Paroxysmal Movement Disorders Research Articles

P147 – 2569: Adenylosuccinate lyase deficiency presenting as a paroxysmal movement disorder

Objective To describe an atypical presentation of the Adenylosuccinate lyase (ADSL) deficiency syndrome. Methods A three year-old boy presented to our clinic because of intractable epilepsy. At the age of 10 months he had a single episode of status epilepticus. At the age of 16 months recurrent episodes of rapid semi-rhythmic jerking movements occurred, involving mostly the upper limbs and spreading homolaterally or contralaterally to other body parts. These attacks started without any apparent trigger, lasted up to a few hours, constantly disappeared during sleep and returned a few minutes after awakening. There was no change of consciousness or behavior. Some of the paroxysms were accompanied by dystonic limb or neck posturing. Ictal Electroencephalogram (EEG) did not show epileptic activity. Ictal surface Electromyography (EMG) demonstrated irregular bursts lasting less than 50 msec. with a frequency of 14 Hz. Results Whole exome sequencing revealed homozygous mutations in the ADSL gene. The diagnosis was confirmed by highly elevated urine succinyladenosine. Treatment with ketogenic diet significantly improved these paroxysmal attacks. Conclusion ADSL deficiency is a defect of purine metabolism. The disorder can present with a wide spectrum of symptoms. Epilepsy, usually intractable is the most constant feature. Our patient has a unique phenotype: only one unprovoked seizure and a paroxysmal movement disorder which is the most predominant feature.

Clinical movement disorders - a new era.

We are proud to announce the launch of a new open access journal, entitled Journal of Clinical Movement Disorders (JCMD). The aim of the journal is to offer an open access forum for the publication of scientifically rigorous articles focusing on the natural history, etiology, evaluation and treatment of patients with a wide range of movement disorders [1]. We are interested in topics important to the practicing clinician, but especially in articles that explore the ways in which translational approaches are changing the evaluation and treatment of patients with movement disorders in the 21st century. We are particularly pleased to launch JCMD as a fully open access journal. At present, only one journal devoted to movement disorders (Tremor and Other Hyperkinetic Disorders) offers an open access format. We believe that the open access format offers numerous advantages to traditional publication strategies. Articles will be published as soon as they are accepted, markedly decreasing the time for evaluation and distribution. All articles are available online at no cost throughout the world, enabling the widest possible reach for the journal irrespective of a reader’s location or economic resources [2]. Authors will continue to hold copyright for their work, and articles will be archived in PubMed Central. The online format allows articles to be published without constraints on length of text. Further, the format is a perfect vehicle for publication of videos demonstrating salient features in articles, a critical feature of clinical movement disorders. JCMD will publish articles covering the full spectrum of movement disorders including hypokinetic disorders (Parkinson’s disease, atypical parkinsonian disorders such as multiple system atrophy, progressive supranuclear palsy, corticobasal syndrome, dementia with Lewy body disease, frontotemporal dementia, etc.), and usual and unusual hyperkinetic disorders (myoclonus, chorea, tics, tremor, dystonia, stereotypies, paroxysmal movement disorders, etc.). We are interested in both descriptive articles that illustrate phenotype, as well as hypothesis driven articles that explore the etiology, genetics, pharmacology, neuroimaging and treatment options for these patients. Submissions on both adult and pediatric disorders are welcome, and we are particularly interested in novel treatments such as deep brain stimulation. Special features that we plan to integrate into the journal include a clinical phenomenology section, a section devoted to current controversies, and reviews that summarize important findings in the field. All manuscripts submitted to JCMD will be independently reviewed by two experts in the field, by a member of the Editorial Board and by the Editor-in-Chief. We are interested in publishing articles of the highest quality and novelty, but will also consider research that may be of more general interest to readers due to novel clinical or examination features, clinical relevance or unusual phenotype, even if the findings are confirmatory. We believe that clinical movement disorders is unique as a subspecialty area in neurology, in that many are drawn to the field by the complexity and subtlety of the clinical examination, and the way in which 21st century technologies can help illuminate these findings. The Editorial Board of JCMD includes 43 members from 11 different countries [3]. We believe that the broad expertise and international reputations of the Editorial Board attests to their confidence that JCMD will succeed. We hope that you enjoy JCMD, and that you will consider it when deciding where to publish your work. Welcome!

Paroxysmal dyskinesias revisited: a review of 500 genetically proven cases and a new classification.

Paroxysmal movement disorders are a heterogeneous group of conditions manifesting as episodic dyskinesia with sudden onset and lasting a variable duration. Based on the difference of precipitating factors, three forms are clearly recognized, namely, paroxysmal kinesigenic (PKD), non-kinesigenic (PNKD), and exercise induced (PED). The elucidation of the genetic cause of various forms of paroxysmal dyskinesia has led to better clinical definitions based on genotype-phenotype correlations in the familial forms. However, it has been increasingly recognized that (1) there is a marked pleiotropy of mutations in such genes with still expanding clinical spectra; and (2) not all patients clinically presenting with either PKD, PNKD, or PED have mutations in these genes. We aimed to review the clinical features of 500 genetically proven cases published to date. Based on our results, it is clear that there is not a complete phenotypic-genotypic correlation, and therefore we suggest an algorithm to lead the genetic analyses. Given the fact that the reliability of current clinical categorization is not entirely valid, we further propose a novel classification for paroxysmal dyskinesias, which takes into account the recent genetic discoveries in this field.

Pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections: an overview

The acronym PANDAS (pediatric autoimmune neuropsychiatric disorders associated with streptococcal infections) has been used to describe a syndrome characterized by various obsessions, compulsions, tics, hyperactivity, motor stereotypies, and paroxysmal movement disorders that are correlated with prior infection by group A beta-hemolytic Streptococcus pyogenes (GABHS) infections. Five clinical criteria can be used to diagnose PANDAS: (1) the presence of obsessive-compulsive disorder (OCD) and/or any other tic disorders; (2) prepuberal onset (between 3 years of age and the start of puberty); (3) abrupt onset and relapsing-remitting symptom course; (4) a distinct association with GABHS infection; and (5) association with neurological abnormalities during exacerbations (adventitious movements or motoric hyperactivity). The exact pathogenesis of PANDAS remains unclear, and several theories that focus on multiple etiologic or contributive factors have emerged. PANDAS appears to be a neurobiological disorder that potentially complicates GABHS infections in genetically susceptible individuals. The current standard of care for PANDAS patients remains symptomatic, and cognitive behavioral therapy, such as exposure and response prevention, combined with family counseling and psychoeducation, should be the first approach for treating PANDAS. This review examines current theories of PANDAS pathogenesis, identifies possible treatments for managing this complex condition, and highlights areas for future research. Moving forward, developing more standardized diagnostic criteria and identifying specific laboratory markers to facilitate PANDAS diagnoses are crucial.

Successful control with carbamazepine of family with paroxysmal kinesigenic dyskinesia of PRRT2 mutation.

Paroxysmal kinesigenic dyskinesia (PKD), a rare paroxysmal movement disorder often misdiagnosed as epilepsy, is characterized by recurrent, brief dyskinesia attacks triggered by sudden voluntary movement. Pathophysiological mechanism of PKD remains not well understood. Ion channelopathy has been suggested, since the disease responds well to ion channel blockers. Mutations in proline-rich transmembrane protein 2 (PRRT2) were recently identified in patients with familial PKD. To extend these genetic reports, we studied a family with clinical manifestations of familial PKD responding well to low dose carbamazepine. Therapeutic dose ranged from 1.5 to 2.0 mg/ kg/day, below that in seizure control. One insertion mutation c.649_650insC (p.P217fsX7) was identified in three patients of the family. This study avers PRRT2’s high sensitivity for PKD phenotype. Identification of genes underlying pathogenesis will enhance diagnosis and treatment. Function of PRRT2 and its role in PKD warrant further investigation.

GLUT1 deficiency syndrome into adulthood: a follow-up study

GLUT1 deficiency syndrome (GLUT1DS) is a treatable neurometabolic disorder in which glucose transport into the brain is disturbed. Besides the classic phenotype of intellectual disability, epilepsy, and movement disorders, other phenotypes are increasingly recognized. These include, for example, idiopathic generalized epilepsy and paroxysmal exercise-induced dyskinesia. Since the disorder has only been recognized for two decades and is mostly diagnosed in children, little is known about the disease course. Our purpose was to investigate the disease course of GLUT1DS patients with the classic, complex phenotype from infancy into adulthood. We performed a systematic literature review as well as a cohort study, including GLUT1DS patients aged 18 years and older. The literature search yielded a total of 91 adult GLUT1DS patients, of which 33 patients (one-third) had a complex phenotype. The cohort study included seven GLUT1DS patients with a complex phenotype who were prospectively followed up in our clinic from childhood into adulthood. Our results show that epilepsy is a prominent feature during childhood in classic GLUT1DS patients. During adolescence, however, epilepsy diminishes or even disappears, but new paroxysmal movement disorders, especially paroxysmal exercise-induced dyskinesia, either appear or worsen if already present in childhood. Intellectual disability was not systematically assessed, but cognitive functions appeared to be stabile throughout life. Like children, adolescents may benefit from a ketogenic diet or variants thereof.

Idiopathic head tremor in english bulldogs

Idiopathic head tremor (IHT) syndrome is a recognized but poorly characterized movement disorder in English bulldogs (EBs). The data analyzed were collected via a detailed online questionnaire and video recordings. Thirty-eight percent of the population demonstrated IHT. The first presentation was early in life. There was no sex or neutered status predisposition. The condition disappeared with time in 50% of the cases. The direction of the head movement was vertical or horizontal. The number of episodes per day and the duration of the episodes were greatly variable. The majority of episodes occurred at rest. Most of the episodes were unpredictable. And there was no alteration of the mental status for most dogs during the episodes. Stress has been reported as a suspected trigger factor. IHT in EBs can be considered an idiopathic paroxysmal movement disorder.

Gratification Disorder Mimicking Childhood Epilepsy in an 18-month-old Nigerian Girl: A Case Report and Review of the Literature

Gratification disorder is common in younger children, but is often unrecognized because unlike in adolescents, it does not involve manual genital manipulation and the clinical features are quite variable; therefore a thorough history, physical examination, and video recording of the events will go a long way in making the correct diagnosis, otherwise it could easily be misdiagnosed as epilepsy, nonepileptic paroxysmal movement disorder, or even gastrointestinal disorder.

Late onset of atypical paroxysmal non-kinesigenic dyskinesia with remote history of Graves’ disease

Paroxysmal non-kinesigenic dyskinesia (PNKD) is a rare hyperkinetic movement disorder and falls under the category of paroxysmal movement disorders. In this condition, episodes are spontaneous, involuntary, and involve dystonic posturing with choreic and ballistic movements. Attacks last for minutes to hours and rarely occur more than once per day. Attacks are not typically triggered by sudden movement, but may be brought on by alcohol, caffeine, stress, fatigue, or chocolate. We report a patient with multiple atypical features of PNKD. She had a 7-year history of this condition with onset at the age of 59, and a remote history of Graves’ disease requiring total thyroidectomy. The frequency of attacks in our case ranged from five to six times a day to a minimum of twice per week, and the duration of episode was short, lasting not more than 2 min. Typically, PNKDs occur at a much younger age and have longer attack durations with low frequency. Administering clonazepam worked to reduce her symptoms, although majority of previous research suggests that pharmacological interventions have poor outcomes.

Psychogenic paroxysmal movement disorders – Clinical features and diagnostic clues

BackgroundThe diagnosis of psychogenic paroxysmal movement disorders (PPMD) can be challenging, in particular their distinction from the primary paroxysmal dyskinesias (PxD) remains difficult. MethodsHere we present a large series of 26 PPMD cases, describe their characteristics, contrast them with primary PxD and focus on their distinguishing diagnostic features. ResultsMean age at onset was 38.6 years, i.e. much later than primary PxD. Women were predominantly affected (73%). Most subjects (88.4%) had long attacks, and unlike primary PxD there was a very high within-subject variability for attack phenomenology, duration and frequency. Dystonia was the most common single movement disorder presentation, but 69.2% of the patients had mixed or complex PxD. In 50% of PPMD cases attack triggers could be identified but these were unusual for primary PxD. 42.3% of patients employed unusual strategies to alleviate or stop the attacks. Response to typical medication used for primary PxD was poor. Precipitation of the disorder due to physical or emotional life events and stressors were documented in 57.6% and 65.3% of the cases respectively. Additional interictal psychogenic signs were documented in 34.6% and further medically unexplained somatic symptoms were present in 50% of the cases. 19.2% of patients had a comorbid organic movement disorder and 26.9% had pre-existing psychiatric comorbidities. ConclusionAlthough the phenotypic presentation of PPMD can be highly diverse, certain clinical characteristics help in distinguishing this condition from the primary forms of PxD. Recognition is important as multidisciplinary treatment approaches led to significant improvement in most cases.

Movement disorders in GLUT1 deficiency syndrome respond to the modified Atkins diet

Movement disorders are a prominent feature of glucose transporter-1 (GLUT1) deficiency syndrome (GLUT1DS). First-choice treatment is a ketogenic diet, but compliance is poor. We have investigated the effect of the modified Atkins diet as an alternative treatment for movement disorders in GLUT1DS. Four patients with GLUT1DS ages 15 to 30 years who had movement disorders as the most prominent feature were prospectively evaluated after initiation of the modified Atkins diet. Movement disorders included dystonia, ataxia, myoclonus, and spasticity, either continuous or paroxysmal, triggered by action or exercise. Duration of treatment ranged from 3 months to 16 months. All patients reached mild to moderate ketosis and experienced remarkable improvement in the frequency and severity of paroxysmal movement disorders. Cognitive function also improved subjectively. The modified Atkins diet is an effective and feasible alternative to the ketogenic diet for the treatment of GLUT1DS-related paroxysmal movement disorders in adolescence and adulthood.

Clinical and polygraphic study of familial paroxysmal kinesigenic dyskinesia with PRRT2 mutation

Paroxysmal kinesigenic dyskinesia is a neurological condition characterised by brief attacks of involuntary movements triggered by sudden voluntary movements. We describe the clinical, polygraphic, and genetic features of an Italian family with paroxysmal kinesigenic dyskinesia. Paroxysmal kinesigenic dyskinesia manifested as brief choreoathetosic-dystonic attacks precipitated by sudden movements, varying in severity and frequency, amongst the four affected family members. The disorder follows an autosomal dominant transmission and affects female members. Mutation of SLC2A1, MR1, CACNA1A, and ATP1A2 genes was excluded by direct sequencing. Mutation analysis of the PRRT2 gene revealed a single nucleotide duplication, c.649dupC, resulting in the frameshift mutation p.Arg217Profs*8 in all affected members. Paroxysmal kinesigenic dyskinesia is the most common type of paroxysmal movement disorder and is often misdiagnosed clinically as epilepsy. We describe a family with paroxysmal kinesigenic dyskinesia associated with PRRT2 gene mutation, mild intrafamilial clinical heterogeneity, and benign course. [Published with video sequences].

PRRT2-related disorders: further PKD and ICCA cases and review of the literature

Recent studies reported mutations in the gene encoding the proline-rich transmembrane protein 2 (PRRT2) to be causative for paroxysmal kinesigenic dyskinesia (PKD), PKD combined with infantile seizures (ICCA), and benign familial infantile seizures (BFIS). PRRT2 is a presynaptic protein which seems to play an important role in exocytosis and neurotransmitter release. PKD is the most common form of paroxysmal movement disorder characterized by recurrent brief involuntary hyperkinesias triggered by sudden movements. Here, we sequenced PRRT2 in 14 sporadic and 8 familial PKD and ICCA cases of Caucasian origin and identified three novel mutations (c.919C>T/p.Gln307, c.388delG/p.Ala130Profs 46, c.884G>A/p.Arg295Gln) predicting two truncated proteins and one probably damaging point mutation. A review of all published cases is also included. PRRT2 mutations occur more frequently in familial forms of PRRT2-related syndromes (80-100 %) than in sporadic cases (33-46 %) suggesting further heterogeneity in the latter. PRRT2 mutations were rarely described in other forms of paroxysmal dyskinesias deviating from classical PKD, as we report here in one ICCA family without kinesigenic triggers. Mutations are exclusively found in two exons of the PRRT2 gene at a high rate across all syndromes and with one major mutation (c.649dupC) in a mutational hotspot of nine cytosines, which is responsible for 57 % of all cases in all phenotypes. We therefore propose that genetic analysis rapidly performed in early stages of the disease is highly cost-effective and can help to avoid further unnecessary diagnostic and therapeutic interventions.

PRRT2 gene mutations

The proline-rich transmembrane protein (PRRT2) gene was recently identified using exome sequencing as the cause of autosomal dominant paroxysmal kinesigenic dyskinesia (PKD) with or without infantile convulsions (IC) (PKD/IC syndrome). Episodic neurologic disorders, such as epilepsy, migraine, and paroxysmal movement disorders, often coexist and are thought to have a shared channel-related etiology. To investigate further the frequency, spectrum, and phenotype of PRRT2 mutations, we analyzed this gene in 3 large series of episodic neurologic disorders with PKD/IC, episodic ataxia (EA), and hemiplegic migraine (HM). The PRRT2 gene was sequenced in 58 family probands/sporadic individuals with PKD/IC, 182 with EA, 128 with HM, and 475 UK and 96 Asian controls. PRRT2 genetic mutations were identified in 28 out of 58 individuals with PKD/IC (48%), 1/182 individuals with EA, and 1/128 individuals with HM. A number of loss-of-function and coding missense mutations were identified; the most common mutation found was the p.R217Pfs*8 insertion. Males were more frequently affected than females (ratio 52:32). There was a high proportion of PRRT2 mutations found in families and sporadic cases with PKD associated with migraine or HM (10 out of 28). One family had EA with HM and another large family had typical HM alone. This work expands the phenotype of mutations in the PRRT2 gene to include the frequent occurrence of migraine and HM with PKD/IC, and the association of mutations with EA and HM and with familial HM alone. We have also extended the PRRT2 mutation type and frequency in PKD and other episodic neurologic disorders.

Clinical Reasoning: A middle-aged man with episodes of gait imbalance and a newly found genetic mutation

A 47-year-old right-handed man, with no history of alcohol use, presented with episodic unsteadiness that began in his early 20s. During the episodes, which last several hours, he is unable to walk steadily and has poor control of his limbs. These attacks are often brought on by emotional stress and occurred 1 to 2 times per month into his 30s. There is no association with headache or head movement, no diplopia, tinnitus, or hearing loss. His earlier evaluation included a brain MRI and routine EEG, which were normal. Though his diagnosis was unknown, he was given a trial of acetazolamide at age 38, and became attack-free on the medication. ### Questions for consideration: 1. What is the differential diagnosis for paroxysmal episodes of neurologic dysfunction based on the time course and age at onset? 2. How can medication responsiveness and examination findings be helpful? GO TO SECTION 2 In this patient, the likely etiologies for paroxysmal neurologic events are seizures, migraine, vestibular syndromes, and paroxysmal movement disorders. Pure ataxia as seizure semiology has not been described: cerebellar cortex is not known to be epileptogenic. Vertiginous partial seizures may be localized to the posterior part of superior temporal neocortex, are rare, and typically seen in conjunction with other temporal lobe localizing symptoms (auditory, olfactory). Postictal state of complex partial or generalized seizures can result in gait unsteadiness associated with fatigue and confusion and gradual improvement. In migraine, paroxysmal neurologic symptoms may occur without headache. Classified as typical aura without headache, the aura must include visual or sensory symptoms, and last less than …

Identification of PRRT2 as the causative gene of paroxysmal kinesigenic dyskinesias

Paroxysmal kinesigenic dyskinesias is a paroxysmal movement disorder characterized by recurrent, brief attacks of abnormal involuntary movements induced by sudden voluntary movements. Although several loci, including the pericentromeric region of chromosome 16, have been linked to paroxysmal kinesigenic dyskinesias, the causative gene has not yet been identified. Here, we identified proline-rich transmembrane protein 2 (PRRT2) as a causative gene of paroxysmal kinesigenic dyskinesias by using a combination of exome sequencing and linkage analysis. Genetic linkage mapping with 11 markers that encompassed the pericentromeric of chromosome 16 was performed in 27 members of two families with autosomal dominant paroxysmal kinesigenic dyskinesias. Then, the whole-exome sequencing was performed in three patients from these two families. By combining the defined linkage region (16p12.1–q12.1) and the results of exome sequencing, we identified an insertion mutation c.649_650InsC (p.P217fsX7) in one family and a nonsense mutation c.487C>T (p.Q163X) in another family. To confirm our findings, we sequenced the exons and flanking introns of PRRT2 in another three families with paroxysmal kinesigenic dyskinesias. The c.649_650InsC (p.P217fsX7) mutation was identified in two of these families, whereas a missense mutation, c.796C>T (R266W), was identified in another family with paroxysmal kinesigenic dyskinesias. All of these mutations completely co-segregated with the phenotype in each family. None of these mutations was identified in 500 normal unaffected individuals of matched geographical ancestry. Thus, we have identified PRRT2 as the first causative gene of paroxysmal kinesigenic dyskinesias, warranting further investigations to understand the pathogenesis of this disorder.

Exome sequencing identifies truncating mutations in PRRT2 that cause paroxysmal kinesigenic dyskinesia

Paroxysmal kinesigenic dyskinesia is the most common type of paroxysmal movement disorder and is often misdiagnosed clinically as epilepsy. Using whole-exome sequencing followed by Sanger sequencing, we identified three truncating mutations within PRRT2 (NM_145239.2) in eight Han Chinese families with histories of paroxysmal kinesigenic dyskinesia: c.514_517delTCTG (p.Ser172Argfs*3) in one family, c.649dupC (p.Arg217Profs*8) in six families and c.972delA (p.Val325Serfs*12) in one family. These truncating mutations co-segregated exactly with the disease in these families and were not observed in 1,000 control subjects of matched ancestry. PRRT2 is a newly discovered gene consisting of four exons encoding the proline-rich transmembrane protein 2, which encompasses 340 amino acids and contains two predicted transmembrane domains. PRRT2 is highly expressed in the developing nervous system, and a truncating mutation alters the subcellular localization of the PRRT2 protein. The function of PRRT2 and its role in paroxysmal kinesigenic dyskinesia should be further investigated.

Clinical/Scientific Notes

Paroxysmal kinesigenic dyskinesia (PKD) is a rare paroxysmal movement disorder characterized by involuntary movements triggered by sudden voluntary movements.1 PKD is a genetic disorder due to de novo mutations or autosomal dominant pedigree inheritance. Genetic linkage studies have generally localized the dominant gene to the pericentromic region of chromosome 16 (16p11-q21).2 Despite extensive searching, including mutation analysis of 157 genes in this region, the PKD gene remains unidentified.2

An inherited episodic head tremor syndrome in Doberman pinscher dogs

Episodic head tremor anecdotally occurs in the Doberman pinscher dog breed, but it is not described in sufficient detail in the literature. We evaluated 87 Doberman pinschers affected with episodic head tremor and appropriate controls. The data analyzed were collected through detailed questionnaires, elaborate telephone interviews, and video recordings. Affected dogs underwent clinical, neurological, and laboratory examination, and a detailed diagnostic workup was conducted in 5 affected dogs. Pedigrees of affected dogs were collected and reviewed. The affected dogs expressed individual phenotypes of either horizontal or vertical head movements, but rarely did a dog exhibit head movements in both directions. There was considerable variation in duration (10 seconds to 3 hours; median: 3 minutes), frequency of occurrence (1-20 episodes/day; median: 2/day) of head tremor and length of the period without head tremor (1-1,800 days; median: 60 days). Subtle dystonic posturing of the head and neck during head tremor was evident on video recordings of 5 dogs. Certain exceptional conditions such as illness, surgery, some medications, heat, pseudopregnancy, or pregnancy triggered episodes. Two main important forms of episodic head tremor were identified: a familial early-onset form (age < 1 year) that affected littermates and a sporadic form. Affected dogs were traced back to 1 common sire, also including sporadic cases. Episodic head tremor is an inherited, paroxysmal movement disorder that affects the Doberman pinscher breed. Identification of the causative genes in the future will allow us to obtain a more detailed description of the syndrome.

Extrapyramidal epilepsy

Non-epileptic paroxysmal movement disorders are rare extrapyramidal diseases. The clinical signs are often excluded from differential diagnosis due to their paucity on clinical examination and low prevalence. The paroxysmal, stereotypical nature of non-epileptic paroxysmal movement disorders, as well as the potential response to benzodiazepines, renders these disorders susceptible to misdiagnosis as epileptic seizures. We report a case of paroxysmal non-kinesigenic dyskinesia which was misdiagnosed and treated as epilepsy for decades. The major pathophysiological, diagnostic and therapeutic hallmarks of the disease are summarised. Familiarity and inclusion of the disease in the list of conditions that mimic epilepsy, as well as prolonged video-EEG recordings, may prevent diagnostic delays and unnecessary or belated treatments. [Published with video sequences].