides a clear indication of chaos in
G. W. Frank; T. Lookman; M. A. H. Nerenberg; C. Essex; J. Lemieux; W. Blume
Objective: The fundamental mechanisms by which childhood absence epilepsy (CAE) changes neural networks even between seizures remain poorly understood. During seizures, cortical and subcortical networks exhibit bihemspheric synchronous activity based on prior EEG-fMRI studies. Our aim was to investigate whether this abnormal bisynchrony may extend to the interictal period, using a blood oxygen level–dependent (BOLD) resting functional connectivity approach. Methods: EEG-fMRI data were recorded from 16 patients with CAE and 16 age- and gender-matched controls. Three analyses were performed. 1) Using 16 pairs of seizure-related regions of interest (ROI), we compared the between-hemisphere interictal resting functional connectivity of patients and controls. 2) For regions showing significantly increased interhemispheric connectivity in CAE, we then calculated connectivity to the entire brain. 3) A paired-voxel approach was performed to calculate resting functional connectivity between hemispheres without the constraint of predefined ROIs. Results: We found significantly increased resting functional connectivity between hemispheres in the lateral orbitofrontal cortex of patients with CAE compared to normal controls. Enhanced between-hemisphere connectivity localized to the lateral orbitofrontal cortex was confirmed by all 3 analysis methods. Conclusions: Our results demonstrate abnormal increased connectivity between the hemispheres in patients with CAE in seizure-related regions, even when seizures were not occurring. These findings suggest that the lateral orbitofrontal cortex may play an important role in CAE pathophysiology, warranting further investigation. In addition, resting functional connectivity analysis may provide a promising biomarker to improve our understanding of altered brain function in CAE during the interictal period.
Bai, X.; Guo, J.; Killory, B.; Vestal, M.; Berman, R.; Negishi, M.; Danielson, N.; Novotny, E.J.; Constable, R.T.
A 37-year-old female presented following a witnessed generalised tonic clonic seizure while washing her car. Witnesses reported the patient hit her head on the car bumper upon falling. She was investigated for a cause of a second seizure with blood tests, CT and MR brain which were normal. On day 3 of her admission she was still unable to walk since the seizure and a neurological examination was performed which revealed signs consistent with a cervical myelopathy. A cervical MRI scan revealed a disc prolapse with cord compression at C5/6. This was successfully operated and the patient made a good recovery. Given that many of our medical assessment units and emergency department frequently assess patient with seizures, there was an important lesson for us to be more aware of the consequences as well as the cause of seizures. PMID:22691593
Satia, Imran; Goodwin, Lynsey; Madi, Salem; Hamdalla, Hisham
Oxidative stress resulting from excessive free-radical release is likely implicated in the initiation and progression of epilepsy. Therefore, antioxidant therapies aimed at reducing oxidative stress have received considerable attention in epilepsy treatment. However, much evidence suggests that oxidative stress does not always have the same pattern in all seizures models. Thus, this review provides an overview aimed at achieving a better understanding of this issue. We summarize work regarding seizure models (i.e., genetically epilepsy-prone rats, kainic acid, pilocarpine, pentylenetetrazol, and trimethyltin), oxidative stress as an etiologic factor in epileptic seizures (i.e., impairment of antioxidant systems, mitochondrial dysfunction, involvement of redox-active metals, arachidonic acid pathway activation, and aging), and antioxidant strategies for seizure treatment. Combined, this review highlights pharmacological mechanisms associated with oxidative stress in epileptic seizures and the potential for neuroprotection in epilepsy that targets oxidative stress and is supported by effective antioxidant treatment.
Shin, Eun-Joo; Jeong, Ji Hoon; Chung, Yoon Hee; Kim, Won-Ki; Ko, Kwang-Ho; Bach, Jae-Hyung; Hong, Jau-Shyong; Yoneda, Yukio; Kim, Hyoung-Chun
A case report is presented of a 36-year-old U.S. Coast Guard aviator who had a single seizure while operating a helicopter on the ground. His seizure activity produced a loss of consciousness during which he pushed the cyclic to the left anterior quadrant that resulted in a ground mishap. No risk factors were identified in an extensive neurological workup. The current guidance for handling seizures in military aviation personnel is reviewed, along with considerations for treatment. Although the military aviation selection process carefully screens applicants for seizure history and potential, occasional seizures in the aviation population remain possible. Such events may result in military aircraft mishaps despite careful risk factor surveillance, as demonstrated by this case. PMID:18419038
Simon, Esan; Watts, Darron; Bohnker, Bruce K
Neonatal seizures are often refractory to treatment with initial antiseizure medications. Consequently, clinicians turn to alternatives such as levetiracetam, despite the lack of published data regarding its safety, tolerability, or efficacy in the neonatal population. We report a retrospectively identified cohort of 23 neonates with electroencephalographically confirmed seizures who received levetiracetam. Levetiracetam was considered effective if administration was associated with a greater than 50% seizure reduction within 24 hours. Levetiracetam was initiated at a mean conceptional age of 41 weeks. The mean initial dose was 16 ± 6 mg/kg and the mean maximum dose was 45 ± 19 mg/kg/day. No respiratory or cardiovascular adverse effects were reported or detected. Levetiracetam was associated with a greater than 50% seizure reduction in 35% (8 of 23), including seizure termination in 7. Further study is warranted to determine optimal levetiracetam dosing in neonates and to compare efficacy with other antiseizure medications.
Abend, Nicholas S.; Gutierrez-Colina, Ana M.; Monk, Heather M.; Dlugos, Dennis J.; Clancy, Robert R.
Triheptanoin, the triglyceride of heptanoate, is used to treat certain hereditary metabolic diseases in USA because of its anaplerotic potential. In two chronic mouse seizure models this clear tasteless oil was found to be reproducibly anticonvulsant. Here we investigated the effects of triheptanoin feeding in C3H and CD1 mice using standard acute seizure models. Feeding 30-40% triheptanoin (caloric intake) consistently elevated blood propionyl-carnitines, but inconsistent anticonvulsant effects were observed in the fluorothyl, pentylenetetrazole and 6Hz seizure models. A 2mA consistent increase in the maximal electroshock threshold was found after 3 weeks of 35% triheptanoin feeding (p=0.018). In summary, triheptanoin shows a unique anticonvulsant profile in seizure models, compared to other treatments that are in the clinic. Therefore, despite small and/or inconsistent effects of triheptanoin in acute seizure models, triheptanoin remains of interest as a potential add-on treatment for patients with medically refractory epilepsy. PMID:22260920
Thomas, Nicola K; Willis, Sarah; Sweetman, Lawrence; Borges, Karin
The time-varying dynamics of epileptic seizures and the high interindividual variability make their detection difficult. Osorio et al. (1998, Epilepsia, 39, 6) developed an algorithm that has had success in detecting seizures. We present a new strategy for adapting this algorithm or other algorithms to an individual's seizure fingerprint using both seizure and non-seizure training segments and a novel performance criterion that directly incorporates the non-linearity and lack of differentiability of the algorithm. The joint optimization of a linear filter chosen from a bank of candidate filters and of a percentile used in order statistic filtering provides an empirical solution that is both practical and useful, which should translate into improved sensitivity, specificity and detection speed. This premise is strongly supported by the results obtained in a large validation study and the examples illustrated in this article. This strategy is generalizable to other detection algorithms with modular architecture and spectral filters.
Haas, Shane M.; Frei, Mark G.; Osorio, Ivan
Great effort has been made toward defining and characterizing the pre-ictal state. Many studies have pursued the idea that there are recognizable electrographic (EEG-based) features that occur before overt clinical seizure activity. However, development of reliable EEG-based seizure detection and prediction algorithms has been difficult. In this review, we discuss the concepts of seizure detection vs. prediction and the pre-ictal “clinical milieu” and “EEG milieu”. We proceed to discuss novel concepts of seizure detection based on the pre-ictal “physiological milieu”; in particular, we indicate some early evidence for the hypothesis that pre-ictal cell swelling/extracellular space constriction can be detected with novel optical methods. Development and validation of optical seizure detection technology could provide an entirely new translational approach for the many patients with intractable epilepsy.
Binder, Devin K.; Haut, Sheryl R.
Disturbances in GABAergic and glutamatergic neurotransmission in the thalamocortical loop are involved in absence seizures. Here, we examined potential disturbances in metabolism and interactions between neurons and glia in 5-month-old genetic absence epilepsy rats from Strasbourg (GAERS) and nonepileptic rats (NER). Animals received [1-13C]glucose and [1,2-13C]acetate, the preferential substrates of neurons and astrocytes, respectively. Extracts from cerebral cortex, thalamus, and
Torun M Melø; Ursula Sonnewald; Monique Touret; Astrid Nehlig
The organization of sleep during and after frequentative convulsions, consisting of 2, 3, or 5 comparatively rare seizures (following one another with a 90-minute interval) or of 3, 5 or 9 comparatively frequent seizures (following one another with a 45-minute interval) of generalized tonic-clonic character in Krushinskii-Molodkina strain rats with inherited predisposition to audiogenic convulsions, was studied. In frequentative convulsions with rare seizures, between separate seizures, passive wakefulness (75.2 +/- 4.6% time) prevailed under low (24.8 +/- 4.3%) slow-wave sleep and full absence of fast-wave sleep. In rats under frequentative convulsions with frequent seizures, in interictal period, only passive wakefulness was observed under reduction of slow-wave sleep and fast-wave sleep, i.e. total sleep deprivation. Minimal latensy of first episodes of the slow-wave sleep after frequentative convulsions was 59.9 +/- 10.8, and of fast-wave sleep: 158.2 +/- 13.4 min. First episodes of slow-wave sleep and fast-wave sleep had normal structure, though they were lesser and shorter than in control experiments. In spite of long-lasting (up to 7 hrs) absence of slow-wave sleep during seizure and prolonged (8.5 hrs) reduction of fast-wave sleep with no subsequent compensatory increase, these conditions occurred in the wakefulness-sleep cycle during 12-hour reconstruction after convulsions. The reconstruction period after frequentative convulsions was characterized by increase in general share of wakefulness and reduction of total slow-wave and fast-wave sleep as compared with control data. Paroxysmal status seems to disorganize work of the brain somnogenic structures. The function of systems responsible for slow-wave sleep are affected to a lesser extent, but disorganization of the system responsible for fast-wave sleep is more significant and associated with mechanisms of starting the phase of sleep in the first place. PMID:16206617
Vataev, S I
Coadministration of azoles and vincristine has been shown to increase vincristine neurotoxic effects due to the inhibition of cytochrome P450 (CYP) isoform 3A4, for which vincristine is a substrate. Despite the absence of any casual relationship between seizure and coadministration of azoles, few case reports of vincristine-induced seizure have been documented after coadministration of fluconazole or posaconazole in children. In this paper we are reporting the first young female adult who experienced generalized seizure after coadministration of posaconazole and vincristine. The 19-year-old female was diagnosed with acute lymphoblastic leukemia. She started induction phase of Berlin Frankfurt Muenster protocol along with posaconazole 200?mg three times daily as prophylactic antifungal therapy. Five days after the third vincristine dose, she developed generalized seizure accompanied by high blood pressure and SIADH. Her neurological exam/CT scan did not show any abnormality. In conclusion, this study reports a novel finding in the sense that all previous case reports pertaining to posaconazole-vincristine-induced seizure in literature involved children. Physicians should be made aware of this rare possible outcome to closely monitor their patients and take appropriate measures to prevent such possible adverse effect.
Hamdy, Dalia A.; El-Geed, Hager; El-Salem, Samah; Zaidan, Manal
Low grade oligodendrogliomas (LGO) are diffusely infiltrating World Health Organization (WHO) grade II gliomas, 20 - 30% of which show contrast enhancement. Seizures are a common presenting feature. It has been suggested that 1p19q co-deletion is associated with occurrence of seizures in adults, however, to date, the relationship of tumor genetics and seizure activity has not been extensively investigated. We sought to assess the influence of 1p19q co-deletion, IDH1-R132H positivity, and radiological variables on seizure activity in LGO patients. Specifically, we examined whether these characteristics were associated with seizure at initial presentation, or if they could predict outcome in terms of seizure free survival. In 62 LGOs, neither tumor location nor tumor enhancement were associated with seizures. 1p19q co-deletion status did not predict seizures when controlled for mutant IDH1-R132H expression, tumor location, or enhancement status (odds ratio (OR) 0.9, 95% confidence interval (CI) 0.1 - 4.3). This study, although of limited statistical power, did not demonstrate an association between 1p19q status and seizure occurrence in LGO's. Replication in a larger cohort would further support our hypothesis that 1p19q status alone cannot be used as a reliable predictor of seizure occurrence in LGO's. PMID:24986208
Mulligan, Linda; Ryan, Elizabeth; O'Brien, Margaret; Looby, Seamus; Heffernan, Josephine; O'Sullivan, Joanne; Clarke, Mary; Buckley, Patrick; O'Brien, David; Farrell, Michael; Brett, Francesca Mary
Neuroleptic malignant syndrome is a serious and potentially fatal adverse effect of antipsychotic medications. Although diagnostic criteria for neuroleptic malignant syndrome have been established and are widely accepted and used, it should be recognized that atypical presentations occur, particularly during treatment with atypical antipsychotics. However, it remains unclear whether these atypical presentations represent early or impending neuroleptic malignant syndrome. Furthermore, since neuroleptic malignant syndrome is a diagnosis of exclusion, careful consideration of other neuropsychiatric conditions should occur. Relying on creatine phosphokinase elevation may result in an incorrect diagnosis of atypical neuroleptic malignant syndrome. We wish to present a case of this diagnostic dilemma in a patient with catatonia.
Surber, Susan A.
Using a measure of nonlinear dynamical changes we term marginal predictability, we report evidence of robust changes in this parameter on scalp EEG in a cohort of patients with medically refractory mesiobasal temporal lobe epilepsy (MBTLE). In the baseline (interictal) state there are distinct differences in this nonlinear measure between epileptic and neurologically normal subjects. At baseline, in patients with MBTLE there are differences in these measures between electrodes adjacent to the ictal onset zone and more remotely placed electrodes. The character of these differences evolves over a period of approximately 30 min before a seizure. We discuss and integrate our findings with two emerging concepts in epileptology, first, the concept of a preictal or transition phase rather than an abrupt movement from interictal to ictal activity, and second, the notion of an epileptic neural network with changes in areas of brain remote from what has traditionally been considered the ictal onset zone influencing "ictogenesis." PMID:14597320
Drury, Ivo; Smith, Brien; Li, Dingzhou; Savit, Robert
A dissociation between clinical and electroencephalographic phenomena is often observable in neonatal seizures. This finding raises important questions, including those related to the management of these patients. Another characteristic of neonatal convulsions is represented by their increased tendency towards status epilepticus. In order to examine the electroclinical correlation and its possible relationship to the occurrence of status epilepticus, recorded video-electroencephalograms of 17 newborns were submitted to detailed analysis. Time of onset, duration and other characteristics of all clinical and electrical events were noted. Five degrees of correlation were observed, from constant concurrence to complete dissociation. Examining the role of different parameters, it was observed that the incidence of electric discharges related significantly to the occurrence of electroclinical dissociation. PMID:10726833
Biagioni, E; Ferrari, F; Boldrini, A; Roversi, M F; Cioni, G
The attainment of developmental milestones was examined and compared in 162 infants and toddlers with developmental disabilities, including Down Syndrome (n = 26), Cerebral Palsy (n = 19), Global Developmental Delay (n = 22), Premature birth (n = 66), and Seizure Disorder (n = 29). Toddlers in the Seizures Disorder group began crawling at a…
Horovitz, Max; Matson, Johnny L.
A lens coloboma is not a true coloboma; it is just a zonular absence that causes a defect in the lens equator and hence a more spherical lens. It can be isolated or in association with iris, choroid or retinal colobomas. Typically the defect is present at the site of the embryonic fissure, but it can be present elsewhere. This congenital defect can cause amblyopia, especially when it is associated with cataract. We describe a case of a 39-year-old male with an atypical coloboma that was managed successfully with phacoemulsification, capsular tension ring and intraocular lens implantation. Scheimpflug image analysis and full ophthalmological assessment were performed. Scheimpflug images demonstrated a notching of the lens equator and an absence of zonulae. During the surgical procedure an ophthalmic viscosurgical device was used to tamponade the vitreous, and after continuous curvilinear capsulorhexis the capsular tension ring was inserted to allow the surgeon to perform a safe phacoemulsification.
Hernadez-Camarena, Julio C.; Ayup-Arguijo, Eduardo; Chavez-Mondragon, Eduardo; Ramirez-Miranda, Arturo
Introduction A laugh-induced seizure is an unrecognized condition and to the best of our knowledge no case has been reported in the medical literature until now. We present an interesting and extremely rare case in which laughing generated the seizure activity that was recorded and confirmed by video electroencephalography. Case presentation A 43-year-old obese Caucasian man with history of bipolar disorder and chronic headache presented with multiple episodes of seizures, all induced by laughter while watching comedy shows. Each episode lasted approximately five seconds. In each instance, he started laughing, then his arms started shaking and he felt like ‘his consciousness was being vacuumed away’. A physical examination revealed normal findings. He had been maintained on valproic acid for bipolar disorder and topiramate for his chronic headache, but this did not control his symptoms. His sleep-deprived electroencephalography and brain magnetic resonance imaging were normal except for an arachnoid cyst measuring 4.2 × 2.1cm in the anterior right middle cranial fossa. His video electroencephalography demonstrated laugh-induced seizure activities. He was then placed on carbamazepine. Following treatment, he had two episodes of mild staring but no frank seizures, and his seizures have remained well controlled on this regimen for more than a year. Conclusions Laugh-induced seizure is a most unusual clinical entity without any previous case report. Confirmatory diagnosis can be made by video electroencephalography recording of seizure activities provoked by laughing. As in gelastic seizure without hypothalamic hamartoma, our case responded well to polytherapy with topiramate and carbamazepine on top of laugh-provocation avoidance. Further study is required to establish the standard treatment of this condition.
Seizures are more common in the neonatal period than any other time in the human lifespan. A high index of suspicion for seizures should be maintained for infants who present with encephalopathy soon after birth, have had a stroke, central nervous system (CNS) infection or intracranial hemorrhage or have a genetic or metabolic condition associated with CNS malformations. Complicating the matter, most neonatal seizures lack a clinical correlate with only subtle autonomic changes and often no clinical indication at all. Over the last three decades, several tools have been developed to enhance the detection and treatment of neonatal seizures. The use of electroencephalography (EEG) and the later development of amplitude-integrated EEG (aEEG), allows for Neurologists and non-Neurologists alike, to significantly increase the sensitivity of seizure detection. When applied to the appropriate clinical setting, time to diagnosis and start of therapy is greatly reduced. Phenobarbital maintains the status of first-line therapy in worldwide use. However, newer anti-epileptic agents such as, levetiracetam, bumetanide, and topiramate are increasingly being applied to the neonatal population, offering the potential for seizure treatment with a significantly better side-effect profile. Seizures in premature infants, continue to confound clinicians and researchers alike. Though the apparent seizure burden is significant and there is an association between seizures and adverse outcomes, the two are not cleanly correlated. Compounding the issue, GABA-ergic anti-epileptic drugs are not only less effective in this age group due to reversed neuronal ion gradients but may cause harm. Selecting an appropriate treatment group remains a challenge. PMID:24796413
Vesoulis, Zachary A; Mathur, Amit M
Wilson's disease is a rare metabolic disease involving copper metabolism. Neuroimaging plays an important part in evaluation of patients with a neuropsychiatric presentation. We present a case of a 14-year-old girl with atypical confluent white matter disease and cystic degeneration on MRI, with a rapidly progressive course, who succumbed to complications despite treatment with trientine. Wilson's disease should be considered as a differential for leucoencephalopathy in young patients with progressive neurological disease for its early recognition and optimum outcome. PMID:24907221
Patell, Rushad; Dosi, Rupal; Joshi, Harshal K; Storz, Dennis
Seizures can occur spontaneously and in a recurrent manner, which defines epilepsy; or they can be induced in a normal brain under a variety of conditions in most neuronal networks and species from flies to humans. Such universality raises the possibility that invariant properties exist that characterize seizures under different physiological and pathological conditions. Here, we analysed seizure dynamics mathematically and established a taxonomy of seizures based on first principles. For the predominant seizure class we developed a generic model called Epileptor. As an experimental model system, we used ictal-like discharges induced in vitro in mouse hippocampi. We show that only five state variables linked by integral-differential equations are sufficient to describe the onset, time course and offset of ictal-like discharges as well as their recurrence. Two state variables are responsible for generating rapid discharges (fast time scale), two for spike and wave events (intermediate time scale) and one for the control of time course, including the alternation between 'normal' and ictal periods (slow time scale). We propose that normal and ictal activities coexist: a separatrix acts as a barrier (or seizure threshold) between these states. Seizure onset is reached upon the collision of normal brain trajectories with the separatrix. We show theoretically and experimentally how a system can be pushed toward seizure under a wide variety of conditions. Within our experimental model, the onset and offset of ictal-like discharges are well-defined mathematical events: a saddle-node and homoclinic bifurcation, respectively. These bifurcations necessitate a baseline shift at onset and a logarithmic scaling of interspike intervals at offset. These predictions were not only confirmed in our in vitro experiments, but also for focal seizures recorded in different syndromes, brain regions and species (humans and zebrafish). Finally, we identified several possible biophysical parameters contributing to the five state variables in our model system. We show that these parameters apply to specific experimental conditions and propose that there exists a wide array of possible biophysical mechanisms for seizure genesis, while preserving central invariant properties. Epileptor and the seizure taxonomy will guide future modeling and translational research by identifying universal rules governing the initiation and termination of seizures and predicting the conditions necessary for those transitions. PMID:24919973
Jirsa, Viktor K; Stacey, William C; Quilichini, Pascale P; Ivanov, Anton I; Bernard, Christophe
We studied head turning in 239 complex partial seizures with or without generalization, in 32 patients with unilateral temporal lobe epilepsy. Head turns occurred in 73% of seizures that did not evolve to focal jerking or secondary generalization, and in all 41 seizures that secondarily generalized. In seizures without focal jerking or secondary generalization the most common pattern was that
Bassel Abou-Khalil; Toufic Fakhoury
Summary: Purpose: Noninvasive magnetic resonance imaging was used to assess the evolution of seizure-induced pathology in epileptic, carrier, and normal chickens. Our objective was to determine whether repetitively evoked seizures in an epilep- tic fowl model of generalized seizures resulted in altered brain development. Methods: Data were obtained from seizure and control groups at 45, 90, and 180 days after
Zhao Gong; Andre Obenaus; Nan Li; Gordon E. Sarty; Edward J. Kendall
We report 3 cases presenting ictal vomiting during partial seizures of temporal lobe origin. Two patients had complex partial seizures accompanying vomiting characteristics. Ictal vomiting occurred early in the course of the seizure when rhythmic discharges involved predominantly the left hemispere, the language dominance hemispere. The other patient had ictal vomiting in simple partial seizures which originated from the right
Chien Chen; Der-Jen Yen; Chun-Hing Yiu; Yang-Hsin Shih; Hsiang-Yu Yu; Ming-Shung Su
Background Atypical clinical and imaging findings in Reversible Posterior Leukoencephalopathy Syndrome are recognized with increasing frequency. Case report We report a case of an adult in his 5th decade immunosupressed with methilprednisolone, tacrolimus and micophenolate who two months after renal transplantation, multiple infections and an episode of humoral rejection became hypertensive with severe headaches, visual field abnormalities, seizures, left hemiparesis and hemineglect. Computed Tomography scan of the brain showed a hypo dense lesion in the left occipital lobe. Ischemic stroke was diagnosed and aspirin and permissive hypertension were indicated. Twelve hours later he developed left sided motor seizures and cortical blindness. Magnetic Resonance Image showed hyper intensity in T2 and FLAIR in both occipital lobes and a small area of cortical restricted diffusion in Diffuson Weighted Images in the left occipital lobe. With a diagnosis of Reversible Posterior Leukoencephalopathy Syndrome his blood pressure was controlled with intravenous labetalol, and two days later the neurologic findings returned to baseline and most Computed tomography findings resolved. Conclusion This case underscores that in the appropriate setting Reversible Posterior Leukoencephalopathy Syndrome should be suspected and the clinician should not be misled by atypical clinical or imaging findings. In contrast to other pathologies that resemble Reversible Posterior Leukoencephalopathy Syndrome, with the right and timely treatment, signs, symptoms and images can be completely reversible.
This study investigated treatment patterns at discharge in infants with neonatal seizures and evaluated the impact of outpatient phenobarbital prophylaxis on the frequency of seizure recurrence and the long-term neurodevelopmental outcome at 1 to 11 years. Infants with neonatal seizures during a 12-year period were identified retrospectively (n = 146), and data were obtained by medical chart review. Outcomes were ascertained by standardized telephone survey. Thirty-three infants (23%) were taking phenobarbital, and 99 infants (68%) were taking no anticonvulsants at discharge. Comparisons were made between these 2 groups. Phenobarbital prophylaxis did not improve neurologic outcomes, either with respect to seizure recurrence or neurologic development. These data have important implications at a time when many are questioning the practice of prophylaxis after neonatal seizures and when newer anticonvulsants are being recommended for treatment of acute neonatal seizures. PMID:17621516
Guillet, Ronnie; Kwon, Jennifer
Amyloid beta-related angiitis (ABRA) of the central nervous system (CNS) is a very rare inflammatory disorder that causes destruction of CNS arteries and subsequent neuronal injury. Most patients with ABRA are old and present with cognitive dysfunction and stroke; however, some patients may present atypically. In this article, we report a 44-year-old man who presented with a first-time seizure but was otherwise neurologically intact and denied any headache. Brain MRI showed right hemispheric and bilateral medial frontal lobe hyperintensities and microhemorrhages that were most suspicious for a mass lesion. An extensive diagnostic evaluation including CSF analysis and catheter angiography was unremarkable. A brain biopsy with specific stains for amyloid surprisingly demonstrated ABRA and led to immunosuppressive treatment. The patient has remained neurologically intact and seizure-free 1 year after presentation. This case demonstrates that ABRA can occur in young patients without headache or neurologic deficits, and should be considered in patients with new-onset seizures and mass lesions. It also reinforces the need to consider a brain biopsy in patients with idiopathic brain lesions and negative non-invasive testing, as it is virtually impossible to confirm the diagnosis of ABRA otherwise. PMID:24707337
Ishii, Makoto; Lavi, Ehud; Kamel, Hooman; Gupta, Ajay; Iadecola, Costantino; Navi, Babak B
Amyloid beta-related angiitis (ABRA) of the central nervous system (CNS) is a very rare inflammatory disorder that causes destruction of CNS arteries and subsequent neuronal injury. Most patients with ABRA are old and present with cognitive dysfunction and stroke; however, some patients may present atypically. In this article, we report a 44-year-old man who presented with a first-time seizure but was otherwise neurologically intact and denied any headache. Brain MRI showed right hemispheric and bilateral medial frontal lobe hyperintensities and microhemorrhages that were most suspicious for a mass lesion. An extensive diagnostic evaluation including CSF analysis and catheter angiography was unremarkable. A brain biopsy with specific stains for amyloid surprisingly demonstrated ABRA and led to immunosuppressive treatment. The patient has remained neurologically intact and seizure-free 1 year after presentation. This case demonstrates that ABRA can occur in young patients without headache or neurologic deficits, and should be considered in patients with new-onset seizures and mass lesions. It also reinforces the need to consider a brain biopsy in patients with idiopathic brain lesions and negative non-invasive testing, as it is virtually impossible to confirm the diagnosis of ABRA otherwise.
Ishii, Makoto; Lavi, Ehud; Kamel, Hooman; Gupta, Ajay; Iadecola, Costantino; Navi, Babak B.
We report the successful surgical intervention in a case of constrictive pericarditis after long-term use of atypical antipsychotics. Pericarditis developed in our patient with a longstanding history of schizophrenia treated with atypical antipsychotics. Pericardiectomy was undertaken, and the patient's presenting symptom of shortness of breath resolved subsequently with an uneventful postoperative course.
Chen, Kuan-chin Jean; Goela, Aashish; Teefy, Patrick; Guo, L. Ray
Atypical antipsychotics were a great advance in the treatment of schizophrenia. But, there is still no atypical antipsychotic with an exceptional efficacy and safety profile for all patients. Clinicians are required to draw on their experiential knowledge to examine suitable options for individual patients. Following its suspension in 1998, the safety and efficacy of sertindole have been investigated in several
OBJECTIVE—Despite a high incidence of nocturnal hypoglycemia documented by the use of continuous glucose monitoring (CGM), there are no reports in the literature of nocturnal hypoglycemic seizures while a patient is wearing a CGM device. RESEARCH DESIGN AND METHODS—In this article, we describe four such cases and assess the duration of nocturnal hypoglycemia before the seizure. RESULTS—In the cases where patients had a nocturnal hypoglycemic seizure while wearing a CGM device, sensor hypoglycemia (<60 mg/dl) was documented on the CGM record for 2.25–4 h before seizure activity. CONCLUSIONS—Even with a subcutaneous glucose lag of 18 min when compared with blood glucose measurements, glucose sensors have time to provide clinically meaningful alarms. Current nocturnal hypoglycemic alarms need to be improved, however, since patients can sleep through the current alarm systems.
Buckingham, Bruce; Wilson, Darrell M.; Lecher, Todd; Hanas, Ragnar; Kaiserman, Kevin; Cameron, Fergus
In front of any clinical paroxysmal event in childhood, the first step is to make a positive diagnostic of an epileptic seizure; for this it is necessary to eliminate non epileptic seizures which are different according to age. Then the type of seizures has to be precised, being focal or generalized. EEG will contribute to determine the epileptic syndrome according to interictal and/or ictal findings. The epilepsy syndrome is the main entity to go further in etiology and treatment. According to the type of epilepsy syndrome it will be possible to look for a structural or metabolic cause, or to perform a genetic study. The present classification of seizures and syndromes as proposed by the International League Against Epilepsy (ILAE) allows a common language in the world community as in clinical and therapeutic research. PMID:23424915
Plouin, Perrine; Kaminska, Anna; Eisermann, Monika; Soufflet, Christine
... information: Long QT syndrome (The Basics) Patient information: Myoclonus (The Basics) Patient information: Seizures (The Basics) Patient ... High blood pressure emergencies (The Basics) Patient information: Myoclonus (The Basics) Patient information: Time to stop driving? ( ...
There are an estimated two million people with epilepsy in the United States. Many of these people do not respond to anti-epileptic drug therapy. Two devices can be developed to assist in the treatment of epilepsy. The first is a microcomputer-based system designed to process massive amounts of electroencephalogram (EEG) data collected during long-term monitoring of patients for the purpose of diagnosing seizures, assessing the effectiveness of medical therapy, or selecting patients for epilepsy surgery. Such a device would select and display important EEG events. Currently many such events are missed. A second device could be implanted and would detect seizures and initiate therapy. Both of these devices require a reliable seizure detection algorithm. A new algorithm is described. It is believed to represent an improvement over existing seizure detection algorithms because better signal features were selected and better standardization methods were used.
Olsen, Dale E.; Harris, John C.; Cutchis, Protagoras N.; Cristion, John A.; Lesser, Ronald P.; Webber, W. Robert S.
Coexistence of 2 idiopathic epilepsy syndromes (ie, childhood absence and Rolandic epilepsy), as evidenced by electroencephalographic (EEG) findings with or without clinical features of the 2 conditions, is uncommon and remains controversial. Few case reports support this coexistence either as a continuum or drug-induced conversion, whereas a large sample case review did not find such co-occurrence. The authors report a case of conversion of typical absence to Rolandic spikes after treatment with ethosuximide. An 11-year-old girl was diagnosed with typical childhood absence epilepsy at the age of 6 years with classic clinical and EEG features. She became seizure-free on ethosuximide but her follow-up EEGs consistently recorded right centrotemporal and centroparietal spikes without associated clinical seizures. This case may suggest simultaneous presence of these 2 common childhood idiopathic epilepsies either as a continuum or a drug-induced conversion. PMID:22467741
Anyanwu, Chinekwu; Ghavami, Forough; Schuelein, Marianne; Motamedi, Gholam K
Background: To evaluate seizure characteristic among multiple sclerosis patients with coexistent seizure activity compared to control group. Materials and Methods: This study is a cross-sectional study which was conducted by reviewing the clinical records of patients with definite diagnosis of MS according to McDonald's criteria from March 2007 to June 2011, who referred to the MS clinic of the university. Results: A total of 920 patients with a diagnosis of MS were identified, among whom 29 patients (3.15%) with seizure activity (case) due to MS with the mean age of 32.6 ± 6.23 years were analyzed. Also, fifty MS patients without any seizure occurrence with the mean age of 33.7 ± 7.4 years were used as our control group. In case group, seizure was general tonic clonic in 23 patients (79.3%), complex partial in four (13.8%), and simple partial in two (5.9%). The 26 available interictal EEGs in MS patients showed abnormal EEG pattern in 22 (84.6%) of them, including focal epileptic form discharge or focal slowing in 10 (38.5%), generalized discharge (spike-wave, polyspike, or general paroxysmal fast activity) in 10 (38.5%), and general slowing activity in 10 record (38.5%). MRI reviews of the 26 available brain MRIs showed subcortical white mater lesions in 22 (84.6%) of patients with seizure. All MRIs were performed within one month after the first seizure episode. Amongst 48 available MRIs in our control group, 91.7% (44 cases) showed periventricular lesions and in 8.3% (4 cases) subcortical white matter lesions were reported. Conclusion: The result of this study demonstrated the higher rate of subcortical whit matter lesion in MS patients with seizure occurrence compared to control group.
Shaygannejad, Vahid; Ashtari, Fereshteh; Zare, Mohammad; Ghasemi, Majid; Norouzi, Rasul; Maghzi, Helia
Effective treatment of seizures associated with central nervous system (CNS) infection and inflammation depends on rapid diagnosis\\u000a and early attainment of bactericidal activity in the cerebrospinal fluid with appropriate antimicrobial agents, or appropriate\\u000a management of vasculitis-induced cerebral complications. Despite the rarity of these disorders, there is nothing specific\\u000a regarding the management in the intensive care unit of seizures in these
Wendy C. Ziai
A 9 year old girl presented with seizures, weight gain and early morning behavioural changes. She had been commenced on anticonvulsants and was subsequently diagnosed with hyperinsulinaemic hypoglycaemia. This case demonstrates the importance of blood glucose monitoring in children presenting with new-onset seizures and/or with early morning or fasting behavioural changes, the challenges in localizing the lesion, as well as the difficulties in achieving normoglycaemia prior to, and immediately following, surgery. PMID:24698060
Kao, Kung-Ting; Simm, Peter J; Brown, Justin
This hospital based cross sectional analytic study was carried out in department of Pediatrics Dhaka Medical College Hospital and Combined Military Hospital from July 2009 to June 2010 to find out the relationship of iron status with febrile seizure in children. Sixty children aged 6 months to 5 years having febrile seizure were enrolled as cases after taking proper consent by purposive sampling. Sixty age matched children with fever but no seizures were taken as control after taking proper consent. Children having a febrile seizure, developmental delay, hemorrhagic disorder, severe malnutrition or micronutrient deficiency and abnormal CSF findings were excluded from the study. In cases after control of seizure 2 ml of CSF was taken for cytology, biochemistry, Gram & AFB staining. Two milliliter of blood from cases as well as control was collected, released to EDTA bottle for Hb, MCV and MCHC. Again 2 ml of blood was taken in plain test tube for testing serum iron, TIBC and for serum ferritin. Serum iron, TIBC, serum ferritin was estimated by automated analyzer (PENTRA for serum iron, DADE BEHRING for TIBC, IMMULITE 1000 for serum ferritin). There was no significant difference between two groups regarding age (p>0.05), sex (p>0.05). High fever (p<0.001) and continuous fever (p<0.001) was significantly associated with febrile seizure. Seizure was generalized in all with positive family history in 83.3% cases. The Mean±SD of blood Hb (Case 9.8±1.6; Control 10.7±0.9), MCH (Case 22.1±2.3; Control 24.3±3.6) & serum ferritin (Case 55.1±29.3; Control 99.6±81.9) level were significantly low associated with febrile seizure (p<0.05). No significant difference was found between cases and control regarding MCV, serum iron and serum TIBC. PMID:23715348
Choudhury, M A; Zaman, M; Mollah, A H; Hoque, M A; Fatmi, L E; Islam, M N; Bhuiyan, K J; Hossain, M A
Thinking epilepsy is a rare form of reflex epilepsy that can be induced by specific cognitive tasks, and occurs mainly in idiopathic generalized epilepsies. We report a case of complex partial seizures triggered by thinking in a young man with acute bacterial meningitis and a remote head injury. This case illustrates that thinking-induced reflex seizures can be partial and can be provoked by an acute brain insult. PMID:23114680
Nevler, Naomi; Gandelman-Marton, Revital
The dramatic improvement in knowledge concerning celiac disease (CD) has disclosed the pattern of the associated clinical manifestations and the often atypical or silent presentation of this disease, which makes clinical diagnosis difficult. Also oral manifestations, mostly recurrent apthous stomatitis (RAS) and dental enamel hypoplasia, are atypical signs of CD. Our opinion about the possibility of performing mass-screening to reveal atypical or silent CD is in agreement whit who is asserting that a sistematical case-finding is, at present, the most suitable epidemiological approach. So, we think that patients affected by RAS, or dental enamel hypoplasia, should be considered, even in the absence of any gastrointestinal symptom, at-risk subjects, and should therefore undergo diagnostic procedure for CD. PMID:15560296
Pastore, Luca; De Benedittis, Michele; Petruzzi, Massimo; Tatò, Daniela; Napoli, Christian; Montagna, Maria Teresa; Catassi, Carlo; Serpico, Rosario
Background: There is a broad variation in reported frequencies of seizure in multiple sclerosis (MS). In this study, the seizure and its characteristics analyzed among a large group of patients with MS. Patients and Methods: We reviewed the medical records of all definite MS patients referred to the MS Clinic of Kashani hospital, Isfahan, Iran, between 2007 and 2011. Results: Altogether, 34 cases with seizure activity identified among the 920 definite MS subjects (3.69%). Five excluded due to the other probable etiologies rather than MS. In the remained 29 patients (3.15%), the type of seizure was mostly generalized (79.3%); interictal electroencephalography showed an abnormal pattern in 84.6%, brain magnetic resonance imaging revealed subcortical white mater lesions in 84.6% of patients. The mean duration of MS onsets was 8.17 years and the mean interval between MS onset and the first seizure occurrence was 3.7 years. In general, response to antiepileptic treatment was excellent. Conclusion: Seizures can occur at any stage during the course of MS, but it is more common during the early stages.
Zare, Mohammad; Norouzi, Rasul; Shayegannejad, Vahid; Ashtari, Fereshteh; Ghasemi, Majid; Tavahen, Hemaseh; Masaeli, Ali
A 25-year-old woman with recurrent syncopal episodes presented with a first time generalized tonic clonic (GTC) seizure. She had experienced two prior fainting spells lasting seconds and associated with diet pills and dehydration. She had another similar spell prior to falling, sustaining a laceration to the right posterior occiput, and having a witnessed GTC seizure. Her scalp electroencephalography (EEG) showed left temporal slowing with sharp features. T1-weighted and T2-weighted MRI revealed two moderately enhancing focal lesions within the left frontal and temporal regions. These findings raised the possibility of an underlying seizure focus. Repeat imaging studies of this patient 1 month later, however, demonstrated resolution of these findings and an area of encephalomalacia, consistent with a traumatic coup contrecoup injury. A repeat EEG was normal. Therefore, the cause of the loss of consciousness was due to syncope with the consequent head injury giving rise to an isolated seizure. Understanding the underlying cause of a seizure is important in dictating treatment. In this setting the patient was not initiated on seizure medication and has done well. PMID:22245277
Sheen, Volney L
This review focuses on anoxic seizures induced by self terminating syncopes in the young. Anoxic seizures are nonepileptic events consequent upon abrupt interruption of the energy supply to metabolically active cerebral neurones. Anoxic seizures are the most common paroxysmal events misdiagnosed as epilepsy. Neurally mediated syncopes have numerous appellations, especially in the young. This proliferation of terminology likely results from uncertainty regarding pathophysiology. The most important type of self-limiting syncope from the point of view of diagnostic difficulty has been called neurocardiogenic or vasovagal syncope and reflex anoxic seizure, amongst other names: this review includes a video clip of such a child with prolonged asystole. It also includes a detailed case history emphasising the feelings of a patient with this type of syncope who was misdiagnosed as having epilepsy for many years. The second class of self-terminating syncope discussed and illustrated on video is the so-called breath-holding spell of young children. The third example illustrated is the compulsive Valsalva manoeuvre of individuals with autistic spectrum disorder, in which anoxic seizures - as shown on the video clips - are easily misdiagnosed as epileptic seizures, with unfortunate consequences. PMID:11313215
Stephenson, J B
Most intellectually normal children with focal epilepsy have partial complex or focal with secondary generalization seizures without a precise epilepsy syndrome. Their long-term outcome is largely unknown. Cases were identified from the population-based Nova Scotia Childhood Epilepsy cohort. Those eligible had seizure onset at 1 month to 16 years between 1977 and 1985, normal intelligence, ?10 years of follow-up, only focal seizures and no benign epilepsy syndromes. There were 108 patients with partial complex with or without secondary generalization as the only seizure type(s) throughout (partial complex group) and 80 with secondary generalization as the only seizure type (secondary generalization group). Average age ± standard deviation at onset was 7.3 ± 4.5 years and follow-up was 27.9 ± 5.4 years. At follow-up, 57% of the partial complex group were in remission versus 81% of the secondary generalization group (P = 0.001). The partial complex group was more likely to be intractable or have undergone epilepsy surgery (36% versus 5%, P = 0.000). In the partial complex group, 28% had <5 years seizure free versus 5% in the secondary generalized group (P = 0.000). More patients in the partial complex group had undergone mental health assessments (59% versus 32%, P = 0.000), and 33% had a psychiatric diagnosis versus 15% in the secondary generalized group (P = 0.004). More patients with partial complex seizures had specific learning disorders (63% versus 45%, P = 0.03). Seven markers of poor social outcome were more common in patients with partial complex seizures (>2 markers: 34% versus 10%, P = 0.000). During 25-30 years of follow-up, >50% of intellectually normal patients with childhood-onset partial complex seizures had difficult-to-control seizures and learning and psychiatric/social problems. Most with secondary generalized seizures only had remission and better academic and psychiatric/social outcomes. PMID:23378221
Camfield, Carol S; Camfield, Peter R
Limited information is available regarding the methodology required to characterize hashish seizures for assessing the presence or the absence of a chemical link between two seizures. This casework report presents the methodology applied for assessing that two different police seizures were coming from the same block before this latter one was split. The chemical signature was extracted using GC-MS analysis and the implemented methodology consists in a study of intra- and inter-variability distributions based on the measurement of the chemical profiles similarity using a number of hashish seizures and the calculation of the Pearson correlation coefficient. Different statistical scenarios (i.e., a combination of data pretreatment techniques and selection of target compounds) were tested to find the most discriminating one. Seven compounds showing high discrimination capabilities were selected on which a specific statistical data pretreatment was applied. Based on the results, the statistical model built for comparing the hashish seizures leads to low error rates. Therefore, the implemented methodology is suitable for the chemical profiling of hashish seizures. PMID:24008198
Cadola, Liv; Broséus, Julian; Esseiva, Pierre
This paper describes the sensor technology and associated electronics of a monitor designed to detect the onset of a seizure disorder called status epilepticus. It is a condition that affects approximately 3-5 percent of those individuals suffering from epilepsy. This form of epilepsy does not follow the typical cycle of start-peak-end. The convulsions continue until medically interrupted and are life threatening. The mortality rate is high without prompt medical treatment at a suitable facility. The paper describes the details of a monitor design that provides an inexpensive solution to the needs of those responsible for the care of individuals afflicted with this disorder. The monitor has been designed as a cooperative research and development effort involving the United States Army Armament Research, Development, and Engineering Center's Benet Laboratories (Benet) and the Cerebral Palsy Center for the Disabled (Center), in association with the Department of Neurology at Albany Medical College (AMC). Benet has delivered a working prototype of the device for field testing, in collaboration with Albany Medical College. The Center has identified several children in need of special monitoring and has agreed to pursue commercialization of the device.
Johnson, Mark; Simkins, Thomas
LAY ABSTRACT Diagnosis of an autism spectrum disorder (ASD) requires a qualitative assessment of social aptitude: one person judging whether another person interacts in a ‘typical’ way. Quantitative or computerized assessment of social aptitude cannot substitute for this subjective judgment. We hypothesized that mice could be used to make a similar judgment if they prefer ‘typical’ over ‘atypical’ social interactions with mouse models relevant to ASD. We used typical C57BL/6 (B6) mice as ‘judges’ and evaluated their preference for a chamber containing a ‘typical’ or an ‘atypical’ mouse. For our atypical mice, we chose two strains with well-documented social phenotypes, as well a mutant line with abnormal social behavior and seizures. Overall, we observed a characteristic pattern of behavior over the course of 30 minutes, with the judges preferring the typical mouse chamber to the atypical mouse chamber during the last 10 minutes of the test. When we evaluated the individual stimulus pairings, two of the three showed a similar pattern as the overall results, and the other stimulus comparison showed a trend for a preference for the typical mouse chamber across the entire test. We repeated the experiments using the 129S6 strain of typical mice as judges and found a much less strong preference pattern across time. These data suggest that a characteristic pattern of exploration in B6 mice can distinguish some socially atypical animals from controls. SCIENTIFIC ABSTRACT Diagnosis of an autism spectrum disorder (ASD) requires a qualitative assessment of social aptitude: one person judging whether another person interacts in a ‘typical’ way. We hypothesized that mice could be used to make a similar judgment if they prefer ‘typical’ over ‘atypical’ social interactions with mouse models relevant to ASD. We used wildtype C57BL/6 (B6) mice as ‘judges’ and evaluated their preference for a chamber containing a ‘typical’ (B6 or 129S6) or an ‘atypical’ mouse. For our atypical mouse stimuli, we chose two inbred strains with well-documented social phenotypes (BTBR and BALB/c), as well a mutant line with abnormal social behavior and seizures (Gabrb3 +/?). Overall, we observed a stimulus by time interaction (P < 0.0001), with B6 mice preferring the typical mouse chamber during the last 10 minutes of the 30-minute test. For two of the individual stimulus pairings, we observed a similar chamber by time interaction (BALB/c vs. 129S6, P = 0.0007; Gabrb3 +/? vs. 129S6, P = 0.033). For the third stimulus pairing, we found a trend for preference of the typical mouse across time (BTBR vs. B6, P = 0.051). We repeated the experiments using 129S6 mice as judges and found a significant overall interaction (P = 0.034), but only one stimulus pairing reached significance on its own (BALB/c vs. 129S6, P = 0.0021). These data suggest that a characteristic pattern of exploration in B6 mice can distinguish some socially atypical animals from controls.
Shah, Charisma R.; Forsberg, Carl Gunnar; Kang, Jing-Qiong; Veenstra-VanderWeele, Jeremy
TREM2 mutations were first identified in Nasu-Hakola disease, a rare autosomal recessive disease characterized by recurrent fractures because of bone cysts and presenile dementia. Recently, homozygous and compound heterozygous TREM2 mutations were identified in rare families with frontotemporal lobar degeneration (FTLD) but without bone involvement. We identified a p.Thr66Met heterozygous mutation in a new consanguineous Italian family. Two sibs had early onset autosomal recessive FTLD without severe bone disorders. Atypical signs were present in this family: early parietal and hippocampus involvement, parkinsonism, epilepsy, and corpus callosum thickness on brain magnetic resonance imaging. This study further demonstrates the implication of TREM2 mutations in FTLD phenotypes. It illustrates the variability of bone phenotype and underlines the frequency of atypical signs in TREM2 carriers. This and previous studies evidence that TREM2 mutation screening should be limited to autosomal recessive FTLD with atypical phenotypes characterized by: (1) a very young age at onset (20-50 years); (2) early parietal and hippocampal deficits; (3) the presence of seizures and parkinsonism; (4) suggestive extensive white matter lesions and corpus callosum thickness on brain magnetic resonance imaging. PMID:24910390
Le Ber, Isabelle; De Septenville, Anne; Guerreiro, Rita; Bras, José; Camuzat, Agnès; Caroppo, Paola; Lattante, Serena; Couarch, Philippe; Kabashi, Edor; Bouya-Ahmed, Kawtar; Dubois, Bruno; Brice, Alexis
In WAG/Rij rats with a genetic predisposition to absence epilepsy, age-related dynamics of seizure activity in the frontal EEG was studied. In parallel, cellular content in substantia nigra was analyzed at the age of 1 and 10 months. In 10-months animals, the number of neurons in substantia nigra showed a profound decrease, whereas the number of glial calls increased. In parallel to that, the incidence of epileptic discharges and their duration significantly increased between 5 and 9 months of age. Positive correlations were revealed between the incidence of epileptic discharges and the number of neurons in substantia nigra suggesting that animals with lower number of neurons in SN expressed fewer absence seizures, and vice versa. It was suggested that reduction of neurons in substantia nigra is an adaptive process to prevent progressive development of absence seizures. PMID:23227734
Sitnikova, E Iu; Egorova, T N; Raevski?, V V
This study evaluated the incidence, prevalence, and clinical features of seizures in a pedigreed captive colony of baboons. The association of seizures with subspecies, age, sex, and various clinical features was assessed. Records for 1527 captive, pedigreed baboons were reviewed, and 3389 events were identified in 1098 baboons. Of these events, 1537 (45%) represented witnessed seizures, whereas the remaining 1852 presented with craniofacial trauma or episodic changes in behavior that were suggestive, but not diagnostic, of seizure activity. Seizures were generalized myoclonic or tonic–clonic, with two thirds of the events witnessed in the morning. Seizure onset occurred in adolescence (age, 5 y), with an average of 3 seizures in a lifetime. The incidence and prevalence of seizures were 2.5% and 26%, respectively, whereas the prevalence of recurrent seizures (that is, epilepsy) was 15%. Seizures were more prevalent in male baboons, which tended to present with earlier onset and more seizures over a lifetime than did female baboons. Seizures were equally distributed between the subspecies; age of onset and seizure recurrences did not differ significantly between subspecies. Clinical features including age of onset, characteristics, and diurnal presentation of seizures in baboons suggested similarities to juvenile myoclonic epilepsy in humans. Facial trauma may be useful marker for epilepsy in baboons, but its specificity should be characterized.
Szabo, C Akos; Knape, Koyle D; Leland, M Michelle; Cwikla, Daniel J; Williams-Blangero, Sarah; Williams, Jeff T
This study evaluated the incidence, prevalence, and clinical features of seizures in a pedigreed captive colony of baboons. The association of seizures with subspecies, age, sex, and various clinical features was assessed. Records for 1527 captive, pedigreed baboons were reviewed, and 3389 events were identified in 1098 baboons. Of these events, 1537 (45%) represented witnessed seizures, whereas the remaining 1852 presented with craniofacial trauma or episodic changes in behavior that were suggestive, but not diagnostic, of seizure activity. Seizures were generalized myoclonic or tonic-clonic, with two thirds of the events witnessed in the morning. Seizure onset occurred in adolescence (age, 5 y), with an average of 3 seizures in a lifetime. The incidence and prevalence of seizures were 2.5% and 26%, respectively, whereas the prevalence of recurrent seizures (that is, epilepsy) was 15%. Seizures were more prevalent in male baboons, which tended to present with earlier onset and more seizures over a lifetime than did female baboons. Seizures were equally distributed between the subspecies; age of onset and seizure recurrences did not differ significantly between subspecies. Clinical features including age of onset, characteristics, and diurnal presentation of seizures in baboons suggested similarities to juvenile myoclonic epilepsy in humans. Facial trauma may be useful marker for epilepsy in baboons, but its specificity should be characterized. PMID:23561888
Szabó, C Ákos; Knape, Koyle D; Leland, M Michelle; Cwikla, Daniel J; Williams-Blangero, Sarah; Williams, Jeff T
It is generally accepted that sleep deprivation contributes to seizures. However, it is unclear whether a seizure occurring in the setting of sleep deprivation should be considered as provoked or not and whether this is influenced by seizure type and etiology. This information may have an important impact on epilepsy diagnosis and management. We prospectively analyzed the influence of sleep deprivation on the risk of seizure recurrence in patients with first-ever unprovoked seizures and compared the findings with patients with first-ever provoked seizures. Of 1026 patients with first-ever unprovoked seizures, 204 (20%) were associated with sleep deprivation. While the overall likelihood of seizure recurrence was slightly lower in sleep-deprived patients with first-ever seizures (log-rank p=0.03), sleep deprivation was not an independent predictor of seizure recurrence on multivariate analysis. Seizure recurrence following a first-ever unprovoked seizure associated with sleep deprivation was far more likely than for 174 patients with a provoked first-ever seizure (log-rank p<0.0001). Our findings support the International League Against Epilepsy recommendation that seizures occurring in the setting of sleep deprivation should not be regarded as provoked. PMID:24657503
Lawn, Nicholas; Lieblich, Sam; Lee, Judy; Dunne, John
Objectives Partial seizures are often believed to be associated with EEG signals of low complexity because seizures are associated with increased neural network synchrony. The investigations reported here provide an assessment of the signal complexity of epileptic seizure onsets using newly developed quantitative measures. Methods Using the Gabor atom density (GAD) measure of signal complexity, 339 partial seizures in 45 patients with intracranial electrode arrays were analyzed. Segmentation procedures were applied to determine the timing and amplitude of GAD changes relative to the electrographic onset of the seizure. Results 330 out of 339 seizures have significant complexity level changes, with 319 (97%) having an increase in complexity. GAD increases occur within seconds of the onset of the partial seizure but are not observed in channels remote from the focus. The complexity increase is similar for seizures from mesial temporal origin, neocortical temporal and extra-temporal origin. Conclusions Partial onset seizures are associated with early increases in signal complexity as measured by GAD. This increase is independent of the location of the seizure focus. Significance Despite the often predominant rhythmic activity that characterizes onset and early evolution of epileptic seizures, partial seizure onset is associated with an early increase in complexity. These changes are common to partial seizures originating from different brain regions, indicating a similar seizure dynamic.
Jouny, Christophe C; Bergey, Gregory K; Franaszczuk, Piotr J
Parvovirus B19 is the etiologic agent of erythema infectiosum (fifth disease), a fever-rash illness occurring in childhood. We present a 10 month old child with high grade fever for 10 days, generalized tonic-clonic seizure, bilateral cervical lymphadenopathy, generalized maculopapular rash, hematemesis and malena. Bone marrow aspiration and liver biopsy were done. EBV serology and parvovirus PCR were also performed. Bone marrow aspiration and biopsy showed giant pro-erythroblast consistent with parvovirus infection. PCR showed amplification of parvovirus genomic sequences. Present case highlights an atypical presentation of Parvovirus B19 infection as fever, rash and hepatosplenomegaly. PMID:21760806
Kamlesh, Yadav; Pallav, Gupta; Manjula, Murari; Rohan, Malik
Ten patients who presented with dorsolateral or frontocentral seizures were studied with chronic subdural grid electrodes. Cortical mapping, sensory-evoked potentials and chronic electrocorticography were obtained for each patient. Seizures were classified as focal, regional or dipolar. At the time of explanation, a selective functional corticectomy was performed. Surgical outcome is presented at a mean follow-up of 36 months. Two patients are seizure-free and 7 patients had a significant reduction in seizure frequency. One patient had no change in seizure pattern. Dorsolateral frontal lobe seizures have a focal functional anatomy and can be surgically treated by selective cortectomy. PMID:1439335
Levesque, M F; Sutherling, W W; Crandall, P H
Five patients who developed seizures following a general anesthetic are described. It is not possible to determine retrospectively whether or not the initial attacks were definitely epileptic, but these patients all subsequently received a diagnosis of psychological nonepileptic convulsions/seizures (also known as pseudoseizures, psychogenic nonepileptic seizures, and nonepileptic attack disorder) established by video/EEG telemetry or ictal EEG recordings. In two cases there was evidence of concurrent epilepsy. We suggest that nonepileptic seizures may develop following postanesthetic seizures and that a psychogenic basis for seizures occurring after general anesthetics needs to be considered. PMID:15582852
Lichter, Ida; Goldstein, Laura H; Toone, Brian K; Mellers, John D C
We studied 10 patients with intractable epilepsy being evaluated for epilepsy surgery for preictal changes in spiking. All patients were implanted with intracranial electrodes and underwent continuous EEG/audiovisual monitoring. Interictal spikes were detected and recorded continuously by a dedicated computerized system. Edited spikes were counted during 0-5, 5-10, and 0-60 min epochs before each seizure, during epochs of unvarying state of arousal (awake or sleep stage II). When comparing by repeated measures, 1-way ANOVA, total spiking (in all recording channels) did not differ among the different preictal epochs (0-5, 5-10, 0-60 min) in 45 seizures (F = 0.88, P = 0.40, using the Geisser-Greenhouse adjustment--GGA). Likewise, no significant differences were obtained during those same epochs when comparing spiking originating from the channel of seizure onset in 5 patients with 28 seizures of localized onset (F = 1.19, P = 0.38 using the GGA). Our findings indicate that in patients with intractable epilepsy, no changes in spiking occur in the 5 min prior to seizures, when compared to more distant preictal epochs. PMID:1713830
Katz, A; Marks, D A; McCarthy, G; Spencer, S S
The relation between interhippocampal seizure propagation time (IHSPT) and anatomic alterations in the human epileptic hippocampus may provide insight into the pathophysiology of temporal lobe epilepsy (TLE). Using depth electrode recordings, we measured the time required for spontaneous seizures with onset in one hippocampus to become manifest in the contralateral hippocampus in 50 patients who underwent resection of the temporal lobe of seizure origin. Cell densities in individual hippocampal subfields were determined and correlated with mean IHSPT for each patient. Mean IHSPT was significantly and inversely correlated with cell counts in CA4 only (r = -0.38, p less than 0.01, Pearson's product correlation; r = -0.52, p less than 0.001, Spearman's rank order correlation). In 5 patients with bilateral independent hippocampal seizure onset who had temporal lobectomy and a diagnosis of mesial temporal sclerosis, mean IHSPT was consistently longer from the sclerotic temporal lobe than to it. These observations suggest that anatomic changes associated with chronic epilepsy alter propagation patterns. Because CA4 is believed to modulate the output of dentate granule cells and also has commissural connections to the contralateral homotopic area, the association of decreased CA4 cells with prolongation of IHSPT suggests that the observed anatomic alterations may actively (through increased inhibition) or passively (through decreased recruitment) interfere with various routes of seizure propagation. PMID:1396429
Spencer, S S; Marks, D; Katz, A; Kim, J; Spencer, D D
Seizures are a frequent complication associated with several neurogenetic disorders. Antiepileptic medications remain the mainstay of treatment in these patients. We summarized the available data associated with various antiepileptic therapies used to treat patients with neurogenetic disorders who experienced recurrent seizures. A MEDLINE search was conducted to identify articles and abstracts describing the use of antiepileptic therapy for the treatment of various neurogenetic syndromes. Of all the neurogenetic syndromes, only autism spectrum disorders, Angelman syndrome, Rett syndrome, Dravet syndrome, and tuberous sclerosis complex were identified as having sufficient published information to evaluate therapy. Some efficacy trends were identified, including frequent successes with valproic acid with clonazepam for epilepsy with Angelman syndrome; valproic acid, stiripentol, and clobazam (triple combination therapy) for epilepsy with Dravet syndrome; and vigabatrin for infantile spasms associated with tuberous sclerosis complex. Due to a paucity of information regarding the mechanisms by which seizures are generated in the various disorders, approach to seizure control is primarily based on clinical experience and a limited amount of study data exploring patient outcomes. Although exposure of the developing brain to antiepileptic medications is of some concern, the control of epileptic activity is an important undertaking in these individuals, as the severity of eventual developmental delay often appears to correlate with the severity of seizures. As such, early aggressive therapy is warranted. PMID:23400943
Faulkner, Michele A; Singh, Sanjay P
Although antiepileptic drugs are often effective in the control of seizures, some patients show little or no improvement. As alternative treatments, different dietary modifications were shown to be beneficial for patients with poor tolerance for AEDS. Previous reports have shown that rice-based oral electrolyte hydration therapy is effective in seizure control in patients with refractory absence seizures. In the present study, using an animal model of absence epilepsy, we showed that the occurrence of spike-and-wave discharges significantly decreases upon switching to electrolyte therapy. We also showed that consumption of solution with the same osmolarity as rice-based oral electrolyte solution leads to a decrease in the number of spike-and-wave discharges per hour. We suggest that the antiepileptic effect of rice-based oral electrolyte hydration therapy can be at least in part due to hyperosmolarity of the ingested solution. PMID:22749239
Talnov, Arkadij N; Isaeva, Elena; Savotchenko, Alina V; Dovgalets, Galina V; Ochoa, Juan G; Holmes, Gregory L; Isaev, Dmytro
Seizures are a frequent sequela of impaired brain development and can be expected to affect more children with radiation-related brain damage than children without such damage. This report deals with the incidence and type of seizures among survivors prenatally exposed to the atomic bombing of Hiroshima and Nagasaki, and their association with specific stages of prenatal development at the time of irradiation. Fetal radiation dose was assumed to be equal to the dose to the maternal uterus. Seizures here include all references in the clinical record to seizure, epilepsy, or convulsion. Histories of seizures were obtained at biennial routine clinical examinations starting at about the age of 2 years. These clinical records were used to classify seizures as febrile or unprovoked (without precipitating cause). No seizures were ascertained among subjects exposed 0-7 weeks after fertilization at doses higher than 0.10 Gy. The incidence of seizures was highest with irradiation at the eighth through the 15th week after fertilization among subjects with doses exceeding 0.10 Gy and was linearly related to the level of fetal exposure. This obtains for all seizures without regard to the presence of fever or precipitating causes, and for unprovoked seizures. When the 22 cases of severe mental retardation were excluded, the increase in seizures was only suggestively significant and only for unprovoked seizures. After exposure at later stages of development, there was no increase in recorded seizures.
Dunn, K.; Yoshimaru, H.; Otake, M.; Annegers, J.F.; Schull, W.J. (Radiation Effects Research Foundation, Hiroshima (Japan))
Congenital absence of uterus and vagina, Mayer–Rokitansky–Kuster–Hauser (MRKH) syndrome, is mullerian agenesis and is the\\u000a second most frequent cause of primary amenorrhea. Only atypical form of MRKH (type B) is associated with renal skeletal and\\u000a ovarian abnormalities. We report the management of an unusual case of atypical MRKH, unilateral gonadal agenesis, and solitary\\u000a ectopic pelvic kidney with pelviureteric junction obstruction
Anup Kumar; Saurabh Mishra; P. N. Dogra
The aim of this study was to review the efficacy and safety of atypical antipsychotics, comparing within class, placebo, or compared to another active treatment for delirium. A literature search was conducted using PubMed, EMBASE, and the Cochrane database (1 January 1990-5 November 2012). Selection criteria for review were prospective, controlled studies (comparison studies), using validated delirium rating scales as outcome measures. A total of six prospective, randomized controlled studies were included in the review. It was found that atypical antipsychotics are effective and safe in treating delirium, even though there seemed to be no difference between each agent. In particular, comparison studies with haloperidol showed that the efficacy of atypical antipsychotics was similar to that of low-dose haloperidol. It was concluded that atypical antipsychotics appear to be effective and tolerable in the management of delirium, even though the evidence is limited. PMID:23859663
Wang, Hee Ryung; Woo, Young Sup; Bahk, Won-Myong
Psychogenic non-epileptic seizures (PNES) are frequently encountered in epilepsy monitoring units (EMU) at Veterans Affairs Medical Centers (VAMCs) and cause significant long-term disability. An understanding of psychiatric factors associated with PNES could aid in earlier diagnosis and treatment. We studied 50 consecutive veterans diagnosed with PNES and 37 veterans diagnosed with epileptic seizures (ES), evaluated at a VAMC EMU. We reviewed all available mental health evaluations prior to EMU evaluation. Univariate comparisons included axis I diagnoses, axis II diagnoses, and psychiatric hospitalizations. Predictive models of seizure classification were evaluated by logistic regression. A diagnosis of post-traumatic stress disorder (PTSD) preceded the diagnosis of PNES in 58% of patients and the diagnosis of ES in 13.5% (p<0.001). On logistic regression, PTSD was the only significant psychiatric diagnosis (odds ratio 9.2). Major depression and alcohol abuse were common diagnoses but did not differentiate PNES and ES groups. PMID:23103308
Salinsky, Martin; Evrard, Collette; Storzbach, Daniel; Pugh, Mary Jo
The usual differential diagnoses of nocturnal events in children include parasomnias, nocturnal seizures, nocturnal reflux (Sandifer syndrome), hypnic jerks, periodic limb movements of sleep, and sleep disordered breathing. We report a previously healthy young girl who presented to the sleep clinic for evaluation of nocturnal events which were diagnosed as medically refractory nocturnal seizures. It was not until a syncopal event occurred in the daytime, which prompted referral for cardiac evaluation, the diagnosis of idiopathic pulmonary arterial hyper-tension (IPAH) was made. Sleep physicians should consider IPAH in the differential diagnosis of nocturnal events in children. Citation: Izzo A; McSweeney J; Kulik T; Khatwa U; Kothare SV. “Nocturnal seizures” in idiopathic pulmonary arterial hypertension. J Clin Sleep Med 2013;9(10):1091-1092.
Izzo, Anthony; McSweeney, Julia; Kulik, Thomas; Khatwa, Umakanth; Kothare, Sanjeev V.
An atypical presentation of diabetes mellitus was described in black subjects, initially in adolescents by Winter et al. then, in adult populations. The principal characteristics of "African" diabetes are an acute onset with severe hyperglycemia and ketosis, and a clinical course of type 2 diabetes mellitus. In the subsequent clinical course after initiation of insulin therapy, prolonged remission is often possible with cessation of insulin therapy and maintenance of appropriate metabolic control. In the subsequent clinical course after initiation of insulin therapy, prolonged remission is often possible with cessation of insulin therapy and maintenance of appropriate metabolic control. The molecular mechanisms underlining the insulin secretory dysfunction are still to be understood and may involve glucolipotoxicity processes. The HLA alleles associated with susceptibility to type 1 diabetes were reported of high frequency in some populations with this form of diabetes, in the absence of makers of pancreatic beta cell autoimmunity. The aim of the present review is to discuss two cases of African diabetes and review the specific diagnostic, metabolic, pathogenic and management features of this atypical diabetes. PMID:17692810
Belhadi, L; Chadli, A; Bennis, L; Ghomari, H; Farouqi, A
Background. A few studies have found somatosensory abnormalities in atypical odontalgia (AO) patients. The aim of the study is to explore the presence of specific abnormalities in facial pain patients that can be considered as psychophysical factors predisposing to AO. Materials and Methods. The AO subjects (n = 18) have been compared to pain-free (n = 14), trigeminal neuralgia (n = 16), migraine (n = 17), and temporomandibular disorder (n = 14). The neurometer current perception threshold (CPT) was used to investigate somatosensory perception. Structured clinical interviews based on the DSM-IV axis I and DSM III-R axis II criteria for psychiatric disorders and self-assessment questionnaires were used to evaluate psychopathology and aggressive behavior among subjects. Results. Subjects with AO showed a lower A?, A?, and C trigeminal fiber pain perception threshold when compared to a pain-free control group. Resentment was determined to be inversely related to A? (rho: 0.62, P < 0.05), A? (rho: 0.53, P < 0.05) and C fibers (rho: 0.54, P < 0.05), and depression was inversely related with C fiber (rho: 0.52, P < 0.05) perception threshold only in AO subjects. Conclusion. High levels of depression and resentment can be considered predictive psychophysical factors for the development of AO after dental extraction.
Ciaramella, A.; Paroli, M.; Lonia, L.; Bosco, M.; Poli, P.
Schizophrenia and other psychotic disorders are treated principally by a class of medications called anti-psychotic drugs. These medications act principally by blocking the stimulation of dopamine receptors by the neurotransmitter dopamine. There are two main classes of anti-psychotic drugs. One is called the first generation, or typical, or conventional anti-psychotic drugs, and the second is called the second generation or atypical anti-psychotic drugs. The difference between these two, even though they both have similar properties and act as anti-psychotics, is that the newer medicines do not produce the disabling neurologic side-effects like stiffness, and slowness and tremor that the first generation drugs produced. In addition, they have a much lower affinity for dopamine receptors so they're kinder, gentler pharmacological agents as opposed to the very potent high-affinity dopamine receptor antagonist that the first generations are. And then thirdly, they may be a little better in some ways terms of their therapeutic efficacy. They alleviate symptoms better, they prevent relapse, and they may work on a broader range of the symptoms of schizophrenia.
The aims of this study were to characterize the alterations in total and free carbamazepine (CBZ) and in total and free carbamazepine-epoxide (CBZ-EPO) clearances during pregnancy, to calculate the change in free fractions of CBZ and CBZ-EPO during pregnancy, and to determine whether seizure worsening is associated with a low ratio to nonpregnant baseline concentration of total or free CBZ or CBZ-EPO. Women on CBZ were enrolled before conception or during pregnancy in this prospective, observational study. Concomitant medications and seizure frequency were recorded. Serum total and free CBZ and CBZ-EPO were collected at each visit. Changes in the clearance of all four compounds and free fractions of CBZ and CBZ-EPO were compared with nonpregnant baseline. During pregnancy, the ratios to baseline concentrations of total and free CBZ and CBZ-EPO were compared for months with and without increased seizure frequency. Total and free CBZ and CBZ-EPO clearances were calculated in 15 pregnancies in 12 women. Clearances did not change for any of these compounds during pregnancy. The free fraction of CBZ increased from 0.23 at baseline to a maximum of 0.32 in the third trimester (p=0.008). In the six women on CBZ monotherapy with adequate seizure diaries and blood sampling, seizure worsening did not correspond to a ratio to baseline concentration of less than 0.65 for total or free CBZ or CBZ-EPO. In conclusion, total and free CBZ and CBZ-EPO clearances did not change substantially during pregnancy, and seizure frequency worsening was not associated with decreased concentrations of total or free CBZ; therefore, therapeutic drug monitoring may not be necessary for all women on CBZ during pregnancy. Further studies with larger sample sizes are needed before definitive recommendations can be made. Carbamazepine monotherapy may be a relatively safe and cost effective treatment option for women with focal epilepsy syndromes during pregnancy. PMID:24632353
Johnson, Emily L; Stowe, Zachary N; Ritchie, James C; Newport, D Jeffrey; Newman, Melanee L; Knight, Bettina; Pennell, Page B
Methylmalonic acidemias are inherited metabolic disorders characterized by methylmalonate (MMA) accumulation and neurological dysfunction, including seizures. Dietary fatty acids are known as an important energy source and reduce seizure activity in selected acute animal models. This study investigated whether chronic treatment with fish oil or with oleic acid attenuates MMA-induced seizures and whether maintenance of Na(+),K(+)-ATPase activity was involved in such an effect. Adult male Wistar rats were given fish oil (85 mg/kg), oleic acid (85 mg/kg) or vehicle (0.42% aqueous Cremophor EL™, 4 mL/kg/body weight/day), p.o., for 75 days. On the 73th day a cannula was implanted in the right lateral ventricle with electrodes over the parietal cortex for EEG recording. On the 76th day the animals were injected with NaCl (2.5 ?mol/2.5 ?L, i.c.v.), or with MMA (2.5 ?mol/2.5 ?L, i.c.v.), and seizure activity was measured by electroencephagraphic (EEG) recording with concomitant behavior monitoring. The effect of prostaglandin E2 (PGE2) on Na(+),K(+)-ATPase activity of slices of cerebral cortex from NaCl-injected animals was determined. Fish oil increased the latency to MMA-induced tonic-clonic seizures, reduced the mean amplitude of ictal EEG recordings, and prevented PGE2-induced decrease of Na(+),K(+)-ATPase activity in cortical slices in vitro. Oleic acid decreased mean amplitude of ictal EEG recordings. The results support that fish oil decreases MMA-induced seizures. The decreased sensitivity of Na(+),K(+)-ATPase to the inhibitory effect of PGE2 in fish oil-treated animals may be related to the currently reported anticonvulsant activity. PMID:23375884
Banderó, Cristina Ruedell Reschke; Salvadori, Mirian G S S; Gomes, Anajara Teixeira; Dal Ri, Nadja M K; Furian, Ana Flávia; Oliveira, Mauro Schneider; Rambo, Leonardo Magno; Scorza, Fulvio A; Cysneiros, Roberta M; Emanuelli, Tatiana; Mello, Carlos Fernando
The process by which the brain transitions into an epileptic seizure is unknown. In this study, we investigated whether the transition to seizure is associated with changes in brain dynamics detectable in the wideband EEG, and whether differences exist across underlying pathologies. Depth electrode ictal EEG recordings from 40 consecutive patients with pharmacoresistant lesional focal epilepsy were low-pass filtered at 500 Hz and sampled at 2,000 Hz. Predefined EEG sections were selected immediately before (immediate preictal), and 30 seconds before the earliest EEG sign suggestive of seizure activity (baseline). Spectral analysis, visual inspection and discrete wavelet transform were used to detect standard (delta, theta, alpha, beta and gamma) and high-frequency bands (ripples and fast ripples). At the group level, each EEG frequency band activity increased significantly from baseline to the immediate preictal section, mostly in a progressive manner and independently of any modification in the state of vigilance. Preictal increases in each frequency band activity were widespread, being observed in the seizure-onset zone and lesional tissue, as well as in remote regions. These changes occurred in all the investigated pathologies (mesial temporal atrophy/sclerosis, local/regional cortical atrophy, and malformations of cortical development), but were more pronounced in mesial temporal atrophy/sclerosis. Our findings indicate that a brain state change with distinctive features, in the form of unidirectional changes across the entire EEG bandwidth, occurs immediately prior to seizure onset. We postulate that these changes might reflect a facilitating state of the brain which enables a susceptible region to generate seizures.
Perucca, Piero; Dubeau, Francois; Gotman, Jean
When dealing with seizure detection/prediction problems, there are three main performance metrics that must be optimized: false positive rate, false negative rate, detection delay or, if the problem is seizure prediction, it is desirable to obtain the gre...
R. Esteller J. Echauz M. D. Alessandro G. Vachtsevanos B. Litt
Results of the studies indicate that acute administration of Delta tetrahydrocannabinol(THC), in sufficiently high doses, will abolish spontaneous seizures in the sensitive strain of gerbils. The dose required to prevent the seizures gave rise to a marked...
B. Cox M. ten Ham W. J. Loskota P. Lomax
Midazolam (Versed), the first water-soluble benzodiazepine, has had widespread acceptance as a parenteral anxiolitic agent. Its antiepileptic properties were studied in adult patients with good results. Midazolam was administered intramuscularly to 48 children, ages 4 months to 14 years, with 69 epileptic episodes of various types. In all but 5 epileptic episodes, seizures stopped 1-10 min after injection. These results suggest that midazolam administered intramuscularly may be useful in a variety of epileptic seizures during childhood, specifically when attempts to introduce an intravenous line in convulsing children are unsuccessful. PMID:1622519
Lahat, E; Aladjem, M; Eshel, G; Bistritzer, T; Katz, Y
A 35-year-old, 50-kg female with a history of epilepsy was scheduled for elective breast surgery (fibroadenoma) under general anaesthesia. She was given glycopyrrolate 0.2 mg, ondansetron 4 mg and tramadol 100 mg i.v. as premedication. Within 5 min, she had an acute episode of generalised tonic-clonic seizure that was successfully treated with 75 mg thiopentone i.v. and after 30 min, she was given general anaesthesia with endotracheal intubation. Surgery, intra-operative period, extubation and post-operative period were uneventful. We conclude that tramadol may provoke seizures in patients with epilepsy even within the recommended dose range. PMID:22529421
Raiger, Lalit Kumar; Naithani, Udita; Bhatia, Sonali; Chauhan, Sandeep Singh
Lung carcinomas have a high propensity to metastasize to the liver, adrenal, bone or brain. However, certain atypical intracranial sites may occur. We report two cases of adenocarcinoma of the lung discovered by peculiar brain metastases. The first patient presented with an orbital apex syndrome due to a metastasis to the cavernous sinus with intraorbital extension. The second patient presented with headache and visual loss due to metastasis to the pituitary. Intrasellar and parasellar metastases are rare sequelae of pulmonary neoplasms. Etiological diagnosis is based on imaging and sampling or biopsy. They are often associated with a poor prognosis. The differential diagnosis of an intracranial process in the area of the sella should include metastasis even in the absence of a known primary cancer. PMID:23083771
Lachhab, L; Fikri, M; Aitbenhaddou, E H; Arkha, Y; Regragui, W; Jiddane, M; Benomar, A; Yahyaoui, M
We report a case of a 42-year-old male who developed generalized tonic-clonic seizure with sudden, brief decrease in bispectral index (BIS) value while undergoing emergency kidney transplantation. Few reports have been made on intraoperative pitfall of BIS value associated with seizure. This case report suggests seizure should be taken into account as a reason for such brief fall of BIS, especially while under general anesthesia or in other specific cases in which clinical signs of seizure are unseen.
Summary Acute isolated seizure, repetitive or recurrent seizures, and status epilepticus are all deemed medical emergencies. Mortality\\u000a and worse neurologic outcome are directly associated with the duration of seizure activity. A number of recent reviews have\\u000a described consensus statements regarding the pharmacologic treatment protocols for seizures when patients are in pre-hospital,\\u000a institutional, and home-bound settings. Benzodiazepines, such as lorazepam, diazepam, midazolam,
Daniel P. Wermeling
Seizures have both local and remote effects on nervous system function. While propagated seizures are known to disrupt cerebral activity, little work has been done on remote network effects of seizures that do not propagate. Human focal temporal lobe seizures demonstrate remote changes including slow waves on electroencephalography (EEG) and decreased cerebral blood flow (CBF) in the neocortex. Ictal neocortical slow waves have been interpreted as seizure propagation, however we hypothesize that they reflect a depressed cortical state resembling sleep or coma. To investigate this hypothesis, we performed multi-modal studies of partial and secondarily-generalized limbic seizures in rats. Video/EEG monitoring of spontaneous seizures revealed slow waves in the frontal cortex during behaviorally mild partial seizures, contrasted with fast poly-spike activity during convulsive generalized seizures. Seizures induced by hippocampal stimulation produced a similar pattern, and were used to perform functional magnetic resonance imaging (fMRI) weighted for blood oxygenation (BOLD) and blood volume (CBV), demonstrating increased signals in hippocampus, thalamus and septum, but decreases in orbitofrontal, cingulate, and retrosplenial cortex during partial seizures; and increases in all these regions during propagated seizures. Combining these results with neuronal recordings and CBF measurements, we related neocortical slow waves to reduced neuronal activity and cerebral metabolism during partial seizures, but found increased neuronal activity and metabolism during propagated seizures. These findings suggest that ictal neocortical slow waves represent an altered cortical state of depressed function, not propagated seizure activity. This remote effect of partial seizures may cause impaired cerebral functions, including loss of consciousness.
Englot, Dario J.; Mishra, Asht M.; Mansuripur, Peter K.; Herman, Peter; Hyder, Fahmeed; Blumenfeld, Hal
\\u000a Fulminant hepatic failure and other causes of multiorgan dysfunction can be associated with seizures. These seizures can be\\u000a convulsive or nonconvulsive, and may significantly affect the pathobiology of the patient’s critical condition. The use of\\u000a continuous electroencephalography has become very important in the identification and treatment of seizures in critically\\u000a ill patients with hepatic or other metabolic disorders. Seizures arise
Andrew Beaumont; Paul M. Vespa
All open wounds are primarily contaminated and subsequently colonized by microorganisms, predominantly bacteria. Only about 30% of chronic wounds are also infected. Factors which favor the development of infection are the following: large quantity of bacteria, presence of virulence factors, their quantity and number, predominantly the synergy of aerobic and anaerobic bacteria, and formation of biofilm. Common agents of infection of acute and chronic wounds are Staphylococcus aureus, MRSA, Streptococcus beta-haemolyticus, Pseudomonas aeruginosa, Bacteroides spp., and Candida albicans. Difference between acute and chronic wound is in the predominance of individual agents, with an observation that Staphylococcus aureus is predominant in both cases. Atypical agents of chronic wound infection are rare, unusual, not found in the area in which we live, not proven by standard microbiological methods, but molecular methods are needed instead. They are predominantly opportunists, varying in the expression of virulence factors, or they have changed their phenotype characteristics and are not the agents of primary wound infections. They are the agents of secondary infections. Atypical agents of the chronic wound infection are diverse, from the anaerobe group, Peptoniphilus spp., Anaerococcus spp., Bacteroides ureolyticus, Finegoldia magma, the group of gram positive rods of the Corynebacterium genus, the group of bacteria from aquatic environment Mycobacterium fortuitum complex, and Vibrio alginolyticus. The targeted samples are biopsy sample as the "gold standard" and/or aspirate, when a significant quantity of exudate is present. Targeted samples are obligatory when there is a progression and decomposition of the base of the wound, increase in the size or depth of the wound, isolation of multiresistant microbes, or absence of clinical response to empirical antimicrobial therapy. In the diagnosis of opportunistic pathogens or atypical agents of chronic wound infection, it is necessary to be thorough, meticulous, and conduct revision of the patient, diagnosis, treatment and samples. Crucial for the detection of the agents atypical wound infection is discussion and agreement with clinical microbiologist. Essential for the quality diagnosis is decontamination of the wound before taking targeted samples. The targeted sample is biopsy specimen as the "gold standard", less frequently aspirate, depending on the quantity and content of the wound. Swab as a sample is not recommended. If there is no other choice, only exceptionally surface swabs may be taken, but only under the following conditions: decontamination of the wound with the application of Levine's or Z-technique of taking of swabs. PMID:23193825
Focal, secondarily generalizing epileptic seizures were released by magnetic stimulation in a patient with focal epilepsy. The stimulation induced seizures had a similar clinical appearance to the patient's spontaneous seizures. They were released exclusively by an angulated “figure-of-8” coil which stimulates the brain more focally as compared to the commonly used flat round coil. The epileptic focus could be located
J. Classen; O. W. Witte; G. Schlaug; R. J. Seitz; H. Holthausen; R. Benecke
Alterations of consciousness are critical factors in the diagnosis of epileptic seizures. With these alterations in consciousness, some persons report sensations of separating from the physical body, experiences that may in rare cases resemble spontaneous out-of-body experiences. This study was designed to identify and characterize these out-of-body-like subjective experiences associated with seizure activity. Fifty-five percent of the patients in this study recalled some subjective experience in association with their seizures. Among our sample of 100 patients, 7 reported out-of-body experiences associated with their seizures. We found no differentiating traits that were associated with patients' reports of out-of-body experiences, in terms of either demographics; medical history, including age of onset and duration of seizure disorder, and seizure frequency; seizure characteristics, including localization, lateralization, etiology, and type of seizure, and epilepsy syndrome; or ability to recall any subjective experiences associated with their seizures. Reporting out-of-body experiences in association with seizures did not affect epilepsy-related quality of life. It should be noted that even in those patients who report out-of-body experiences, such sensations are extremely rare events that do not occur routinely with their seizures. Most patients who reported out-of-body experiences described one or two experiences that occurred an indeterminate number of years ago, which precludes the possibility of associating the experience with the particular characteristics of that one seizure or with medications taken or other conditions at the time. PMID:24592228
Greyson, Bruce; Fountain, Nathan B; Derr, Lori L; Broshek, Donna K
Autoimmunity has aroused interest in the last years as a contributory mechanism of epilepsy, especially in epilepsies with unknown cause or therapy resistance. Since the relationship of absence epilepsy (AE) with calcium channels is well established, we aimed to investigate related antibodies in patients diagnosed with AE. Consecutive patients with typical absence seizures having either childhood absence epilepsy (CAE) or juvenile absence epilepsy (JAE) with generalized spike and wave discharges on electroencephalography (EEG) were included after their consent. The patients were diagnosed according to the International League Against Epilepsy (ILAE) 2010 criteria. Antibodies against P-Q type voltage gated calcium channels (VGCC) and T-type VGCC subunit Cav3.2 (encoded by the CACNA1H gene) were investigated by RIA and ELISA, respectively. We searched for these antibodies in 32 patients with AE and 53 patients with focal epilepsy of unknown cause (FEOUC) as the disease control group; furthermore, 30 healthy persons served as the healthy controls. Eleven patients (34.3%) with AE had CAE and the remaining patients had JAE. Only a 47-year-old female FEOUC patient, who also had systemic lupus erythematosus with normal MRI scans showed antibodies against P-Q type VGCC, whereas no antibody positivity could be found in other FEOUC and AE patients and healthy controls. Our results might suggest that calcium channel antibodies do not play an important role in the pathophysiology of AE. Further studies with larger groups of other epileptic syndromes are needed to confirm our results. PMID:24147594
Tektürk, P?nar; Baykan, Betül; Ekizo?lu, Esme; Ulusoy, Canan; Aydin-Özemir, Zeynep; Içöz, Sema; K?nay, Demet; Tüzün, Erdem
Automated detection of seizures is still a challenging problem. This study presents an approach to detect seizure segments in Laplacian electroencephalography (tEEG) recorded from rats using the tripolar concentric ring electrode (TCRE) configuration. Three features, namely, median absolute deviation, approximate entropy, and maximum singular value were calculated and used as inputs into two different classifiers: support vector machines and adaptive boosting. The relative performance of the extracted features on TCRE tEEG was examined. Results are obtained with an overall accuracy between 84.81 and 96.51%. In addition to using TCRE tEEG data, the seizure detection algorithm was also applied to the recorded EEG signals from Andrzejak et al. database to show the efficiency of the proposed method for seizure detection.
Feltane, Amal; Boudreaux-Bartels, G. Faye; Besio, Walter
Of all partial seizures, those of frontal lobe origin (FLPS) are most bizarre and are often mistaken for psychogenic seizures (PS). The reverse can also be true. To clarify the confusing clinical presentation of these different seizure types, we compared the clinical ictal characteristics of 63 FLPS in 11 patients with 29 PS in 12 patients. Patients with PS had significantly later age at onset and longer ictal duration. There was no statistically significant difference between the two groups with respect to history of psychiatric disorder, ictal pelvic thrusting, rocking of body, side-to-side head movements, or rapid postictal recovery, all of which previously have been reported as characteristic features of PS. Turning to a prone position during the seizure occurred only in FLPS. Nocturnal occurrence, short ictal duration, younger age at onset, stereotyped patterns of movements, and MRI and EEG abnormality suggested FLPS. PMID:1620332
Saygi, S; Katz, A; Marks, D A; Spencer, S S
Kepler photometry shows that most A-type stars have low frequency variations which can be understood in terms of rotational modulation. Indeed, the distribution of equatorial velocities derived from the photometric periods agrees with the distribution of equatorial velocities of A-type stars in the general field. The amplitude of the rotational frequency varies by 20-30 percent as might be expected of star spots. From the light amplitudes we estimate that most spots are considerably larger than typical sunspots but generally smaller than the largest sunspots. The rotation peaks in the periodograms of a significant fraction of A-type stars have a peculiar structure which is not understood. Although peaks corresponding to the rotation frequency can be identified in many ? Scuti stars, the low frequency peaks in these stars are too numerous to be caused by rotational modulation. It thus appears that while the variability of non-pulsating A stars can be explained by rotation, the low-frequency variability in A-type ? Sct stars requires a new pulsation mechanism. We also find several ? Dor stars much hotter than the theoretical hot edge of the instability strip. We find 13 new A-type flare stars, which means that about 1.5 percent of A stars flare. Less dramatic flares may be common in all A-type stars. We show that these superflares cannot be attributed to normal flares on a cool companion. We conclude that A-type stars are active and, like cooler stars, have starspots and flares. Surprisingly, there does not seem to be a drop in activity as the granulation boundary is crossed.
Balona, L. A.
Although functional imaging studies described networks associated with generalized epileptic activity, propagation patterns within these networks are not clear. In this study, electroencephalogram (EEG)-based coherent source imaging dynamic imaging of coherent sources (DICS) was applied to different types of generalized epileptiform discharges, namely absence seizures (10 patients) and photoparoxysmal responses (PPR) (eight patients) to describe the representation and propagation of these discharges in the brain. The results of electrical source imaging were compared to EEG-functional magnetic resonance imaging (fMRI) which had been obtained from the same data sets of simultaneous EEG and fMRI recordings. Similar networks were described by DICS and fMRI: (1) absence seizures were associated with thalamic involvement in all patients. Concordant results were also found for brain areas of the default mode network and the occipital cortex. (2) Both DICS and fMRI identified the occipital, parietal, and the frontal cortex in a network associated with PPR. (3) However, only when PPR preceded a generalized tonic-clonic seizure, the thalamus was involved in the generation of PPR as shown by both imaging techniques. Partial directed coherence suggested that during absences, the thalamus acts as a pacemaker while PPR could be explained by a cortical propagation from the occipital cortex via the parietal cortex to the frontal cortex. In conclusion, the electrical source imaging is not only able to describe similar neuronal networks as revealed by fMRI, including deep sources of neuronal activity such as the thalamus, but also demonstrates interactions interactions within these networks and sheds light on pathogenetic mechanisms of absence seizures and PPR. PMID:22431268
Moeller, Friederike; Muthuraman, Muthuraman; Stephani, Ulrich; Deuschl, Günther; Raethjen, Jan; Siniatchkin, Michael
Two patients with eyelid myoclonia with absences (EMA) are described. Videotape of the eyelid myoclonia in one patient is presented. An interesting feature in one patient was the induction of clinical seizures only with daylight, and in another the presence of rare, focal, epileptiform discharges during drowsiness. Valproic acid only partially controlled eyelid myoclonia in both cases. Lamotrigine, alone or in combination with valproate, can be used as an alternative but was ineffective in our cases. [Published with video sequences]. PMID:15634626
Burneo, J G; Miller, S; Bebin, E M; Prasad, A
Generalized epilepsy with febrile seizures plus (GEFS+) is an early onset febrile epileptic syndrome with therapeutic responsive (a)febrile seizures continuing later in life. Dravet syndrome (DS) or severe myoclonic epilepsy of infancy has a complex phenotype including febrile generalized or hemiclonic convulsions before the age of 1, followed by intractable myoclonic, complex partial, or absence seizures. Both diseases can result from mutations in the Nav1.1 sodium channel, and initially, seizures are typically triggered by fever. We previously characterized two Nav1.1 mutants—R859H (GEFS+) and R865G (DS)—at room temperature and reported a mixture of biophysical gating defects that could not easily predict the phenotype presentation as either GEFS+ or DS. In this study, we extend the characterization of Nav1.1 wild-type, R859H, and R865G channels to physiological (37°C) and febrile (40°C) temperatures. At physiological temperature, a variety of biophysical defects were detected in both mutants, including a hyperpolarized shift in the voltage dependence of activation and a delayed recovery from fast and slow inactivation. Interestingly, at 40°C we also detected additional gating defects for both R859H and R865G mutants. The GEFS+ mutant R859H showed a loss of function in the voltage dependence of inactivation and an increased channel use-dependency at 40°C with no reduction in peak current density. The DS mutant R865G exhibited reduced peak sodium currents, enhanced entry into slow inactivation, and increased use-dependency at 40°C. Our results suggest that fever-induced temperatures exacerbate the gating defects of R859H or R865G mutants and may predispose mutation carriers to febrile seizures.
Kahlig, Kristopher M.; Das, Joost H.G.; van Kempen, Marjan J.A.; Lindhout, Dick; Koeleman, Bobby P.C.; Rook, Martin B.
... neurons) in the brain, where they transport potassium ions out of cells. These channels transmit a particular type of electrical signal called ... 4 months. It has been suggested that potassium channels formed from the KCNQ2 and KCNQ3 proteins play a ... neuron ; potassium ; seizure ; symptom You may ...
Neonatal seizures are a potentially life-threatening pediatric problem with a variety of causes, such as birth trauma, asphyxia, congenital anomalies, metabolic disturbances, infections, and drug withdrawal or intoxication. Thorough and timely evaluations of such patients are necessary to identify and treat the underlying etiology, therefore reducing potential morbidity and mortality. We review neonatal seizures and hypocalcemia and present the case of a 6-day-old male infant who presented to a tertiary pediatric emergency department with seizure-like episodes. He was found to have markedly low serum calcium, magnesium, and parathyroid hormone concentrations, as well as a significantly elevated serum phosphate concentration. The etiology of these abnormalities was found to be maternal ingestion of extremely high doses of calcium carbonate during the third trimester of her pregnancy, an occurrence that has been reported only once in the literature. Education pertaining to the dangers of excessive calcium carbonate intake during pregnancy may be an important piece of anticipatory guidance for pregnant mothers with symptoms of gastroesophageal reflux, and questioning the mother of a neonate presenting with seizures about such over-the-counter medications may help to elucidate the diagnosis. PMID:24084610
Thornton, Matthew D; Chen, Lei; Langhan, Melissa L
Introduction: Occurrence of generalized tonic-clonic seizures (GTCS) is one of the most important risk factors of seizure-related complications and comorbidities in patients with epilepsy. Their prevention is therefore an important aspect of therapeutic management both in idiopathic generalized epilepsies and in focal epilepsies. Areas covered: It has been shown that the efficacy of antiepileptic drugs (AEDs) varies across epilepsy syndromes, with some AEDs efficacious against focal seizures with secondary GTCS (sGTCS) but aggravating primary GTCS (pGTCS). In patients with pGTCS, evidence-based data support the preferential use of valproic acid, lamotrigine, levetiracetam and topiramate. In patients with sGTCS, all AEDs approved in the treatment of focal epilepsies might be used. Expert opinion: Both in pGTCS and sGTCS, additional data are required, specifically to inform about the relative efficacy of AEDs in relation to each other. Although valproic acid might be the most efficacious drug in idiopathic generalized epilepsies, it should be avoided in women of childbearing age due to its safety profile. In patients with sGTCS, AEDs for which the impact on this seizure type has been formally evaluated and which have demonstrated greater efficacy than placebo might preferentially be used, such as lacosamide, perampanel and topiramate. PMID:24798217
Rheims, Sylvain; Ryvlin, Philippe
As many as 325,000 school-age children, ages 5-14, have epilepsy in the U.S. Thankfully, with medication, surgery, a special diet or vagus nerve stimulation, most go to school and fully participate in school activities. Children who continue to have seizures, however, may run into problems. Many of these problems can be overcome or prevented…
Fever can precipitate ventricular tachycardia in adults with Brugada syndrome, but such a link has not been reported in children. A 21-month-old white girl presented repeatedly with decreased conscious level and seizures during fever. During a typical episode, rapid ventricular tachycardia was documented. The resting 12-lead electrocardiogram revealed a Brugada electrocardiogram signature. Resting electrocardiograms of the asymptomatic brother and mother
Jonathan Robert Skinner; Seo-Kyung Chung; Carey-Anne Nel; Andrew Neil Shelling; Jackie Robyn Crawford; Neil McKenzie; Ralph Pinnock; John Kerswell French; Mark Ian Rees
The pharmacokinetics of levetiracetam were determined prospectively in 18 neonates with seizures. Neonates were found to have lower clearance, higher volume of distribution, and a longer half-life as compared with older children and adults. Mild somnolence was the only adverse effect.
Merhar, Stephanie L.; Schibler, Kurt R.; Sherwin, Catherine M.; Meinzen-Derr, Jareen; Shi, Jing; Balmakund, Tonya; Vinks, Alexander A.
Background: Atomoxetine (Strattera™), has recently been approved for the treatment of Attention Deficit Hyperactivity Disorder (ADHD) in adolescents and adults. Atomoxetine acts by inhibiting the reuptake of norepinephrine. There are limited reports of the effects of atomoxetine in overdose. We report a case of isolated atomoxetine overdose resulting in seizure and mild cardiac toxicity. Case Report: A 17-year-old female ingested
John Kashani; Anne-Michelle Ruha
We utilized a novel ratiometric nanoquantum dot fluorescence resonance energy transfer (NQD-FRET) optical sensor to quantitatively measure oxygen dynamics from single cell microdomains during hypoxic episodes as well as during 4-aminopyridine (4-AP)-induced spontaneous seizure-like events in rat hippocampal slices. Coupling oxygen sensing with electrical recordings, we found the greatest reduction in the O2 concentration ([O2]) in the densely packed cell body stratum (st.) pyramidale layer of the CA1 and differential layer-specific O2 dynamics between the st. pyramidale and st. oriens layers. These hypoxic decrements occurred up to several seconds before seizure onset could be electrically measured extracellularly. Without 4-AP, we quantified a narrow range of [O2], similar to the endogenous hypoxia found before epileptiform activity, which permits a quiescent network to enter into a seizure-like state. We demonstrated layer-specific patterns of O2 utilization accompanying layer-specific neuronal interplay in seizure. None of the oxygen overshoot artifacts seen with polarographic measurement techniques were observed. We therefore conclude that endogenously generated hypoxia may be more than just a consequence of increased cellular excitability but an influential and critical factor for orchestrating network dynamics associated with epileptiform activity. PMID:24598521
Ingram, Justin; Zhang, Chunfeng; Cressman, John R; Hazra, Anupam; Wei, Yina; Koo, Yong-Eun; Ziburkus, Jok?bas; Kopelman, Raoul; Xu, Jian; Schiff, Steven J
The 15-month study aimed at validating the epileptic seizure warning system designed by McDonnell Douglas Astronautics Corporation found it to be reasonably efficient in a test population of 13. Besides the history and description of the study, technical ...
S. S. Viglione
The treatment of neonatal seizures has not changed significantly over the last 50 years despite advances in antiepileptic drug (AED) development for older children and adults. Recently new drugs have emerged some of which address age-specific challenges or mechanisms and will be discussed in this review. The loop diuretic bumetanide blocks the neuronal NKCC1 co-transporter and is thought specifically to supress seizures in the immature brain. Levetiracetam has been used in children and infants with good efficacy, an excellent safety profile, and near-ideal pharmacokinetic characteristics. Randomised controlled trials are now underway to test the efficacy of some newer AEDs for neonatal seizures. Topiramate has been shown to have neuroprotective properties in addition to its antiepileptic action and trials in babies with hypoxic-ischaemic encephalopathy are now planned. There is an urgent need to develop age-specific AEDs for preterm and term babies. These drugs must be evaluated with multicentre, collaborative trials using innovative methods and high ethical standards to overcome age-specific challenges with the ultimate aim of improving the outcome for neonates with seizures. PMID:23688938
Pressler, Ronit M; Mangum, B
The syndrome of malignant migrating partial seizures in infancy (MMPSI) was first reported in 1995, and is now included among the childhood epileptic syndromes in the revision proposal of the ILAE Commission on classification and terminology. The main clinical features are seizure onset in the first 6 months of life, occurrence of almost continuous migrating polymorphous focal seizures, associated with multifocal ictal EEG discharges, progressive deterioration of psychomotor development combined with frequent evolution of acquired microcephaly, and lack of a significant familial and etiological context. Eventually, children develop major axial hypotonia, pyramidal and extrapyramidal signs with athetotic movements and strabismus. Neuroradiological, biochemical, and genetic investigations thus far have note contributed to our knowledge about this syndrome. Etiology is still unknown, though it appears reasonable to suspect a genetic etiology for MMPSI; a channelopathy may be responsible for the age-dependent cortical neuronal hyperexcitability. Seizures are markedly drug resistant and outcome is generally severe. However, some patients may respond favourably to bromide, stiripentol associated with clonazepam, and, more recently, to levetiracetam. Vagus nerve stimulation and a ketogenic diet have been tried also but with poor or inconclusive results. Based on age at onset, MMPEI may be placed between early epileptic encephalopthies and infantile spasms. PMID:23622207
Article abstract—Objective: To evaluate the effects of sleep on partial seizures arising from various brain regions. Methods: The authors prospectively studied 133 patients with localization-related epilepsy undergoing video-EEG moni- toring over a 2-year period. Seizure type, site of onset, sleep\\/wake state at onset, duration, and epilepsy syndrome diagnosis were recorded. Periorbital, chin EMG, and scalp\\/sphenoidal electrodes were used. A subset
S. T. Herman; T. S. Walczak; C. W. Bazil
Background: The risk of developing immediate postoperative seizures in patients undergoing supratentorial brain tumor surgery without anti-epileptic drug (AED) prophylaxis is 15-20%. Patients who present with pre-operative seizures and patients with supratentorial meningioma or supratentorial low grade gliomas are at significantly higher risk. There is little data on the efficacy of levetiracetam as a prophylactic AED in the immediate postoperative period (within 7 days of surgery) in these patients. Methods: We conducted a retrospective chart review of 165 adult patients classified as higher risk for postoperative seizures who underwent brain tumor resection at Duke University Hospital between time May 2010 and December 2011. All patients had received levetiracetam monotherapy in doses of 1000-3000 mg/day in the immediate postoperative period. Results: We identified 165 patients with following tumor locations: Frontal 83 (50.3%), Temporal 37 (22.4%), Parietal 30 (18.2%), Occipital 2 (1.2%) and 13 (7.8%) with single lesions involving more than one lobe. Histology revealed: Glioma 98 (59.4%), Meningioma 57 (34.5%) and Brain Metastases 6 (3.6%). Preoperatively, 88/165 (53.3%) patients had presented with seizures. 12/165 patients (7.3%) developed clinical seizures (generalized 10, partial 2) in the immediate post-operative period. Other than somnolence in 7 patients (4.2%), no major side-effects were noted. Conclusions: The incidence of seizures was significantly lower in patients treated with levetiracetam (7.3%) when compared with the expected (15-20%) rate without AED prophylaxis based on the previous literature. Levetiracetam appears effective and safe for seizure prevention in patients undergoing brain tumor resection and who are at significantly higher risk of developing post-operative seizures. These findings warrant confirmation in a prospective randomized trial.
Gokhale, Sankalp; Khan, Shariq Ali; Agrawal, Abhishek; Friedman, Allan H.; McDonagh, David L.
The neurobiological doctrine governing the concept of neurogenesis has undergone a revolution in the past few years. What was once considered dubious is now well accepted: new neurons are born in the adult brain. Science fiction is quickly becoming a reality as scientists discover ways to convert skin, bone, or blood cells into neurons. In the epilepsy arena, widespread interest has developed because of the evidence that neurogenesis increases after seizures, trauma, and other insults or injuries that alter seizure susceptibility. This review discusses some of the initial studies in this field, and their often surprising functional implications. The emphasis will be on the granule cells of hippocampus, because they are perhaps more relevant to epilepsy than other areas in which neurogenesis occurs throughout life, the olfactory bulb and subventricular zone. In particular, the following questions will be addressed:Do granule cells that are born in the adult brain become functional, and what are the limits of their function? Do they behave homogeneously? Results from our own laboratory have focused on cells that become established outside the normal boundaries of the granule cell layer, forming a group of “ectopic” granule cells in the hilar region.Is increased neurogenesis beneficial, or might it actually exacerbate seizures? Evidence is presented that supports the hypothesis that new granule cells may not necessarily act to ameliorate seizures, and might even contribute to them. Furthermore, cognitive deficits following seizures might in part be due to new circuits that develop between new cells and the host brain.How do the new cells interact with the host brain? Several changes occur in the dentate gyrus after seizures, and increased neurogenesis is only one of many. What is the interdependence of this multitude of changes, if any?Is neurogenesis increased after seizures in man? Research suggests that the data from human epileptics are actually inconsistent with the studies in animal models of epilepsy, because there is little evidence of increased neurogenesis in epileptic tissue resected from intractable epileptics. Yet neurogenesis has been shown to occur in humans throughout adult life. What might be the reasons for these seemingly disparate results?
Scharfman, Helen E.
Objectives To define the incidence of and explore risk factors for seizures and epilepsy in children with spontaneous intracerebral hemorrhage (ICH). Design Prospective cohort study. Setting Three tertiary care pediatric hospitals. Participants Seventy-three pediatric subjects with spontaneous ICH including 20 perinatal (?37 weeks gestation to 28 days) and 53 childhood subjects (>28 days to <18 years at presentation). Main outcome measures Acute symptomatic seizures (clinically evident and electrographic-only within 7 days), remote symptomatic seizures, and epilepsy. Results Acute symptomatic seizures occurred in 35 subjects (48%). Acute symptomatic seizures as a presenting symptom of ICH occurred in 12 (60%) perinatal and 19 (36%) childhood subjects, P=.07. Acute symptomatic seizures after presentation occurred in 7 children. Electrographic-only seizures were present in 9/32 (28%) with continuous EEG monitoring. One-and two-year remote symptomatic seizure-free survival were 82% (95% CI 68%–90%) and 67% (95% CI 46%–82%), respectively. One- and two-year epilepsy-free survival were 96% (95% CI 83%–99%) and 87% (95% CI 65%–95%), respectively. Elevated intracranial pressure requiring acute intervention was a risk factor for acute seizures after presentation, remote symptomatic seizures, and epilepsy (P=.014, P=.025 and P=.0365, respectively log-rank test). Conclusions Presenting seizures are common in perinatal and childhood ICH. Continuous EEG may detect electrographic seizures in some subjects. Single remote symptomatic seizures occur in many, and development of epilepsy is estimated to occur in 13% at two-years. Elevated intracranial pressure requiring acute intervention is a risk factor for acute seizures after presentation, remote symptomatic seizures, and epilepsy.
Beslow, Lauren A; Abend, Nicholas S; Gindville, Melissa C; Bastian, Rachel A; Licht, Daniel J; Smith, Sabrina E; Hillis, Argye E.; Ichord, Rebecca N; Jordan, Lori C
Postictal values of prolactin, LH and FSH have been recorded in patients with both generalised tonic-clonic and partial seizures. Elevations of prolactin and LH were seen immediately and at 20 minutes in males and females with generalised attacks. At sixty minutes values for prolactin had fallen to baseline levels, but LH remained elevated. FSH values were increased in females only, at twenty and sixty minutes. Following partial seizures prolactin was elevated, especially with complex partial seizures, at twenty minutes. These results are discussed in the light of known electrophysiological mechanisms relating to partial seizures, and clinical guidelines for the use of neurohormonal tests in the evaluation of seizures are suggested.
Dana-Haeri, J; Trimble, M r; Oxley, J
Some antiepileptic drugs (AEDs) have been reported to aggravate generalized seizures. We have seen three children whose myoclonic seizures increased on starting treatment with Levetiracetam. In all seizures aggravation was temporally associated to the introduction of the drug. All became seizure-free on withdrawal of levetiracetam with a switch to an alternative antiepileptic drug and this persisted for at least 6 months. This suggests that some children with myoclonic seizures may have an aggravation on starting treatment with levetiracetam but this requires further studies. PMID:22990039
Liu, Yong-Hong; Wang, Xiao-Li; Deng, Yan-Chun; Zhao, Gang
Neonatal seizures are inherently different from seizures in the child and the adult. The phenotype, often exhibiting electroclinical dissociation, is unique: neonatal seizures can be refractory to antiepileptic drugs otherwise effect for older patients. Recent experimental and human-based research reveals that the mechanism of neonatal seizures, as well as their long-term sequelae on later brain development, appears to involve a large number of age-specific factors. These observations help explain the resistance of neonatal seizures to conventional therapy as well as identify potential areas of risk for later neurocognitive development. Emerging targets from this research may suggest new therapies for this unique population of patients.
Jensen, Frances E.
The lifespan risk of seizures is highest in the neonatal period. Currently used therapies have limited efficacy. Although the treatment of neonatal seizures has not significantly changed in the last several decades, there has been substantial progress in understanding developmental mechanisms that influence seizure generation and responsiveness to anticonvulsants. Here we provide an overview of current approaches to the diagnosis and treatment of neonatal seizures, identifying some of the recent insights about the pathophysiology of neonatal seizures that may provide the foundation for better treatment.
Jensen, Frances E.
Bithalamic infarctions initially presenting as a convulsive seizure are rarely reported and, to our best knowledge, have never been reported in China. Here, we present a patient with convulsive seizure at the onset of bilateral paramedian thalamic infarction. The diffusion-weighted imaging revealed that the infarct area is supplied by Percheron artery. Associated with the relationship between seizure and centrencephalic system and reticular formation as previously reported, we suggest that seizure could be the onset symptom of paramedian thalamic infarction. Physicians should recognize this condition, because both seizure control and early ischemic stroke management are required. PMID:23762679
Wang, Jianping; Fu, Xiaojie; Jiang, Chao; Liu, Hengfang; Zhao, Yuanzheng; Han, Wei
Bithalamic infarctions initially presenting as a convulsive seizure are rarely reported and, to our best knowledge, have never been reported in China. Here, we present a patient with convulsive seizure at the onset of bilateral paramedian thalamic infarction. The diffusion-weighted imaging revealed that the infarct area is supplied by Percheron artery. Associated with the relationship between seizure and centrencephalic system and reticular formation as previously reported, we suggest that seizure could be the onset symptom of paramedian thalamic infarction. Physicians should recognize this condition, because both seizure control and early ischemic stroke management are required.
Wang, Jianping; Fu, Xiaojie; Jiang, Chao; Liu, Hengfang; Zhao, Yuanzheng; Han, Wei
Posterior reversible encephalopathy syndrome (PRES) is a clinical and radiologic entity with typical symptoms including headache, seizures, visual disturbance, altered mental status, vomiting, nausea and focal neurologic signs. In this article, we report recurrent and atypical PRES in a child with hypertension due to end-stage renal disease (ESRD) who was on a peritoneal dialysis program for 6 months. After the second hypertension attack, PRES findings did not recover and persisted as encephalomalacia. As far as we know, this case is the first child with ESRD who developed encephalomalacia after recurrent episodes of PRES. When a patient with a history of PRES presented with new clinical and neuroradiological findings, recurrent PRES should be considered.
Komur, Mustafa; Delibas, Ali; Arslankoylu, Ali Ertug; Okuyaz, Cetin; Kara, Engin
Stimulation of the vagus nerve has become an effective method for desynchronizing the highly coherent neural activity typically associated with epileptic seizures. This technique has been used in several animal models of seizures as well as in humans suffering from epilepsy. However, application of this technique has been limited to unilateral stimulation of the vagus nerve, typically delivered according to a fixed duty cycle, independently of whether ongoing seizure activity is present. Here, we report that stimulation of another cranial nerve, the trigeminal nerve, can also cause cortical and thalamic desynchronization, resulting in a reduction of seizure activity in awake rats. Furthermore, we demonstrate that providing this stimulation only when seizure activity begins results in more effective and safer seizure reduction per second of stimulation than with previous methods. Seizure activity induced by intraperitoneal injection of pentylenetetrazole was recorded from microwire electrodes in the thalamus and cortex of awake rats while the infraorbital branch of the trigeminal nerve was stimulated via a chronically implanted nerve cuff electrode. Continuous unilateral stimulation of the trigeminal nerve reduced electrographic seizure activity by up to 78%, and bilateral trigeminal stimulation was even more effective. Using a device that automatically detects seizure activity in real time on the basis of multichannel field potential signals, we demonstrated that seizure-triggered stimulation was more effective than the stimulation protocol involving a fixed duty cycle, in terms of the percent seizure reduction per second of stimulation. In contrast to vagus nerve stimulation studies, no substantial cardiovascular side effects were observed by unilateral or bilateral stimulation of the trigeminal nerve. These findings suggest that trigeminal nerve stimulation is safe in awake rats and should be evaluated as a therapy for human seizures. Furthermore, the results demonstrate that seizure-triggered trigeminal nerve stimulation is technically feasible and could be further developed, in conjunction with real-time seizure-predicting paradigms, to prevent seizures and reduce exposure to nerve stimulation. PMID:11050139
Fanselow, E E; Reid, A P; Nicolelis, M A
In this paper numerous alternative treatments in addition to pharmacological therapy are proposed for their use in epileptic patients. Epileptic animal models can play a crucial role in the performance evaluation of new therapeutic techniques. The objective of this research is to first develop various epileptic rat models; second, develop a portable wireless closed-loop seizure controller including on-line seizure detection and real-time electrical stimulation for seizure elimination; and third, apply the developed seizure controller to the animal models to perform on-line seizure elimination. The closed-loop seizure controller was applied to three Long-Evans rats with spontaneous spike-wave discharges (non-convulsive) and three Long-Evans rats with epileptiform activities induced by pentylenetetrazol (PTZ) injection (convulsive) for evaluation. The seizure detection accuracy is greater than 92% (up to 99%), and averaged seizure detection latency is less than 0.6 s for both spontaneous non-convulsive and PTZ-induced convulsive seizures. The average false stimulation rate is 3.1%. Near 30% of PTZ-induced convulsive seizures need more than two times of 0.5 s electrical stimulation for suppression and 90% of the non-convulsive seizures can be suppressed by only one 0.5 s electrical stimulation.
Liang, Sheng-Fu; Liao, Yi-Cheng; Shaw, Fu-Zen; Chang, Da-Wei; Young, Chung-Ping; Chiueh, Herming
The physical behavior of several atypical amphiphilic molecules was studied in various environments including micelles, model bilayer membranes, and emulsions. The molecules under investigation were nor-chenodeoxycholic acid (nor-CDCA), ursodeoxycholic acid (UDCA), sphingosine (Sp), sphingosine hydrochloride (Sp?HCl), and tetrahydrolipstatin (THL). The bile acids, nor-CDCA and UDCA, were studied using 13C-Nuclear Magnetic Resonance ([13C) -NMR) in micelles of taurocholate and in bilayers of phosphatidylcholine. The pK a values of the bile acids in each environment were determined by [13C) -NMR and are as follows: 6.08 ±.03 for nor-CDCA and 6.27 ±.01 for UDCA in micelles, and 7.04 ± 12 for nor-CDCA and 6.89 ±.05 for UDCA in vesicles. Using line shape analysis, the transbilayer movement rate at 36oC for nor-CDCA and UDCA was calculated to be 580 sec--1 and 409 sec-1, respectively. [13C) -NMR titration of Sp gave pK a values of 9.09 ±.02 in micelles and 9.69 ±.21 in bilayers. Differential scanning calorimetry (DSC) and X-ray diffraction were used to establish the Sp?water and Sp?HCl?water phase diagrams. Anhydrous and hydrated samples ranging from 5- 90% water were analyzed. The DSC thermograms traced out the transition temperatures of each molecule while the X- ray diffraction patterns revealed their chain and crystalline lattice packing structures. In general, sphingosine exists as a hydrated crystal with ? packing phase below 43oC and melts into an L? phase. Sphingosine hydrochloride, however, exists as a gel phase (L_beta or /beta/sp') below 42oC that swells to 61% hydration. At low water concentrations (0-64%), a lamellar liquid crystal phase (L_alpha) is formed above the chain melting transition of 42oC. At medium concentration (65%), a Hexagonal I phase is present, and at high water concentrations (66-90%), a micellar phase is present. THL, a specific inhibitor of lipases, was analyzed with [ 13C) -NMR to study its behavior in various environments, ranging from carbon tetrachloride to water to pure triolein. THL was also incorporated into phosphatidylcholine bilayers and into microemulsions of triolein and phosphatidylcholine. [ 13C) -NMR analysis revealed that THL gets incorporated into the surface of vesicles, and into both the surface and core of microemulsion particles.
Epilepsy is more common in patients with multiple sclerosis (MS) than in the general population, occurring in 2-3% of patients. Convulsions may be either tonic–clonic in nature or partial complex. In these individuals, seizures most likely result from lesions present in the cerebral cortex and subcortical white matter. A Jacksonian seizure is a type of simple partial seizure characterized by abnormal movements that begin in one group of muscles and progress to adjacent groups of muscles. We describe a case of Jacksonian seizure as the relapse symptom of MS. Focal motor seizures of this patient have been observed before and presumably marking the clinical onset or during acute bouts of MS. In this case, Jacksonian seizures appear to be the sign of a flare of MS, while the majority of seizures had been reported occur unrelated to MS relapses.
Najafi, Mohammad Reza; Chitsaz, Ahmad; Najafi, Mohammad Amin
Meningiomas, in particular the Atypical (grade 2), vary greatly in their behaviour and prognosis. Over a 19 year period, we operated on 169 meningiomas (on 86 patients) and of those, 9 cases of atypical meningiomas were found which met the 2007 World Health Organization (WHO) classification. The 9 patients represented 5.3% of all meningiomas. The average presenting age was 51 years and average follow-up was 103 months with 5 patients passing away between 38 and 219 months after diagnosis. The time to first recurrence was 24 months with 1 patient suffering 12 recurrences and 2 cases having metastases. Although we had a small number of atypical meningiomas, we believe our paper highlights the unpredictable and difficult nature of these tumours. PMID:22285882
Andric, Marko; Dixit, Shreya; Dubey, Arvind; Jessup, Peter; Hunn, Andrew
To describe early symptomatic and late seizures in a cohort of patients with acute cerebral vein and dural sinus thrombosis (CVDST) and to identify their determinants, we performed a prospective registry and follow-up study of CVDST patients admitted to 20 Portuguese hospitals, from June 1995 to June 1998. Of 91 registered patients, 31 (34%) had early symptomatic seizures; 29 (31.9%) as a presenting feature and 2 (2.1%) after admission. Early symptomatic seizures were more frequent in patients with motor and sensory deficits and in those with focal oedema/ischaemic infarcts or haemorrhages on admission CT/MR. On multivariate logistic regression analysis, sensory defects (OR = 7.8; 95% CI = 0.8-74.8) and a parenchymal lesion on admission CT/MR (OR = 3.7, 95% CI = 1.4-9.4) were found to be significant predictors of early symptomatic seizures. Seizures were directly related to acute death in 2 patients. Eight (9.5%) patients had late seizures, which were multiple in 4 (4.8%). Late seizures were more frequent in patients with early symptomatic seizures and with haemorrhage on admission CT/MR. Neither early symptomatic seizures nor late seizures were related to functional prognosis at the last follow-up (median = 1 year). There is a moderate risk of seizure recurrence early in the course and during the first year after CVDST. Seizures can be a cause of acute death, but might not have an independent influence on functional outcome. Pharmacological prevention of seizures after CVDST should probably be limited to patients with early symptomatic seizures and cerebral lesions on admission CT/MR. PMID:12499715
Ferro, J M; Correia, M; Rosas, M J; Pinto, A N; Neves, G
Background Mesial temporal lobe epilepsy (mTLE) has been suggested to follow a circadian rhythm. Previous research found an afternoon peak in mTLE seizure occurrence. We evaluated the pattern of seizure occurrence in patients with well-localized mTLE and hypothesized that peak seizure frequency would occur in the afternoon, and that this pattern would not be altered by age, gender, or seizure focus. Methods We retrospectively identified consecutive mTLE patients with a seizure-free outcome following anterior temporal lobectomy from 1993-2004 with video-EEG captured seizures. We recorded and plotted the 24-hour clock time for each seizure and performed cosinor analysis. SAS Proc GLIMMIX was used to fit the linearized transform of the cosinor model. Negative binomial regression fitted by the generalized estimating equations (GEE) method was also performed to estimate and compare the mean seizure rates over a 24-hour day. Results Sixty mTLE patients monitored between 2-16 days were analyzed. Mean (standard deviation), median number of seizures per subject were 10.47(7.86), 9.00. Cosinor plots indicated that the function had two modes: 7-8 a.m. and 4-5 p.m. GEE analysis was consistent with peak seizure frequency occurrence at 6-8 a.m. (p<0.0001) and 3-5 p.m. (p<0.01). Conclusions We found a bimodal pattern of seizure occurrence in human mTLE, with peak seizure frequencies occurring between 6-8 a.m. and 3-5 p.m. confirming an afternoon peak, as well as a previously unsuspected morning peak in seizure occurrence that provides rationale for future investigations of antiepileptic drug chronopharmacology and informs patient counseling regarding patterns of seizure occurrence.
Karafin, Matthew; St. Louis, Erik K.; Zimmerman, M. Bridget; Sparks, Jon David; Granner, Mark A.
In 76 patients treated at the Department of Conservative Stomatology, Institute of Stomatology, Medical Academy in Lublin congenital absence of anlages of permanent teeth was found. The patients were aged 8 to 18 years, including 49 girls and 27 boys. In the whole group absence of 214 teeth was noted, i.e. 136 in girls and 78 in boys. In the examinations it was observed that congenital absence of permanent teeth was more frequent in girls than in boys, 2) most frequently only one tooth was absent in a given patient, 3) upper teeth were lacking more frequently than lower teeth, and absence of right teeth was more frequent than left teeth, 4) the greatest group among absent teeth were upper lateral incisors, while second upper molars in the maxilla were least frequently absent. PMID:2104345
Struzak-Wysoki?ska, M; Kami?ska, K; Wysoki?ska-Miszczuk, J
In Ayurveda, Anacyclus pyrethrum has been used as a brain tonic. The present study evaluates the effect of hydroalcoholic extract of A. pyrethrum (HEAP) root against seizures, seizure-induced oxidative stress and cognitive impairment in experimental models of seizures. Male Wistar rats were used in the study. HEAP was administered in doses of 50, 100, 250, 500 in pentylenetetrazole (PTZ) model and 250, 500 and 1000 mg/kg in maximal electroshock (MES) model. Myoclonic jerk latency and generalized tonic clonic seizures (GTCS) were noted in PTZ whereas occurrence of tonic hind limb extension (THLE) was observed in MES seizures. Cognitive deficit was assessed using elevated plus maze and passive avoidance tests. Whole brain reduced glutathione, malondialdehyde levels and cholinesterase activity were measured. HEAP showed 50, 66.7, 83.3 and 100% protection at 50,100, 250 and 500 mg/kg, respectively against GTCS in PTZ induced seizures. In MES induced seizures, HEAP produced 16.7, 33.3 and 50% protection against THLE at 250, 500 and 1000 mg/kg, respectively. HEAP administration significantly prevented seizure induced oxidative stress and cognitive impairment in a dose-dependent manner. HEAP also normalized the decrease in cholinesterase activity caused by seizures. Thus, HEAP showed protective effect against seizures, seizure-induced oxidative stress and cognitive impairment in rats. PMID:21993359
Pahuja, Monika; Mehla, Jogender; Reeta, K H; Joshi, Sujata; Gupta, Yogendra Kumar
1. At least twelve subgroups of atypical Type II pneumococcus may be recognized by specific agglutination reactions. They have been designated Subgroups IIa, IIb, IIc, IId, IIe, IIf, IIg, IIh, IIj, IIk, IIl, and IIm. 2. These subgroups have an incidence of 11 per cent in lobar pneumonia, and of 18 per cent in normal mouths. 3. Certain groups, IIb, IIc, IIf, and IIm, occur in normal mouths. 4. Subgroups IIa and IIh are met with largely in connection with disease. 5. The mortality of acute lobar pneumonia due to these atypical Type II pneumococci is fairly high—32 per cent.
Stillman, Ernest G.
Patient: Female, 60 Final Diagnosis: Cholangiocarcinoma Symptoms: Abdominal pain • abdominal discomfort Medication: — Clinical Procedure: — Specialty: Oncology Objective: Unusual natural history/clinical course Background: Cholangiocarcinoma remains to be a challenging case to diagnose and manage as it usually presents in advanced stage and survival rate remains dismal despite the medical breakthroughs. It is usually classified as intrahepatic, perihilar or distal tumor which can lead to bile duct obstruction causing sluggish flow of bile through the biliary tract and promoting increased absorption of bilirubin, bile acids and bile salts into systemic circulation accounting for the occurrence of jaundice, dark-colored urine and generalized pruritus. It usually becomes symptomatic when the tumor has significantly obstructed the biliary drainage causing painless jaundice and deranged liver function with cholestatic pattern. Jaundice occurs in 90% of the cases when the tumor has obstructed the biliary drainage system. A markedly dilated gallbladder as initial presenting feature in the absence of other typical obstructive clinical manifestations of an advanced stage of the cholangiocarcinoma is rare. Case Report: This case report presents an atypical case of an elderly woman who presented with advanced metastatic ductal cholangiocarcinoma with markedly dilated gallbladder and liver mass without other clinical manifestations and laboratory evidence of cholestatic jaundice. Conclusions: The mere presence of Courvoisier’s sign, even in the absence of other signs of biliary obstruction, could be suggestive of advanced neoplastic process along the biliary tract. Laboratory evidence of cholestasis might lag behind the clinical severity of the biliary obstruction in cholangiocarcinoma.
Salvador, Vincent Bryan; Samrao, Pushkinder; Leytin, Anatoly; Basith, Mohammed
Absence epilepsy (AE) is a common type of genetic generalized epilepsy (GGE), particularly in children. AE and GGE are complex genetic diseases with few causal variants identified to date. Gria4 deficient mice provide a model of AE, one for which the common laboratory inbred strain C3H/HeJ (HeJ) harbors a natural IAP retrotransposon insertion in Gria4 that reduces its expression 8-fold. Between C3H and non-seizing strains such as C57BL/6, genetic modifiers alter disease severity. Even C3H substrains have surprising variation in the duration and incidence of spike-wave discharges (SWD), the characteristic electroencephalographic feature of absence seizures. Here we discovered extensive IAP retrotransposition in the C3H substrain, and identified a HeJ-private IAP in the Pcnxl2 gene, which encodes a putative multi-transmembrane protein of unknown function, resulting in decreased expression. By creating new Pcnxl2 frameshift alleles using TALEN mutagenesis, we show that Pcnxl2 deficiency is responsible for mitigating the seizure phenotype – making Pcnxl2 the first known modifier gene for absence seizures in any species. This finding gave us a handle on genetic complexity between strains, directing us to use another C3H substrain to map additional modifiers including validation of a Chr 15 locus that profoundly affects the severity of SWD episodes. Together these new findings expand our knowledge of how natural variation modulates seizures, and highlights the feasibility of characterizing and validating modifiers in mouse strains and substrains in the post-genome sequence era.
Frankel, Wayne N.; Mahaffey, Connie L.; McGarr, Tracy C.; Beyer, Barbara J.; Letts, Verity A.
Absence epilepsy (AE) is a common type of genetic generalized epilepsy (GGE), particularly in children. AE and GGE are complex genetic diseases with few causal variants identified to date. Gria4 deficient mice provide a model of AE, one for which the common laboratory inbred strain C3H/HeJ (HeJ) harbors a natural IAP retrotransposon insertion in Gria4 that reduces its expression 8-fold. Between C3H and non-seizing strains such as C57BL/6, genetic modifiers alter disease severity. Even C3H substrains have surprising variation in the duration and incidence of spike-wave discharges (SWD), the characteristic electroencephalographic feature of absence seizures. Here we discovered extensive IAP retrotransposition in the C3H substrain, and identified a HeJ-private IAP in the Pcnxl2 gene, which encodes a putative multi-transmembrane protein of unknown function, resulting in decreased expression. By creating new Pcnxl2 frameshift alleles using TALEN mutagenesis, we show that Pcnxl2 deficiency is responsible for mitigating the seizure phenotype - making Pcnxl2 the first known modifier gene for absence seizures in any species. This finding gave us a handle on genetic complexity between strains, directing us to use another C3H substrain to map additional modifiers including validation of a Chr 15 locus that profoundly affects the severity of SWD episodes. Together these new findings expand our knowledge of how natural variation modulates seizures, and highlights the feasibility of characterizing and validating modifiers in mouse strains and substrains in the post-genome sequence era. PMID:25010494
Frankel, Wayne N; Mahaffey, Connie L; McGarr, Tracy C; Beyer, Barbara J; Letts, Verity A
Interictal and ictal fluorodeoxyglucose scans were obtained with positron CT from four patients with spontaneous recurrent partial seizures, one with epilepsia partialis continua, and one with a single partial seizure induced by electrical stimulation of the hippocampus. Ictal metabolic patterns were different for each patient studied. Focal and generalized increased and decreased metabolism were observed. Ictal hypermetabolism may exceed six times the interictal rate and could represent activation of excitatory or inhibitory synapses in the epileptogenic region and its projection fields. Hypometabolism seen on ictal scans most likely reflects postictal depression and may indicate projection fields of inhibited neurons. No quantitative relationship between alterations in metabolism and EEG or behavioral measurements of ictal events could be demonstrated.
Engel, J. Jr.; Kuhl, D.E.; Phelps, M.E.; Rausch, R.; Nuwer, M.
Objective To investigate the feasibility of using noninvasive EEG source imaging approach to image continuous seizure activity in pediatric epilepsy patients. Methods Nine pediatric patients with medically intractable epilepsy were included in this study. Eight of the patients had extratemporal lobe epilepsy and one had temporal lobe epilepsy. All of the patients underwent resective surgery and seven of them underwent intracranial EEG (iEEG) monitoring. The ictal EEG was analyzed using a noninvasive dynamic seizure imaging (DSI) approach. The DSI approach separates scalp EEGs into independent components and extracts the spatio-temporal ictal features to achieve dynamic imaging of seizure sources. Surgical resection and intracranial recordings were used to validate the noninvasive imaging results. Results The DSI determined seizure onset zones (SOZs) in these patients were localized within or in close vicinity to the surgically resected region. In the seven patients with intracranial monitoring, the estimated seizure onset sources were concordant with the seizure onset zones of iEEG. The DSI also localized the multiple foci involved in the later seizure propagation, which were confirmed by the iEEG recordings. Conclusions Dynamic seizure imaging can noninvasively image the seizure activations in pediatric patients with both temporal and extratemporal lobe epilepsy. Significance EEG seizure imaging can potentially be used to noninvasively image the SOZs and aid the pre-surgical planning in pediatric epilepsy patients.
Lu, Yunfeng; Yang, Lin; Worrell, Gregory A.; Brinkmann, Benjamin; Nelson, Cindy; He, Bin
Sudden unexplained death in toddlers has been associated with febrile seizures, family history of febrile seizures, and hippocampal anomalies. We investigated the mode of inheritance for febrile seizures in these families. A three-generation pedigree was obtained from families enrolled in the San Diego Sudden Unexplained Death in Childhood Research Project, involving toddlers with sudden unexplained death, febrile seizures, and family history of febrile seizures. In our six cases, death was unwitnessed and related to sleep. The interval from last witnessed febrile seizure to death ranged from 3 weeks to 6 months. Hippocampal abnormalities were identified in one of three cases with available autopsy sections. Autosomal dominant inheritance of febrile seizures was observed in three families. A fourth demonstrated autosomal dominant inheritance with incomplete penetrance or variable expressivity. In two families, the maternal and paternal sides manifested febrile seizures. In this series, the major pattern of inheritance in toddlers with sudden unexplained death and febrile seizures was autosomal dominant. Future studies should develop markers (including genetic) to identify which patients with febrile seizures are at risk for sudden unexplained death in childhood, and to provide guidance for families and physicians. PMID:22490769
Holm, Ingrid A; Poduri, Annapurna; Crandall, Laura; Haas, Elisabeth; Grafe, Marjorie R; Kinney, Hannah C; Krous, Henry F
Sudden unexplained death in toddlers has been associated with febrile seizures, family history of febrile seizures, and hippocampal anomalies. We investigated the mode of inheritance for febrile seizures in these families. A three-generation pedigree was obtained from families enrolled in the San Diego Sudden Unexplained Death in Childhood Research Project, involving toddlers with sudden unexplained death, febrile seizures, and family history of febrile seizures. In our six cases, death was unwitnessed and related to sleep. The interval from last witnessed febrile seizure to death ranged from 3 weeks to 6 months. Hippocampal abnormalities were identified in one of three cases with available autopsy sections. Autosomal dominant inheritance of febrile seizures was observed in three families. A fourth demonstrated autosomal dominant inheritance with incomplete penetrance or variable expressivity. In two families, the maternal and paternal sides manifested febrile seizures. In this series, the major pattern of inheritance in toddlers with sudden unexplained death and febrile seizures was autosomal dominant. Future studies should develop markers (including genetic) to identify which patients with febrile seizures are at risk for sudden unexplained death in childhood, and to provide guidance for families and physicians.
Holm, Ingrid A.; Poduri, Annapurna; Crandall, Laura; Haas, Elisabeth; Grafe, Marjorie R.; Kinney, Hannah C.; Krous, Henry F.
Diffuse low-grade gliomas are highly epileptogenic brain tumours. We aimed to explore the natural course of epileptic seizures, their predictors and the prognostic significance of their occurrence in adult patients harbouring a diffuse low-grade glioma. An observational retrospective multicentre study examined 1509 patients with diffuse low-grade gliomas to identify mutual interactions between tumour characteristics, tumour course and epileptic seizures. At diagnosis, 89.9% of patients had epileptic seizures. Male gender (P = 0.003) and tumour location within functional areas (P = 0.001) were independent predictors of a history of epileptic seizures at diagnosis. Tumour volume, growth velocity, cortical location, histopathological subtype or molecular markers did not significantly affect epileptic seizure occurrence probability. Prolonged history of epileptic seizures (P < 0.001), insular location (P = 0.003) and tumour location close to functional areas (P = 0.038) were independent predictors of uncontrolled epileptic seizures at diagnosis. Occurrence of epileptic seizures (P < 0.001), parietal (P = 0.029) and insular (P = 0.002) locations were independent predictors of uncontrolled epileptic seizures after oncological treatment. Patient age (P < 0.001), subtotal (P = 0.007) and total (P < 0.001) resections were independent predictors of total epileptic seizure control after oncological treatment. History of epileptic seizures at diagnosis and total surgical resection were independently associated with increased malignant progression-free (P < 0.001 and P < 0.001) and overall (P < 0.001 and P = 0.016) survivals. Epileptic seizures are independently associated with diffuse low-grade glioma prognosis. Patients diagnosed with epileptic seizures and those with complete and early surgical resections have better oncological outcomes. Early and maximal surgical resection is thus required for diffuse low-grade gliomas, both for oncological and epileptological purposes. PMID:24374407
Pallud, Johan; Audureau, Etienne; Blonski, Marie; Sanai, Nader; Bauchet, Luc; Fontaine, Denys; Mandonnet, Emmanuel; Dezamis, Edouard; Psimaras, Dimitri; Guyotat, Jacques; Peruzzi, Philippe; Page, Philippe; Gal, Beatriz; Párraga, Eduardo; Baron, Marie-Hélène; Vlaicu, Michaela; Guillevin, Rémy; Devaux, Bertrand; Duffau, Hugues; Taillandier, Luc; Capelle, Laurent; Huberfeld, Gilles
In order to observe the pattern of behaviour, the underlying Stressors and possible association with psychosocial factors\\u000a in Pediatric Non-Epileptic Seizure (NES); 22 children with unusual feature of poor control of their symptoms were studied.\\u000a Detailed historical account, examination including psychiatric evaluation, EEG and CT Scan in all and video recording with\\u000a provocative procedures were done wherever feasible. Ten males
S. K. Tamer
Objective : To characterize non-epileptic seizures (NES) in the elderly and compare their features with NES of a younger control group. Methods : The database of the epilepsy monitoring unit of the Cleveland Clinic Foundation (CCF) was searched for patients aged 60 years and older having undergone long-term video-\\/EEG monitoring between 1994 and 2002,with the subsequent diagnosis of NES. Videotapes
Christoph Kellinghaus; Tobias Loddenkemper; Dudley S. Dinner; Deepak Lachhwani; Hans O. Lüders
A previously unreported epileptic condition characterised by onset before 6 months of age, nearly continuous electroencephalographic seizures involving multiple independent areas originating in both hemispheres, no identifiable cause, and poor outcome has been described by Coppola et al. We report three cases presenting the same clinical and EEG pictures. They show a peculiar epileptic condition unlike the other early epileptogenic encephalopathies, so they may represent a new infantile epileptic syndrome. PMID:11395285
Veneselli, E; Perrone, M V; Di Rocco, M; Gaggero, R; Biancheri, R
Electroconvulsive therapy (ECT) has unparalleled antidepressant efficacy, but its cognitive side effects may be persistent. Research suggests that the side effects may be at least partially dissociable from the therapeutic effects of ECT, suggesting that distinct cortical networks may underlie them and introducing a role for focal seizure induction as a means of minimizing side effects. In magnetic seizure therapy (MST), magnetic fields avoid tissue impedance and induce electrical currents confined to superficial cortex, facilitating focal seizure induction. The translational development strategy for MST has included: (1) device development, (2) feasibility in animals and initial human trials, (3) testing in nonhuman primates on safety and mechanisms of action (with neuroanatomical, neurophysiological and cognitive endpoints), (4) safety testing in patients, (5) initial efficacy testing in patients, (6) dosage optimization, and (7) randomized comparison with ECT. These stages have been iterative, with results of early clinical testing prompting device enhancements that were, in turn, tested in nonhuman primates prior to human trials. Safety testing was aided by development of a nonhuman primate model of human ECT, and the validation of a cognitive battery for the monkey that is sensitive to the range of effects of ECT on human memory. Human testing has been facilitated by the development of an international consortium of centers addressing various aspects of technique and dose/response relationships. Challenges facing MST are common to other device based therapies: characterizing dose/response relationships, optimizing efficacy, and developing efficient and reliable methods to induce lasting therapeutic change in the circuitry underlying depression.
Rowny, Stefan; Benzl, Karla; Lisanby, Sarah H.
Patients with ecstatic epileptic seizures report an altered consciousness, which they describe as a sense of heightened perception of themselves – they “feel very present” – and an increased vividness of sensory perceptions. Recently, the anterior insula has been proposed as the region where these seizures originate, based on the results of ictal nuclear imaging in three patients, the first induction of ecstatic auras by electrical stimulation, and the functional characteristics of the anterior insula in neuroimaging literature. Specifically, the anterior insula is thought to play a key role in integrating information from within the body, the external world, as well as the emotional states. In addition, the anterior insula is thought to convert this integrated information into successive global emotional moments, thus enabling both the construct of a sentient self as well as a mechanism for predictive coding. As part of the salience network, this region is also involved in switching from mind wandering toward attentional and executive processing. In this review, we will summarize previous patient reports and recap how insular functioning may be involved in the phenomenon of ecstatic seizures. Furthermore, we will relate these hypotheses to the results from research on meditation and effects of drug abuse. PMID:24436968
Picard, Fabienne; Kurth, Florian
Flint Hills Scientific, L.L.C. (FHS) has invented what is believed to be the first real-time epileptic seizure detection and short-term prediction method in the world. They have demonstrated an IBM PC prototype with a multi-channel EEG monitoring configuration. This CRADA effort applied AlliedSignal FM and T hardware design, manufacturing miniaturization, and high quality manufacturing expertise in converting the prototype into a small, portable, self-contained, multi-channel EEG epileptic seizure detection and warning device. The purpose of this project was to design and build a proof-of-concept miniaturized prototype of the FHS-developed PC-based prototype. The resultant DSP prototype, measuring 4'' x 6'' x 2'', seizure detection performance compared favorably with the FHS PC prototype, thus validating the DSP design goals. The very successful completion of this project provided valuable engineering information for FHS for future prototype commercialization as well as providing AS/FM and T engineers DSP design experience.
Elarton, J.K.; Koepsel, K.L.
A new case of Foreign Accent Syndrome is described. This American woman presented with a British- or Australian- sounding accent after stroke, which resulted in a lacunar infarct in the left internal capsule. The atypical etiology and apparent changes in lexical use are described. It is hypothesized that an abnormally tense vocal tract posture may…
Ryalls, Jack; Whiteside, Janet
The authors present 3 cases of asphyxia caused by atypical compression of the neck by the metal bed bars fitted at the sides of the bed to prevent falling out. These occurred in 3 elderly women living in nursing homes, confined to bed by severe neuro- psychiatric disturbances. In all 3 cases, the minor nature of the skin lesions and
Nunzio Di Nunno; Michele Vacca; Fulvio Costantinides; Cosimo Di Nunno
Background and purpose The risk of atypical fracture of the femur is associated with bisphosphonate use. While characterizing atypical fractures from a previous nationwide study in radiographic detail, we had the impression that the fractures were located either in the subtrochanteric region or in the shaft. We determined whether there is a dichotomy in this respect. Patients and methods The distance between the atypical fractures and the lesser trochanter was measured on plain radiographs from 129 of 160 patients with atypical fractures, taken from 2008 through 2010. Analysis of the distances measured showed 2 clusters, which were then analyzed with regard to bisphosphonate use and age. Results The distribution of the distances would be best described as 2 clusters, with a dichotomy at 8 cm. The proximal (subtrochanteric) cluster comprised 25 patients who were generally younger (median 71 years) than the 104 patients in the cluster with shaft fractures (median 80 years). The 95% CI for the difference between medians was 4–11 years. Of the patients with subtrochanteric fractures, 18 of 25 used bisphosphonates as compared to 89 of 104 with shaft fractures. Interpretation The younger age and possibly smaller proportion of bisphosphonate users in the subtrochanteric cluster may be compatible with a greater influence of mechanical stress in the underlying pathophysiology of proximal fractures.
We present a patient with atypical pyoderma gangrenosum (APG), which involved the patient's arm and hand. Hemorrhagic bullae and progressive ulcerations were initially thought to be secondary to an infectious process, but a biopsy revealed PG. Awareness of APG by infectious disease services may prevent unnecessary use of broad-spectrum antibiotics.
To, Derek; Wong, Aaron; Montessori, Valentina
Pneumorrhachis, caused by intraspinal air, is an exceptional but important radiographic finding that is accompanied by different etiologies. Pneumorrhachis, by itself, is usually asymptomatic and gets reabsorbed spontaneously. Therefore, the patients with pneumorrhachis are mostly managed conservatively. We encountered a unique case of atypical traumatic pneumorrhachis accompanied by paraparesis.
Kim, Kweon Young; Kang, Jung Hun; Lee, Min Hong; Han, Yong
Children with autism spectrum disorders (ASD) frequently encounter difficulties in visuomotor tasks, which are possibly caused by atypical visuoperceptual processing. This was tested in children (aged 9-12 years) with pervasive developmental disorder (PDD; including PDD-NOS and Asperger syndrome), and two same-age control groups (Tourette syndrome…
Schlooz, Wim A. J. M.; Hulstijn, Wouter
Restricted stimulus control refers to discrimination learning with atypical limitations in the range of controlling stimuli or stimulus features. In the study reported here, 4 normally capable individuals and 10 individuals with intellectual disabilities (ID) performed two-sample delayed matching to sample. Sample-stimulus observing was recorded…
Dube, William V.; Dickson, Chata A.; Balsamo, Lyn M.; O'Donnell, Kristin Lombard; Tomanari, Gerson Y.; Farren, Kevin M.; Wheeler, Emily E.; McIlvane, William J.
Introduction: A growing body of literature suggests atypical cerebral asymmetry and interhemispheric interaction in ADHD. A common means of assessing lateralized brain function in clinical populations has been to examine the relative proportion of EEG alpha activity (8-12 Hz) in each hemisphere (i.e., alpha asymmetry). Increased rightward alpha…
Hale, T. Sigi; Smalley, Susan L.; Hanada, Grant; Macion, James; McCracken, James T.; McGough, James J.; Loo, Sandra K.
The ability to decode atypical and degraded speech signals as intelligible is a hallmark of speech perception. Human adults can perceive sounds as speech even when they are generated by a variety of nonhuman sources including computers and parrots. We examined how infants perceive the speech-like vocalizations of a parrot. Further, we examined how…
Vouloumanos, Athena; Gelfand, Hanna M.
Recently, there has been increased concern about the occurrence of diabetes associated with the use of atypical antipsychotic (AAP) drugs. The relationship between diabetes, schizophrenia, and antipsychotic drugs is complex and intriguing, as untreated patients with schizophrenia are known to suffer from diabetes more often than the general population. Thirty individual case reports of clozapine-, 26 cases of olanzapine- and
Jambur Ananth; Ravi Venkatesh; Karl Burgoyne; Sarath Gunatilake
SUMMARY Morphological and biochemical features of $2 strains of 'atypical' myco- bacteria and one strain of Mycobacterium tubmmbsis were studied. Of the $2 atypid mycobaderia, 16 were originally classified in Runyon's pup I, 4 in pup II, 19 in group 111, and 8 in group IV. The characteristics studied were bacdlary morphology and staining properties on Kirschner and Lowenstein- Jensen
Hydatid disease is an infectious disease caused by the larval stage of Echinococcus granulosus. It constitutes a major public health problem worldwide. This disease may present in several atypical forms, causing difficulty in diagnosis. In this article it is stressed that the diagnosis of hydatid disease should be kept in mind in patients presenting with hemoptysis, although this presentation is very rare. PMID:23619052
Özsürekçi, Yasemin; Parlakay, Asl?nur Özkaya; Cengiz, Ali Bülent; Çelik, Melda; Karada? Öncel, Eda; Kara, Ate?; Kiper, Nural; Cinel, Güzin; O?uz, Berna
A 1-year-old female spayed Birman cat was presented with a history of inappropriate urination and defecation, lethargy, anorexia, and weight loss. After intermittent responses to non-specific therapy she was diagnosed with atypical hypoadrenocorticism from the results of an adrenocorticotropic hormone stimulation test.
Hock, Colleen E.
Histological assessment of melanocytic naevi constitutes a substantial proportion of a dermatopathologist’s daily workload. Although they may be excised for cosmetic reasons, most lesions encountered are clinically atypical and are biopsied or excised to exclude melanoma. Although dysplastic naevi are most often encountered, cytological atypia may be a feature of several other melanocytic lesions, including genital type naevi, acral naevi,
K S Culpepper; S R Granter; P H McKee
In order to evaluate the following potential mechanisms underlying atypical gaze following in autism, impaired reflexive gaze following, difficulty integrating gaze and affect, or reduced understanding of the referential significance of gaze, we administered three paradigms to young children with autism (N = 21) and chronological (N = 21) and nonverbal mental age (N = 21) matched controls. Children with autism exhibited impaired reflexive gaze following. The absence of evidence of integration of gaze and affect, regardless of diagnosis, indicates ineffective measurement of this construct. Reduced gaze following was apparent among children with autism during eye-tracking and in-person assessments. Word learning from gaze cues was better explained by developmental level than autism. Thus, gaze following may traverse an atypical, rather than just delayed, trajectory in autism.
Gillespie-Lynch, K.; Elias, R.; Escudero, P.; Hutman, T.; Johnson, S. P.
Epilepsy may result from abnormal function of ion channels, such as those caused by genetic mutations. Recently, pathological alterations of the expression or localization of normal channels have been implicated in epilepsy generation, and termed ‘acquired channelopathies’. Altered expression levels of the HCN channels--that conduct the hyperpolarization-activated current, Ih--have been demonstrated in hippocampus of patients with severe temporal lobe epilepsy as well as in animal models of temporal lobe and absence epilepsies. Here we probe the mechanisms for the altered expression of HCN channels which is provoked by seizures. In organotypic hippocampal slice cultures, seizure-like events selectively reduced HCN type 1 channel expression and increased HCN2 mRNA levels, as occurs in vivo. The mechanisms for HCN1 reduction involved Ca2+-permeable AMPA receptors-mediated Ca2+ influx, and subsequent activation of Ca2+/calmodulin-dependent protein kinase II. In contrast, upregulation of HCN2 expression was independent of these processes. The data demonstrate an orchestrated program for seizure-evoked transcriptional channelopathy involving the HCN channels, that may contribute to certain epilepsies.
Richichi, Cristina; Brewster, Amy L.; Bender, Roland A.; Simeone, Timothy A.; Zha, Qinqin; Yin, Hong Z.; Weiss, John H.; Baram, Tallie Z.
Despite numerous neuroendocrinological studies of seizures, the influence of estrogen and progesterone on seizures and epilepsy remains unclear. This may be due to the fact that previous studies have not systematically compared distinct endocrine conditions and included all relevant controls. The goal of the present study was to conduct such a study using pilocarpine as chemoconvulsant. Thus, age and weight-matched, intact or ovariectomized rats were tested to determine incidence of status epilepticus and to study events leading to status. Intact female rats were sampled at each cycle stage (proestrus, estrus, metestrus, or diestrus 2). Convulsant was administered at the same time of day, 10:00-10:30 a.m. Statistical analysis showed that there was a significantly lower incidence of status on the morning of estrus, but differences were attenuated in older animals. Ovariectomized rats were distinct in their rapid progression to status. These results show that the incidence of status in female rats following pilocarpine injection, and the progression to pilocarpine-induced status, are influenced by reproductive state as well as age. The hormonal milieu present specifically on the morning of estrus appears to decrease susceptibility to pilocarpine-induced status, particularly at young ages. In contrast, the chronic absence of reproductive steroids that characterizes the ovariectomized rat leads to a more rapid progression to status. This dissociation between incidence vs. progression provides new insight into the influence of estrogen and progesterone on seizures. PMID:16084511
Scharfman, Helen E; Goodman, Jeffrey H; Rigoulot, Marie-Aude; Berger, Russell E; Walling, Susan G; Mercurio, Thomas C; Stormes, Kerry; Maclusky, Neil J
In this study, we investigated the possibility of differential diagnosis of patients with epileptic seizures (ES) and patients with psychogenic non-epileptic seizures (PNES) by an advanced analysis of dynamics of the patients' scalp electroencephalograms (EEG). The underlying principle was the presence of resetting of brain's pre-ictal spatiotemporal entrainment following onset of ES and the absence of resetting following PNES. Long-term (days) scalp EEGs recorded from five ES and six PNES patients were analyzed. It was found that: (a) Pre-ictal entrainment of brain sites was reset by epileptic seizures (p<0.05) in 4 out of the 5 patients with ES, and not reset (p=0.28) in the fifth patient. (b) Resetting did not occur (p>0.1) in any of the 6 patients with PNES. These preliminary results in patients with ES are in agreement with our previous findings from intracranial EEG recordings on resetting of brain dynamics at ES and it is expected to constitute the basis for the development of a reliable and supporting tool in the differential diagnosis between ES and PNES. Finally, we believe that these results shed a novel light on the electrophysiology of psychogenic epilepsy by showing that occurrence of PNES does not assist patients to overcome a pathological entrainment of brain dynamics.
Krishnan, Balu; Faith, Aaron; Vlachos, Ioannis; Roth, Austin; Williams, Korwyn; Noe, Katie; Drazkowski, Joe; Tapsell, Lisa; Sirven, Joseph; Iasemidis, Leon
The aim of this study was to examine clinical characteristics in patients with psychogenic nonepileptic seizures and to analyze the Minnesota Multiphasic Personality Inventory (MMPI) profiles and their relation to psychopathology. Thirty patients with nonepileptic seizures confirmed through video-electroencephalography were included. A structured clinical interview (Structured Clinical Interview for DSM-III-R), a measure of personality variables (MMPI), and several structured interviews designed for collecting data on clinical and personal history were administered. Descriptive and comparative statistical methods were used. Of the sample, 67.7% met criteria for two or more simultaneous Axis I diagnoses, and 60% for an Axis II personality disorder. The most frequently elevated scales of the MMPI were Schizophrenia and Depression. There were multiple scale elevations in 12 profiles, the 91.7% of which had elevated "neurotic" and "psychotic" scales. The subgroup with personality disorders showed higher scores on the MMPI Paranoia and Hypomania scales, and the subgroup with traumatic experiences showed higher scores on the MMPI Hypomania scale. Our sample comprising patients with nonepileptic seizures showed a significant degree of psychopathology and absence of a unique character substrate. According to grades of clinical severity of pseudoseizures, several subgroups and different therapeutic implications may be defined. PMID:15234827
Baillés, Eva; Pintor, Luis; Fernandez-Egea, Emili; Torres, Xavier; Matrai, Silvia; De Pablo, Joan; Arroyo, Santiago
Wet dog shakes (WDS) and head shakes (HS) are associated with experimentally induced convulsive seizures. We sought to determine whether these behaviors are correlated or not with major (status epilepticus (SE) or fully kindled animals) or minor (non-SE or partially kindled animals) seizure severity. WDS are directly correlated with SE induced by intracerebral star fruit extract (Averrhoa carambola) injection and with kindled animals in the amygdala fast kindling model. On the other hand, WDS are inversely correlated with SE induced by intracerebral bicuculline and pilocarpine injections. Systemic pilocarpine in animals pretreated with methyl-scopolamine barely induced WDS or HS. The role of shaking behaviors may vary from ictal to anticonvulsant depending on the experimental seizure model, circuitries involved, and stimulus intensity. The physical presence of acrylic helmets may per se inhibit the HS response. Also, methyl-scopolamine, a drug incapable of crossing the blood-brain barrier, can induce HS in animals without acrylic helmets. PMID:15820339
Rodrigues, Marcelo Cairrão Araújo; Rossetti, Franco; Foresti, Maira Licia; Arisi, Gabriel Maisonnave; Furtado, Márcio Araújo; Dal-Cól, Maria Luiza Cleto; Bertti, Poliana; Fernandes, Artur; Santos, Francisco Leite; Del Vecchio, Flávio; Garcia-Cairasco, Norberto
We recorded occipitotemporal seizures induced by intermittent photic stimulation in three children with brain injuries, aged 10 to 13 years. All had a history of seizures and showed occipital spikes on EEG, but were seizure free and were not being treated at the time of investigation. In all, photic stimulation induced seizures in the right occipital lobe. They were followed by clinical and EEG signs suggesting infrasylvian spreading to ipsilateral mesiotemporal limbic structures and by vomiting, appearing at late stages of the attacks. Seizure spread was very slow in two patients in whom attacks lasted 16 and 25 minutes. Patients with occipital epileptiform abnormalities presenting with ictal vomiting are often diagnosed as having vague migraine-epilepsy syndromes. We conclude that vomiting can be a late ictal phenomenon resulting from temporal lobe spread of seizures originating in the occipital lobe. PMID:8309569
Guerrini, R; Ferrari, A R; Battaglia, A; Salvadori, P; Bonanni, P
Seizures are one of the most common pediatric neurologic disorders. Many complications secondary to seizures have been described in the literature including head trauma, fractures, drowning and burns. However, to the best of our knowledge, rupture of the myotendinous insertion of the temporalis muscle on the mandible secondary to a seizure has never been described in the literature. We report the case of a unilateral temporalis muscle rupture in a 16-year-old boy who developed unilateral facial swelling following new onset tonic-clonic seizures. We emphasize on the computed tomography and magnetic resonance imaging findings in this case report. Two mechanisms have been proposed to explain such an injury. The favored mechanism in our patient is a pull on the temporalis myotendinous insertion on the mandible following vigorous and brisk deviation of the head and neck during seizure. Radiologists should be familiar with this type of injury following seizures in order to prevent misdiagnosis and subsequently mistreatment.
Naffaa, Lena N; Tandon, Yasmeen K; Rubin, Michael
Seizures are one of the most common pediatric neurologic disorders. Many complications secondary to seizures have been described in the literature including head trauma, fractures, drowning and burns. However, to the best of our knowledge, rupture of the myotendinous insertion of the temporalis muscle on the mandible secondary to a seizure has never been described in the literature. We report the case of a unilateral temporalis muscle rupture in a 16-year-old boy who developed unilateral facial swelling following new onset tonic-clonic seizures. We emphasize on the computed tomography and magnetic resonance imaging findings in this case report. Two mechanisms have been proposed to explain such an injury. The favored mechanism in our patient is a pull on the temporalis myotendinous insertion on the mandible following vigorous and brisk deviation of the head and neck during seizure. Radiologists should be familiar with this type of injury following seizures in order to prevent misdiagnosis and subsequently mistreatment. PMID:24976940
Naffaa, Lena N; Tandon, Yasmeen K; Rubin, Michael
It is unusual for a newborn to have the classic "tonic-clonic" seizure experienced by adults and older children. Signs of seizure in newborns are either subtle or may become clinically silent. Therefore, the electroencephalogram (EEG) is becoming the most reliable tool for detecting neonatal seizure. Being non-stationary and multicomponent, EEG signals are suitably analyzed using time-frequency (TF) based methods. In this paper, we present a seizure detection method using a new measure based on the matching pursuit (MP) decomposition of EEG data. Signals are represented in the TF domain where seizure structural characteristics are extracted to form a new coherent TF dictionary to be used in the MP decomposition. A new approach to set data-dependent thresholds, used in the seizure detection process, is proposed. To enhance the performance of the detector, the concept of areas of incidence is utilized to determine the geometrical correlation between EEG recording channels. PMID:19162804
Khlif, M S; Mesbah, M; Boashash, B; Colditz, P
The sensitivity of the Seizure Severity Questionnaire (SSQ) was evaluated using pooled data from open-label extensions of two clinical trials in patients with partial-onset seizures. The SSQ includes questions relating to frequency and helpfulness of warning signs as well as frequency, severity, and bothersomeness of ictal and postictal effects. Differences between mean change from baseline for each SSQ item for responders and nonresponders were described and compared between patients solely with complex partial seizures (CPSs: responders, n=166; nonresponders, n=127) and those solely with secondarily generalized partial seizures (SGPSs: responders, n=26; nonresponders, n=24) at baseline. Seizure Severity Questionnaire total score and individual SSQ items related to ictal movement, consciousness, bothersomeness of postictal effects, and frequency of postictal emotional effects showed differentiation between seizure type responders. These data provide further validation of the SSQ by demonstrating its sensitivity in describing treatment effects. PMID:24275520
Borghs, Simon; de la Loge, Christine; Brabant, Yves; Cramer, Joyce