Non-epileptic Paroxysmal Events Research Articles

CACNA1A-associated epilepsy: Electroclinical findings and treatment response on seizures in 18 patients

CACNA1A pathogenic mutations are involved in various neurological phenotypes including episodic ataxia (EA2), spinocerebellar ataxia (SCA6), and familial hemiplegic migraine (FHM1). Epilepsy is poorly documented. We studied 18 patients (10 males) carrying de novo or inherited CACNA1A mutations, with median age of 2,5 years at epilepsy onset. Eight mutations were novel. Two variants known leading to gain of function (GOF) were found in 5 patients. Five other patients had non-sense variants leading to loss of function (LOF). Seizures were most often revealed by either status epilepticus (SE) (n = 8), eventually triggered by fever (n = 5), or absences/behavioural arrests (n = 7). Non-epileptic paroxysmal events were frequent and consisted in recurrent hemiplegic accesses (n = 9), jitteriness in the neonatal period (n = 6), and ocular paroxysmal events (n = 9). Most of the patients had early permanent cerebellar dysfunction (n = 16) and early moderate to severe global developmental delay (GDD)/intellectual deficiency (ID) (n = 17). MRI was often abnormal, with cerebellar (n = 8) and/or cerebral (n = 6) atrophy. Stroke-like occurred in 2 cases. Some antiepileptic drugs including topiramate, levetiracetam, lamotrigine and valproate were effective on seizures. Acetazolamide and calcium channel blockers were often effective when used. More than half of the patients had refractory epilepsy. CACNA1A mutation should be evoked in front of 2 main electro-clinical phenotypes that are associated with permanent cerebellar dysfunction and moderate to severe GDD/ID. The first one, found in all 5 patients with GOF variants, is characterized by intractable seizures, early and recurrent SE and hemiplegic accesses. The second, less severe, found in 5 patients with LOF variants, is characterized by refractory early onset absence seizures.

Profiles of Indigenous Epileptic Patients in a Brazilian Village

Background: Epilepsy affects 50 million people worldwide, most of whom are low-income countries. Patients with epilepsy who live in socially isolated communities often require more medical attention. In Brazil, the estimated prevalence of epilepsy is approximately 2 million. Methods: This descriptive, cross-sectional study investigated the incidence of epilepsy among indigenous people residing in Jaguapiru Village, Dourados, Mato Grosso do Sul, Brazil. We reviewed the clinical histories of the participants and the results of electroencephalography (EEG), neuroimaging, and/or laboratory tests. Additionally, patients with nonepileptic paroxysmal events were identified. Findings: Of the 2,994 respondents, 49 had a confirmed diagnosis of epilepsy, and 22 had self-limited epileptic syndromes; these numbers exceeded the global prevalence but were similar to those reported in the Brazilian population. Focal epilepsy, predominantly of temporal lobe origin, was the most prevalent epileptic syndrome (71.4%). Febrile seizures occurred in only 0.4% of respondents -- much lower than that reported in the general population, which can be attributed to the cross-sectional study design. The aetiology of epilepsy was undetermined in 82.6% of cases, with the remaining cases attributed to head trauma, hypoxic-ischaemic encephalopathy, and brain malformations. A history of delayed neuropsychomotor development was significantly associated with epilepsy. Monotherapy with first-generation antiepileptic drugs was greatly effective in most epilepsy cases (87.7%). EEG helped to define focal epilepsies and diagnose seizures of nonepileptic origin; the latter mostly included cases of psychogenic crises and, less frequently, syncope. Interpretation: Identifying the epidemiological determinants of epilepsy in traditional communities is essential to establish public policies in countries such as Brazil. Funding: This study was conducted without external financial support. EEG was performed using the authors’ own equipment. Travel to the village for questionnaire application was funded by the authors as well. Imaging examinations (cranial computed tomography, magnetic resonance imaging) and laboratory tests, when performed, were provided by the National Health System. Declaration of Interest: The authors declare no financial conflicts of interest. Ethical Approval: This study was performed between November 2012 and November 2017 and was approved by the Research Ethics Committee Involving Human Beings of the Federal University of Grande Dourados and by the National Research Ethics Commission (CONEP; references 6810 and 64479, respectively), as well as the appropriate regulatory bodies and indigenous authorities. All of the participants or their legal representatives signed a free and informed consent form translated into the Guarani-Kaiwa language.

The influence of the abundance and morphology of epileptiform discharges on diagnostic accuracy: How many spikes you need to spot in an EEG

ObjectiveThe operational definition of interictal epileptiform discharges (IEDs) of the International Federation of Clinical Neurophysiology (IFCN) described six morphological criteria. Our objective was to assess the impact of pattern-repetition in the EEG-recording, on the diagnostic accuracy of using the IFCN criteria. For clinical implementation, specificity over 95% was set as target. MethodsInterictal EEG-recordings of 20-minutes, containing sharp-transients, from 60 patients (30 with epilepsy and 30 with non-epileptic paroxysmal events) were evaluated by three experts, who first marked IEDs solely based on expert opinion, and then, independently from the first session evaluated the presence of the IFCN criteria for each sharp-transient. The gold standard was derived from long-term video-EEG recordings of the patientś habitual paroxysmal episodes. ResultsPresence of at least one discharge fulfilling five criteria provided a specificity of 100% (sensitivity: 70%). For discharges fulfilling fewer criteria, a higher number of discharges was needed to keep the specificity over 95% (5 discharges, when only 3 criteria were fulfilled). A sequential combination of these sets of criteria and thresholds provided a specificity of 97% and sensitivity of 80%. ConclusionsPattern-repetition and IED morphology influence diagnostic accuracy. SignificanceSystematic application of these criteria will improve quality of clinical EEG interpretation.

Her İstemsiz Hareket Epileptik midir?

Paroxysmal nonepileptic events are episodic changes in behavior, sensation, or consciousness that are similar to epileptic seizures but not associated with abnormal ictal brain electrophysiological discharges. Here, a case treated as epileptic seizure was presented in order to draw attention to paroxysmal nonepileptic events in differential diagnosis.
 A 4 years old girl sent to our hospital with the diagnose of status epilepticus due to change in her consciousness, contractions and abnormal movements in her body, arms and legs those started after taking 6 spoonfull syrup of Peditus® (Containing 120 mg paracetamol, 50 mg guaifenesin, 6.25 mg pyrilamine maleate, 5 mg phenylephrine hcl in 5 ml scale) and 5 Medikinet® 10 mg capsules (10 mg methylphenidate hydrochloride in 1 capsule). She was conscious and cooperate and has involuntary snake-like movements throughout her body on admission. The patient's movement disturbances thought as methylphenidate-induced choreoathetosis responded to given haloperidol treatment and any sign of poisoning were not observed in the patient's follow up.
 Chorea side effects were observed in our patient but not any poisoning symptoms, who received a toxic dose of methylphenidate for her age. This suggests that methylphenidate, a central nervous system stimulant, may have therapeutic, toxic dose limits and side effects profile those associated with individual pharmacogenetic variations. Accurate distinction of chorea from drug-related paroxysmal nonepileptic events will ensure early effective treatment of patients and to protect patients from unnecessary drug risks.

Shuddering attacks in children: A retrospective analysis of 19 cases from a single-center in Turkey

BackgroundShuddering attacks (SA) are one of the most common childhood paroxysmal nonepileptic events (PNEs). These attacks usually start between the first 4th and 6th months of life with rapid tremors of the head and adduction of the arms and knees. A number of factors including eating, breastfeeding, and playing stimulating games have been shown to trigger the attacks; however, the exact pathogenesis remains unknown. It has been stated that there is no need for further research in patients diagnosed, and spontaneous regression is expected. PurposeThis study aimed to identify the causes, accompanying clinical conditions, possible differential diagnosis of SA, and the role of video-electroencephalogram (V-EEG) recording for accurate diagnosis. MethodsNineteen cases with SA have been collected from the database of Erciyes University Pediatric Neurology Clinic, where 52.6% are boys (n = 10) and 47.6% are girls (n = 9). The relationship between the onset and disappearance of SA symptoms and variables including family history, birth history, age, sleep, teething during SA, video-EEG recordings, brain imaging, and accompanying conditions such as epilepsy have been investigated by retrospective analysis. ResultsFour cases were found to have gastroesophageal reflux, one had epilepsy, and one had Marcus Gunn Jaw Winking Syndrome. No accompanying conditions could be identified for rest of the cases. It was observed that onset of symptoms in 15 (78.9%) of 19 cases coincided remarkably with the period of teething. ConclusionWe speculate that there might be an indirect link between SA and teething and teething may be a triggering or an aggravating factor for SA.

Non-epileptic paroxysmal events in paediatric patients: A single tertiary centre study in Egypt

PurposeThe misdiagnosis of non-epileptic seizures (NES) as epilepsy is one of the most common pitfalls in neuropsychiatric practice. This study aimed to describe the percentage and types of NES among children who were referred for a diagnosis of epilepsy in Upper Egypt. MethodsWe recruited a total of 876 patients who were referred to Sohag University Hospital, a tertiary referral centre in Upper Egypt, for the evaluation of suspected epilepsy. Relevant methods for the diagnosis of epilepsy, including medical history and examination, EEG, video-EEG, laboratory investigations, and brain imaging, were performed for all study participants. ResultsAmong the 876 patients who were referred for the diagnosis of suspected epilepsy during the period from June 2017 to October 2018, 171 patients (19.5 %) were diagnosed as having NES. In general, we found that NES in the paediatric age groups did not differ from that reported in various studies across several different populations. The most prevalent NES in our study was breath-holding spells (32.2 %), followed by syncope (17.5 %), psychogenic nonepileptic seizures (12.3 %), motor tics (9.9 %), and benign sleep myoclonus (7.6 %). Other less frequent NES included infantile masturbation (7 %), spasmus nutans (5.3 %), migraine (2.9 %), benign paroxysmal torticollis (2.9 %), night terrors (1.8 %), and shuddering attacks (0.6 %). ConclusionIdeally, neurologists should not misdiagnose NES as epilepsy, and whenever the diagnosis of NES is uncertain, an accurate diagnosis should be made using long-term video-EEG monitoring, especially in younger paediatric patients.

Indications and yield of ambulatory EEG recordings.

To study the yield of prolonged ambulatory electroencephalogram (aEEG). A retrospective chart review of all patients who underwent aEEG studies between 2013 and 2017 was performed. Reasons for aEEG were classified into five categories: detection of interictal epileptiform discharges (IEDs), capturing clinical events, detection of unrecognized seizures, monitoring IEDs during treatment, and unclassifiable. Ambulatory EEG reports were reviewed to evaluate whether the study answered the clinical question. A total of 1,264 patients were included. Forty studies were excluded for incomplete data and 234 for being a repeat study. The average number of recording days was 1.57 ± 0.73. Based on initial clinical evaluation, patients carried the following presumptive diagnosis: 61% epilepsy, 11% single unprovoked or acute symptomatic seizure and 28% non-epileptic paroxysmal events (PEs). Overall, focal IEDs were seen in 16.1% of studies, generalized IEDs in 10.8%, focal seizures in 4.1%, and generalized seizures in 1.9%. The most frequent reason for ordering aEEG was to detect IEDs for diagnostic purposes (48.1%). For this indication, additional information was provided by the aEEG in 19.1% of cases (58.6% focal IEDs, 33.5% generalized IEDs, 7.9% seizures without IEDs). Ambulatory EEG was ordered with the intent to capture and characterize clinical events in 18.9%, mostly in patients who reported daily or weekly events. In these, aEEG captured either epileptic seizures or PEs in 102 (42.7%) of the studies (83.3% PEs, 16.7% epileptic seizures). Ambulatory EEG was ordered to evaluate unrecognized seizures in 17.8% of patients, and electrographic seizures were identified in 13.3% of these studies. The yield of aEEG varies based on the indication for the study. Ambulatory EEG can be a useful tool for recording IEDs in the outpatient setting and in a select group of patients to capture clinical events or unrecognized seizures.

Role of Video-EEG in Children.

Routine electroencephalogram (EEG) has many limitations, especially the inability to capture reported habitual events in question. A prolonged EEG with synchronized video (video-EEG) overcomes some of these limitations by improving the sensitivity, specificity and the diagnostic yield by attempting to record the habitual events when they are frequent and when indicated. Video-EEG is employed commonly for the diagnosis and classification of epilepsy/epilepsy syndromes, to distinguish between seizures and seizures mimickers, for pre-surgical evaluation and in the management of critically ill children. The duration of recording would vary depending on the indication and frequency of events. Ambulatory EEG is another cost effective and convenient alternative in certain circumstances. However, availability of the machines and expertise, accessibility, affordability and labor intensive nature of the procedure limit widespread use in India. This review explores the role of video-EEG in the management of children with epileptic and non-epileptic paroxysmal events with respect to routine clinical practice in India.

Paroxysmal Nonepileptic Events in a Pediatric Epilepsy Clinic.

ABSTRACTAims:We aimed to study the frequency, age, and gender distribution of paroxysmal nonepileptic events (PNEs) in children referred to epilepsy clinic with the diagnosis of epilepsy. We also evaluated the therapeutic implications of correct diagnosis and co-existence of true epilepsy in this population.Settings and Design:All new patients below 18 years attending the Pediatric epilepsy out-patient clinic of PD Hinduja hospital over 6 months were evaluated.Materials and Methods:Patients with history of paroxysmal events characterized by abrupt changes in consciousness or behavior or movement were included. They were assessed on description of events aided by recorded videos. If the diagnosis was not confirmed by this preliminary evaluation, further investigations were advised.Statistical Analysis Used:Chi-square/Fisher’s exact test was used to analyze differences between categorical variables and Kruskal–Wallis test between continuous variables. The data were analyzed by SAS University Edition. All significance tests were two-tailed with α <0.05.Results:Two hundred new patients presenting with paroxysmal events were enrolled over 6 months. After diagnoses, 19% of these children had PNEs, 80% had epileptic events, and 1% remained undiagnosed. Common nonepileptic events seen were physiological in patients below 5 years and psychogenic in older children. Thirty-four percent of patients with PNEs were on anti-epileptic drugs (AEDs). After confirming nonepileptic attacks, only 2.6% patients needed AEDs for coexisting epilepsy which was statistically significant (P < 0.001) change in treatment.Conclusions:Epilepsy mimics are common in children and are often misdiagnosed causing undue stress. Correct diagnosis leads to a drastic change in management like withdrawal of drugs, commencing new treatment if needed, and appropriate referrals.

Development of a Risk Predictive Scoring System for Epilepsy in Infants with Paroxysmal Motor Events: A Retrospective Single-Center Study

Purpose: Paroxysmal motor events are common clinical symptoms in infants visiting pediatric neurology clinics. Due to the heterogeneous clinical symptoms and the difficulty of interpreting electroencephalography (EEG) in infants, differentiating paroxysmal motor events from epileptic events is challenging. This study aimed to investigate the risk factors for the diagnosis of epilepsy in infants and to develop a scoring system that predicts the risk of epilepsy.Methods: We retrospectively analyzed data from patients who presented with paroxysmal motor events in infancy between January 2008 and December 2009 at Asan Medical Center. Electronic medical records were reviewed for patients’ demographics, medical history, clinical characteristics associated with specific situations, and motor symptoms. Laboratory findings, EEG, and brain magnetic resonance imaging were also reviewed. Results: In total, 111 infants with paroxysmal motor events were enrolled. Non-epileptic paroxysmal motor events (NEPMs) were associated with specific situations (P&lt;0.001). Patients with epilepsy were likely to have focal motor symptoms (P=0.08), a medical history of a neurologic disorder, and/or a family history of epilepsy (P&lt;0.05). A risk scoring system was developed based on these risk factors; using this system, infants with 2 or more points could be diagnosed with epilepsy with 61.76% sensitivity and 88.31% specificity.Conclusion: Infants with paroxysmal motor events were more likely to be diagnosed with NEPMs than with epilepsy. An absence of specific situations for paroxysmal events, focal motor seizures, and a medical history of another illness were associated with the final diagnosis of epilepsy.

Neurodevelopmental outcomes of neonatal non-epileptic paroxysmal events: a prospective study.

To report on psychomotor development and outcomes in term born neonates with non-epileptic paroxysmal events (NEPEs). From October 2017 to March 2019 we enrolled 38 consecutive term born neonates (22 males, 16 females; aged between 0-28d), born at the University Hospital San Marco in Catania, Italy, with NEPEs. We performed the Hammersmith Neonatal Neurological Examination scale (at enrolment), the Hammersmith Infant Neurological Examination (HINE) scale (at age 3, 6, 9, and 12mo), and the Griffiths scale (at age 12mo). The age at onset of first paroxysmal manifestations ranged from birth to 4 days. We recorded a suboptimal global score in 18 out of 38 patients at enrolment and in 10 out of 38 patients at age 3 months (>70% of these infants were male); all events disappeared within 6 months of life. At age 6, 9, and 12 months, all infants scored within normal values on the HINE and Griffiths scale. Patients with NEPEs achieve neurodevelopment optimal scores within their first year of life. Neonates experiencing non-epileptic paroxysmal events (NEPEs) can be examined with the Hammersmith Neonatal Neurological Examination, Hammersmith Infant Neurological Examination, and Griffiths scale at follow-up. Newborn infants with NEPEs achieve optimal scores within the first year of life.

Real-life use of videos in pediatric epilepsy consultations

Paroxysmal events are usually not directly observed by physicians. The diagnosis remains challenging and relies mostly on the description of witnesses. The effectiveness of videos for seizure diagnosis has been validated by several studies, but their place in clinical practice is not yet clear. The aim of our study was to evaluate the real-life use of videos by child neurologists. We conducted a three-month prospective study in which child neurologists were asked to use a short questionnaire to evaluate all videos that were watched in their clinical practice for an initial diagnosis or during follow-up. A click-off meeting during the French pediatric neurology meeting allowed to recruit participants. A total of 165 questionnaires were completed by 15 physicians over the study period. The physicians were child neurologists working in secondary and tertiary/university hospitals, consulting children with epilepsy. Based on the evaluation of child neurologists, 51% of the videos consisted of epileptic seizures; 40%, nonepileptic paroxysmal events; and 9%, psychogenic nonepileptic seizures. Most of the videos were made on parental initiative. The use of video has modified the first diagnosis hypothesis in 35% of cases. The physicians’ feelings regarding the interest of the video used during the diagnostic phase were similar to those of the video used during follow-up. It appears that videos have become a part of the epilepsy clinic and are helpful for diagnosis as well as during follow-up. Unfortunately, one of the limitations of this study is the absence of private practitioner.

CACNA1A Gene Variants in Eight Chinese Patients With a Wide Range of Phenotypes.

Background: The CACNA1A gene encodes the voltage-dependent P/Q-type calcium channel subunit alpha-1A, which is widely expressed throughout the CNS. The biological roles of the P/Q channel are diverse and the phenotypic spectrum caused by CACNA1A mutations is wide. The aim of this study is to demonstrate its phenotypic diversity and analyze the genotype-phenotype correlations in a cohort of Chinese patients.Methods: Patients with hemiplegic migraine, cerebellar ataxia, developmental delay, or epilepsy without known causes were tested by trios whole-exome sequencing. Patients with pathogenic CACNA1A gene variants were recruited. The clinical information of the patients was collected, and the association between the genotype and the phenotype was investigated.Results: In total, eight patients (six females and two males) were found to have CACNA1A gene variants. All the variants were de novo including six missense variants and one frameshift variant. Four de novo missense variants were found in five patients located in the S4, S5, or S6 transmembrane segments of Domain II and III (p.R1352Q, p.G701V, p.A713T, p.V1393M). All of them were correlated with severe phenotypes, including three with sporadic hemiplegic migraine type 1 and epilepsy, and two with developmental and epileptic encephalopathy. The other two missense variants, p.Y62C and p.F1814L, located in the cytoplasmic side of the N-terminus and C-terminus, respectively. The variant p.Y62C was associated with severe hemiconvulsion-hemiplegia-epilepsy syndrome, and p.F1814L was associated with relatively mild phenotypes. All the missense variants were speculated as gain-of-function (GOF) mutations. The only frameshift variant, p.Q681Rfs*100, a lose-of-function (LOF) mutation, was found in a patient with episodic ataxia type 2. Meanwhile, all the patients had developmental delay ranging from mild to severe, as well as cerebellar ataxia including one with congenital ataxia, one with episodic ataxia, and six with non-progressive ataxia.Conclusions: CACNA1A variants could lead to a wide spectrum of neurological disorders including epileptic or non-epileptic paroxysmal events, cerebellar ataxia, and developmental delay. The variants could be both GOF and LOF mutations. There appeared to be some correlations between genotypes and phenotypes.

Shuddering Attacks in an Infant

AbstractShuddering attacks are rare benign nonepileptic paroxysmal events (NEPEs) seen in infancy and early childhood. These movements may look like myoclonus or infantile spasms. Recognition of these movements is important to avoid elaborate workup and antiepileptic medications. Shuddering attacks disappear by the age of 2 years in most of these children. NEPEs are almost as common as epilepsy. It is easy to differentiate the common NEPEs from epilepsy. However, it is difficult to diagnose the rare benign NEPEs not seen before. Shuddering attacks are one of these rare NEPEs. It is commonly diagnosed as infantile spasms/myoclonus unless one observes the actual event or video very carefully.

Clinical phenotypes of infantile onset CACNA1A-related disorder.

CACNA1A-related disorders present with persistent progressive and non-progressive cerebellar ataxia and paroxysmal events: epileptic seizures and non-epileptic attacks. These phenotypes overlap and co-exist in the majority of patients. To describe phenotypes in infantile onset CACNA1A-related disorder and to explore intra-familial variations and genotype-phenotype correlations. This study was a multicenter international collaboration. A retrospective chart review of CACNA1A patients was performed. Clinical, radiological, and genetic data were collected and analyzed in 47 patients with infantile-onset disorder. Paroxysmal non-epileptic events (PNEE) were observed in 68% of infants, with paroxysmal tonic upward gaze (PTU) noticed in 47% of infants. Congenital cerebellar ataxia (CCA) was diagnosed in 51% of patients including four patients with developmental delay and only one neurological sign. PNEEs were found in 63% of patients at follow-up, with episodic ataxia (EA) in 40% of the sample. Cerebellar ataxia was found in 58% of the patients at follow-up. Four patients had epilepsy in infancy and nine in childhood. Seven infants had febrile convulsions, three of which developed epilepsy later; all three patients had CCA. Cognitive difficulties were demonstrated in 70% of the children. Cerebellar atrophy was found in only one infant but was depicted in 64% of MRIs after age two. Nearly all of the infants had CCA, PNEE or both. Cognitive difficulties were frequent and appeared to be associated with CCA. Epilepsy was more frequent after age two. Febrile convulsions in association with CCA may indicate risk of epilepsy in later childhood. Brain MRI was normal in infancy. There were no genotype-phenotype correlations found.

Is it really a seizure? The challenge of paroxysmal nonepileptic events in young infants.

Paroxysmal nonepileptic events (PNE) comprise of a variety of nonepileptic behaviors and are divided into various types. A more accurate diagnosis is possible by examining the video clip provided by the caregiver. In infants, physiologic PNE accounts for the majority of the PNE. It is important to exclude epilepsy, for which blood tests, electroencephalography, and imaging tests can facilitate differential diagnosis. Since most PNE have a benign progress, symptoms often improve with age and without special treatment. Therefore, it is important to reassure the caregivers after making an accurate diagnosis.

Myoclonic tremor status as a presenting symptom of adenylosuccinate lyase deficiency

Adenylosuccinate lyase deficiency is a rare autosomal recessive disorder of purine metabolism. The disorder manifests with developmental delay, postnatal microcephaly, hypotonia, involuntary movements, epileptic seizures, ataxia and autistic features. Paroxysmal non-epileptic motor events are not a typical presentation of the disease. We describe an 8-year-old boy who presented with an infantile onset of prolonged episodes of multifocal sustained myoclonic tremor lasting from minutes to days on a background of global developmental delay and gait ataxia. Ictal EEG during these episodes was normal. Ictal surface EMG of the involved upper limb showed a muscular activation pattern consistent with cortical myoclonus. Brain MRI showed mild cerebral atrophy. Whole exome sequencing revealed a novel homozygous variant in the ADSL gene: c.1027G > A; p. Glu343Lys, inherited from each heterozygous parent. There was a marked elevation of urine succinyladenosine, confirming the diagnosis of adenylosuccinate lyase deficiency. In conclusion, myoclonic tremor status expands the spectrum of movement disorders seen in adenylosuccinate lyase deficiency.

Seizure-like events leading to hospital referrals in infants: A retrospective population-based study.

To identify the aetiology and outcome of seizure-like events leading to hospital referrals in infants and to identify early predictors of epilepsy and delayed neurodevelopment. This Norwegian population-based study focused on all children born in Sør-Trøndelag county, who were up to one year of age in 2014-2015. They were identified by diagnostic codes for seizure-like events and electroencephalography (EEG) examinations. Hospital records were examined up to 1.5years of age. The one-year prevalence of seizure-like events was 1.5% (114/7430). Epilepsy was diagnosed in 17%, 57% had non-epileptic paroxysmal events (NEPE), 16% had febrile seizures, and 10% had other acute symptomatic epileptic seizures. Neurodevelopmental delay occurred in 21%. The cumulative incidence was 0.22% for epilepsy and 0.79% for NEPE. Abnormal brain magnetic resonance imaging, abnormal first EEGs and neonatal care increased the likelihood of epilepsy and delayed development. Identifying situation-related factors decreased the epilepsy risk. Occurrence at a younger age increased the risk of delayed development. Absence of unambiguous motor symptoms was less common in epilepsy than in NEPE. Seizure-like events were common in infants and most were not caused by epilepsy. Specific anamnestic clues, and detailed descriptions of the entire event, helped to predict adverse outcomes.

Non-Epileptic Paroxysmal Events in Infants: stucture, manifestation and risk factors

During 3 years we observed in specialized neurological department of St. Olga Children’s City Hospital 708 babies aged up to 42 months with different paroxysmal disorders. Non-epileptic paroxysmal events (NEPE) were diagnosed in 98 cases. These disorders were qualified (according to ILAE criteria) as differentiated and undifferentiated NEPE. In most cases neurophysiological assessment data, neurovisualization data, and neurological status of infants with NEPE and babies from comparison group varied marginally. It was shown that NEPE development correlated with perinatal factors, and with infants’ developmental diseases (functional gastrointestinal disorders, iron deficiency states, chronic or persistent infections). The obtained data was interpreted according to the developmental neurology principles (optimality concept, in particular).

Approach to the pre-school child with sudden loss of consciousness

Abstract There are several causes for sudden loss of consciousness in pre-school children. These include Transient Loss of Consciousness (T-LOC), which encompasses a group of disorders with the following characteristics: abnormal motor control, loss of responsiveness, amnesia for the period of unconsciousness, and a short duration. There are three main categories of T-LOC in pre-school children: syncope, resulting from a sudden and reversible lack of oxygenated blood supplied to the brain, often caused by transient impairment of cardiac output or systemic arterial hypotension. Secondly, epileptic seizures due to excessive and hypersynchronous cortico-neuronal electrical activity in the brain, and thirdly, “unexplained T-LOC,” which includes cases not yet diagnosed fully, and those for which a pathophysiological mechanism has not yet been identified, despite thorough investigations. Reflex Anoxic Seizures (RAS) are important in the differential diagnosis of non-epileptic paroxysmal events in infants and pre-school children. Parents and carers who witness these episodes are understandably anxious, and the mainstay of management is ensuring that the correct diagnosis is made, reassurance is given, and both the patient and parents are educated about the condition. Diagnosis can be made based off a detailed history, including an eyewitness account of the episode, as well as clinical examinations. This review aims to describe an approach to the diagnosis and management of T-LOC in pre-school children.