Epidemiologic Study of Epilepsy in Young Singaporean Men

PURPOSE: This survey of 20,542 Singaporean men born in 1974 studied the clinical features of young men diagnosed with epilepsy on preenlistment screening. METHODS: All male citizens in Singapore are medically screened at age 18 years before enlistment for compulsory military service. Patients suspected to have epilepsy are then referred to government hospitals for further management. We interviewed the patients and their parents and reviewed their hospital records. RESULTS: Eighty-nine patients with epilepsy were identified, indicating a lifetime prevalence of 4.9/1,000 males by age 18 years. The lifetime prevalence of epilepsy among Chinese, Malays, and Indians were 5.2, 2.8, and 6.4/1,000, respectively; these differences were not statistically significant. The mean age of seizure onset was 11.1 years. Generalized seizures (65.2%) were commoner than partial seizures (34.8%); common seizure types included generalized tonic-clonic seizures (52.8%), complex partial seizures with secondary generalization (24.7%), and myoclonic seizures (5.6%). Common epileptic syndromes included temporal lobe epilepsy (16.9%), juvenile myoclonic epilepsy (5.6%), and frontal lobe epilepsy (2.2%). Eighty-four (94.4%) patients sought medical treatment, and seven (7.9%) patients sought additional traditional treatment. Although 70 (78.7%) patients responded to medication, 14 (15.7%) patients remained refractory to treatment. CONCLUSIONS: The lifetime prevalence of epilepsy in young Singaporean men was 4.9/1,000. The majority (65.2%) had generalized seizures. Temporal lobe epilepsy was the commonest (16.9%) defined epilepsy syndrome. More patients with epilepsy (94.4%) sought medical treatment, although 15.7% remained refractory to medication.     

Levetiracetam monotherapy for primary generalised epilepsy.

PURPOSE: To evaluate the efficacy of levetiracetam in cases of refractory primary generalised epilepsy. METHODS: Three patients with refractory primary generalised epilepsy were treated with levetiracetam monotherapy; one with absence seizures, myoclonic jerks and generalised tonic-clonic (GTC) seizures one with myoclonic jerks and GTC seizures, and one with only GTC seizures. All three patients had generalised spike wave on the EEG and had failed at least three antiepileptic drugs (AEDs) before trying levetiracetam. RESULTS: All three patients tolerated levetiracetam well and became seizure free for at least 6 months. Therapeutic doses of levetiracetam ranged from 1250 to 3000 mg/day. CONCLUSION: Levetiracetam, a new AED with a novel mechanism(s) of action, should be considered for patients with refractory primary generalised epilepsy.     

Epilepsy in Pakistan: A Population-Based Epidemiologic Study

Summary: A house-to-house, cross-sectional, population study of epilepsy on 24,130 individuals of all ages from southern Pakistan indicates an age-specific prevalence rate of 9·99 in 1,000 (14·8 in 1,000 in rural and 7·4 in 1,000 in urban areas) for recurrent, non febrile "active" epilepsy in Pakistan. Mean onset of epilepsy was 13·3 years, and 74·3% epileptic persons were aged <19 years at onset of the disorder. The most common seizure type was ton-icclonic in 77% [primary generalized tonic-clonic (GTC) in 59% and secondarily generalized in 18%], simple partial (SPS) in 5%, complex partial (CPS) in 6%, generalized absence in l%, tonic in 3%, and myoclonic in 3% cases. Multiple seizure types in the same person were evident in 9·6% of only the generalized group. A putative cause could be suggested in 38·4% of cases: 32% had a positive family history of epilepsy, most common among siblings. Common perceived precipitants included fever in 29·2% and emotional disturbances in 16·6%. Only 3% of epileptic persons believed that their illness was due to supernatural causes. Treatment status was very poor, with only 2% rural and 27% urban epileptic persons receiving antiepileptic drugs (AEDs) at the time of the survey. We discuss the logistic and management problems of population-based epidemiologic studies in developing countries.     

Long-Term Course of Childhood Epilepsy with Intractable Grand Mal Seizures

Abstract: Twenty-nine children with childhood epilepsy characterized by frequent grand mal (generalized tonic-clonic) seizures in spite of maximal doses of antiepileptic drugs and by an early onset of seizures (before 1 :year of age) were followed up for more than 5 :years. The children were divided into 3 :groups: severe myoclonic epilepsy in infancy (SME), no SME, and intractable childhood epilepsy with generalized tonic-clonic seizures (GTC). In all the 3 :groups, the grand mal seizures persisted, whereas the other types of seizures tended to disappear as the patients aged, and the prognosis for mental development was poor. In the majority of cases in all the 3 :groups, the waking grand mal seizures altered to sleep grand mal seizures with aging. Two pairs of monozygotic twins with SME suggested that genetic factors play a role in this epileptic syndrome. Intractable childhood epilepsy with GTC is distinguished by the absence of other types of generalized seizures. It cannot be regarded as an epileptic syndrome, but its pathogenesis and treatment require further studies.     

Refractory grand mal seizures with onset during infancy including severe myoclonic epilepsy in infancy.

In 1978, Dravet proposed a clinical entity called severe myoclonic epilepsy in infancy (SMEI). In the same year, a patient group, which was later called high voltage slow wave-grand mal syndrome (HVSW-GM), is reported in Japan. Both syndromes are very similar, except for seizure manifestation: generalized tonic-clonic convulsions (GTC) with myoclonic and other polymorphic seizures in SMEI vs. GTC only in HVSW-GM. To study the pathophysiology of these refractory epilepsies, the author formulated new clinical diagnostic criteria common to both syndromes as follows: GTC with onset before the age of 1 year as the principal seizure type; an epilepsy entity unclassifiable either as partial or generalized by all the clinical data including EEG findings; mental and motor dysfunction absent prior to seizure onset but appearing later; absence of epileptiform activities on EEG in the initial stage; stubborn refractoriness to conventional antiepileptic medication. Twenty-two patients meeting all of five clinical criteria above mentioned were recruited in the study. Detailed analysis of clinico-electrical features and long-term follow-up of these patients led the author to the conclusion that GTC in combination with seizures of other types will contribute to an unfavorable pathophysiological or prognostic conditions, and, especially when GTC exists in combination with myoclonic seizures, the severity of epilepsy will increase. The author claimed that the three clinical entities, SMEI, HVSW-GM, and their variant form, share certain characteristics in common and may constitute a unique epilepsy syndrome for which a new name of infantile refractory grand mal syndrome (IRGMS) was offered. This is a more basic concept with broader spectrum than SMEI, encompassing not only SMEI but also related borderlands like HVSW-GM. More recently, the author observed that early zonisamide medication within 1 year after seizure onset may improve seizure prognosis in IRGMS, by preventing the development of myoclonic seizures.     

Juvenile Myoclonic Epilepsy: A 5-Year Prospective Study

Summary: We made a long term prospective study of 66 patients with juvenile myoclonic epilepsy (JME). Prevalence was 10.2% among 672 patients with epilepsies. Sex distribution was equal. Sixty-three were not diagnosed on referral; JME was not initially recognized in the epilepsy clinic in 22. Clinical typical absence seizures were reported in 33.3%, myoclonic jerks in 97% and generalized tonic-clonic seizures (GTC) in 78.8% of the patients. Mean age (±SD) at onset was 10.5 ± 3.4 years (range 5–16 years) for absence seizures, 15 ± 3.5 years (range 8–26 years) for myoclonic jerks, and 16 ± 3.5 years (9–28) for GTC. Absence predated myoclonic jerks by 3.9 ± 2.3 years (range-1–9 years) and GTC by 4.4 ± 2.7 years (range 1–8 years) in 14 (21.2%) patients who manifested all three types of seizure. Absence were never antedated by myoclonic jerks or GTC. Myoclonic jerks occurred on awakening in 87.5% of the patients. GTC occurred mainly on awakening, but other patients had nocturnal or diurnal GTC with no circadian distribution. Neurologic examination was normal for all patients except for tremor of the hands similar to essential tremor, noted in 35% of patients. Computed tomography (CT) brain scans were normal: 93% of patients had precipitating factors: sleep deprivation (89.5%), fatigue (73.7%), photosensitivity (36.8%; television and video games 8.8%), menstruation (24.1% of women), mental concentration (22.8%), and stress (12.3%). Incidence of JME among siblings (13 of 41 examined families) implies an autosomal recessive mode of inheritance for this Arab population. EEGs were frequently normal in treated patients. At least one abnormal EEG was recorded in 56 (84.9%) patients. Abnormalities consisted mainly of generalized discharges of spike/double spike and/or polyspike and slow wave. Frequent multiple spikes and discharge fragmentations varied from 0.5to 20-s duration (mean 6.8 s). Twenty (30.3%) had focal abnormalities, and 18 (27.3%) had photoconvulsive discharges. Eighty-eight percent of patients remained seizure-free for 3 years of follow-up. Effective treatment was achieved with valproate (VPA); control of myoclonic jerks was improved with clonazepam (CZP). CZP monotherapy did not consistently prevent GTC. Adding small doses of CZP with simultaneous reduction of VPA was the most effective and better tolerated form of medication, particularly in patients demonstrating an adverse reaction or requiring a large VPA dosage. VPA dosage was successfully reduced in 15 patients who were seizure-free for >2 years and had infrequent seizures before treatment, but 9 of 11 patients relapsed after VPA discontinuation.     

EEG discharges on awakening: a marker of idiopathic generalized epilepsy

In a series of 24-hour ambulatory EEG recordings from 1,000 consecutive adult outpatients (44.5% with generalized and 55.5% with partial epilepsy, one recording per patient), the authors found only 46 (4.6%) activations of epileptiform discharges on awakening. All recordings came from patients with idiopathic generalized epilepsy, predominantly with juvenile myoclonic epilepsy and generalized tonic-clonic seizures on awakening. Multiple spike discharges that develop with an unusually delayed onset after arousal (more than 10 minutes) might help to discriminate juvenile myoclonic epilepsy.     

Observations on the misdiagnosis of generalized epilepsy as partial epilepsy: causes and consequences.

More therapeutic options (surgical and pharmacologic) are available for partial than for generalized epilepsies. This report describes and analyzes a possible bias to diagnose focal epilepsies. Data were prospectively collected on patients who underwent noninvasive prolonged EEG-video monitoring over a 2-year period at an epilepsy program. Cases where the diagnosis of 'partial seizures' (after monitoring) was questionable were identified and the data reviewed. Sixteen cases were identified. (a) Six had an idiopathic generalized epilepsy. All had generalized tonic-clonic (GTC) seizures, two had myoclonic seizures, and three had typical absences. All patients had generalized spikes and spike-wave complexes. All had normal IQs and normal brain imaging. One patient underwent invasive EEG. (b) Ten patients had a symptomatic or cryptogenic generalized epilepsy. IQs ranged from 49 to 74 (mean: 63). All patients had diffuse EEG slowing, and generalized ictal EEG patterns. Interictal EEG showed generalized spike-wave complexes in nine, and multifocal spikes in five. Seizures included GTC in all, generalized tonic in four, and atypical absences in two. Two of the 10 patients underwent invasive EEG. The misdiagnosis of generalized epilepsy as partial epilepsy occurs for both idiopathic and cryptogenic or symptomatic generalized epilepsies, more often in the latter case. Risk factors may include: asymmetry in EEG or seizure semeiology, the eagerness to enroll in drug studies or surgical programs, and the lack of team thinking involving several epileptologists. This problem is almost certainly under-reported and may occasionally result in unwarranted invasive procedures.     

Worsening of seizures by oxcarbazepine in juvenile idiopathic generalized epilepsies

PURPOSE: Several studies have shown that carbamazepine (CBZ) may aggravate idiopathic generalized epilepsy (IGE). Oxcarbazepine (OXC) is a new drug chemically related to CBZ. We report six cases of juvenile IGE with a clear aggravation by OXC. METHODS: We retrospectively studied all patients with IGE first referred to our epilepsy department between January 2001 and June 2003 and treated with OXC. RESULTS: During this period, six patients were identified. All had an aggravation of their epilepsy in both clinical and EEG activities. OXC had been used because of an incorrect diagnosis of focal epilepsy or generalized tonic-clonic seizures (GTCSs) of undetermined origin (no syndromic classification of the epilepsy). Before OXC, only one patient had experienced a worsening of seizures with an inadequate drug (CBZ). Four had juvenile myoclonic epilepsy, one had juvenile absence epilepsy, and one had IGE that could not be classified into a precise syndrome. OXC (dosage range, 300-1,200 mg/day) was used in monotherapy in all of them except for one patient. Aggravation consisted of a clear aggravation of myoclonic jerks (five cases) or de novo myoclonic jerks (one case). Three patients had exacerbation of absence seizures. One patient had worsened dramatically and had absence status, and one had de novo absences after OXC treatment. The effects of OXC on GTCSs were less dramatic, with no worsening in frequency in three and a slight increase in three. CONCLUSIONS: OXC can be added to the list of antiepileptic drugs that can exacerbate myoclonic and absence seizures in IGE.     

Distinct white matter abnormalities in different idiopathic generalized epilepsy syndromes

Purpose: By definition idiopathic generalized epilepsy (IGE) is not associated with structural abnormalities on conventional magnetic resonance imaging (MRI). However, recent quantitative studies suggest white and gray matter alterations in IGE. The purpose of this study was to investigate whether there are white and/or gray matter structural differences between controls and two subsets of IGE, namely juvenile myoclonic epilepsy (JME) and IGE with generalized tonic–clonic seizures only (IGE‐GTC). Methods: We assessed white matter integrity and gray matter volume using diffusion tensor tractography–based analysis of fractional anisotropy and voxel‐based morphometry, respectively, in 25 patients with IGE, all of whom had experienced generalized tonic–clonic convulsions. Specifically, 15 patients with JME and 10 patients with IGE‐GTC were compared to two groups of similarly matched controls separately. Correlations between total lifetime generalized tonic–clonic seizures and fractional anisotropy were investigated for both groups. Key Findings: Tractography revealed lower fractional anisotropy in specific tracts including the crus of the fornix, body of corpus callosum, uncinate fasciculi, superior longitudinal fasciculi, anterior limb of internal capsule, and corticospinal tracts in JME with respect to controls, whereas there were no fractional anisotropy differences in IGE‐GTC. No correlation was found between fractional anisotropy and total lifetime generalized tonic–clonic seizures for either JME or IGE‐GTC. Although false discovery rate–corrected voxel‐based morphometry (VBM) showed no gray matter volume differences between patient and control groups, spatial extent cluster–corrected VBM analysis suggested a trend of gray matter volume reduction in frontal and central regions in both patient groups, more lateral in JME and more medial in IGE‐GTC. Significance: The findings support the idea that the clinical syndromes of JME and IGE‐GTC have unique anatomic substrates. The fact that the primary clinical difference between JME and IGE‐GTC is the occurrence of myoclonus in the former raises the possibility that disruption of white matter integrity may be the underlying mechanism responsible for myoclonus in JME. The cross‐sectional study design and relatively small number of subjects limits the conclusions that can be drawn here; however, the absence of a correlation between fractional anisotropy and lifetime seizures is suggestive that the white matter abnormalities observed in JME may not be secondary to seizures.     

Electroclinical features of epilepsy in patients with juvenile type dentatorubral-pallidoluysian atrophy

Purpose: To clarify the electroclinical characteristics of epileptic seizures in patients with juvenile type dentatorubral‐pallidoluysian atrophy (DRPLA). Methods: Seventeen patients with juvenile type DRPLA confirmed by genetic analysis were studied retrospectively. The clinical records of all 17  patients and the ictal video electroencephalography (EEG) recordings from 12 of the 17 patients were reviewed. Results: Electroclinical studies in 12 patients identified 11 habitual seizures in 6 patients as partial seizures on ictal video EEG recordings. Clinical manifestations composed mainly of versions of the head and loss of consciousness. These partial seizures were persistently recorded throughout the clinical course. Brief generalized seizures (atypical absence and myoclonic seizure) were observed in 6 of 12 patients at the early stage. In contrast, generalized tonic–clonic seizures (GTCS) were recorded in four advanced stage patients who were almost bedridden. Semiological studies in 17 patients showed that the prevalence of partial seizures was significantly higher in patients with younger epilepsy onset (below 10 years of age; χ² test, p < 0.05) and that the age of epilepsy onset was significantly lower in patients with partial seizures than in those without partial seizures (Mann‐Whitney U test, p = 0.02). However, the number of CAG repeats and age at clinical onset were not significantly different between two groups. Discussion: Partial seizure is one of the common epileptic features in juvenile type DRPLA, especially in patients with younger epilepsy onset. Seizure types may be affected in an age‐dependent manner and change evolutionally during progression of the clinical stage.     

Corpus callosotomy in refractory idiopathic generalized epilepsy

RATIONALE: A small percentage of patients with idiopathic generalized epilepsy (IGE) do not respond to medical therapy. Generalized tonic-clonic (GTC) seizures are especially debilitating and can be associated with severe injuries. The benefit, safety and effect of corpus callosotomy (CC) in patients with IGE have not been studied. METHODS: We reviewed patients with presumed IGE who underwent CC between 1991 and 2000. Criteria for selection included history, examination, brain imagining, interictal and ictal EEG. All patients had refractory and debilitating tonic-clonic seizures (GTCS) and had failed four or more antiepileptic drugs. Seizure frequency was calculated per month over the last year and pre-operative baseline was compared to last follow-up using paired t-tests. IQ, executive function, language and verbal, non-verbal memory and quality of life (QOL) was compared before and after surgery. Serial EEGs after surgery were reviewed. RESULTS: There were nine patients (seven men), mean age 37.9 (range: 22-49), mean IQ 87.3 (range: 75-107). All had anterior CC. Mean follow-up time was 5.4 years (range: 0.6-10.3 years). One patient died from sudden death in epilepsy 9 months after surgery. There was a significant reduction of GTC seizures from 6.3 to 1.1 (p<0.005). Four patients had more than 80% and eight more than 50% reduction. Of five patients with absence seizures, two became seizure free and one had more than 80% reduction and two worsened slightly, and of three with myoclonic seizures one had more than 90% reduction. One patient had completion of the CC with improvement of myoclonus and absence seizures, but not of GTC seizures and suffered a disconnection syndrome. Another had right frontal focal resection without improvement after new seizures of focal onset. Cognitive testing showed a good outcome (improved or no change) in all cognitive domains. Post-surgical EEG showed new focal slowing and sharp waves. There was no change in QOL. CONCLUSION: CC can be effective in reducing GTC, absence and myoclonic seizures in patients with refractory IGE. These findings suggest that interhemispheric communication of the cerebral cortices plays an important role in the generation of seizures in IGE. Anterior CC appears safe while complete callosotomy has a risk of disconnection syndrome.     

Prevalence of epilepsy in Okayama Prefecture: a neuroepidemiologic study

On the prevalence day, 2,378 epileptic children were identified. Therefore, the prevalence rate for epilepsy was 8.2 per 1,000. The lowest prevalence rate was 1.2 in children under one year of age, and the highest was 11.0 at five years of age. The rate was higher for males than females. The annual incidence rate for epilepsy was estimated at 145.0 per 100,000 for 1975. The onset of seizures was high in the first three years, totaling 1,795 cases (77.7%), and this decreased after four years of age. Primary generalized epilepsy was found in 577 cases (31.7%), secondary generalized epilepsy in 167 (9.2%), partial epilepsy with elementary symptomatology in 205 (11.3%), with complex symptomatology in 61 (3.3%) and partial seizures secondarily generalized in 801 (44.5%). The study on such a number of cases had hitherto never been reported in the world.     

Focal clinical and electroencephalographic features in patients with juvenile myoclonic epilepsy

Jayalakshmi SS, Srinivasa Rao B, Sailaja S. Focal clinical and electroencephalographic features in patients with juvenile myoclonic epilepsy.
Acta Neurol Scand: 2010: 122: 115–123.
© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Objective – To identify prevalence and factors associated with occurrence of focal clinical and electroencephalogram (EEG) abnormalities in patients with juvenile myoclonic epilepsy (JME). Materials and methods – Clinical asymmetries in the seizures and focal EEG abnormalities were analyzed in 266 patients with JME. Results – All the patients had myoclonic jerks (MJ) and generalized tonic‐clonic seizures (GTCS); 56 (21%) had absence seizures. Asymmetry in clinical seizures was reported in 45 (16.9%) and focal EEG abnormalities were noted in 92 (45.5%) patients. Amplitude asymmetry or focal onset of generalized discharges was noted in 41 (44.6%) and independent focal EEG abnormalities in 30 (32.6%) patients. A statistically significant association was seen with the presence of GTCS and MJ (P = 0.007), a family history of epilepsy (P = 0.001) and drug resistance (P = 0.04) and the occurrence of focal EEG abnormalities. Conclusion – Patients with JME showed focal clinical and EEG features. These features should not be misinterpreted as indicative of partial epilepsy.     

Absence seizures with evolution into generalized tonic-clonic activity: clinical and EEG features

PURPOSE: To report the clinical and electrographic features of absence seizures evolving into generalized tonic-clonic (GTC) activity in six patients with idiopathic generalized epilepsy. METHODS: All patients were referred for evaluation of refractory seizures and underwent video-EEG monitoring after discontinuation of their antiepileptic drugs (AEDs). We analyzed the video-EEG recordings for seizure semiology as well as ictal and interictal activity. We also reviewed the initial clinical data in all patients. RESULTS: All patients were women, with a mean age of 27 years (range, 14-43 years). The mean age at seizure onset was 12 years (range, 5-15 years). Family history was positive for epilepsy in four patients. All patients had recorded seizures with an onset that was characteristic of generalized absence clinically and electrographically, with evolution into GTC activity. The EEG onset was with generalized 2.5-to 5-Hz spike-and-wave discharges, with evolution into faster rhythmic activity. Interictal EEG recordings showed generalized 2-to 5-Hz spike-and-wave discharges. All had normal background activity. All patients were treated with divalproex monotherapy. Five patients have been seizure free, and one had a single breakthrough GTC seizure during a follow-up period of 12-36 months. CONCLUSIONS: GTC activity may evolve from typical absence seizures. This seizure type should be included in the International Classification of Seizures. Its recognition and distinction from complex partial seizures with secondary generalization are important for appropriate therapy.     

Prevalence of the Epilepsies in Children and Adolescents

The prevalence of epilepsy in children and adolescents from birth through age 19 years was determined for residents of two counties in central Oklahoma. Cases, identified from hospitals, clinics, private physicians' offices, and EEG laboratory and emergency room records, numbered 1,159, yielding a prevalence rate of 4.71 per 1,000. The prevalence was highest in children aged 1-4 years. Overall, males had a slightly higher prevalence rate than females (M:F = 1.1). However, the male/female ratio varied by age, with the group aged less than 1 year having the highest ratio (M:F = 1.5), and by type of epilepsy, with males having higher rates of simple partial epilepsy (M:F = 1.8) and infantile spasms (M:F = 1.5). The prevalence of epilepsy was higher in blacks than in whites. Differences in prevalence by race were confined primarily to generalized epilepsies (B:W = 1.8). The most common types of epilepsy were tonic, clonic, and tonic-clonic (1.14 per 1,000), complex partial (0.39 per 1,000), and partial seizures secondarily generalized (0.33 per 1,000). Approximately 70% of cases were considered idiopathic. Among the presumed causes were perinatal factors (7%), trauma (4%), central nervous system (CNS) infection (3%), and congenital/developmental factors (3). Sixty-five percent of cases had at least one additional medical problem. The most common types of comorbidity were motor handicap (13%) and developmental delay (24%).     

Carbamazepine-exacerbated epilepsy in children and adolescents

Forty-nine children and adolescents whose seizures reportedly worsened while receiving carbamazepine (CBZ) were studied retrospectively. Twenty-six patients met criteria for excellent documentation of carbamazepine-exacerbated seizures. Four epileptic syndromes were particularly affected: childhood absence epilepsy; focal symptomatic, frontal lobe epilepsy; Lennox-Gastaut syndrome; and severe myoclonic epilepsy of infancy. Eight of the 26 patients developed new-onset absence seizures and three patients with established absence epilepsy experienced absence status. Other seizure types, including atonic, tonic-clonic, and myoclonic, developed in eight patients treated with CBZ, and new generalized spike-and-wave discharges were observed in electroencephalograms of nine patients. CBZ is a widely used, effective antiepileptic drug, particularly for partial or partial complex seizures; however, if uncontrolled, generalized seizures occur after CBZ is prescribed for children or adolescents with absence or mixed seizures, a trial of CBZ discontinuation is warranted. The data reported here do not permit calculation of the incidence of this phenomenon.     

Rotatory seizures in juvenile myoclonic epilepsy

Rotatory seizures have been reported in association with focal intracranial lesions. This type of seizure was also described in patients with primary generalized epilepsies. To our knowledge, there is only one previous publication denoted an association between juvenile myoclonic epilepsy (JME) and rotatory seizures. We present two female patients with JME and rotatory seizures together. The onset of myoclonic jerks and generalized tonic clonic (GTC) seizures was in their midteens. Their interictal EEGs showed bilateral symmetric spike and polyspike wave discharges. The rotatory seizures of the patients started at age of 26 and 33 years, respectively. In one of the patients, turning to the left was followed by three or four complete turns, after then, she had GTC seizures. The other patient has turned to the right with only one or two turns and sometimes continued with GTC seizures. Neuroradiologic investigations including brain CT, MRI, and SPECT were performed. Response to valproate therapy of rotatory seizures was good. We believe that rotatory seizures are rarely seen in JME patients, and this causes false diagnosis which lead unsuitable drug choice.     

Coexistence of temporal lobe and idiopathic generalized epilepsies.

OBJECTIVE: To assess the interrelation of idiopathic generalized epilepsy (IGE) and temporal lobe epilepsy (TLE) when they coexist in the same patient. METHODS: The authors reviewed the electroclinical features of 350 consecutive patients who had temporal resection between 1975 and 1997 at the Maudsley and King's College Hospitals, London. RESULTS: Two patients had the unusual combination of TLE and IGE (0.57%). In the first, the clinical onset of juvenile myoclonic epilepsy followed the surgical resolution of his partial seizures but had been heralded for at least 5 years by subclinical spontaneous and photically induced generalized spike-wave discharges. In the second, TLE and juvenile absence epilepsy had a long parallel course before surgery. After surgery he had no further partial seizures. CONCLUSION: These cases suggest that when an idiopathic absence or myoclonic syndrome manifests in a patient with symptomatic TLE, the phenotype may not be a merged syndrome. Rather, the two conditions can retain their inherent electroclinical profile, responsiveness to treatment, and prognosis.     

Reflex Seizures are Frequent in Patients with Down Syndrome and Epilepsy

In a retrospective study of 30 Down syndrome (DS) patients with epilepsy, we found 6 cases (20%) with reflex seizures. One patient had benign myoclonic epilepsy of infancy with clinical photosensitivity. The other 5 cases had all startle-induced epileptic seizures and a form of symptomatic epilepsy. Three patients had a Lenox-Gastaut syndrome, one had generalized symptomatic epilepsy, and one had partial symptomatic epilepsy (PSE). Reflex epilepsy was also used as a classification category in the PSE case, as most or all seizures were stimulus-related in this patient. Seizures precipitated by stimuli were stereotyped in 4 patients, but 2 patients responded to stimuli with different types of seizures. The actual occurrence of reflex seizures in DS patients with epilepsy is probably underestimated. These cases seem to confirm previous reports showing deficiencies in cortical inhibition in the brain of DS patients.