Understanding Lennox–Gastaut syndrome: insights from focal epilepsy patients with Lennox–Gastaut features

To delineate the clinical and EEG features of adults with focal epilepsy associated with a generalized paroxysmal fast activity (GPFA) pattern on EEG who developed refractory seizures, notably drop attacks, but do not fulfill the classical triad for the diagnosis of Lennox–Gastaut syndrome (LGS) and provide further insight into LGS mechanisms. Among 957 patients admitted to video-EEG monitoring between 2002 and 2015, we retrospectively research adult patients with refractory focal epilepsy, drop attacks and GPFA on EEG. We collected demographic, anamnestic, and clinical data from medical records. We reviewed for all patients the interictal and ictal video-EEG recordings. We identified ten patients with focal epilepsy and electro-clinical features of LGS. As compared to classical LGS patients, our patients: (1) began epilepsy later (15.4 ± 8 years); (2) exhibited exclusively focal onset seizures, including drop attacks seizures linked to focal asymmetrical tonic posturing seizures; (3) had a stable cognition over time and (4) evolved favourably with a good secondary response to treatments in 80% of cases. Interestingly, all patients exhibited apparent diffuse interictal and ictal EEG abnormalities but a detailed analysis revealed that 50% had asymmetrical GPFA and 70% secondary bilateral synchrony processes. We may hypothesize here that a process of “secondary LGS” occurred which produced a worsening of seizures with the apparition of drop attacks and GPFA on EEG. This study brings arguments to consider that some cases of LGS could be linked to the development of a “secondary epileptic network” driven by a primary focal epileptic zone.     

Long-Term Prognosis in Childhood Epilepsy: Survival and Seizure Prognosis

All children aged 0-19 years who had active epilepsy in a defined Swedish population were traced and given a clinical and psychometric investigation. Twelve years later, a follow-up study was carried out. Eleven of the 194 children had died, 8 of whom had had signs of neurodeficit, i.e., abnormal neurology and/or mental retardation. A long-standing remission of seizures occurred in 124 of the 194 children. Signs of neurodeficit, frequent seizures, and many types of seizures were negative prognostic factors. The presence of all these factors carried a bad prognosis, seizures persisting during 12 years in greater than 80%. For those who were mentally and neurologically normal and had low seizure frequency, prognosis was excellent, only 11% still having active epilepsy after 12 years. A study of the annual remission rate showed that each year approximately 13% of the children without neurodeficit had remission from epilepsy the next year. This rate appeared to be stable over the 12 years studied. Among those children with neurodeficit, the annual remission rate was high only during the first years after onset, later falling to 3% a year.     

Epidemiological and Clinical Study of Epilepsy in Benghazi, Libya

An epidemiologic study of adult patients (aged greater than 15 years) with epilepsy was made over a 2-year period, through the policlinics, EEG laboratory, and university hospitals in Benghazi, northeastern Libya. Based on a total of 568 patients (310 male and 258 female), the prevalence rate on 31 December 1984 was 2.3/1,000 in the population above the age of 15 years (1.9, if age standardized). The age-specific prevalence per 1,000 population decreased from 3.6 in the 15-24 year age group to 1 in the greater than 60-year age group. For 70.8% of the patients the onset of epilepsy occurred in the first 2 decades of life, and for 7.6% it commenced after the age of 40. The duration of epilepsy when first seen in the study period was less than 1 year in 14.6%, and greater than 10 years in 25.7%. The percentages of primary generalized, partial seizures secondarily generalized, simple partial, and partial complex seizures were 55.6, 18.8, 10.4, and 14.6%, respectively. Antecedents that could be considered potential causes of epilepsy were found in 17.5% of cases. Fifty-five patients had status epilepticus, 24 of whom had symptomatic epilepsy and eight recurrent status. EEG revealed generalized epileptic activity in 199 cases and focal seizure activity in 118. Computerized tomography (CT) scan, done in 219 patients, disclosed abnormal results in 50. Among those with abnormal CT scans, 84% had partial seizures. Monotherapy with carbamazepine or phenytoin was used in the majority of cases. The influence of social, cultural, and religious factors in the diagnosis and management of epilepsy as seen in Benghazi are discussed.     

Incidence of Epilepsy in Rural Central Ethiopia

Summary: Purpose: To study the incidence of epilepsy in a rural area of Ethiopia.
Methods: A community-based study was performed in a random sample of villages with 61,686 inhabitants in a rural area of central Ethiopia. In a door-to-door survey, all inhabitants in the study area were interviewed about seizures. A standardized protocol was used. All new cases with epilepsy that had occurred since a previous study was made 3.5 years earlier were included. Fifty-three of the subjects were investigated with EEG.
Results: One-hundred thirty-nine incident cases were identified, corresponding to an annual incidence of 64 in 100,000 inhabitants [95% confidence interval (CI) 44–84]. The corresponding rate for males was 72 (CI 42–102); for females, it was 57 (CI 31–84). The highest age-specific incidence occurred in the youngest age groups (0–9 years); the next highest was in the group aged 10–19 years. Generalized convulsive seizures occurred in 69%, partial seizures occurred in 20%, and unclassifiable seizures occurred in 11%. Seizures occurred daily in 10% and weekly in another 14%; 33% had monthly seizures. Twenty-two percent had a family history of epilepsy. A history of head trauma was ascertained in 5.7% and was the most common possible etiologic factor identified. Thirteen percent were treated with antiepileptic drugs (AEDs).
Conclusions: The incidence of epilepsy in Ethiopia is high. A high incidence in combination with a prevalence of epilepsy in the study area comparable to that in the rest of the world may be explained by a high degree of spontaneous remission of epilepsy and/or a high mortality due to epilepsy. Despite health education on epilepsy given to the community, a minority of subjects were treated with AEDs, which may reflect the inadequacies of the health services and transportation difficulties faced by the patients.     

Symptoms accompanying epileptic attacks

The author analysed 124 cases of grand mal seizures in patients aged above 16 years observed for 2 to 15 years finding sporadic tongue or oral mucosa biting in 55.7% of cases, involuntary passing of urine in 49%, simultaneous occurrence of both these signs in 37% defection in 8.1%, ejaculation in 1.6%, conjunctival o cutaneous ecchymoses in 29.8%, and significant traumas in 20.2% (72% of these cases were men). Assuming that these signs occurred as a rule during each attack these per cent proportions were as follows: 17.7%, 15.3%, 9.7%, 2.4%, 0.8%, 10.5% and 2.4% respectively. Most frequently these signs appeared during the first attacks before treatment beginning, during attacks appearing after several years without seizures, after arbitrary stopping of drug taking and as a result of irregular treatment and alcohol drinking. In 14.5% of patients during observation lasting from 10 to 20 years no peri-ictal signs appeared. In a group of young unmarried people these signs were often concealed, while they were exaggerated by a small group applying for disability pension or wanting to have that pension increased. Patients from rural areas seemed to give less attention to these concomitant signs.     

Diagnosis, management and prognosis of a group of 128 patients with non-epileptic attack disorder. Part I.

Three hundred and forty three patients with attack disorder labelled as epilepsy were admitted for assessment to a Neuropsychiatry ward in a small English mental hospital over a 5 year period. After assessment it was decided that 63% (215) of these patients had epilepsy, but in 128 (37%) a diagnosis of non-epileptic seizures was made. Just over a third of these patients (46) had an additional history of present or past epileptic seizures as well, so that 24% of the total population had non-epileptic seizures only. The methods used to make this diagnosis are reviewed and an attempt made to classify the non-epileptic attacks from which the patients were suffering. A variety of management strategies were offered and at discharge from hospital the majority of patients had practically lost their non-epileptic seizures. At follow-up 2 years later, seizures had returned in most patients. In 8% of the patients it was clear that the diagnosis of non-epilepsy had been erroneous. The importance of classifying the kind of non-epileptic event the patient suffers from and of translating treatment in hospital to the community is emphasized.     

Frequent association of autism spectrum disorder in patients with childhood onset epilepsy

Autism spectrum disorder (ASD) has a close relationship with epilepsy. This study retrospectively examined patients with epilepsy associated with ASD. Among the 519 patients with epilepsy, 79 patients (15.2%) had ASD. Sixty-two patients had idiopathic ASD and 17 had secondary ASD. The epilepsy patients with idiopathic ASD were retrospectively analyzed. There were 47 males and 15 females, ranging from 2 to 43 years of age (median 11 years). The most frequent age at the onset of seizures was 4 years, and 85% occurred before 10. ASD was detected after the onset of epilepsy in 29 cases (46.8%), and eight of them had been overlooked for more than five years. Most of these were high-functioning ASD cases. The most frequent type of seizure was a complex partial seizure (CPS; 68%). Paroxysmal activities on EEG were localized in the frontal area in about half of the cases. Multiple anti-epileptic drugs were used in 33.8% cases (two in 17.7%, three in 16.1%), and 67.3% of the patients were seizure-free for more than two years. An amelioration of the autistic symptoms occurred after epilepsy treatment in five cases (8%).     

Seizures in rural Zambia

PURPOSE: To describe the period prevalence of epilepsy and febrile seizures in a bush hospital and discuss the medical sequelae and social impact of seizures in this population. METHODS: For 13 weeks, an evaluation of inpatients was made at Chikankata Hospital in rural Zambia. Inpatients identified as having seizures, "fits," "spells," or "fainting," were evaluated by a medical records review, basic demographic data, a neurological history and physical examination, and a treatment history. A semistructured questionnaire was administered to evaluate the social impact of seizures and assess factors associated with delayed care seeking. RESULTS: Seizures composed 44% of all inpatient neurologic disease and resulted in 84 admissions. Epilepsy patients received treatment primarily from traditional healers; only 31% reported ever receiving antiepileptic drugs (AEDs). Among those who had received treatment, AEDs were frequently underdosed. Patients with epilepsy had significantly less education than their sex-matched siblings. Patients with untreated epilepsy for >2 years were more likely to have experienced serious burns or falls requiring hospitalization. Children with febrile seizures whose parents held supernatural beliefs regarding seizures were more likely to be treated with traditional medicines, had higher malarial parasite counts, and required longer hospitalizations than children with febrile seizures whose parents recognized the association between seizures and hyperthermia. CONCLUSIONS: Epilepsy and febrile seizures are responsible for a significant burden of disease in rural Zambia. Serious medical complications often result from seizures, especially if untreated for >2 years. Social stigma decreases educational opportunities and misperceptions regarding seizures may result in delayed care for children with febrile seizures. Some evidence suggests that epilepsy is underreported, underrecognized, and undertreated in this population.     

Prevalence and Pattern of Epilepsy (Lath/Mirgi/Laran) in Rural Kashmir, India

The rural population of 63,645 living in the mountainous Kuthar Valley of South Kashmir, Northwest India was surveyed to determine the prevalence of major neurologic disorders, including epilepsy (called Lath/Mirgi/Laran in the local language). The survey was done according to a World Health Organization protocol (1981). House-to-house screening was done by Anganwadi workers to identify people with possible epilepsy. The screening questionnaire was translated into local vernacular. Persons who had some indication of a history of seizures or other neurologic disease were subsequently examined by a neurologic team. The diagnostic criteria of Hauser and Kurland (1975) were used to define cases of active epilepsy and seizure classification (ILAE, 1981) was done only with clinical data. One hundred fifty-seven cases of active epilepsy were detected, giving a crude prevalence rate of 2.47/1,000 general population. In those aged less than 14 years, prevalence was 3.18/1,000. Ninety-five (60.5%) of all cases were male; 91% of active epilepsy cases had onset of seizures before age 30 years. Mean age of onset in males was 5.3 years, and in females it was 7.1 years. Mean duration of seizures was 6 years; 78.9% cases had generalized seizures, 74.5% cases were receiving no specific treatment, 99.4% cases were born of home delivery, and 8.9% cases had a positive family history of seizures. Mental retardation was the most common associated abnormality in 22.9% of cases.     

Car drivers with epilepsy (Pilot Hungarian EPIMED Database Evaluation)

We investigated aspects of driving among Hungarian patients with epilepsy. Nineteen percent (148/753) of our population had a driving license (DL), including 26% (38/148) who used it. Among persons without a DL, 29% (178/605) desired one. Among those who drove (n=38), 24 worked and 8 had experienced seizures in the last 3 months. Each of these seizures occurred during sleep (5/8), were preceded by known auras and warning signs (2/8) or were provoked by extreme external factors (1/8).     

Prevalence and Prognosis of Epilepsy in Patients with Multiple Sclerosis

An analysis of 599 clinically definite multiple sclerosis (MS) patients including all known cases of the southern province of Finland in January 1, 1979 revealed epileptic seizures in 21 (3.5%) patients. On that date, 12 patients needed treatment (2.0%). The age-adjusted prevalence of active epilepsy was significantly higher than that in the general population. The percentage of partial seizures (67%) was significantly increased in proportion to a series of 100 adult epilepsy patients, with a comparable age distribution. In 10 patients (including three patients with symptomatic epilepsy), the attacks appeared before the MS symptoms. The mean follow-up after the first seizures was 19.3 years. In 10 patients, the seizures disappeared totally during the surveillance until September 1985. Our results show an increased comorbidity between MS and epilepsy. In most cases, however, the prognosis of epilepsy was good and there seemed not to be any clear correlation between the severity of MS and epilepsy.     

A comprehensive community epilepsy programme: the Nakuru project

The methodology of a programme of investigation of epilepsy carried out at a community health level in Nakuru district, Kenya, East Africa, and the characteristics of patients with tonic-clonic epilepsy in this community, are presented. The study identified a group of 302 patients with untreated generalised tonic-clonic seizures (with or without other seizure types) from the general population. Case finding was carried out using the key informant method over a 12-month period. The clinical characteristics of this cohort of patients, the majority of whom had never had previous contact with a formal medical system, is described. Most were young, living in a rural area, with a mean age of seizure onset of 14 years and a mean duration of seizures of 7 years. Thirty-eight per cent had a clinically evident focal disturbance associated with the tonic-clonic seizures. In 77% of the cases, no aetiology was established. Most cases had had a substantial number of seizures during the course of their condition and only about a third of the patients had less than 1 seizure a month in the previous year. Only 26% had ever had previous anti-epileptic drug treatment. In addition to the medical inquiry, psychological and sociological studies were also conducted and a prospective treatment programme was carried out. Throughout the study, strict definitions and standardised instruments were used, and the study was the result of a collaboration between local investigators, an international neurological team, the Kenyan Government agencies and the pharmaceutical industry. We consider this a model for community-based investigations for the management of epilepsy in developing countries.     

Longterm follow-up of childhood epilepsy with absences. II. Absence-epilepsy with initial grand mal

The study deals with 83 patients with absence epilepsy which had started with generalized tonic clonic seizures. Only those patients were included, who could be followed up to an age older than eighteen years. The patient population is heterogeneous; it includes numerous older patients in whom therapy had been instituted at a time when the present standard medication with ethosuximide and valproate was not available. Therefore the data cannot be used as a basis for global statements concerning the prognosis of absence epilepsy with grand mal onset. About 80% of the patients treated with standard therapy became seizure free. An unfavourable course was mainly preceded by incorrect, irregular and quantitatively inadequate therapy. Standard therapy cannot prevent singular generalised tonic clonic seizures in the late course. The social status of adult patients is mainly favourable if they are seizure free. Sporadic attacks usually will not impair social integration. In all, absence epilepsy starting with grand mal responds not as well to therapy and has a more unfavourable social prognosis than epilepsy starting with absences.     

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.     

Vagus nerve stimulation: clinical experience in drug-resistant pediatric epileptic patients.

INTRODUCTION: Vagus nerve stimulation (VNS) is an effective alternative treatment for patients with partial refractory epilepsy. Nevertheless, information regarding VNS in children is still limited. MATERIALS AND METHODS: The clinical efficacy, safety and neuropsychological effects of VNS in 34 children (mean age 11.5 years) with drug-resistant epilepsy were studied. Mean follow-up was 30.8 months. Nine patients have been diagnosed with Lennox-Gastaut Syndrome, nine patients were affected by severe partial epilepsy with bisynchronous EEG and drop attacks, and 16 patients suffered from partial epilepsy without bisynchronous EEG and fall seizures. Forms were designed for prospective data collection on each patient's history, seizures, implants, device settings, quality of life (QOL), neuropsychological assessment and adverse events. Surgical technique was performed both by standard two incisions and single neck incision. RESULTS: Mean reduction in total seizures was 39% at 3 months, 38% at 6 months, 49% at 12 months, 61% at 24 months and 71% at 36 months. Significant better results were obtained in partial epilepsy, with and without drop attacks, than in Lennox-Gastaut syndrome--three patients being seizure-free. No operative morbidity was reported. Side-effects were minor and transient--the most common were voice alteration and coughing during stimulation. In two patients, electrode breakage occurred 3 years after surgical procedure; in both cases, a new device was implanted after removing the vagal electrode coils and generator. CONCLUSION: VNS can be considered an appropriate strategy as an add-on treatment in children affected by drug-resistant partial epilepsy and ineligible for resective epilepsy surgery.     

De novo psychogenic nonepileptic attacks after adult epilepsy surgery: An underestimated entity

We retrospectively assessed all patients in a large cohort of patients with epilepsy surgery at the National Hospital for Neurology and Neurosurgery (NHNN) over 12 years, to identify patients with postoperative psychogenic nonepileptic attacks (PNEA). Twenty‐nine patients (23 women) were identified of a total of 790 patients, a frequency of 3.7%. Female gender and presurgical psychiatric diagnosis, other than psychosis, were significant risk factors for PNEA development. In female patients with a preoperative psychiatric diagnosis the chance of developing PNEA after epilepsy surgery was 8.5%. PNEA developed between 2 weeks and 10 years after epilepsy surgery, independently of outcome of epileptic seizures. In most cases, PNEA differed from the present or past epileptic seizures, and motor symptoms were the most common manifestations. Seizures after epilepsy surgery should be carefully evaluated. Physicians should consider the possibility of PNEA, especially in female patients with preoperative psychiatric comorbidity developing “atypical” seizures with motor manifestations postoperatively, even many years after epilepsy surgery.     

Zur Problematik der Früherkennung von Hirngeschwülsten,die als einziges Symptom epileptische Anfälle hervorrufen

The diagnosis of brain tumour could not be made in 91 cases at the first investigation in a group of 1155 brain tumours. Slowly growing gliomas causing only epileptic fits and no other symptoms are especially difficult to diagnose. Of 21 personal observations of tumour seizures, in which the diagnosis of the neoplasm was missed at the first investigation in hospital, 9 were oligodendrogliomas, 5 astrocytomas, 3 glioblastomas, 2 spongioblastomas, 1 gangliocytoma and 1 a metastasis. They were all located in the frontal or centroparietal region. In most cases the seizures appeared during the third or fourth decade. The average interval between the first epileptic fit and the tumour diagnosis was 8.2 years in cases of oligodendrogliomas and 2.2 years in astrocytomas. 5 patients had major seizures, 2 had psychomotor attacks and all the others suffered from partial epilepsy. Anticonvulsive therapy was often successfull; either the frequency of the fits diminished or, in 2 cases, the character of the seizures changed. 18 patients had a normal neurostatus at time of the first investigation. Only 3 patients had a slight difference of physiological reflexes, but no other pathological signs. In none of the patients did investigation of the CSF, skull X-rays, brain scanning, pneumencephalography or cerebral angiography first lead to the diagnosis of a brain tumour. The EEG alone showed focal signs corresponding to the location of the tumour in about 50% of the cases.     

Mah-Jong-induced epilepsy: a special reflex epilepsy in Chinese society.

We present five patients of epilepsy in which seizures were triggered by playing or watching the traditional Chinese gambling game "Mah-Jong." One patient also experienced seizures while playing a computer version of the Mah-Jong game. This condition appeared to have a predominance of males (80%) and middle-aged onset (39.4 years). Four patients had generalized tonic-clonic seizures and one patient had partial seizures with secondary generalization. No spontaneous seizure occurred in these patients. Three patients had been receiving antiepileptic drug therapy, but without effective control over their seizures. Mah-Jong-induced epilepsy is a very peculiar form of complex reflex epilepsy that involves the higher mental activities. This phenomenon may consist of distinct pathophysiologic mechanisms from other reflex epilepsy induced by thinking and spatial tasks in idiopathic generalized epilepsies. This unusual reflex epilepsy is relatively benign in nature and antiepileptic drug therapy has uncertain benefits. It may be necessary to avoid playing the Mah-Jong game in order to prevent seizures.     

Clinical and etiological profile of epilepsy in elderly: a hospital-based study from rural India

To determine the clinical profile and etiology of epilepsy in elderly patients in rural population of India which is in a phase of demographic transition with steadily growing geriatric population. A cross-sectional, prospective, hospital-based clinicoepidemiological study was performed from October 2014 to November 2015. Patients having onset of epilepsy after 60 years were incorporated in the study. We excluded the acute symptomatic seizures. One hundred and ten patients were enrolled and were divided into three standard subgroups: subgroup A (aged 60–70), subgroup B (aged 71–80), and subgroup C (aged 81–90). Out of 110 patients, 72 (65.45%) were male and 38 (34.54%) were female. The most common etiology was cerebrovascular disease (46.36%), followed by focal cerebral calcifications single or multiple (11.81%), tumors (9.09%), trauma (6.36%) dementias (6.36%) and unknown (16.63%). In our study, the frequency of epilepsy decreases with advancing age which was 77.27% in group A (less than 70 years), 16.36% in group B and 6.36% in group C (70–90 years).The most common type of seizure in the group studied was focal 59.09% followed by generalized seizures 37.27%. Hypertension was the most common co-morbidity found in 40 (36.36%) patients. The present study proposes that epilepsy in the elderly patients have etiological relationship with stroke, focal cerebral calcifications, tumors and dementias. CNS infections account for a significant number of cases of remote symptomatic epilepsy in elderly in our region where neurocysticercosis is endemic.     

Prevalence of Epilepsy in a Rural Community of Guatemala

Summary: We conducted a cross-sectional epidemiological study to assess the prevalence and patterns of epilepsy in a small rural village of Guatemala (population 2,111); 1,882 subjects (97.3%) were surveyed. By administering the World Health Organization (WHO) standard questionnaire and performing neurological examinations, we detected 16 cases of epilepsy. The crude prevalence rate for this community was 8.5 in 1,000 general population. Eleven cases were further classified as active epilepsy, giving a prevalence rate of 5.8 in 1,000 general population for this form. The most common type of seizure was generalized tonicclonic seizures (GTCS, 50%), followed by complex partial seizures (CPS, 37.5%), simple partial seizures (SPS, 6.2%) and generalized atonic seizures (6.2%). The age-specific prevalence ratio was highest among the group aged 20–29 years, although the difference between that group and the other age groups was not statistically significant ( z < 2, p > 0.05). Fourteen persons (87.5%) had sought medical care for their seizures at least once in their lifetime, 5 (31.25%) were receiving an antiepileptic drug (AED), and 9 (56.25%) had previously received treatment and 2 (12.5%) had never been treated for their illness. Phenobarbital was the most common AED prescribed; 7 persons had positive family history of epilepsy, 5 reported a history of significant head trauma, 4 had history of central nervous system disease, and 1 had a history of chronic alcohol intake.