Nonketotic hyperglycemia-related reflex epileptic seizures induced by Mah-Jong playing

Seizures related to nonketotic hyperglycemia (NKH) are often encountered in clinical practice. Among such seizures, reflex seizures are rare, and most of them are movement-induced focal seizures. We describe five patients with NKH and reflex seizures induced exclusively by playing Mah-Jong, a traditional and popular game in Chinese society. Four patients had partial seizures and one had a generalized seizure. All the patients manifested NKH on occurrence of the seizure. During Mah-Jong in the presence of NKH, seizures could arise from the frontal and temporal lobes, which are highly activated during the game. Both avoidance of Mah-Jong playing and control of glucose intake help prevent this disorder.     

Reflex epilepsy induced by playing Go-stop or Baduk games

PurposeSeizures can be triggered by complex mental activities. The aim of this study was to investigate the clinical characteristics of reflex epilepsy induced by playing Go-stop or Baduk games.MethodsThe study comprised 11 patients with this type of reflex epilepsy identified from our patient database. We collected data on clinical features, EEG, and brain MRI as well as seizure outcomes.ResultsThe patients had a late age of onset (range, 43–65 years, except for one patient with an age of onset of 11 years). An MRI abnormality and interictal EEG abnormalities were found in one patient respectively. The seizures exhibited diverse semiological features suggesting a focal or generalized onset. No myoclonic seizures were observed. Individualized strategies such as avoiding the precipitating game or reducing exposure to the stimulus were most effective in preventing the seizures.ConclusionThe pathophysiological mechanisms underlying Go-stop- or Baduk-induced seizures may be different from other cognition-associated reflex epileptic phenomena. This type of reflex epilepsy may be a heterogeneous syndrome manifesting in a range of diverse semiological features.     

Effects of zonisamide on the electroencephalogram of a patient with juvenile myoclonic epilepsy

Zonisamide, a newer antiepileptic drug (AED) with multiple mechanisms of action, has been increasingly used in patients with generalized epilepsies. This case study focuses on the effects of zonisamide on the EEG of a patient with juvenile myoclonic epilepsy. The patient had complete resolution of myoclonic jerks and absence and generalized seizures on introduction and titration of therapy with zonisamide; his EEG showed almost complete resolution of generalized spike and wave discharges and electrographic absence seizures. This case report suggests that zonisamide may be effective in the treatment of medication-resistant idiopathic generalized epilepsy and that it may lead to a significant improvement of the EEG.     

麻将游戏诱发癫痫发作14例报告

目的 分析玩麻将诱发癫痫的临床特点。方法 回顾性分析1996－2000年在我院癫痫中心诊治的14例因玩麻将引起癫痫发作的患者。结果 2例患者表现为部分性发作继发全身性发作，余12例表现为全身强直－阵挛性发作。发作间歇期脑电图检查13例正常，1例呈爆发性双侧对称性同步高波幅3次／s的棘慢波综合。14例均未服用抗癫痫药。结论 玩麻将诱发的癫痫属于反射性癫痫。其发生机制可能与打麻将时思考、空间任务和决定有关，是一种良性癫痫，避免打麻将则癫痫发作停止。     

Reflex occipital lobe epilepsy.

Photosensitivity is a typical feature of photosensitive epilepsy which is usually considered a form of idiopathic generalized epilepsy. Partial seizures featuring visual symptoms are rarely reported in photosensitive epilepsy. In this study, we describe 13 neurologically normal patients in whom daytime seizures were always induced by television and began with elementary visual hallucinations, followed frequently by vomiting, headache and then secondary generalization. Three patients additionally reported nocturnal seizures, which have not been described in previous studies. Two of these latter patients had generalized tonic-clonic seizures, the other always awoke from sleep and could describe typical visual hallucinations at the beginning of the seizure. EEG features included normal background activity and occipital spikes or spike-waves in all but two patients. Eight patients also showed generalized epileptiform activity during intermittent photic stimulation. Seizure frequency was low in all. Apart from two patients, who refused treatment, all patients received antiepileptic drugs. Only one patient continued to have rare seizures after treatment; in the others seizure control was achieved with monotherapy. We conclude that reflex occipital lobe epilepsy is an idiopathic form of the benign partial epilepsies, which may overlap with one another.     

Possibilities of non-pharmacological conservative treatment of epilepsy.

This study set out to assess the effect of non-pharmacological conservative (NPC) interventions as alternatives to antiepileptic pharmacotherapy. A prospective follow-up cohort study was conducted in an outpatient seizure clinic of a referral center for epilepsy. Twenty-five patients (nine males, 16 females) aged 16-45, with at least two well-described epileptic seizures, were included who had rejected antiepileptic pharmacotherapy. Twelve had idiopathic generalized epilepsy, 11 had symptomatic or cryptogenic localization-related epilepsy, and two had epilepsy with generalized and focal signs. Twenty-three of the patients were followed for more than 2 years. The patients were treated with arrest after focal seizure onset (2 cases), sensory protection against reflex seizures (3 cases), avoidance of non-specific seizure-precipitating factors ('life hygiene', 16 cases), and/or miscellaneous interventions (8 cases). The main outcome measures were complete seizure control (more than 2 years) or sufficient improvement to continue with NPC treatment alone. Eight of the 23 patients were completely seizure free for more than 4 years, and three were sufficiently improved to continue NPC treatment without drugs. Trends were observed for patients with idiopathic generalized epilepsies with less than seven convulsive seizures, and with only one seizure type to respond better to NPC treatment. The duration of epilepsy, and the finding of generalized epileptiform discharge in the EEG had no influence on the outcome. Rational NPC treatments which are aimed at specific factors in the precipitation and development of epileptic seizures can be useful therapeutic alternatives for patients with milder forms of epilepsy. Apart from photosensitive patients, those most likely to profit are patients with idiopathic generalized epilepsy, a maximum of six generalized tonic-clonic seizures which were precipitated by lack of sleep or excessive alcohol intake, and with no or rare concomitant absences. In such cases, NPC treatment may be as effective as pharmacotherapy and gives the patient a positive experience of regained self-control.     

Hot water epilepsy: clinical and electrophysiologic findings based on 21 cases

PURPOSE: Our aim is to outline the clinical and electroencephalographic (EEG) features of patients with hot water epilepsy (HWE), a rare and unique form of reflex epilepsy. METHODS: Twenty-one patients with HWE, seen in our clinic until 1999, were studied. Male outnumbered female subjects in a ratio of 3:1. The age at the onset of seizures ranged from 19 months to 27 years (mean age at onset, 12 years). RESULTS: The main factors precipitating seizures were bathing with hot water and/or pouring water over the head. Six patients reported self-induction, either by increasing the heat or the amount of water and/or recalling earlier bathing experiences. Nine patients expressed feeling pleasure during the seizures. Twenty patients had partial seizures, eight of whom also had secondarily generalized seizures. One patient had apparent generalized seizures only. Spontaneous seizures were present in 62% of the cases. Interictal epileptogenic abnormalities were documented in the EEGs of eight patients; the other eight had normal EEGs. The major sites of epileptogenic activity were over the unilateral temporal regions (in 40% of patients). Neuroimaging studies available for 12 patients (four cranial computed tomography and eight cranial magnetic resonance imaging scans) revealed normal findings. Seizure control in patients who were followed up was achieved by reducing the temperature or the duration of the bath or shower; several of the patients required medication. CONCLUSIONS: The major findings of this study are that HWE has a male preponderance, can be self-induced, is often done for pleasure, has complex triggering factors, and shows temporally located abnormalities in the EEGs. Although HWE is generally known to be self-limited, antiepileptic drug treatment may sometimes be necessary to control seizures. Hot water epilepsy should be classified separately among the epileptic syndromes.     

Adult‐onset photosensitivity: clinical significance and epilepsy syndromes including idiopathic (possibly genetic) photosensitive occipital epilepsy

To evaluate the clinical associations of adult‐onset photosensitivity, we studied the clinical and EEG data of patients who were referred due to a possible first seizure and who had a photoparoxysmal response on their EEG. Patients with clinical evidence of photosensitivity before the age of 20 were excluded. Of a total of 30 patients, four had acute symptomatic seizures, two had vasovagal syncope, and 24 were diagnosed with epilepsy. Nine of the 24 patients had idiopathic (genetic) generalized epilepsies and predominantly generalized photoparoxysmal response, but also rare photically‐induced seizures, while 15 had exclusively, or almost exclusively, reflex photically‐induced occipital seizures with frequent secondary generalization and posterior photoparoxysmal response. Other important differences included a significantly older age at seizure onset and paucity of spontaneous interictal epileptic discharges in patients with photically‐induced occipital seizures; only a quarter of these had occasional occipital spikes, in contrast to the idiopathic (genetic) generalized epilepsy patients with typically generalized epileptic discharges. On the other hand, both groups shared a positive family history of epilepsy, common seizure threshold modulators (such as tiredness and sleep deprivation), normal neurological examination and MRI, a generally benign course, and good response to valproic acid. We demonstrated that photosensitivity can first occur in adult life and manifest, either as idiopathic (possibly genetic) photosensitive occipital epilepsy with secondary generalization or as an EEG, and less often, a clinical/EEG feature of idiopathic (genetic) generalized epilepsies. Identification of idiopathic photosensitive occipital epilepsy fills a diagnostic gap in adult first‐seizure epileptology and is clinically important because of its good response to antiepileptic drug treatment and fair prognosis.     

Treatment of epilepsy in adults. Options and strategies

Currently, epilepsy can be treated with antiepileptic drugs and, in patients with focal and/or secondarily generalized seizures (focal epilepsy), by means of surgery and vagus nerve stimulation. In the choice of monotherapy possible negative drug related effects on cognitive, endocrine, and psychic symptoms must be considered. Newly developed antiepileptic drugs help to establish an individualized strategy, especially in antiepileptic drug monotherapy. Additionally these antiepileptic drugs have proven to be effective and well tolerated when combined with other antiepileptic drugs. Surgery of focal epilepsy offers the chance of complete cure. Vagus nerve stimulation is a nonmedical treatment option used in addition to antiepileptic drugs in patients with focal epilepsy. Tolerability and safety data should be considered to establish a long-term medical treatment tolerated and accepted by the patient.     

Video game epilepsy.

Reflex epilepsy is the commonest form of epilepsy in which seizures are provoked by specific external stimulus. Photosensitive reflex epilepsy is provoked by environmental flicker stimuli. Video game epilepsy is considered to be its variant or a pattern sensitive epilepsy. The mean age of onset is around puberty and boys suffer more commonly as they are more inclined to play video games. Television set or computer screen is the commonest precipitants. The treatment remains the removal of the offending stimulus along with drug therapy. Long term prognosis in these patients is better as photosensitivity gradually declines with increasing age. We present two such case of epilepsy induced by video game.     

Childhood epilepsy syndromes with both focal and generalized seizures

Childhood is a time of considerable importance for the onset of epilepsy syndromes. Selection of an appropriate antiepileptic drug (AED) is central to its successful management. Different AEDs have various effects depending on whether seizures are focal or generalized and this is often used as a rational basis for drug selection. Syndromes, in which features of both focal and generalized seizures are associated in a single patient, present particular problems for pediatric prescribing and epilepsy management since an AED suitable for one seizure type may be ineffective for, or even aggravate, another seizure type. This review discusses the syndromes with different seizures types, focal and generalized, examines the treatment options that may be useful in each case and highlights the potential of some of the newer AEDs in managing these difficult syndromes.     

Adult absence semiology misinterpreted as mesial temporal lobe epilepsy

Correct diagnosis of seizure type and epilepsy syndrome is the foundation for appropriate antiepileptic drug selection. Inappropriate medication choices occur in the treatment of generalized epilepsy and may aggravate some seizure types, including absence seizures, potentially leading to pseudo‐drug resistance. Fortunately, a correct diagnosis of absence seizures is usually not difficult, though rarely demonstrates electroclinical overlap with focal seizures. EEG can be especially misleading when secondary bilateral synchronous discharges occur in patients with focal seizures. However, the semiology of focal seizures associated with mesial temporal lobe epilepsy has a characteristic and consistent semiology that is the mark of this common epilepsy syndrome in adulthood. We recently encountered a 53‐year‐old female with refractory seizures and a semiology strongly suggesting mesial temporal lobe epilepsy. Instead of focal seizures, prolonged absence seizures were validated by video‐EEG monitoring and she became seizure‐free after a change to broad‐spectrum antiepileptic drugs. This case further expands our understanding of the complexity of semiology in electroclinical classification and the spectrum that may occur in adult absence seizures. It serves to underscore the need for ictal EEG recordings and the importance of concordance with the clinical course during the pre‐surgical evaluation of patients with lesions and drug‐resistant epilepsy. [Published with video sequences]     

Surgical treatment for epilepsy

Nearly one-third of patients with newly diagnosed epilepsy will develop medically refractory seizure disorders. The initial response to antiepileptic drug therapy is highly predictive of long-term outcome. Patients with intractable epilepsy may have a progressive disorder that is medically, physically, and socially disabling. Surgical resection of the epileptogenic zone or lesional pathology, or both, may significantly reduce seizure tendency in selected patients. The present review supports the position that early and effective epilepsy surgery may not only render the patient with intractable partial epilepsy seizure-free, but also allow the individual to become a participating and productive member of society. Patients with surgically remediable epileptic syndromes should be identified early in the evaluation and treatment of their seizure disorders. Favorable candidates for focal cortical resection include individuals with medial temporal lobe epilepsy and partial seizures related to selected lesional pathology, e.g. primary brain tumor or vascular anomalies. In conclusion, surgical treatment of intractable partial epilepsy has been shown to compare favorably to antiepileptic drug therapy. Individuals rendered seizure-free may experience a significant improvement in quality of life. Patients who fail to respond to initial antiepileptic drug therapy should be “triaged” to a presurgical evaluation. Ictal semiology combined with structural magnetic resonance imaging and the electroclinical correlation may permit identification of candidates for early and effective surgical treatment.     

A patient with myoclonic epilepsy in infancy followed by myoclonic astatic epilepsy

Myoclonic epilepsy in infancy (MEI) is a primary generalized epilepsy. According to the literature, the outcome of MEI is usually benign. Here we report a patient who developed myoclonic astatic epilepsy at age four, having been seizure free without antiepileptic drug treatment for 2 years after his recovery from MEI. At age four, a video-EEG-recording showed frequent head nodding (atonic seizures) and myoclonic astatic seizures associated with diffuse spikes or polyspikes and waves. The interictal EEG revealed frequent bursts of generalized 100-200 μV, 2-4 Hz spike-and-slow-wave complexes. Despite a general favorable outcome, more severe epilepsy syndromes may develop after MEI, and mental retardation is sometimes observed. Our case and the previous literature suggest that epilepsies following on from MEI often involve myoclonic seizures.     

Effect of vagus nerve stimulation on adults with pharmacoresistant generalized epilepsy syndromes.

INTRODUCTION: Although vagus nerve stimulation (VNS) therapy is approved for the treatment of partial onset seizures, its efficacy for generalized seizures has not been fully evaluated. This Investigational Device Exemption assessed the outcome of VNS therapy among patients with generalized epilepsy syndromes. METHODS: Sixteen patients with pharmacoresistant generalized epilepsy syndromes and stable antiepileptic drug (AED) regimens were implanted with the VNS therapy device and were evaluated for changes in seizure frequency and type between baseline and follow-up of 12-21 months. RESULTS: The patients experienced a statistically significant overall median seizure frequency reduction of 43.3% (P = 0.002, Wilcoxon signed rank test) after 12-21 months of VNS therapy. Types of seizures that may involve a fall or collapse decreased with reductions in the frequency of myoclonic (60% reduction, n = 9; P = 0.016, Wilcoxon signed rank test), tonic (75% reduction, n = 8, NS), atonic (98.6%, n = 3, NS), and clonic seizures (86.7%, n = 1, NS). Conclusion: The benefits of reduced seizure frequency and reduced risk of injury merit consideration of VNS therapy for patients with pharmacoresistant generalized seizure syndromes.     

Exceptional response to brivaracetam in a patient with refractory idiopathic generalized epilepsy and absence seizures

Brivaracetam is currently indicated as adjunctive therapy for patients with focal‐onset seizures with or without secondary generalization. However, it has been suggested that it could provide broad‐spectrum efficacy given its similarity to levetiracetam and based on the results from preclinical studies and others of patients with generalized epilepsy. We present the case of a woman with refractory idiopathic generalized epilepsy and absence seizures with dramatic response to brivaracetam. Our report supports a consideration of treatment with this new antiepileptic drug on a case‐by‐case basis in patients with refractory generalized epilepsy, while we await further studies on this topic.     

Focal semiologic and electroencephalographic features in patients with juvenile myoclonic epilepsy

PURPOSE: A few reports have described focal electroencephalographic or clinical features or both of juvenile myoclonic epilepsy (JME), but without video-EEG documentation. We examined focal clinical and EEG features in patients with JME who underwent video-EEG monitoring. METHODS: Twenty-six patients (nine males and 17 females) who had seizures recorded during video-EEG monitoring were included. Age at seizure onset was 0 to 22 years (mean, 12.3 years), and age at monitoring was 12 to 44 years (mean, 26.5 years). In one patient with left parietooccipital epilepsy, primary generalized tonic-clonic seizures developed after resection of the parietal tumor. Two patients had both temporal lobe epilepsy and JME. Videotaped seizures in each patient were analyzed. Interictal and ictal EEG also were analyzed for any focal features. RESULTS: Focal semiologic features were observed in 12 (46%) of 26 patients. Six patients had focal myoclonic seizures, and two had Figure 4 sign: one with version to the left, and another had left version followed by Figure 4 sign, and left arm clonic seizure. Their ictal EEGs were generalized at onset but with a lateralized evolution over the right hemisphere. The patient who had both JME and left parietooccipital epilepsy, right arm clonic seizure, and Figure 4 sign was seen during a generalized EEG seizure. Interictally, one patient had temporal sharp waves, and another had run of spikes in the right frontal region. CONCLUSIONS: Fourteen (54%) of 26 patients with JME exhibited focal semiologic or electroencephalographic features or both. Video-EEG was essential in reaching a correct diagnosis and choosing an appropriate antiepileptic drug regimen.     

Levetiracetam in the treatment of epilepsy

Epilepsy is a common chronic disorder that requires long-term antiepileptic drug therapy. Approximately one half of patients fail the initial antiepileptic drug and about 35% are refractory to medical therapy, highlighting the continued need for more effective and better tolerated drugs. Levetiracetam is an antiepileptic drug marketed since 2000. Its novel mechanism of action is modulation of synaptic neurotransmitter release through binding to the synaptic vesicle protein SV2A in the brain. Its pharmacokinetic advantages include rapid and almost complete absorption, minimal insignificant binding to plasma protein, absence of enzyme induction, absence of interactions with other drugs, and partial metabolism outside the liver. The availability of an intravenous preparation is yet another advantage. It has been demonstrated effective as adjunctive therapy for refractory partial-onset seizures, primary generalized tonic-clonic seizures, and myoclonic seizures of juvenile myoclonic epilepsy. In addition, it was found equivalent to controlled release carbamazepine as first-line therapy for partial-onset seizures, both in efficacy and tolerability. Its main adverse effects in randomized adjunctive trials in adults have been somnolence, asthenia, infection, and dizziness. In children, the behavioral adverse effects of hostility and nervousness were also noted. Levetiracetam is an important addition to the treatment of epilepsy.     

Lacosamide in refractory mixed pediatric epilepsy: a prospective add-on study

Lacosamide is a new antiepileptic drug that is currently approved by the US Food and Drug Administration (FDA) for adults 17 years or older for partial-onset seizures. The authors reviewed 21 pediatric patients (<17 years) with various seizure types who were started on oral lacosamide as part of a prospective add-on study as adjunctive therapy for refractory epilepsy. Five patients were excluded due to less than 3 months of meaningful follow-up. Maintenance dosages used ranged from 2.4 to 19.4 mg/kg/d. Eight of 16 (50%) patients had greater than 50% reduction in seizure frequency with adjunctive lacosamide therapy. Eight (50%) patients had generalized epilepsy including 4 with Lennox-Gastaut syndrome. Lacosamide was effective therapy for most seizure types but was particularly effective for partial-onset seizures. Lacosamide was effective in treating 5 of 8 (62.5%) localization-related epilepsies but only 2 of 8 (25%) generalized epilepsies, both Lennox-Gastaut syndrome patients with greater than 90% seizure reduction. None of these very refractory patients remained seizure free.     

Long-Term Outcome of Pattern-Sensitive Epilepsy

Summary:  Purpose: To evaluate the long-term outcome of patients with pattern-sensitive epilepsy.
Methods: We prospectively studied 35 patients (21 females and 14 males) with pattern-sensitive epilepsy (follow up ≥5 years; mean 13.9; range 6.4 – 27.6). All cases had regular clinical examinations and serial electroencephalographic (EEG) recordings. Photosensitivity and pattern sensitivity were ascertained neurophysiologically in all cases. Outcome was evaluated according to the following variables: duration of photosensitivity, rate of remission (seizure-free period ≥ 2 years), withdrawal of therapy, and recurrence after drug discontinuation.
Results: The epilepsy was generalized in 18 cases (17 idiopathic, one symptomatic) and partial in 17 (13 idiopathic, four symptomatic). Sixteen patients (46%) had a family history of seizures. The mean age at the last examination was 21.4 years (range 11.2–35.5 years). Five patients (14%) had only reflex seizures. The most common type of spontaneous seizures was generalized (60%), whereas reflex seizures were more frequently partial (74%). Mean epilepsy duration was 8.7 ± 6.3 years. Patients with only reflex seizures were instructed to avoid precipitating stimuli and were not treated with antiepileptic drugs. Treatment was gradually withdrawn in 10 out of 30 treated patients, with relapse in only two cases. At the end of follow up, 28 patients (80%) were seizure-free.
Conclusion: The long-term outcome of patients with pattern-sensitive epilepsy indicates a good prognosis with a favorable course for both spontaneous and reflex seizures.