Self-induced Seizures Research Articles

Electroclinical Features and Long-term Seizure Outcome in Patients With Eyelid Myoclonia With Absences.

Eyelid myoclonia (EM) with absences (EMA) is a generalized epilepsy syndrome with a prognosis and clinical characteristics that are still partially undefined. We investigated electroclinical endophenotypes and long-term seizure outcome in a large cohort of patients with EMA. In this multicenter retrospective study, patients with EMA with ≥5 years of follow-up were included. We investigated prognostic patterns and sustained terminal remission (STR), along with their prognostic factors. Moreover, a 2-step cluster analysis was used to investigate the presence of distinct EMA endophenotypes. We included 172 patients with a median age at onset of 7 years (interquartile range [IQR] 5-10 years) and a median follow-up duration of 14 years (IQR 8.25-23.75 years). Sixty-six patients (38.4%) displayed a nonremission pattern, whereas remission and relapse patterns were encountered in 56 (32.6%) and 50 (29.1%) participants. Early epilepsy onset, history of febrile seizures (FS), and EM status epilepticus significantly predicted a nonremission pattern according to multinomial logistic regression analysis. STR was achieved by 68 (39.5%) patients with a mean latency of 14.05 years (SD ±12.47 years). Early epilepsy onset, psychiatric comorbid conditions, and a history of FS and generalized tonic-clonic seizures were associated with a lower probability of achieving STR according to a Cox regression proportional hazards model. Antiseizure medication (ASM) withdrawal was attempted in 62 of 172 patients, and seizures recurred in 74.2%. Cluster analysis revealed 2 distinct clusters with 86 patients each. Cluster 2, which we defined as EMA-plus, was characterized by an earlier age at epilepsy onset, higher rate of intellectual disability, EM status epilepticus, generalized paroxysmal fast activity, self-induced seizures, FS, and poor ASM response, whereas cluster 1, the EMA-only cluster, was characterized by a higher rate of seizure remission and more favorable neuropsychiatric outcome. Early epilepsy onset was the most relevant prognostic factor for poor treatment response. A long latency between epilepsy onset and ASM response was observed, suggesting the effect of age-related brain changes in EMA remission. Last, our cluster analysis showed a clear-cut distinction of patients with EMA into an EMA-plus insidious subphenotype and an EMA-only benign cluster that strongly differed in terms of remission rates and cognitive outcomes.

Pattern-sensitive patients with epilepsy use uncomfortable visual stimuli to self-induce seizures

Sensory stimuli can induce seizures in patients with epilepsy and predisposed subjects. Visual stimuli are the most common triggers, provoking seizures through an abnormal response to light or pattern. Sensitive patients may intentionally provoke their seizures through visual stimuli. Self-induction methods are widely described in photo-sensitive patients, while there are only a few reports of those who are pattern-sensitive.We analyzed 73 images of environmental visual triggers collected from 14 pattern-sensitive patients with self-induced seizures. The images were categorized according to their topics: 29 Objects (43%); 19 Patterns (28%); 15 External scenes (22%); 4 TV or computer screens (6%). Six photos were of poor quality and were excluded from analysis. Images were analyzed by an algorithm that calculated the degree to which the Fourier amplitude spectrum differed from that in images from nature. The algorithm has been shown to predict discomfort in healthy observers. The algorithm identified thirty-one images (46%) as “uncomfortable”. There were significant differences between groups of images (ANOVA p = .0036; Chi2 p < .0279), with higher values of difference from nature in the images classified as “Objects” (mean 6,81E+11; SD 6,72E+11; n.17, 59%) and “Pattern” (mean 9,05E+11; SD 6,86E+11; n.14, 74%). During the semi-structured face-to-face interviews, all patients described the visual triggers as ‘uncomfortable’; the appearance of enjoyable visual epileptic symptoms (especially multi-colored hallucinations) transformed uncomfortable images into pleasant stimuli. Patients considered self-induction as the simplest and most effective way to overcome stressful situations, suggesting that self-inducing pattern-sensitive patients often use uncomfortable visual stimuli to trigger their seizures. Among the reasons for the self-inducing behavior, the accidental discovery of pleasurable epileptic symptoms related to these “uncomfortable” visual stimuli should be considered.

Self-induced epileptic seizures: Prevalence, Causes and Treatment

Self-induced seizures were first described in 1827. A majority of authors found that in unselected patients with epilepsy, the prevalence rate of these seizures was 1%. In patients with photosensitive epilepsy, there was roughly a 25% prevalence. Apart from visual stimulation, many other mechanisms of self-induction have been described. A feeling of pleasure or relaxation during seizures may be a reason for self-inductive behaviour. But often the procedure of self-induction is experienced as involuntary. Treatment is always difficult. Behavioral therapy has been proven effective in some patients. In patients with photosensitive epilepsy, sunglasses are recommended. Fenfluramine, clonazepam and valproate seem to be a bit more effective than other drugs. After all, the treatment effect depends on the motivation of the patient to change the condition.

Clinical and electrographic features of sunflower syndrome

BackgroundSunflower Syndrome describes reflex seizures – typically eyelid myoclonia with or without absence seizures – triggered when patients wave their hands in front of the sun. While valproate has been recognized as the best treatment for photosensitive epilepsy, many clinicians now initially treat with newer medications; the efficacy of these medications in Sunflower Syndrome has not been investigated. We reviewed all cases of Sunflower Syndrome seen at our institution over 15 years to describe the clinical course, electroencephalogram (EEG), and treatment response in these patients. MethodsSearch of the electronic medical record and EEG database, as well as survey of epilepsy providers at our institution, yielded 13 cases of Sunflower Syndrome between 2002 and 2017. We reviewed the records and EEG tracings. ResultsPatients were mostly young females, with an average age of onset of 5.5 years. Seven had intellectual, attentional or academic problems. Self-induced seizures were predominantly eyelid myoclonia ± absences and 6 subjects also had spontaneous seizures. EEG demonstrated a normal background with 3–4 Hz spike waves ± polyspike waves as well as a photoparoxysmal response. Based on both clinical and EEG response, valproate was the most effective treatment for reducing or eliminating seizures and improving the EEG; 9 patients tried valproate and 66% had significant improvement or resolution of seizures. None of the nine patients on levetiracetam or seven patients on lamotrigine monotherapy achieved seizure control, though three patients had improvement with polypharmacy. ConclusionsValproate monotherapy continues to be the most effective treatment for Sunflower Syndrome and should be considered early. For patients who cannot tolerate valproate, higher doses of lamotrigine or polypharmacy should be considered. Levetiracetam monotherapy, even at high doses, is unlikely to be effective.

CHD2-epilepsy: Polygraphic documentation of self-induced seizures due to fixation-off sensitivity

CHD2 gene has been described in association with different types of childhood myoclonic epilepsy and is emerging as a gene involved in photosensitivity alone or combined with epilepsy. Recent studies suggest that CHD2 could be responsible for a proper phenotype characterized by infantile-onset generalized epilepsy, intellectual disability, and photosensitivity and in particular with self-induced seizures.We report the case of a child with CHD2 mutation and mild developmental impairment that since the age of 3 years started with myoclonic seizures apparently well responding to antiepileptic drugs and that subsequently developed intractable self-induced seizures.Through an accurate Video-EEG polygraphic analysis, we demonstrated that seizures are related to an abnormal increase of epileptiform activity after eye-closure or loss of fixation as observed in the Fixation-Off Sensitivity (FOS) phenomenon.In conclusion our study adds relevant features of the CHD2-epilepsy phenotype and confirms that CHD2 mutations produce a distinctive form of myoclonic epilepsy with visual-sensitive seizures.

CHD2 myoclonic encephalopathy is frequently associated with self-induced seizures.

To delineate the phenotype of early childhood epileptic encephalopathy due to de novo mutations of CHD2, which encodes the chromodomain helicase DNA binding protein 2. We analyzed the medical history, MRI, and video-EEG recordings of 9 individuals with de novo CHD2 mutations and one with a de novo 15q26 deletion encompassing CHD2. Seizures began at a mean of 26 months (12-42) with myoclonic seizures in all 10 cases. Seven exhibited exquisite clinical photosensitivity; 6 self-induced with the television. Absence seizures occurred in 9 patients including typical (4), atypical (2), and absence seizures with eyelid myoclonias (4). Generalized tonic-clonic seizures occurred in 9 of 10 cases with a mean onset of 5.8 years. Convulsive and nonconvulsive status epilepticus were later features (6/10, mean onset 9 years). Tonic (40%) and atonic (30%) seizures also occurred. In 3 cases, an unusual seizure type, the atonic-myoclonic-absence was captured on video. A phenotypic spectrum was identified with 7 cases having moderate to severe intellectual disability and refractory seizures including tonic attacks. Their mean age at onset was 23 months. Three cases had a later age at onset (34 months) with relative preservation of intellect and an initial response to antiepileptic medication. The phenotypic spectrum of CHD2 encephalopathy has distinctive features of myoclonic epilepsy, marked clinical photosensitivity, atonic-myoclonic-absence, and intellectual disability ranging from mild to severe. Recognition of this genetic entity will permit earlier diagnosis and enable the development of targeted therapies.

Self‐induction seizures in sunflower epilepsy: a video‐EEG report

Seizures triggered by visual stimuli are the most common type of reflex seizure. Self-induced seizures produced by stimulation of natural light are rare and self-induction is a mode of seizure precipitation employed by either intellectually disabled or healthy photosensitive individuals. Absences and myoclonic jerks are the most common seizure types in self-induction. We report on a girl with normal intelligence who self-induced seizures by waving her outspread fingers in front of a bright light. This situation is called sunflower epilepsy.

Self-induced seizures presumably by peri-orbital somatosensory self-stimulation: A report of two cases

Self-induced seizures by somatosensory stimulation are rare. We describe two epileptic patients with self-induced seizures presumably by peri-orbital somatosensory stimulation.Two infants with severely delayed psychomotor development and poor visual acuity after acute subdural hemorrhage in early infancy had been diagnosed as having West syndrome. They evolutionally became to show serial self-induced seizures preceded by rubbing eye with finger in one case and touching right eyebrow with the back of left hand in the other case. Video-electroencephalography (EEG) monitoring was useful to confirm self-induced seizure by peri-orbital self-stimulation. In patients with serial seizures preceded by peculiar behaviors, we need to consider the possibility of self-induced seizures, even if they have a history of West syndrome and severe psychomotor retardation.

P2.13 EEG features of patients with visually self-induced seizures

P2.13 EEG features of patients with visually self-induced seizures

自己誘発発作を主症状とし特異な経過を示した頭部外傷後てんかんの男児例

自己誘発発作を主症状とし特異な経過を示した頭部外傷後てんかんの男児例

Self-Induced "Therapeutic Seizures" for the Treatment of Depression

Back to table of contents Previous article LETTERFull AccessSelf-Induced “Therapeutic Seizures” for the Treatment of DepressionDonald F. Weaver M.D., Ph.D., F.R.C.P.(C),Donald F. Weaver M.D., Ph.D., F.R.C.P.(C)Search for more papers by this author,Published Online:1 Jul 2009https://doi.org/10.1176/jnp.2009.21.3.355AboutSectionsPDF/EPUB ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail To the Editor: Early 20th century research postulated an antagonism between epilepsy and psychosis, leading von Meduna 1 to introduce convulsive therapy for schizophrenia. Although modern research questions this postulate, there is a complex and understudied interplay between seizures and behavioral disorders. I report on a woman with epilepsy who used self-induced seizures to treat her depression. Case ReportA 43-year-old woman was diagnosed with epilepsy at age 29. She experienced 6–8 complex-partial or generalized tonic-clonic seizures per month for many years, failing to respond to a range of anticonvulsant drugs. However, when she was treated with a combination therapy of phenytoin (300 mg/day) and gabapentin (1600 mg/day), her seizures were completely suppressed. Regrettably, after 6 months without seizures, she noticed the onset of depression. Her personality underwent a profound change; she became sad, feeling that life was no longer worth living. She had trouble sleeping and had early morning awakening. Food lost its taste and she lost weight. Although she was depressed, there were no symptoms of psychosis. Her family confirmed the marked change in her mood. Although she had experienced some depressive episodes in her early 20s, since developing epilepsy she had been a happy, positive-thinking person, despite the personal difficulties arising from her seizure disorder.Several antidepressants were tried, but none were of benefit; she remained depressed for more than a year. Ultimately, due to the depression, she quit taking her anticonvulsants stating that “life just is not worth it.” This led to a series of complex partial and generalized tonic-clonic seizures, after which her mood promptly became cheerful and optimistic again. She felt that seizures had done more for her mood than the antidepressant drugs. She was restarted on phenytoin and gabapentin, but did not receive any further antidepressants. After several years of unsupervised self-experimentation, she disclosed a new approach to her seizure disorder. She would abruptly stop taking gabapentin for five consecutive days during the last week of every fourth month. This would result in her having several complex-partial and generalized tonic-clonic seizures (at 3–4 days postwithdrawal); she did not disclose these periodic seizures to family or friends. Since embarking upon this strategy of self-induced “therapeutic seizures,” her depression had completely resolved.Discussion In the 1950s, Landolt 2 observed people with epilepsy who became psychotic when their seizures were controlled—an observation which he attributed to the electroencephalographic phenomenon of “forced normalization.” Conversely, Tellenbach 3 used the term “alternative psychoses” to describe people whose psychosis resolved with the return of seizures. Although these notions are now regarded as outdated and inaccurate, there is a close biological link between seizures and psychiatric (not necessarily psychotic) disorders. This case demonstrates the relationship between seizures and depression. In particular, it demonstrates that the dynamic neurochemical milieu of the postseizure state may exhibit prolonged anticonvulsant properties. Postseizure increases in brain levels of dopamine and serotonin, as well as alterations in norepinephrine metabolism, have been consistently observed in animal models of epilepsy. 4 , 5 Analogously, studies concerning the use of electroconvulsive seizures to treat depression likewise reveal increased release of norepinephrine and increased serotonergic activity (including upregulated 5-HT 2a receptors and increased 5-HT 2a mRNA levels) in the postseizure state. 6 Since such neurochemical changes have antidepressant effects, it is reasonable to assert that “naturally occurring” seizures also produce antidepressant effects. Although abrupt alterations in anticonvulsant dosing cannot be recommended, this woman with epilepsy used periodic self-induced seizures precipitated by intermittent medication withdrawal to treat her depression.Departments of Medicine (Neurology) and Chemistry, Dalhousie University, Halifax, Nova Scotia, Canada

A study of 63 cases with eyelid myoclonia with or without absences: Type of seizure or an epileptic syndrome?

Purpose Eyelid myoclonia and absences (EMA) induced by eye closure associated with brief, fast, and generalized paroxysms of polyspikes and waves was considered as an epileptic syndrome and a type of seizure as well. We analyzed the electroclinical features and evolution of EMA, and tried to determine if it represents a well-defined epileptic syndrome or a non-specific condition associated to other epilepsies. Methods Between June 1994 and June 2005, 63 patients who met diagnostic criteria of EMA were enrolled in the study and have been followed up to the present time. Results Two main groups could be identified. The first group was divided into two subgroups. One subgroup of 28 patients presented EMA associated with infrequent generalized tonic–clonic seizures (GTCS), and the other 1 of 9 patients presented early-onset EMA refractory to antiepileptic drugs (AEDs), associated or not with GTCS and mental retardation. Four of them had self-induced seizures. The second group included 26 patients with EMA associated with GTCS and/or massive myoclonias, or GTCS induced by intermittent photic stimulation. All these patients had electroclinical features compatible with idiopathic generalized epilepsies. Conclusion In the first group, EMA should be considered as a photosensitive idiopathic epileptic syndrome. A subgroup of early-onset of EMA refractory to AEDs, associated or not with GTCS and mental retardation should also be considered as a variant or a distinct photosensitive idiopathic epileptic syndrome. Finally, in the second group EMA may correspond to a type of seizures in idiopathic generalized epilepsies.

Overlap cases of eyelid myoclonia with absences and juvenile myoclonic epilepsy

Eyelid myoclonia with absences (EMA) and juvenile myoclonic epilepsy (JME) are two separate epileptic syndromes included in the new classification of epilepsies and epileptic syndromes by ILAE in 2001. Both are idiopathic generalized epilepsies with their clinical onset in the first two decades. EMA is characterized by eyelid myoclonia associated with absences and photosensitivity. Self-induced seizures are frequently seen in EMA. It can be associated with mildly mental retardation and resistance to treatment. JME includes three types of generalized seizures: typical absences, myoclonic jerks and generalized tonic-clonic seizures. The myoclonic jerks occur almost exclusively on awakening, involve preferently the upper extremities, may rarely affect the lower extremities or the entire body. More severe attacks may be accompanied by a fall. The myoclonic jerks occur rarely in EMA. They are usually mild and are freqently restricted to the upper extremities. Generalized tonic-clonic seizures, photosensitivity and generalized polyspike-wave discharges provoked by eye closure are features of both epileptic syndromes. In this study, we describe four female patients with eyelid myoclonia associated with absences, myoclonic jerks causing falling down and rare generalized tonic-clonic seizures. All patients had good school performance and total seizure control under sodium valproate treatment. Their EEGs show generalized polyspike-wave discharges with a frequency of 3.5-6Hz always appearing a few seconds after eye closure and photoparoxysmal response. These patients show the characterictics of both epileptic syndromes. It is clinically important to make a syndromic diagnosis for an optimum advise on treatment, lifestyle restrictions and prognosis. In this study, we have gathered evidence that EMA and JME are dynamic syndromes that tend to evolve into one another.

Psychiatric aspects of self-induced epileptic seizures.

To review the literature on self-induced epileptic seizures and apply psychiatric knowledge to define possible causes and treatment recommendations. A review of MEDLINE literature on self-induced epileptic seizures was conducted, followed by cross-referencing with the relevant neurologic, psychiatric and paediatric books and journals. The reported prevalence of self-induction varies depending on the setting. In most instances, self-inducers are people affected by photosensitive primary generalized epilepsy, in whom self-induction is by intermittent photic stimulation or, more rarely, pattern stimulation or eye closure. Self-induced seizures are most commonly absences with spike-wave, though eyelid myoclonia and generalized myoclonic jerks occur. Earlier studies indicate that the majority of self-inducers were of subnormal intelligence but more recent ones indicate that as a group they could not be considered mentally retarded. Monitoring in a well-lit environment is indicated in any therapy-resistant photosensitive patient to determine whether or not self-induction occurs. Self-induced epilepsy is notoriously resistant to therapy and reasons for this resistance are discussed. An explanatory model for the initiation and maintenance of self-induction behaviour is also included. It is difficult to determine from published papers just what level of clinical difficulty self-induction of seizures actually posed to the patients and clinicians. In some cases there is contradictory evidence as to the degree of willfulness or self-induction. Self-induced epileptic seizures may be an important aspect of poor seizure control in a subgroup of epilepsy sufferers. These seizures might be modifiable by psychiatric intervention or behavioural modification.

Eyelid myoclonia with absences in adults: a clinical and video-EEG study.

We report clinical and video-EEG findings in 11 adults with the syndro me of eyelid myoclonia with absences (EMA). Prevalence was 2.7% among all epilepsies and 12.9% among idiopathic generalized epilepsies (IGE) with typical absences. All patients with EMA were women, with a mean age of 30.9 years and a mean age of 7.8 years at reported onset of eyelid myoclonia. The characteristic seizures, studied with video-EEG in 10 patients, began with and were mainly manifested by eyelid myoclonia, either alone if brief (< or = 2 s) or with associated mild impairment of consciousness if longer. The ictal EEG was characterized by polyspike and slow waves at 3-6 Hz. Ictal clinical and EEG manifestations occurred mainly after eye closure and were inhibited by total darkness. All patients were photosensitive, but photosensitivity decreased with age; nonpatient had self-induced seizures. Infrequent generalized tonic-clonic seizures (GTCS) occurred in all but the youngest patient; they were usually infrequent and were precipitated mainly by flickering lights, sleep deprivation, fatigue, and menstruation. Mild myoclonic jerks of the upper limbs occurred in 6 patients. Eyelid myoclonia was resistant to medication and persisted despite control of other seizures.

Von Recklinghausen neurofibromatosis: EEG features in 4 pediatric cases

Eyelid myoclonia and absences (EMA) induced by eye closure associated with brief, fast, and generalized paroxysms of polyspikes and waves was considered as an epileptic syndrome and a type of seizure as well. We analyzed the electroclinical features and evolution of EMA, and tried to determine if it represents a well-defined epileptic syndrome or a non-specific condition associated to other epilepsies.Between June 1994 and June 2005, 63 patients who met diagnostic criteria of EMA were enrolled in the study and have been followed up to the present time.Two main groups could be identified. The first group was divided into two subgroups. One subgroup of 28 patients presented EMA associated with infrequent generalized tonic–clonic seizures (GTCS), and the other 1 of 9 patients presented early-onset EMA refractory to antiepileptic drugs (AEDs), associated or not with GTCS and mental retardation. Four of them had self-induced seizures. The second group included 26 patients with EMA associated with GTCS and/or massive myoclonias, or GTCS induced by intermittent photic stimulation. All these patients had electroclinical features compatible with idiopathic generalized epilepsies.In the first group, EMA should be considered as a photosensitive idiopathic epileptic syndrome. A subgroup of early-onset of EMA refractory to AEDs, associated or not with GTCS and mental retardation should also be considered as a variant or a distinct photosensitive idiopathic epileptic syndrome. Finally, in the second group EMA may correspond to a type of seizures in idiopathic generalized epilepsies.

Self-Induced Photogenic Seizures

The characteristics of photogenic self-induced seizures and their treatment by optical filters in a 2-year-old boy with severe myoclonic epilepsy in infancy are reported from the National Epilepsy Center, Shizuoka Higashi Hospital, Japan.

Inhibition of Self‐Induced Photogenic Seizures

Inhibition of Self‐Induced Photogenic Seizures

Self‐Induced Pattern‐Sensitive Epilepsy in Childhood

We studied five children (1 boy and 4 girls) with self-induced pattern-sensitive epilepsy. All patients had refractory epilepsy with multiform, though mainly myoclonic, seizures and medium grade to severe mental retardation. Spontaneously self-induced seizures were documented in all cases by EEG. All the patients underwent full neurophysiological assessment (baseline EEG recording, with activation: eyelid closed, hyperventilation, ILS, EEG during the randomized presentation of 3 types of spatial structured stimuli, VEP-F and VEP-PR). The pattern that triggered the EEG anomalies was highly specific and selective for each patient. Clinical seizures were reproduced by the same patterns as the ones used by the patients to bring on the seizures at will. Authors stress the importance of identifying such peculiar type of epilepsy and of attempting adequate treatment.

Transport and metabolism of glutamic acid studied in the human placenta by an in vitro perfusion method

Eyelid myoclonia with absences (EMA) and juvenile myoclonic epilepsy (JME) are two separate epileptic syndromes included in the new classification of epilepsies and epileptic syndromes by ILAE in 2001. Both are idiopathic generalized epilepsies with their clinical onset in the first two decades. EMA is characterized by eyelid myoclonia associated with absences and photosensitivity. Self-induced seizures are frequently seen in EMA. It can be associated with mildly mental retardation and resistance to treatment. JME includes three types of generalized seizures: typical absences, myoclonic jerks and generalized tonic-clonic seizures. The myoclonic jerks occur almost exclusively on awakening, involve preferently the upper extremities, may rarely affect the lower extremities or the entire body. More severe attacks may be accompanied by a fall. The myoclonic jerks occur rarely in EMA. They are usually mild and are freqently restricted to the upper extremities. Generalized tonic-clonic seizures, photosensitivity and generalized polyspike-wave discharges provoked by eye closure are features of both epileptic syndromes. In this study, we describe four female patients with eyelid myoclonia associated with absences, myoclonic jerks causing falling down and rare generalized tonic-clonic seizures. All patients had good school performance and total seizure control under sodium valproate treatment. Their EEGs show generalized polyspike-wave discharges with a frequency of 3.5–6 Hz always appearing a few seconds after eye closure and photoparoxysmal response. These patients show the characterictics of both epileptic syndromes. It is clinically important to make a syndromic diagnosis for an optimum advise on treatment, lifestyle restrictions and prognosis. In this study, we have gathered evidence that EMA and JME are dynamic syndromes that tend to evolve into one another.