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Query: UMLS:C0036572 (seizures)
 80,221 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A young female patient, who presented acutely with an unusual movement disorder characterized by hyperkinetic facial movements, was referred to us for hemifacial spasm. The only abnormality on neurological examination was myoclonus of the left perioral and bilateral periorbital muscles, exacerbated by mental tasks. A week later, the patient also presented two generalized tonic-clonic seizures on awakening and was successfully treated with antiepileptic drugs. Laboratory and neuroimaging investigations yielded normal findings. Although we were unable to identify a cortical generator, the concomitant occurrence of generalized seizures, the disappearance of symptoms after treatment and the topography of the myoclonus support an epileptic origin of this myoclonus.
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PMID:Focal motor seizures mimicking hemifacial spasm. 1832 23

Marshall Hall (1790-1857), who graduated from the University of Edinburgh's Medical School in 1812, was considered one of the greatest physiologists of his day. He advanced knowledge in various areas of medicine, in particular elucidating the mechanism of reflex activity in 1833. Hall suggested that convulsive epileptic seizures arose from heightened activity in the afferent limb or the central component of the reflex arc. From 1838 onwards he developed the idea that reflex-mediated neck muscle spasm in seizures obstructed cerebral venous return, congested the brain and thus caused unconsciousness. Associated reflex-mediated laryngeal spasm then caused convulsing. This was the most comprehensive physiologically based explanation of the major features of the convulsive epileptic seizure then available. Hall subsequently advocated and employed tracheotomy to prevent epileptic convulsing. His idea was taken up, modified and made more acceptable by others, and for a generation was the widely acknowledged basis for interpreting epileptogenesis. However, from 1870 onwards it was superseded by John Hughlings Jackson's accumulating evidence that epileptic seizures often arose in the cerebral cortex.
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PMID:Marshall Hall, the reflex arc and epilepsy. 1883 Nov 16

A prospective hospital based study of childhood (<15 yrs) and neonatal tetanus cases from July 2004 - May 2006 was done to study the demography, clinical features and outcome of pediatric and neonatal tetanus cases at BPKIHS. During the study, 24 cases of tetanus were admitted from 9 districts including 5 neonatal tetanus. Among children with tetanus, 31.5% received 3 doses of DPT and 10.5% received TT vaccine as tetanus prophylaxis. In 16.0% children there was no recognizable injury preceding the disease. Otitis media preceded tetanus in 16.0%. All neonatal tetanus cases occurred following umbilical sepsis. Despite their mothers receiving 2 doses of TT during pregnancy, 2 neonates developed tetanus. A neonate delivered in hospital also developed neonatal tetanus. Average incubation period was 7.7 days and average onset time was 16.9 hours. Short onset time predicted the favorable outcome (p=0.005). Generalized tetanus cases were 75.0%, neonatal tetanus 21.0% and cephalic tetanus 4.0%. Generalized spasm was present in all cases. Common autonomic dysfunctions were fever, tachycardia and hypotension. Respiratory failure, aspiration pneumonia, rhabdomyolysis and seizure were common complications. Only one case received Intensive Care Unit (ICU) care. Survival rate was 21.1% for childhood tetanus and 40.0% for neonatal tetanus. Respiratory failure was the cause of death in majority. Study finds tetanus as an important disease in eastern Nepal, with substantial morbidity and mortality, primarily affecting the unvaccinated and inadequately vaccinated individuals. Despite lack of adequate resources, we can still manage tetanus cases with comparable outcome to other case series reported in the literatures.
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PMID:Pediatric and neonatal tetanus: a hospital based study at eastern Nepal. 1925 61

Stingrays are common inhabitants in the northern waters of the Persian Gulf and Oman Sea. In the present study, the clinical aspects of injuries induced in three patients bitten by stingrays in Hormozgan province waters were first examined. The LD(50) of crude venom extract obtained from the most common stingray in Hormozgan province (Himantura gerrardi) was then estimated by up-and-down dosing and double dose methods in mice. Third and finally, the cardiac symptoms induced by injection of the extract from the venomous spines were evaluated in rats. Intense pain was noticed in all human cases. Redness was observed in two cases, and spasm and seizure were each recorded in only one case. LD(50) of the venom extract in mice was about 100 mg kg(-1). The observed cardiac symptoms in rats included an increase in pulse rate and various changes in electrocardiogram (ECG) parameters such as T and Q values, and PR and RR intervals.
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PMID:Study of patient's injuries by stingrays, lethal activity determination and cardiac effects induced by Himantura gerrardi venom. 1956 21

Infantile spasms syndrome (ISS) is a catastrophic pediatric epilepsy with motor spasms, persistent seizures, mental retardation, and in some cases, autism. One of its monogenic causes is an insertion mutation [c.304ins (GCG)(7)] on the X chromosome, expanding the first polyalanine tract of the interneuron-specific transcription factor Aristaless-related homeobox (ARX) from 16 to 23 alanine codons. Null mutation of the Arx gene impairs GABA and cholinergic interneuronal migration but results in a neonatal lethal phenotype. We developed the first viable genetic mouse model of ISS that spontaneously recapitulates salient phenotypic features of the human triplet repeat expansion mutation. Arx((GCG)10+7) ("Arx plus 7") pups display abnormal spasm-like myoclonus and other key EEG features, including multifocal spikes, electrodecremental episodes, and spontaneous seizures persisting into maturity. The neurobehavioral profile of Arx mutants was remarkable for lowered anxiety, impaired associative learning, and abnormal social interaction. Laminar decreases of Arx+ cortical interneurons and a selective reduction of calbindin-, but not parvalbumin- or calretinin-expressing interneurons in neocortical layers and hippocampus indicate that specific classes of synaptic inhibition are missing from the adult forebrain, providing a basis for the seizures and cognitive disorder. A significant reduction of calbindin-, NPY (neuropeptide Y)-expressing, and cholinergic interneurons in the mutant striatum suggest that dysinhibition within this network may contribute to the dyskinetic motor spasms. This mouse model narrows the range of critical pathogenic elements within brain inhibitory networks essential to recreate this complex neurodevelopmental syndrome.
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PMID:A triplet repeat expansion genetic mouse model of infantile spasms syndrome, Arx(GCG)10+7, with interneuronopathy, spasms in infancy, persistent seizures, and adult cognitive and behavioral impairment. 2012 36

A female infant suffered from epilepsy since the neonatal period, which evolved into West syndrome at the age of 2 months. Spasms in series and hypsarrhythmia disappeared after treatment with high-dose phenobarbital; however, single spasms persisted with right-sided predominance, and polyspike activity in the left parieto-temporal areas preceded or coincided with these spasms. Magnetic resonance imaging revealed a small calcification in the right occipital area, and positron emission tomography showed hypometabolism over the right hemisphere. Widespread epileptic discharges gradually increased on electroencephalography (EEG) during sleep thereafter. The patient presented with daytime unresponsiveness at 1 year and 6 months, when diffuse, irregular spike and wave activity characterized the waking EEG. Spasms or brief tonic seizures with right-sided predominance were provoked by auditory stimuli during this period, particularly by her mother's voice, with ictal EEG of right posterior predominant fast activity and subsequent desynchronization. The administration of clobazam resulted in the marked improvement of EEG findings and transient disappearance of spasms. Presumably, certain patients with asymmetrical epileptic spasms may be regarded as a unique type of localization-related epilepsy, and can show an unusual course of evolution in comparison to other cases of epilepsy that evolve after West syndrome.
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PMID:Non-convulsive status epilepticus and audiogenic seizures complicating a patient with asymmetrical epileptic spasms. 1973 86

Infantile spasms are characterized by age-specific expression of epileptic spasms and hypsarrhythmia and often result in significant cognitive impairment. Other epilepsies or autism often ensue especially in symptomatic IS (SIS). Cortical or subcortical damage, including white matter, have been implicated in the pathogenesis of SIS. To generate a model of SIS, we recreated this pathology by injecting rats with lipopolysaccharide and doxorubicin intracerebrally at postnatal day (P) 3 and with p-chlorophenylalanine intraperitoneally at P5. Spasms occurred between P4 and 13 and were associated with ictal EEG correlates, interictal EEG abnormalities and neurodevelopmental decline. After P9 other seizures, deficits in learning and memory, and autistic-like behaviors (indifference to other rats, increased grooming) were observed. Adrenocorticotropic hormone (ACTH) did not affect spasms. Vigabatrin transiently suppressed spasms at P5. This new model of SIS will be useful to study the neurobiology and treatment of SIS, including those that are refractory to ACTH.
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PMID:A model of symptomatic infantile spasms syndrome. 1994 33

Rufinamide is an orally active, structurally novel compound (1-[(2,6-difluorophenil1) methyl1]-1 hydro 1,2,3-triazole-4 carboxamide), which is structurally distinct from other anticonvulsant drugs. It was granted orphan drug status for the adjunctive treatment of Lennox-Gastaut syndrome (LGS) in the United States in 2004, and released for use in Europe in 2007. In January 2009, rufinamide was approved by the United States Food and Drug Administration for treatment of LGS in children 4 years of age and older. It is also approved for adjunctive treatment for partial seizures in adults and adolescents. Rufinamide's efficacy mainly against atonic/tonic seizures in patients with LGS seems nowadays indubitable and has been confirmed both in randomized controlled trial and in open label extension studies. More recently, rufinamide was evaluated for the adjunctive treatment of childhood-onset epileptic encephalopathies and epileptic syndromes other than LGS, including epileptic spasms, multifocal epileptic encephalopathy with spasm/tonic seizures, myoclonic-astatic epilepsy, Dravet syndrome and malignant migrating partial seizures in infancy. This review updates the existing literature data on the efficacy and safety/tolerability of rufinamide in childhood-onset epilepsy syndromes.
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PMID:Update on rufinamide in childhood epilepsy. 2179 6

Intermittent vagus nerve stimulation can reduce the frequency of seizures in patients with refractory epilepsy. Stimulation of vagus nerve afferent fibers can also cause vocal cord dysfunction, laryngeal spasm, cough, dyspnea, nausea, and vomiting. Vagus nerve stimulation causes an increase in respiratory rate, decrease in respiratory amplitude, decrease in tidal volume, and decrease in oxygen saturation during periods of device activation. It usually does not cause an arousal, or a change in heart rate or blood pressure. Most patients have an increase in their apnea-hypopnea index (AHI). Patients with VNS can have central apneas, obstructive hypopneas, and obstructive apneas. These respiratory events can be reduced with changes in the vagus nerve stimulator operational parameters or with the use of CPAP. In summary, there are complex relationships between epilepsy and obstructive sleep apneas. In particular, patients with refractory epilepsy need assessment for undiagnosed and untreated obstructive sleep apnea before implantation of vagus nerve stimulator devices. Patients with vagus nerve stimulators often have an increase in apneic events after implantation, and these patients need screening for sleep apnea both before and after implantation.
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PMID:Obstructive sleep apnea and respiratory complications associated with vagus nerve stimulators. 2189 79

This prospective, open label, uncontrolled study was performed to evaluate the efficacy and tolerability of the modified Atkins diet in children with refractory infantile spasms. Fifteen consecutive children aged six months to three years having daily infantile spasms in clusters with electroencephalographic evidence of hypsarrhythmia despite treatment with hormonal treatment (oral corticosteroids/adrenocorticotrophic hormone) and/or vigabatrin, and at least one additional anti-epileptic drug were enrolled. Children with known or suspected inborn errors of metabolism or systemic illnesses were excluded. Carbohydrate intake was restricted to ten grams/day. Among these 12 boys and three girls (median age-24 months), 13 had symptomatic etiology. After three months of diet, six children were spasm free. The time to spasm freedom after diet initiation ranged from two days to two months. The most frequent adverse effect observed was constipation. The modified Atkins diet was found to be effective and well tolerated in children with refractory infantile spasms (ClinicalTrials.gov identifier: NCT01006811).
Seizure 2012 Jan
PMID:Use of the modified Atkins diet in infantile spasms refractory to first-line treatment. 2192 Jul 81


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