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Tonic Seizures (tonic + seizures)
Selected AbstractsPredictive Clinical Factors for the Differential Diagnosis of Childhood Extratemporal SeizuresEPILEPSIA, Issue 8 2005András Fogarasi Summary:,Purpose: To describe predictive clinical factors for the differentiation between childhood frontal lobe epilepsy (FLE) and posterior cortex epilepsy (PCE). Methods: Two independent, blinded investigators analyzed 177 seizures from 35 children (aged 11 months to 12 years) with extratemporal epilepsy selected by postoperative seizure-free outcome. Semiologic seizure components and different periictal signs were observed. Age at onset, auras, seizure frequency, and nocturnal dominance, as well as surgical and histopathologic data, were collected from medical charts. Results: Twenty patients had FLE, and 15 had PCE. Patients from both groups had daily seizures without significant differences in frequency but with higher nocturnal dominance in children with FLE (p < 0.05). Visual aura, nystagmus, and versive seizure were observed exclusively in the PCE group, whereas somatosensory aura and hypermotor seizures appeared only in FLE. Tonic seizures were significantly more frequent in FLE (p < 0.01), whereas the presence of clonic seizure (FLE; p = 0.07) and postictal nose-wiping (PCE; p = 0.05) showed only a trend to localize the seizure-onset zone. Myoclonic seizures, epileptic spasms, psychomotor seizures, atonic seizures, oral and manual automatisms, as well as vocalization and eye deviation appeared in both groups without significant differences in their frequency. Conclusions: Characteristic features described in adults' extratemporal epilepsies were frequently missing during childhood seizures, especially in infants and preschool children. Ictal features help only a little in differentiating childhood FLE from PCE. Nocturnal appearance and the type of aura have high localizing value; therefore an accurate history taking is still an essential element of pediatric presurgical evaluation. [source] Aromatic l -amino acid decarboxylase deficiency associated with epilepsy mimicking non-epileptic involuntary movementsDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 11 2008Susumu Ito MD Aromatic l -amino acid decarboxylase (AADC) deficiency is a rare inborn error of neurotransmitter biosynthesis that leads to a combined deficiency of catecholamines and serotonin and is characterized by global developmental delay, involuntary movements, and autonomic dysfunction. We report the case of an 11-year-old male patient with AADC deficiency who also had epileptic spasms and generalized tonic seizures with asymmetrical features, in addition to frequent involuntary non-epileptic movements. The clinical manifestation of the epileptic attacks apparently resembled that of non-epileptic attacks. It was difficult to differentiate between both attacks without the help of an ictal electroencephalographic study. The epileptic attacks were finally controlled by appropriate antiepileptic drugs. Because an association with epileptic seizures is uncommon in AADC deficiency, some cases may have been regarded as involuntary non-epileptic movements. This indicates that the differentiation of epileptic attacks from non-epileptic ones is indispensable for the adequate treatment of patients with AADC deficiency. [source] Ring chromosome 20 syndrome with intractable epilepsyDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 5 2005Asude Alpman MD Ring chromosome 20 (r[20]) syndrome is characterized by mild to moderate learning disability*, behavioural disorders, epilepsy, and various dysmorphic features. Although still considered rare, r(20) syndrome is being increasingly diagnosed. More than 30 cases have been described in the literature since 1976. Here we report an additional case of a 14-year-old male with r(20). He had moderate to severe learning disability and epileptic seizures manifesting at about 18 months of age. During the 13 years' follow-up period he showed intractable epileptic seizures, behavioural disorders, and mild dysmorphological features including microcephaly, strabismus, micrognathia, down-slanting eyelids, and ear abnormalities. Frequent episodes of atypical absence or non-convulsive status associated with electroencephalogram changes were seen in follow-up. He was treated with several classical and new antiepileptic drugs, including intravenous immunoglobulin, corticotropin, and vagal nerve stimulation, with unsuccessful control of seizures. Finally, surgical treatment (corpus callosotomy) was performed at the age of 13 years; severity of tonic seizures was diminished, but frequency was unchanged. Although his behavioural problems, e.g. hyperactivity, were mild in early childhood they became more severe when he was 11 years old. Aggressiveness, compulsiveness with self-injury, and panic attacks developed at the age of 13 years, and were more pronounced after callosotomy. This case report provides the first description of deterioration in psychological situation in patients with r(20) intractable epilepsy. The patient was diagnosed with r(20) syndrome after 13 years of clinical follow-up. Karyotype analysis should, therefore, be performed in every patient with intractable epilepsy of unknown aetiology. [source] Temporal distribution of clinical seizures over the 24-h day: A retrospective observational study in a tertiary epilepsy clinicEPILEPSIA, Issue 9 2009Wytske A. Hofstra Summary Purpose:, Very few studies have evaluated seizure occurrence in humans over the 24-h day; data from children are particularly scarce. Circadian patterns in seizure occurrence may be of importance in epilepsy research and may have important implications in diagnosis and therapy. Methods:, We have analyzed clinical seizures of 176 consecutive patients (76 children, 100 adults) who had continuous electroencephalography (EEG) and video monitoring lasting more than 22 h. Several aspects of seizures were noted, including classification, time of day, origin, and sleep stage. Results:, More than 800 seizures were recorded. Significantly more seizures were observed from 11:00 to 17:00 h, and from 23:00 to 05:00 h significantly fewer seizures were seen. The daytime peak incidences were observed in seizures overall, complex partial seizures (in children and adults), seizures of extratemporal origin (in children), and seizures of temporal origin (in adults). Incidences significantly lower than expected were seen in the period 23:00 to 05:00 h in seizures overall, complex partial seizures (in children and adults), and in tonic seizures (in children). In addition, significantly fewer seizures of temporal (in children and adults) and extratemporal origin (in children) were observed in this period. Discussion:, The results suggest that certain types of seizures have a strong tendency to occur in true diurnal patterns. These patterns are characterized by a peak during midday and a low in the early night. [source] Interobserver Reliability of Video Recording in the Diagnosis of Nocturnal Frontal Lobe SeizuresEPILEPSIA, Issue 8 2007Luca Vignatelli Summary:,Background: Nocturnal frontal lobe seizures (NFLS) show one or all of the following semeiological patterns: (1) paroxysmal arousals (PA: brief and sudden recurrent motor paroxysmal behavior); (2) hyperkinetic seizures (HS: motor attacks with complex dyskinetic features); (3) asymmetric bilateral tonic seizures (ATS: motor attacks with dystonic features); (4) epileptic nocturnal wanderings (ENW: stereotyped, prolonged ambulatory behavior). Objective: To estimate the interobserver reliability (IR) of video-recording diagnosis in patients with suspected NFLS among sleep medicine experts, epileptologists, and trainees in sleep medicine. Methods: Sixty-six patients with suspected NFLS were included. All underwent nocturnal video-polysomnographic recording. Six doctors (three experts and three trainees) independently classified each case as "NFLS ascertained" (according to the above specified subtypes: PA, HS, ATS, ENW) or "NFLS excluded". IR was calculated by means of Kappa statistics, and interpreted according to the standard classification (0.0,0.20 = slight agreement; 0.21,0.40 = fair; 0.41,0.60 = moderate; 0.61,0.80 = substantial; 0.81,1.00 = almost perfect). Results: The observed raw agreement ranged from 63% to 79% between each pair of raters; the IR ranged from "moderate" (kappa = 0.50) to "substantial" (kappa = 0.72). A major source of variance was the disagreement in distinguishing between PA and nonepileptic arousals, without differences in the level of agreement between experts and trainees. Conclusions: Among sleep experts and trainees, IR of diagnosis of NFLS, based on videotaped observation of sleep phenomena, is not satisfactory. Explicit video-polysomnographic criteria for the classification of paroxysmal sleep motor phenomena are needed. [source] Malignant Refractory Epilepsy in Identical Twins Mosaic for a Supernumerary Ring Chromosome 19EPILEPSIA, Issue 8 2004Amre Shahwan Summary:, We report identical twins with supernumerary ring chromosome 19 mosaicism, who had severe refractory epilepsy at an early age. The epilepsy was dominated largely by severe life-threatening tonic seizures. Both twins died, likely as a consequence of their severe epilepsy. They displayed no dysmorphic features. Eight cases of ring chromosome 19 have been reported in the literature, all to our knowledge without epilepsy. The clinical picture of these twins emphasizes the importance of carrying out a karyotype study on patients with early-onset epilepsy even in the absence of dysmorphic features. [source] Seizure Semiology in the Elderly: A Video AnalysisEPILEPSIA, Issue 3 2004Christoph Kellinghaus Summary: Purpose: To describe the seizure semiology of patients older than 60 years and to compare it with that of a control group of younger adults matched according to the epilepsy diagnosis. Methods: Available videotapes of all patients aged 60 years and older who underwent long-term video-EEG evaluation at the Cleveland Clinic Foundation (CCF) between January 1994 and March 2002 were analyzed by two observers blinded to the clinical data. A younger adult control group was matched according to the epilepsy diagnosis, and their seizures also were analyzed. Results: Fifty-four (3.3%) of the 1,633 patients were 60 years or older at the time of admission. For 21 of them, at least one epileptic seizure was recorded. Nineteen patients had focal epilepsy (nine temporal lobe, two frontal lobe, two parietal lobe, eight nonlocalized), and two patients had generalized epilepsy. Seventy-three seizures of the elderly patients and 85 seizures of the 21 control patients were analyzed. In nine elderly patients and 14 control patients, at least one of their seizures started with an aura. Eleven elderly patients and 19 control patients lost responsiveness during their seizures. Approximately two thirds of the patients in both groups had automatisms during the seizures. Both focal and generalized motor seizures (e.g., clonic or tonic seizures) were seen less frequently in the elderly. Conclusions: Only a small percentage of the patients admitted to a tertiary epilepsy referral center for long-term video-EEG monitoring are older than 60 years. All seizure types observed in the elderly also were seen in the younger control group, and vice versa. Simple motor seizures were seen less frequently in the elderly. [source] Tonic,Absence Seizures: An Underrecognized Seizure TypeEPILEPSIA, Issue 3 2003Tina T. Shih M.D. Summary: ,Purpose: The individual electroclinical patterns,tonic seizures with generalized paroxysmal fast activity (GPFA, activity >13 Hz), and absence seizures with generalized slow spike-and-wave activity (GSS&W, <3 Hz),have been extensively described in the literature. However, only passing reference was made to the pattern of GPFA followed by GSS&W. In addition, these descriptions were formulated in the pre-EMU (Epilepsy Monitoring Unit) era, without benefit of video/clinical correlation. We now characterize this underrecognized seizure type. Methods: We retrospectively reviewed the data from eight patients with seizures that demonstrated this stereotyped EEG and clinical pattern. Results: We identified eight patients (six female patients; age 6,29 years; age at seizure onset, neonate to 10 years) who were evaluated at the Columbia University Epilepsy Monitoring Units between 1993 and 2002. All eight had an International League Against Epilepsy (ILAE) diagnosis of symptomatic generalized epilepsy, with six of eight manifesting multiple seizure types, six of eight with mild static encephalopathy, and two with normal cognition. A total of 29 seizures of this pattern was recorded; 26 of 29 seizures demonstrated GPFA (frequency between 14 and 30 Hz, lasting 2,8 s) followed by GSS&W (frequency range between 1 and 2 Hz, lasting 3,50 s). The predominant clinical correlate was bilateral tonic activity followed by a period of inattentiveness. In general, these seizures were differentiated from the patient's typical tonic seizures by this protracted period of impaired attentiveness. Conclusions: We describe a heretofore underrecognized and poorly characterized seizure type in patients with symptomatic generalized epilepsy, which we have termed tonic,absence seizures. Clinically and electrographically, this consists of a tonic seizure with GPFA followed by an absence seizure with GSS&W. [source] A Detailed Analysis of Symptomatic Posterior Cortex Seizure Semiology in Children Younger Than Seven YearsEPILEPSIA, Issue 1 2003András Fogarasi Summary: ,Purpose: To analyze the semiology of seizure onset and evolution in young children with posterior cortex epilepsy (PCE), compare this with adult reports, and assess age-related differences. Methods: We videotaped and analyzed 110 seizures from 18 patients with PCE, aged 3,81 months. All had a good prognosis after posterior epileptogenic zone removal. Ictal events were categorized by behavioral, consciousness, autonomic, and sensory features, as well as motor patterns, which included myoclonic, tonic, clonic, unclassified motor seizures, and epileptic spasm. A time-scaled data sheet was developed to record each epileptic event as onset, very early, early, or late manifestation. Results: Patients had a high seizure frequency with ,100 attacks/day; one third of them showed a cluster tendency. The mean duration of seizures was 67 s. The most common seizure components were motor manifestations (with myoclonic and tonic seizures), but psychomotor (automotor), hypomotor attacks, and isolated auras also were frequently observed. Clinical seizure spread was frequent; auras and visual sensory signs were difficult to record in this age. Typical phenomena during seizures included behavioral changes, ictal vocalization, smile, flush, head nod, oculomotor features, and late-appearing oral automatisms, whereas hypermotor and secondarily generalized tonic,clonic seizures were not seen. Conclusions: Our results suggest that PCE in infants and young children is very heterogeneous but shows important age-related features. Compared with adults, children with PCE have shorter but more frequent seizures; they rarely report aura or visual sensory signs, only sporadically develop hypermotor and secondarily generalized tonic,clonic seizures, whereas ictal smile, flush, head nod, and behavioral change are typical features at this age. Because of frequent subtle ictal phenomena, long-term video-EEG monitoring is a useful diagnostic tool with infants and young children with PCE. [source] Clinical and Electrographic Features of Epileptic Spasms Persisting Beyond the Second Year of LifeEPILEPSIA, Issue 6 2002Márcio A. Sotero De Menezes Summary: ,Purpose: Few reports detailing the electroclinical features of epileptic spasms persisting beyond infancy have been published. We sought to characterize this unique population further. Methods: We retrospectively reviewed the clinical and video-EEG data on 26 patients (4,17 years; mean, 93 months) with a confirmed diagnosis of epileptic spasms and who were evaluated at our tertiary referral center between 1993 and 2000. Results: In half of our cases, epileptic spasms were associated with disorders of neuronal migration, severe perinatal asphyxia, and genetic anomalies. Interictal EEGs showed generalized slowing in the majority of patients, and a slow-wave transient followed by an attenuation of the background amplitude was the most common ictal EEG pattern associated with an epileptic spasm (19 cases). Other seizure types (number of cases in parentheses) included tonic seizures with or without a preceding spasm (13), partial seizures (11), myoclonic seizures (11), generalized tonic,clonic seizures (six), atypical absence seizures (two), and atonic seizures (one). Cases with a more organized EEG background (especially with frequencies ,7 Hz) were more likely to have better cognition. Continued disorganization of the EEG background and persistence of hypsarrhythmia were associated with poor developmental outcome. Conclusions: Patients with epileptic spasms persisting beyond age 2 years constitute a truly refractory population, one that should be better recognized by clinicians. Interestingly, although many therapies resulted in a >50% reduction in seizures, neither neurocognitive function nor quality of life was substantially improved with intervention. The interictal EEG background is the most helpful in predicting neurologic outcome. [source] Role of Nitric Oxide in Pentylenetetrazol-Induced Seizures: Age-Dependent Effects in the Immature RatEPILEPSIA, Issue 4 2000Anne Pereira de Vasconcelos Summary: Purpose: Seizure susceptibility and consequences are highly age dependent. To understand the pathophysiologic mechanisms involved in seizures and their consequences during development, we investigated the role of nitric oxide (NO) in severe pentylenetetrazol (PTZ)-induced seizures in immature rats. Methods: Four cortical electrodes were implanted in 10-day-old (P10) and 21-day-old (P21) rats, and seizures were induced on the following day by repetitive injections of subconvulsive doses of PTZ. The effects of NG -nitro- l -arginine methyl ester (l -NAME; 10 mg/kg) and 7-nitroindazole (7NI; 40 mg/kg), two NO synthase (NOS) inhibitors, and l -arginine (l -arg; 300 mg/kg), the NOS substrate, were evaluated regarding the mean PTZ dose, seizure type and duration, and mortality rate. Results: At P10, the postseizure mortality rate increased from 18,29% for the rats receiving PTZ only to 100% and 89% for the rats receiving l -NAME and 7NI, respectively; whereas l -arg had no effect. Conversely, at P21, NOS inhibitors did not affect the 82,89% mortality rate induced by PTZ alone, whereas l -arg decreased the mortality rate to 29%. In addition, all NO-related drugs increased the duration of ictal activity at P10, whereas at P21, L -arg and L -NAME affected the first seizure type, producing clonic seizures with L -arg and tonic seizures with L -NAME. Conclusions: The relative natural protection of very immature rats (P10) against PTZ-induced deaths could be linked to a high availability of L -arg and, hence, endogenous NO. At P21, the modulation of seizure type by NO-related compounds may be related to the maturation of the brain circuitry, in particular the forebrain, which is involved in the expression of clonic seizures. [source] Electroclinical Picture of Autosomal Dominant Nocturnal Frontal Lobe Epilepsy in a Japanese FamilyEPILEPSIA, Issue 1 2000Masatoshi Ito Summary: Purpose: Autosomal dominant nocturnal frontal lobe epilepsy (ADNFLE) is the first described partial epilepsy syndrome known to be due to a single gene mutation. We found a first Japanese ADNFLE family with a novel mutation of the neuronal nicotinic acetylcholine receptor (nAChR) ,4 subunit (CHRNA4) gene. The aim of this report is precisely to describe the electroclinical manifestations of ADNFLE in this family and to compare these findings with those of other families reported previously in the literature. Methods: Three affected family members were investigated electroclinically by close clinical observation, interictal EEG, video-EEG monitoring, magnetic resonance imaging, and single-photon-emission tomography. Information about other affected family members was obtained from either the spouse or the parents. Mutations within the CHRNA4 gene were examined in seven family members. Results: The clinical manifestations and diagnostic findings in the members of this family were consistent with ADNFLE. However, there were intrafamilial and interfamilial variations in clinical features. The seizures of the patients were brief tonic seizures, with hyperventilation in children and secondarily generalized tonic,clonic convulsions in adults. The onset of the children's seizures began in infancy and early childhood. The children's seizures were sometimes provoked by movement and sound stimulation, and did not respond to antiepileptic drugs. On the other hand, the adults' seizures disappeared spontaneously or were easily controlled with carbamazepine. Three children showed hyperactivity, and two children had mild mental retardation. All patients had impaired consciousness during their seizures and no auras. A novel missense mutation (c755C>T) in exon 5 of the CHRNA4 gene was found in four affected family members. Conclusions: The electroclinical pictures of a Japanese family with ADNFLE were basically the same as those of other families reported, but with slight differences. ADNFLE is probably not uncommon, and it is very likely that there are unidentified patients with this inherited disorder in Japan. [source] Anticonvulsant activities of nutmeg oil of Myristica fragransPHYTOTHERAPY RESEARCH, Issue 2 2009Abdul Wahab Abstract The purpose of this study was to investigate the anticonvulsant activity of the volatile oil of nutmeg, the dried seed kernel of Myristica fragrans Houtt, using well-established animal seizure models and to evaluate its potential for acute toxicity and acute neurotoxicity. The volatile oil of nutmeg (nutmeg oil) was tested for its effects in maximal electroshock, subcutaneous pentylenetetrazole, strychnine and bicuculline seizure tests. All the experiments were performed at the time of peak effect of nutmeg oil. Nutmeg oil showed a rapid onset of action and short duration of anticonvulsant effect. It was found to possess significant anticonvulsant activity against electroshock-induced hind limb tonic extension. It exhibited dose dependent anticonvulsant activity against pentylenetetrazole-induced tonic seizures. It delayed the onset of hind limb tonic extensor jerks induced by strychnine. It was anticonvulsant at lower doses, whereas weak proconvulsant at a higher dose against pentylenetetrazole and bicuculline induced clonic seizures. Nutmeg oil was found to possess wide therapeutic margin, as it did not induce motor impairment when tested up to 600 µL/kg in the inverted screen acute neurotoxicity test. Furthermore, the LD50 (2150 µL/kg) value was much higher than its anticonvulsant doses (50,300 µL/kg). The results indicate that nutmeg oil may be effective against grand mal and partial seizures, as it prevents seizure spread in a set of established animal models. Slight potentiation of clonic seizure activity limits its use for the treatment of myoclonic and absence seizures. Copyright © 2008 John Wiley & Sons, Ltd. [source] | ||||||||||