Non-epileptic Paroxysmal Events Research Articles

Infantile and early childhood masturbation: Sex hormones and clinical profile

BACKGROUND AND OBJECTIVES:Few studies have explored the hormonal triggers for masturbation in infants and young children. Thus, we aimed to study the sex hormones and clinical profiles of masturbating infants and young children.METHODS:This case-control study involved infants and young children who masturbate and were referred to three pediatric neurology clinics between September 2004 and 2006 (n=13), and a similar control group. All children underwent basic laboratory investigations prior to referral. Other tests included electroencephalography (n=8) and brain neuroimaging (n=9). We measured dehydroepiandrosterone sulfate, 17-hydroxyprogesterone, free testosterone, estradiol, dehydroepiandrosterone, sex hormone-binding globulin (SHBG), and androstenedione in all participants.RESULT:The median age at the first incident was 19.5 months (range, 4-36 months); the median masturbation frequency, 4 times/day; and the median duration of each event, 3.9 min. The subjects masturbated in both prone (n=10) and supine positions (n=3); two subjects used the knee-chest position. All subjects showed facial flushing; 6, friction between the thighs; 5, sweating; 9, sleeping after the event; and 12, disturbance on interruption. EEG was abnormal in one of eight subjects tested, and neuroimages were normal in all of nine subjects examined. The case and control groups had comparable levels of all sex hormones, except estradiol, which showed significantly lower levels in the case group (P=.02).CONCLUSION:Masturbation in children seems to be associated with reduced estradiol levels, but not with other sex hormones. Further studies are needed to confirm our findings.

Evidence of a non-progressive course of alternating hemiplegia of childhood: study of a large cohort of children and adults

Alternating hemiplegia of childhood is a neurological disorder characterized by episodes of hemiplegia, various non-epileptic paroxysmal events and global neurological impairment. Characterization of the evolution and outcome into adulthood has not been sufficiently investigated. The goal of this study was to elucidate the natural history of alternating hemiplegia within a large cohort of 157 patients, as part of the European Network for Research on Alternating Hemiplegia project. A questionnaire was formulated to determine the severity of both paroxysmal and global neurological impairment and address progression of the disorder by allocating data to specific age epochs up to and over 24 years of age. Patients in early age groups were consistently present in subsequent later age groups and for each patient, data were collected for each corresponding age epoch. The study was based on predominantly retrospective and, for a period of 2 years, prospective data. At inclusion, patients were aged from 9 months to 52 years. The median age at diagnosis was 20 months. All patients experienced hemiplegic attacks; 86.5% reported episodes of bilateral weakness, 88% dystonic attacks, 53% epileptic seizures, 72% developed chorea and/or dystonia and 92% mental retardation. When data over the course of the illness were examined for the whole cohort, the severity of symptoms did not appear to change, with the exception of abnormal ocular movements and hypotonia that regressed, but did not disappear into adulthood (from 86 to 36% and 76 to 36%, respectively). No statistically significant correlation between a history of severe paroxysmal hemiplegic/dystonic episodes and a worse neurological outcome was identified. Seven patients died, some of whom experienced severe plegic attacks or epileptic seizures at the time of death. History of severe plegic/dystonic attacks was not found to be an aggravating factor for deceased patients. Our results provide evidence that the natural history of alternating hemiplegia is highly variable and unpredictable for individual patients. However, we did not find evidence to support a steadily progressive and degenerative course of the disorder when patients were analysed as a group. For a minority of patients, a risk of sudden death was associated with more severe neurological impairment. The European Network for Research on Alternating Hemiplegia Registry, validated by our study, includes all major neurological signs and symptoms of alternating hemiplegia and may thus be used as a precedent for the progressive inclusion and follow-up of patients as well as a reference for genetic studies and treatment trials.

Evolution of hemiplegic attacks and epileptic seizures in alternating hemiplegia of childhood

To delineate the evolution of non-epileptic and epileptic paroxysmal events in alternating hemiplegia of childhood (AHC), we reviewed clinical information of nine patients (4-40 years) with AHC. Paroxysmal abnormal ocular movements, head turning, and tonic, clonic, or myoclonic limb movements were the initial symptoms (birth-8m) in each patient. Ictal electroencephalography (EEG) of these episodes, as well as hemiplegic periods that accompanied these symptoms later in infancy showed unremarkable findings or generalized slow background activity. Presumptive epileptic seizures appeared at 2-16y in seven patients: generalized tonic, clonic, myoclonic, tonic-clonic, or complex partial seizures often accompanied by cyanosis or prolonged respiratory arrest. Ictal EEGs recorded in four patients revealed focal slow or fast activities during facial or limb twitching, and widespread sharp waves or polyspike-wave activities during clonic/myoclonic seizures. Four patients with neonatal disease onset showed lower psychomotor developmental achievements compared with other patients, and experienced repeated status epilepticus followed by progressive deterioration. Cerebellar atrophy and hippocampal high signal changes on magnetic resonance imaging were common to this group with severe phenotypes. Apart from the paroxysmal motor symptoms accompanying the hemiplegic episodes, many AHC patients suffer from true epilepsies during childhood. Status epilepticus in AHC is linked to severe outcome with psychomotor deterioration. The variations in clinical phenotypes may imply multiple causative genes for AHC. This variation should be considered while managing patients with this disorder.

The spectrum of movement disorders in Glut‐1 deficiency

To assess the spectrum of movement disorders, we reviewed video recordings and charts of 57 patients with Glut-1 deficiency. Eighty-nine percent of patients with Glut-1 deficiency syndrome had a disturbance of gait. The most frequent gait abnormalities were ataxic-spastic and ataxic. Action limb dystonia was observed in 86% of cases and mild chorea in 75%. Cerebellar action tremor was seen in 70% of patients, myoclonus in 16%, and dyspraxia in 21%. Nonepileptic paroxysmal events occurred in 28% of patients, and included episodes of ataxia, weakness, Parkinsonism and nonkinesogenic dyskinesias. The 40 patients (70%) who were on the ketogenic diet had less severe gait disturbances but more dystonia, chorea, tremor, myoclonus, dyspraxia, and paroxysmal events compared with the 17 patients on a conventional diet. Poor dietary compliance and low ketonuria appear to trigger the paroxysmal events in some patients. Gait disturbances and movement disorders are frequent in patients with Glut-1 deficiency and are signs of chronic and intermittent pyramidal, cerebellar and extrapyramidal circuit dysfunction. These clinical symptoms reflect chronic nutrient deficiency during brain development and may be mitigated by chronic ketosis.

Nonepileptic paroxysmal events in a pediatric population

Nonepileptic paroxysmal events in children are common, and may be more diverse in etiology than those seen in adults. We determined the types of nonepileptic events that are most prominent in the pediatric population, and stratified those events by age group. Ninety-four of 416 pediatric patients monitored during a 3-year period (23%) were found to have had nonepileptic events. Thirty-eight percent of these children were diagnosed with psychogenic nonepileptic seizures, and 72% of those were adolescents. In children younger than 5 years of age, behavioral events and parasomnias were the most common mimickers of epilepsy. Other events, including stereotyped movements and myoclonus, were also diagnosed. We suggest that children with refractory paroxysmal events should be considered for early inpatient monitoring.

Familial frontal lobe epilepsy and its relationship with other nocturnal paroxysmal events

Familial frontal lobe epilepsy and its relationship with other nocturnal paroxysmal events

Role of short-term out patient video electroencephalography in diagnosis of paroxysmal disorders

To distinguish epileptic seizures from non-epileptic paroxysmal events, we often use video electroencephalography (EEG) recording. Long-term video electroencephalography recording for inpatients is of good value, but, it necessitates much resources (financial and manpower). There is some evidence suggesting that short-term video EEG (STVEEG) is useful, but its role in practice has yet to be evaluated. To assess the usefulness of STVEEG in the diagnosis of different paroxysmal disorders and classifying epilepsy if present. Thirty sex patients had STVEEG recordings (for 2h) during a 15 months period. A diagnostic event was recorded in 21 of 36 (58.3%) within the first 2h of monitoring: PNES (n=11), epilepsy (n=5), and other paroxysmal disorders e.g. syncope, hemifacial spasm, movement disorder (n=5). STVEEG is a useful diagnostic technique in diagnosis of different paroxysmal disorders and it can be used as a successful screening test for saving other resources like prolonged inpatient video EEG recording.

Conduite à tenir devant une première crise épileptique du nourrisson et de l’enfant

Seizures are the most common pediatric neurologic disorder. This article describes the guidelines of the French Pediatric Neurology Society, highlighting the importance of a thorough history and examination. Paroxysmal nonepileptic events should be excluded. The role of biological and neuroradiological investigations is discussed. An electroencephalographic recording and advice from a pediatric neurologist are suggested.

Recognition of Nonepileptic Events

Nonepileptic paroxysmal events are behavioral, motor, or sensory episodes that do not result from abnormal cortical electrical activity. They can mimic any type of epileptic seizures including simple partial, complex partial, and generalized tonic-clonic seizures. Nonepileptic paroxysmal events may be physiological or psychogenic in nature. In clinical practice, the most common imitators of epileptic seizures are syncope and psychogenic seizures, but transient ischemic attacks, migraine, movement disorders, and metabolic disturbances must be considered at times in the differential diagnosis. In most cases, the clinical history is enough to make a correct diagnosis. The clinical features suggestive of various types of nonepileptic paroxysmal events, together with useful diagnostic tests, will be reviewed.

Usefulness of video-EEG monitoring in paroxysmal nonepileptic events of children and adolescents

Purpose : In addition to epileptic seizures (ES), a variety of physiologic, organic and psychogenic disorders can manifest as paroxysmal behavioral events. Paroxysmal nonepileptic events (PNEs) are quite encountered in infants, young children, and adolescents. In a substantial proportion of cases, a careful history and examination will elucidate their nature. However, in other cases, it is necessary to differentiate PNEs from ES by video-electroencephalographic (EEG) monitoring. We report our experiences with PNEs in a group of children and adolescents who underwent video-EEG monitoring. Methods : From September, 2004 to June, 2006, one hundred thirty patients were monitored in the Pediatric Epilepsy Monitoring Units of Korea University Guro and Ansan hospitals. Their hospital charts were reviewed and video records of these events were analyzed. We observed all patients after videoEEG monitoring for more than 3 months. Results : Typical spells occurred during monitoring in 33 patients, not associated with a seizure pattern on EEG recordings. Two patients were diagnosed as frontal lobe epilepsy on basis of typical semiology and clinical characteristics, so 31 patients were documented to have PNEs finally. The mean age of patients was 7.2±5.8 years. The male to female ratio was 15 (48.4%) to 16 (51.6%). Among 31 patients, fifteen patients had associated disorders such as epilepsy, developmental delay, cerebral palsy, gastric ulcer, attention deficit hyperactivity disorder or depressive disorder. Somatoform disorder and factitious disorder was frequently seen in children more than 5 years old (P 0.05). Conclusion : Our study suggests that video-EEG monitoring is an important diagnostic tool in the evaluation of paroxysmal behavioral events. With correct diagnosis of the PNEs, several unnecessary treatment could be avoided. (Korean J Pediatr 2008;51:62-66)

Movement disorders in sleep: Guidelines for differentiating epileptic from non-epileptic motor phenomena arising from sleep

Seizures, namely in certain epileptic conditions, may be precipitated by sleep. Nocturnal frontal lobe epilepsy seizures, characterized by bizarre motor behaviour and autonomic activation, appear almost exclusively during sleep. The differential diagnosis between this condition and sleep-related non-epileptic paroxysmal motor phenomena, in particular the parasomnias, is arduous. Moreover, accepted criteria for the diagnosis of nocturnal frontal lobe seizures are lacking and even ictal scalp EEG recording could fail to disclose paroxysmal abnormalities. The clinical and polygraphic features of the different types of seizures in nocturnal frontal lobe epilepsy and of the more common non-epileptic paroxysmal events during sleep are described. The main differentiating features characterizing nocturnal frontal seizures are: onset at any age, several attacks per night at any time during the night, brief duration (s) with stereotyped motor pattern. As video-polysomnographic recordings of the attack, the gold-standard for diagnosis, are expensive and not readily available everywhere, home-made video recordings may be helpful. Further investigations on pathophysiology, genetics and epidemiology are needed to clarify the relationship between epileptic and non-epileptic sleep related paroxysmal phenomena.

Non-epileptic paroxysmal events during sleep - Differentiation from epileptic seizures -

This review describes the wide spectrum of paroxysmal events during sleep in infancy and childhood. The differential diagnosis between sleep-related non-epileptic paroxysmal events and epileptic seizures is difficult in special occasions. The nocturnal frontal lobe seizure and of the more common nonepileptic paroxysmal events during sleep are described. The main differentiating features characterizing parasomnias are: onset in early childhood, rare episodes of long duration, relatively lower frequency per night, absence of stereotypy, gradual disappearance of older age. Video-polysomnography is the gold standard to diagnosing and differentiating parasomnias from nocturnal frontal lobe seizures.

Paroxysmal Nonepileptic Events of Childhood

The paroxysmal nonepileptic events of childhood are a group of disorders, syndromes, and phenomena that mimic true epileptic seizures. Clinical experience and a clear description of the event in question will usually lead to a correct categorization. They span in age from neonate to young adult and are apt to be the most common diagnostic challenges clinicians face regularly. The key to diagnosis is a detailed history and careful observation. Despite the large number of discrete entities enumerated herein, common principles in clinical approach are successful and described. Each entity can pose a significant clinical challenge in identification, etiologic pathophysiology, genetics, and management. A simple division is offered here separating those episodes that are associated with an altered mental status or occurring during sleep and those without an altered mental status or occurring while awake.

Nonepileptic paroxysmal disorders in childhood

Epilepsy is the most common paroxysmal disorders seen in the childhood. But other nonepileptic paroxysmal events are confused with epileptic seizures or have unusual clinical features. Nonepileptic paroxysmal disorders tend to recur episodically. So differential diagnosis between epileptic and nonepileptic disorders in fundamental not only to allow correct management of patients and but also avoid of unnecessary antiepileptic medications. To accurate diagnosis of nonepileptic paroxysmal disorders, the patients' age and accurate description of the events are need.

Paroxysmal Non-Epileptic Events Resembling Seizures in Children with Otitis Media

Children may frequently present with recurrent spells, which are often thought to be seizures. However, there are several benign non-epileptic conditions that mimic seizures. A group of 8 children is described who were diagnosed with otitis media and presented with recurrent, stereotypic spells suggestive of seizures. A paroxysmal phenomenon, in association with otitis media, resembling seizures, has not been previously described. These paroxysmal phenomena resembling seizures may be related to acute otitis media and should be included in the differential diagnosis of paroxysmal non-epileptic events in infants and young children.

Neonatal Seizures and EEG

After completing this article, readers should be able to: 1. Distinguish between epileptiform activity and an electrographic seizure. 2. List clinical events that are most likely and least likely to be associated with electrographic seizures. 3. Define electroclinical dissociation and uncoupling. 4. List interictal EEG abnormalities associated with epileptic seizures. The current terminology used to define a seizure is confusing. Using strict terminology, a “seizure” is a sudden clinical event that has an outward change in neurologic function. (1) Pathophysiologically, a “seizure” may or may not have an epileptic basis, with electroencephalography (EEG) used primarily to differentiate these. An epileptic event is caused by a hypersynchronous neuronal discharge that is seen on EEG as an electrographic seizure. In newborns, however, many clinicians use the term neonatal seizure synonymously with epileptic seizure, thus assuming an epileptic mechanism. The International League Against Epilepsy glossary for seizure classification now uses the descriptor “epileptic” with seizure, (2) which specifically assigns an epileptic mechanism to the clinical event. In older children and adults, determining whether a seizure has an epileptic basis is more straightforward because of a greater association between epilepsy and EEG readings. In the older child, there also is a greater realization that nonepileptic events exist, with specific terminology used, including nonepileptic paroxysmal events and paroxysmal nonepileptic events. (3) The distinction between an epileptic event and a nonepileptic event is not so straightforward in the newborn. Neonates exhibit a broad repertoire of clinical behaviors (events) that are termed seizures. (4) In the article on movement disorders that also appears in this issue of NeoReviews , it is noted that tremor, myoclonus, or dyskinesias may represent seizures, and EEG is used for the evaluation. Determining whether neonatal seizures have an epileptic basis is, therefore, critical for management and determination of whether an antiepileptic drug (AED) is needed. …

Epilepsy in the elderly.

Epilepsy is one of the most common neurologic diseases that affect the elderly population. Underlying etiologies as well as diagnostic and treatment issues vary from that of younger adults and deserve special consideration. The substantially increased risk of seizures and epilepsy in the elderly is associated with medical conditions that affect this group such as stroke, dementia, and metabolic disturbances. In addition, there is an increased incidence and associated mortality of status epilepticus among seniors. Distinguishing epilepsy from paroxysmal nonepileptic events can be a particular challenge. As in the general adult population, EEG and MRI are the cornerstones of diagnostic assessment; however, the clinician must be aware of nonspecific changes associated with aging that do not necessarily indicate an underlying predisposition for epilepsy. Finally, there are unique challenges to the treatment of epilepsy in this population, but fortunately there are multiple treatment options available, including nonpharmacological therapies. Knowledge of the unique challenges in identifying and treating the elderly patient with epilepsy is important for effective management as well as maximizing quality of life. However, further studies in this area are still needed to establish optimal treatment strategies.

Assessment of the utility of paediatric electroencephalography

Electroencephalography (EEG) is an important tool in investigating children with neurological disorders, particularly epilepsy. The objectives were to examine the relationship between clinical indications and EEG results, and assess the predictability of a normal result. 438 consecutive paediatric EEGs were included prospectively. One certified electroencephalographer (EEGer) reviewed EEG requisitions and recorded his prediction of a normal result. EEGs were reviewed separately and the relationship between the clinical indications and EEG abnormalities was recorded. The children’s mean age was 5 years (SD 4.2). Paediatric neurologists ordered 32% of EEGs. The first EEG was studied in 65% of cases. Overall, 55% of the EEGs were abnormal. Repeat EEGs were twice as likely to be abnormal (95% CI 1.3–3, P= 0.001). Established epilepsy, using antiepileptic drugs, and sleep record, highly correlated with an abnormal result ( P< 0.0001). The EEGer predicted 26% of the EEGs to be normal. A normal EEG was correctly predicted in 97% of non-epileptic paroxysmal events, however, normalization of EEG was correctly predicted in only 54% of children with seizures. EEGs of 15 (3.4%) children with epilepsy revealed unexpected findings that completely changed their management. To conclude, a normal EEG is highly predictable in non-epileptic paroxysmal events. EEGs of children with epilepsy are not predictable and may yield unexpected results.

Continuous-display four-channel electroencephalographic monitoring in the evaluation of neonates with paroxysmal motor events.

The objective of this study was to determine the value of continuous-display four-channel electroencephalographic (EEG) monitoring to distinguish epileptic front nonepileptic paroxysmal motor events. Five full-term neonates with paroxysmal motor events were included in the study. Nurses were instructed to print contiguous pages of the continuous-display four-channel EEG monitoring and to activate the event marker on the simultaneously conducted continuous video-EEG telemetry unit during each paroxysmal motor event. The printouts from the continuous-display four-channel EEG monitoring were interpreted and compared with the corresponding segments of continuous video-EEG telemetry. Thirty paroxysmal motor events were captured. Sixteen paroxysmal motor events were epileptic, and 14 were nonepileptic. The interpretation of the printouts of the continuous four-channel EEG monitoring concurred with an independent interpretation of the corresponding video-EEG telemetry segments in all of the events. Continuous-display four-channel EEG monitoring is a valuable tool in the evaluation of neonates with paroxysmal motor events since it reliably distinguishes epileptic and nonepileptic events.

How often does routine pediatric EEG have an important unexpected result?

Electroencephalogram recordings are requested for the assessment of many childhood disorders. To assess the utility of the EEG in children, we studied how often routine EEG results can be correctly predicted from the EEG requisition. Five hundred consecutive initial EEG requests from the IWK Grace Health Centre from two time epochs were examined. All EEGs were 16 channel (10-20 electrode system). Based only on the requisition (patient demographics, referring physician, and reason for EEG), we coded our prediction of the result and then the actual result. When results were discordant from prediction, a judgment was made about the potential importance of the result. Overall, EEG results were correctly predicted in 81%. Prediction for all nonepilepsy reasons was accurate in 91% (n=320) and 96% for paroxysmal nonepileptic events (n=158) but only 59% for epileptic disorders (n=141) (p<0.0001). Neurologists ordered 45% of EEGs, pediatricians 32%, and GP's 17%. Predictions were least accurate for neurologists' requests (p<0.006) however, neurologists were more likely to request EEG for epileptic disorders (p<0.0001). Age of the child and urban versus rural address did not affect the accuracy of prediction. Results of routine pediatric EEG for most nonepilepsy reasons appear highly predictable and therefore, possibly of little value to an experienced clinician. When requested for epilepsy, this "ancient" test remains full of surprises.