Non-epileptic Disorder Research Articles

Conversation analysis can help to distinguish between epilepsy and non-epileptic seizure disorders: A case comparison

Purpose Factual items in patients’ histories are of limited discriminating value in the differential diagnosis of epilepsy and non-epileptic seizures (NES). A number of studies using a transcript-based sociolinguistic research method inspired by Conversation Analysis (CA) suggest that it is helpful to focus on how patients talk. Previous reports communicated these findings by using particularly clear examples of diagnostically relevant interactional, linguistic and topical features from different patients. They did not discuss the sequential display of different features although this is crucially important from a conversation analytic point of view. This case comparison aims to show clinicians how the discriminating features are displayed by individual patients over the course of a clinical encounter. Methods CA-inspired brief sequential analysis of two first 30-min doctor–patient encounters by a linguist blinded to all medical information. A gold standard diagnosis was made by the recording of a typical seizure with video-EEG. Results The patient with epilepsy volunteered detailed first person accounts of seizures. The NES patient exhibited resistance to focusing on individual seizure episodes and only provided a detailed seizure description after repeated prompting towards the end of the interview. Although both patients also displayed some linguistic features favouring the alternative diagnosis, the linguist's final diagnostic hypothesis matched the diagnosis made by video-EEG in both cases. Conclusion This study illustrates the importance of the time point at which patients share information with the doctor. It supports the notion that close attention to how patients communicate can help in the differential diagnosis of seizures.

Benign neonatal sleep myoclonus

Benign neonatal sleep myoclonus is a non-epileptic disorder. This phenomenon of the first weeks of life is characterized by erratic myoclonic jerks occurring only during sleep and with no electroencephalographic changes. It is not associated with perinatal complications, disappears spontaneously within two to four months, and it does not compromise future development. We illustrate with a video this relatively frequent condition, which is often misdiagnosed as epileptic in nature, and discuss the clinical characteristics and differential diagnosis.

Psychiatric diagnoses of patients with psychogenic non-epileptic seizures

Our purpose was to present and discuss the psychiatric diagnoses of patients who presented psychogenic non-epileptic seizures (PNES) during video-electroencephalographic monitoring (VEEG). Out of 98 patients, a total of 28 patients presented PNES during the diagnostic procedure. In those cases in which the PNES that occurred during VEEG were validated by clinical history (clinical validation), and by showing the recorded event on video to an observer close to the patient (observer validation), was defined psychogenic non-epileptic seizure disorder (PNESD). Psychiatric diagnoses were made according to DSM-IV. In 27, psychogenic non-epileptic seizures disorder was diagnosed. Fourteen patients presented only with psychogenic non-epileptic seizure disorder, 13 with both psychogenic non-epileptic seizures disorder and epilepsy, and one patient with epilepsy only. Psychiatric diagnoses were: 17 (63%) patients with conversion disorder, five (19%) with somatization disorder, two (7%) with dissociative disorder NOS, two (7%) with post-traumatic stress disorder and one (4%) with undifferentiated somatoform disorder. Dissociative-conversion non-epileptic seizures are the most frequent finding, representing the pseudoneurological manifestation of mental disorders that have these symptoms as a common feature. Provisionally, they may be defined as dissociative-conversion non-epileptic seizure disorders.

Clinical and Inheritance Profiles of Hyperekplexia in Jordan

Hyperekplexia is a rare nonepileptic disorder characterized by excessive startle response to acoustic, visual, or other stimuli. Patients with hyperekplexia are often misdiagnosed as having epilepsy. The presentation modalities, phenotypes, and the modes of inheritance among patients with hyperekplexia from 9 Jordanian families are described. All families were referred with the preliminary diagnosis of uncontrolled seizures with onset of the disease in the neonatal period and with variable and atypical presenting features. The inheritance profile in 4 families was compatible with autosomal recessive and in 1 family with autosomal dominant inheritance. Four families showed sporadic cases of hyperekplexia. This is the first report of a series of patients with hyperekplexia from Jordan. The clinical manifestations show atypical features that have not been previously reported, pointing to the probable broader clinical spectrum of this entity. Recognition of the syndrome allows for prompt proper management and provision of genetic counseling.

The new ILAE Report on Classification and Evidence‐Based Commentary on Panayiotopoulos Syndrome and Autonomic Status Epilepticus

The new ILAE Report on Classification and Evidence‐Based Commentary on Panayiotopoulos Syndrome and Autonomic Status Epilepticus

Hyperekplexia in two siblings

Hyperekplexia is a rare, hereditary, non-epileptic disorder characterized by an exaggerated startle reaction to unexpected auditory, somatosensory and visual stimuli. The authors describe a one-day-old term neonate, who presented with jitteriness and episodic tonic spasms, and his elder sister with hyperekplexia. Hyperekplexia though is a rare disorder is one of the differential diagnoses for refractory tonic spasms in infancy. The prognosis is generally good in hereditary hyperekplexia. Recent molecular studies have revealed many associated mutations in the glycine receptor alpha and beta subunit genes.

Paroxysmal Motor Disorders of Sleep: The Clinical Spectrum and Differentiation from Epilepsy

The diagnosis of paroxysmal events in sleep represents a significant challenge for the clinician, with the distinction of nocturnal epilepsy from nonepileptic sleep disorders often the primary concern. Diagnostic error or uncertainty is not uncommon in this situation, particularly with respect to nocturnal frontal lobe epilepsy (NFLE), which has a variable and often unusual presentation. Such errors can be minimized if the range of nonepileptic disorders with motor activity in sleep is fully appreciated. Here we review these disorders, before discussing the important clinical and electrographic features that allow their accurate differentiation from seizures. Particular emphasis is placed on the differentiation of nocturnal frontal lobe epilepsy from non-rapid eye movement (NREM) arousal disorders and other parasomnias. The value of recording episodes with video EEG polysomnography is discussed.

Usefulness of the Head-Up Tilt Test in Distinguishing Neurally Mediated Syncope and Epilepsy in Children Aged 5–20 Years Old

Many nonepileptic disorders may mimic epilepsy by history or clinical presentation. Neurally mediated syncope is one of the most important conditions that might be difficult to differentiate from epilepsy on clinical grounds. We investigated the value of the head-up tilt test (HUT) to diagnose syncope in epileptic children. We studied 40 patients (18 girls and 22 boys) between 5 and 20 years old (mean, 11.5 +/- 3.5) who had a previous diagnosis of epilepsy. All patients underwent a HUT test. The HUT test was positive in 26 patients (65%). No statistical difference was observed between the tilt positive and negative groups in sex, age, provocating factors, associated symptoms, family history of syncope and heart disease, findings in physical examination, and electroencephalogram result. There was a history in favor of true syncope in 58% of tilt positive patients compared to 14% of tilt negative patients (p < 0.05). Also, family history of seizure was more frequent in tilt positive patients (p < 0.05). After 18 +/- 6 months of follow-up, 18 of 26 patients with a positive tilt test were completely asymptomatic. Inadequate history taking and overemphasis on positive family history for seizures were important causes of misdiagnosis of epilepsy in our study. The HUT test is a simple, noninvasive diagnostic tool for distinguishing syncope and epilepsy in children and should be considered early in the diagnostic plan and for determining management of selected patients with a history of drop attack and loss of consciousness.

Nonepileptic Uses of Antiepileptic Drugs in Children and Adolescents

Antiepileptic drugs are often prescribed for nonepileptic neurologic and psychiatric conditions. The United States Food and Drug Administration has approved several antiepileptic drugs for the treatment of neuropathic pain, migraine, and mania in adults. For pediatric patients, use of antiepileptic drugs for non-seizure-related purposes is supported mainly by adult studies, open-label trials, and case reports. Summarized here is the published literature for or against the use of antiepileptic drugs for neuropathic pain, migraine, movement disorders, bipolar disorder, aggressive behavior, and pervasive developmental disorders in children and adolescents. Using the American Academy of Neurology's four-tiered classification scheme for a therapeutic article and translation to a recommendation rating, there are no nonepileptic disorders for which antiepileptic drugs have been established as effective for pediatric patients. Valproate and carbamazepine are "possibly effective" in the treatment of Sydenham chorea, and valproate is "probably effective" in decreasing aggressive behavior. Carbamazepine is "probably ineffective" in the treatment of aggression, and lamotrigine is "possibly ineffective" in improving the core symptom of pervasive developmental disorders. Despite the frequent use of antiepileptic drugs in the treatment of juvenile bipolar disorder, migraine, and neuropathic pain, the data are insufficient to make recommendations regarding the efficacy of antiepileptics in these conditions in children and adolescents.

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.

COMPARISON OF EPILEPTIC AND NONEPILEPTIC CASES WITH CENTROTEMPORAL SPIKES IN VIEW OF CLINICAL FINDINGS AND ELECTROENCEPHALOGRAPHIC CHARACTERISTICS

The morphological features of centrotemporal spike discharges (CTSD) and relationship of them with clinical diagnosis in cases with benign epilepsy of childhood with centrotemporal spikes (BECTS) and the other epileptic syndromes of childhood as well as some nonconvulsive neurological disorders were detected in the routine patient population who referred to the authors' EEG laboratory. Thirty-six cases (21 males, 15 females; 8 months–14 years old), in which awake and/or sleep EEGs revealed CTSD were included in this study. The cases were divided into two groups as epileptic and nonepileptic. The cases with seizure were divided into BECTS and the other epilepsies. Of the epileptic cases, 14 (38.8%) patients had typical rolandic seizures. In five cases, there were partial or secondary generalized seizures. Two cases had myoclonic seizures. In the nonepileptic group, there was mental retardation/behavioral disturbances in five cases; there were periodic syndromes of childhood such as migraine and equivalents of migraine in three cases; febrile convulsion in three cases, breath-holding spells in two cases, and primary enuresis nocturna in two cases. In the nonepileptic group, the discharges were significantly fewer than the other groups (p = .014). More frequent discharges occuring for shorter periods were more significantly observed in BECTS group (64%). Typically isolated spike and slow-waves in T3/T4 and C3/C4 location were significantly more common (86%) in rolandic epilepsy group (p = .01). The EEGs of cases with BECTS had more frequency in the cluster of discharges than the other groups (p = .018). Multifocal discharges were observed in 28.5% of cases with BECTS, in 20% of nonepileptic group, and in 71.4% of other epileptics in the trial. Although these epileptic and nonepileptic conditions have some differences in view of frequency and morphology and location, CTSDs could be manifested in the group without seizure. It was concluded that the similar focal abnormalities which could be seen in rolandic epilepsy may be observed in the other epileptic or nonepileptic disorders of childhood and this condition may be originated from the involvement of similar central structures.

Nonepileptic Disorders Imitating Generalized Idiopathic Epilepsies

Differential diagnosis between epileptic and nonepileptic paroxysmal disorders is fundamental not only to allow correct management of patients but also to avoid the burden of unnecessary antiepileptic medication. The focus of this chapter is limited to imitators of idiopathic generalized epilepsies (IGE) which are expressed through myoclonic, tonic-clonic, tonic, atonic, and absence seizures. Apparent losses of consciousness and drop attacks also have to be considered. Benign myoclonus of early infancy is the main nonepileptic disorder in the differential diagnosis of infantile spasms, but is not dealt with here because West syndrome is not an IGE. Hyperekplexia, metabolic disorders, hypnagogic myoclonus, and disturbed responsiveness caused by the use of drugs are listed in Table 1. Other conditions that may imitate more focal epileptic seizures are omitted. Benign neonatal sleep myoclonus, apnea and apparent life-threatening events in infants, cyanotic and pallid breath-holding spells, syncope, staring spells, psychogenic seizures, hyperventilation syndrome, and narcolepsy have been selected based on frequency or difficulties in differential diagnosis with the intention to cover the most conspicuous imitators of IGE in different ages.

Idiopathic Generalized Epilepsies: A Review and Modern Approach

Idiopathic Generalized Epilepsies: A Review and Modern Approach

Epilepsy in sub-Saharan Africa: its socio-demography, aetiology, diagnosis and EEG characteristics in Harare, Zimbabwe

To assess epileptics with regard to socio-demographic characteristics, aetiology, EEG results and classification. A descriptive cohort study from 1997 to 2001. Parirenyatwa Group of Hospitals in Harare, Zimbabwe. A total of 229 consecutive epileptic subjects. The mean (s.d.) follow-up was 3.2 (1.14) years. The mean (s.d.) age of subjects was 23.4(11.8) years and 56% of them were males, over one third (43.1%) were unemployed and had secondary education (48%). Over one fourth (26.6%) of the subjects were first borns and had a family history of epilepsy (25.8%). Most of the subjects had Generalised Tonic Clonic Seizures (TCS). Over half (51.5%) of the subjects had their first onset of seizures after the age of 20 years. Lack of sleep (7.9%) and emotional disturbance (5.4%) were described by subjects as the frequent provoking factors for epileptic attacks. One hundred and fourteen subjects had electroencephalogram (EEG) results. Of these, 96 (41.9%) subjects had abnormal EEG results and only 18 (7.9%) had normal EEG results a majority (43.8%) of whom were of generalised slow waves. Majority of the subjects (52.8%) sought treatments from biomedical drugs, but some started with traditional herbs (20.5%) and then biomedical drugs, whereas others sought biomedical drugs then resorted to traditional herbs (20.1%). Phenobarbital and carbamazepine were taken by over 40% of the subjects. The findings of this study seem to suggest that these referrals to a specialised epilepsy outpatient clinic for further management may be attributed to socio cultural issues in our African settings about the actual nature of the condition, hence the delay in treatments and diagnosis of seizures as well as under diagnoses of non-epileptic seizure disorders that may lead to the condition becomes uncontrollable. Inexpensive drugs such as phenobarbital and phenytoin can be able to control most of the epileptic seizures, if they can be made available in primary health care centres.

Free Papers: Clinical Course and Prognosis

Abstract *Hideji Hattori, *∥Tsunekazu Yamano, †∥Kitami Hayashi, †∥Makiko Osawa, ‡∥Shinichiro Hamano, and §∥Kenichiro Kaneko *Department of Pediatrics, Osaka City University Graduate School of Medicine, Osaka ; †Department of Pediatrics, Tokyo Woman's Medical University, School of Medicine, Tokyo ; ‡Division of Neurology, Saitama Children's Medical Center, Saitama ; §Department of Pediatrics, Juntendo University Urayasu Hospital, Japan ; and ∥Research Committee on Clinical Evidence of Medical Treatment for Status Epileptics in Childhood . Purpose: To evaluate the efficacy of intravenous lidocaine therapy in the management of status epilepticus (SE) in childhood. Subjects and Methods: This was a multiinstitutional, retrospective study. Questionnaires were sent to 30 hospitals with pediatric departments. Patients admitted for SE and managed with lidocaine were included. We collected background information of the patients (underlying diseases, seizure type, etc.), treatment, and efficacy. Results: Case cards (279) were collected. Patients older than 15 years and younger than 1 month were excluded. The data set analyzed included 257 cases. The mean age was 3.8 years, and 66% were younger than 3 years. Epilepsy was the diagnosis in 145 cases. The remaining 112 had nonepileptic diseases. The etiology of epilepsy was determined in 103 cases; of which 79 were from prenatal factors, eight from perinatal factors, and 16 from postnatal factors. Epilepsy type was diagnosed as localization related in 94 cases, generalized in 27 cases, and unclassified in 24 cases. Nonepileptic acute illness included 30 cases of benign infantile convulsion, 30 cases of acute encephalopathy/encephalitis, 13 cases of meningitis, and 39 other causes. Seizure type was diagnosed as generalized in 102 cases, secondarily generalized in 94 cases, and partial in 61 cases. We categorized SE into three groups: continuous (type I); cluster (type II); and frequently repeated (type III). Type III patients regained consciousness between seizures; therefore, type III did not meet the common definition of SE. The numbers of type I, type II, and type III cases were 73, 73, and 111, respectively. The mean dosage of lidocaine in initial loading therapy was 1.65 ± 1.12 mg/kg (206 cases), and the mean starting dosage of maintenance therapy was 2.11 ± 1.19 mg/kg/h (211 cases). The maximum maintenance dosage was 2.60 ± 1.69 mg/kg/h. Lidocaine was infused for a mean of 105 h. Lidocaine was selected as the first drug in 57 cases, the second in 83 cases, the third in 75 cases, and after the fourth in 42 cases. We defined effective as complete seizure control or seizure reduction by >50%, with no or mild adverse side effects. Overall, lidocaine was effective in 57% of cases. The treatment lag time and the order of medication used did not influence the efficacy of lidocaine. No difference in the efficacy rate was found between epileptic and nonepileptic disorders. Patients with benign infantile convulsions showed good responses to lidocaine, whereas SE patients with CNS infection showed poor responses. Efficacy was better in patients with types II and III SE compared with type I. Patients who showed good response to initial loading therapy of lidocaine were well controlled with maintenance therapy, whereas poor responders to initial infusion therapy showed almost no response to maintenance therapy. Therefore if the patients do not respond to the initial therapy, maintenance therapy will be unsuccessful. Adverse side effects were seen in 35 (13.6%) cases, and the risk of side effects was high with maintenance doses >4 mg/kg/h. Conclusions: Lidocaine is useful for the management of SE in childhood. It may be used as a first- or second-line medication for benign infantile convulsions, and patients with types II and III SE are good candidates for lidocaine use. However, lidocaine may be useful in every stage of SE when SE is not controlled by other medications. Patients who do not respond to an initial loading infusion will not achieve control by maintenance infusion.

0016 Neurochemical investigation in children with epilepsy and non-epileptic paroxysmal disorders

0016 Neurochemical investigation in children with epilepsy and non-epileptic paroxysmal disorders

Neonatal Movement Disorders

After completing this article, readers should be able to: 1. Delineate the characteristics that differentiate hyperkinetic neonatal movement disorders from neonatal seizures. 2. Describe the primary characteristics of and treatment for hyperekplexia. 3. Explain the relationship between gastroesophageal reflux and movement disorders in neonates. 4. Describe the primary ocular movement disorders in neonates. Neonates often exhibit paroxysmal behaviors that are nonepileptic in nature, such as oral-buccal-lingual movements, pedaling, stepping, and rotatory arm movements. The electroencephalogram (EEG) is of particular value for distinguishing these nonepileptic behaviors from subtle seizures. EEG and video-EEG studies in such movement disorders do not exhibit seizure discharges during the events. This distinction is important because nonepileptic events do not require treatment with anticonvulsants. Although the absence of surface ictal activity does not exclude entirely the possibility of seizures arising from deeper structures, it generally is accepted that these disorders are not epileptic. For the purposes of this review, we include common nonepileptic paroxysmal (episodic) movements that occur from birth to early infancy (onset between birth and 3 months of age). Many of these movement disorders may persist during infancy or early childhood. We do not discuss more persistent motor problems, such as spasticity, hypotonia, or myotonia. Also, disorders that occur after 3 months of age are not included. We have classified the movement disorders into two major categories: 1) hyperkinetic movements and 2) dystonic or extrapyramidal type of movements. Because the pathophysiologic mechanisms underlying neonatal movement disorders often are unknown, they are categorized on the basis of phenomenology, rather than presumed cause. Some of these disorders may have overlapping symptomatology. A third category encompasses “ocular movement disorders.” ### Jitteriness of the Newborn Jitteriness may be due to many factors, including metabolic disturbance, hypoxic-ischemic encephalopathy, drug withdrawal, hypoglycemia, and hypocalcemia. Although jitteriness may be mistaken for neonatal seizures, a distinguishing feature of jitteriness is that the …

The movement disorders of Coffin–Lowry syndrome

Coffin–Lowry syndrome (CLS) is an X-linked semi-dominant condition with learning difficulties and dysmorphism caused by mutations in the gene RSK2. Originally, epilepsy was reported as a feature. We and others have since described predominantly sound-startle induced drop attacks that have been labelled ‘cataplexy’, abnormal startle response and hyperekplexia. We sought to clarify why there should be controversy over the type of paroxysmal events. Review of the literature and our patients confirmed that each centre had studied only a small numbers of individuals (mean=2). The type of movement disorder varied both with age and between individuals. One individual might have more than one movement disorder. One of our adult patients had several types of movement disorder and epilepsy that merged seamlessly: there was true cataplexy triggered by telling a joke, something close to cataplexy (‘cataplexy’) triggered by sound-startle, a predominantly hypertonic reaction varying from hyperekplexia to a more prolonged tonic reaction resembling startle epilepsy, and true unprovoked epileptic seizures. In the large database of the Coffin–Lowry Syndrome Foundation family support group, 34 of 170 (20%) individuals with CLS and known age had ‘drop attacks’ and an additional 9 (5%) of these had additional epileptic seizures. The onset of such events was usually after age 5 years, prevalence peaking at 15–20 years (27%). Many became wheelchair bound as a result. This unique combination of more than one non-epileptic movement disorder and epilepsy deserves further semiological and genetic study both for the patients with CLS and for the wider implications.

Desviación ocular paroxística benigna infantil: ¿un trastorno no epiléptico?

Benign childhood paroxysmal eye deviation (BCPED) is classified as a 'non-epileptic paroxysmal disorder'. We report the cases of four patients aged between 6 months and 2 years, who suffered brief episodes of upward conjugate gaze deviation, with no clonic movements or associated cognitive deterioration. These episodes, which lasted several seconds, appeared in short repeated bouts that became worse with fatigue. Results of the neurological exploration, laboratory examinations, neuroimaging (CAT, MRI, brain ultrasonography) and a neurophysiological study, which included EEG-video monitoring and EEG performed during the waking state, were all normal. A nocturnal polysomnographic study was later conducted for 7-8 hours and EEG, EMG and EOG readings were recorded. The trace showed focal or generalised paroxysmal discharges during non-REM sleep in the form of polyspike-wave and spike-wave complexes. Sleep analysis (Reschstaffen and Kales) showed only a shortened REM sleep latency, with no clear clinical meaning. Several cases have been reported in the literature with identical symptoms and normal results in the diagnostic tests, including daytime polysomnography. The appearance of these epileptic anomalies in the nocturnal study makes it necessary to perform a complete nocturnal polysomnography. In spite of these findings, BCPED courses favourably and has a benign prognosis both with and without antiepileptic treatment. We therefore believe that BCPED should be classed within the group of 'benign idiopathic epilepsies of childhood'.

Pediatric seizures and their management in the emergency department

Seizures result from paroxysmal involuntary disturbance of brain function. The history and physical examination guide to management, and will help to differentiate seizures from non-epileptic disorders. The studies needed depend on the child’s age, presence or absence of fever, the duration of seizure activity, and clinical examination. Afebrile seizures in children older than 6 months of age require minimal investigation, while younger children are more likely to have an electrolyte abnormality or hypoglycemia that requires treatment in the emergency department. Children with febrile seizures are not at high risk for serious bacterial illness and routine diagnostic evaluation of simple febrile seizures is not indicated. Anti-epileptic drugs should not be routinely initiated in the emergency department in children whose seizures have resolved. We review the management of status epilepticus in this paper.