Epilepsy With Myoclonic Atonic Seizures: An Electroclinical Study of 69 Patients

Epilepsy with myoclonic-atonic seizures is characterized by myoclonic-atonic, absence, tonic-clonic, and eventually tonic seizures, appearing in previously normal children at ages 18-60 months. We analyzed the electroclinical features, treatment, and outcome of 69 patients with myoclonic-atonic seizures; these patients were followed between 1990 and 2012 at the Juan P. Garrahan Pediatric Hospital, Buenos Aires, Argentina. No structural or metabolic etiology was identified. Based on the electroclinical features and evolution, two groups could be distinguished. The first group of 39 patients with myoclonic and myoclonic-atonic seizures with or without generalized tonic-clonic seizures and absences associated with generalized spike- and polyspike-and-wave paroxysms had excellent prognoses. The second group of 30 patients had myoclonic jerks and myoclonic-atonic seizures associated with other seizure types including tonic seizures; some had myoclonic status epilepticus and cognitive deterioration. The interictal EEG showed frequent generalized spike- and polyspike-and-wave paroxysms. In 16 patients, the seizures remitted within 3.6 years. The two groups were distinguished in retrospect, when enough time had elapsed to evaluate cognitive deterioration and different seizure types. In conclusion, epilepsy with myoclonic atonic seizures is an epileptic syndrome with a broad clinical spectrum and variable prognosis.     

Outcome of children with juvenile absence epilepsy

The incidence and natural history of childhood absence epilepsy are well documented, but those of juvenile absence epilepsy are poorly delineated. We conducted a retrospective chart study to evaluate the incidence and outcome of patients with juvenile absence epilepsy by retrieving the medical records of consecutive patients with juvenile absence epilepsy who were evaluated in three pediatric neurology outpatient clinics in Israel. Inclusion criteria included the onset of epilepsy after the age of 10 years and follow-up until at least 15 years of age. The patients with an electroencephalogram (EEG) suggestive of myoclonic epilepsy (polyspike and wave) were excluded from the study. Seventeen patients (10 female and 7 male) fulfilled the inclusion criteria for juvenile absence epilepsy. They presented with epilepsy at a mean age of 11.94 years (range 10-16.5 years). The mean duration of follow-up was 6.05 years (range 2-12 years). Five patients (29.4%) had a family history of epilepsy. All 17 patients had a normal neurodevelopmental status. Eight patients (47%) experienced generalized tonic-clonic seizures. At follow-up, eight patients (43.7%) were seizure free. Only three (37.5%) of the patients who experienced generalized tonic-clonic seizures were seizure free during follow-up compared with five (55.5%) patients without generalized tonic-clonic seizures. Our results indicate that the outcome of patients with juvenile absence epilepsy is less favorable than children with childhood absence epilepsy and that the presence of generalized tonic-clonic seizures is a predictor for poorer outcome.     

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.     

A study on long-term prognosis of epilepsy

Of 631 epileptic patients examined in our seizure clinic in the period between January 1961 and December 1966, 97 (15.4%) have been treated until September 1976, when the long-term prognosis was evaluated. The "good prognosis (completely controlled)" were found in 59% of grand mal, in 55% of focal motor seizure, in 42% of psychomotor and in 33% of the mixed seizure in which more than two types of seizures were combined; in 49% (48 cases) on the average. Seventy-nine percent of the cases of the mixed seizure were combined with psychomotor seizures. In the psychomotor and the mixed seizure groups, the presence of personality disorders tended to lead them to "poor prognosis" which meant that the seizures were not well controlled. Twelve cases manifested psychotic (paranoid) state: a schizophrenic, a case with chronic paranoid-hallucinatory state, and 10 patients with episodic paranoid state, whose episodes may be identified with the paranoid reactions. Out of the 49 "poor prognosis" cases, 17 (35%) had had seizure-free periods for more than three years in the past course of their treatment.     

The genetics of localization-related symptomatic epilepsy: risk of a family history with seizures in patients who have undergone surgery

A family history of epileptic seizures including febrile convulsions was found in 15 of 103 patients (15%) with localization related epilepsy with partial seizures with and without secondary generalization, who were operated on because of drug resistance. This rate was significantly higher than that of the cumulative incidence in the general population (4%). The localization of the brain damage did not play a role (temporal lobe resection left: 15%, right: 17%, extra-temporal lesion excision: 20%, hemispherectomy: 11%). Various family members were involved. Some patients had more than one relative with seizures. Thus, 21 relatives suffered from seizures. Eleven of them had generalized tonic-clonic seizures (one grand mal on awakening), 7 had febrile convulsions (4 complicated), and in 1 patient the grand mal seizures on awakening were preceded by absences; 1 had generalized tonic-clonic and complex partial seizures; 1 after complicated febrile seizures likewise had complex partial seizures; another mentally retarded patient suffered from generalized tonic-clonic, axial tonic and myoclonic-astatic seizures. The seizure type of 3 remote relatives was not known. The first seizure occurred in 16 family members during childhood, in 3 in adolescence and in only 1 in adulthood (1 unknown). Eight showed mental retardation of slight degree in most. It is interesting that only one-third of the patients with a family history with seizures were seizure-free after the operation; 5 still had seizures, mostly reduced in frequency, 3 had seizures and isolated auras and 2 had only isolated auras. On comparing the findings in patients with and without a family history with seizures, those with family members with epileptic seizures showed a lower rate of an intellectual deficit (7 vs 47%) and brain tumours (13 vs 44%). Our earlier findings with a different group of patients are thus confirmed: that genetics play a role in symptomatic epilepsies. Received: 9 July 1996 Received in revised form: 1 April 1997 Accepted: 23 April 1997     

A Study on Long-Term Prognosis of Epilepsy

Of 631 epileptic patients examined in our seizure clinic in the period between January 1961 and December 1966, 97 (15.4%) have been treated until September 1976, when the long-term prognosis was evaiuated. The “good prognosis (completely controlled)” were found in 59% of grand mal, in 55% of focal motor seizure, in 42% of psychomotor and in 33% of the mixed seizure in which more than two types of seizures were combined; in 49% (48 cases) on the average. Seventy-nine percent of the cases of the mixed seizure were combined with psychomotor seizures. In the psycho-motor and the mixed seizure groups, the presence of personality disorders tended to lead them to “poor prognosis” which meant that the seizures were not well controlled. Twelve cases manifested psychotic (paranoid) state: a schizophrenic, a case with chronic paranoid-hallucinatory state, and 10 patients with episodic paranoid state, whose episodes may be identified with the paranoid reactions. Out of the 49 “poor prognosis” cases, 17 (35%) had had seizure-free periods for more than three years in the past course of their treatment.     

EEG Patterns of Grand Mal Seizures Occurring Under Different Recording Conditions

Thirty-five EEG patterns of grand mal seizures recorded under some conditions such as awake-rest, sleep activation, beme-gride activation and intermittent photic stimulation were studied in 31 epileptics and four non-epileptics. Preictal EEG's were generally variable in comparison with ictal EEG's. It was not till 10 sec before seizures that the incidence of seizure discharges changed in most all cases, of which six increased and four decreased. A paroxysmal extreme varied EEG abnormality often continued for several to several ten sec immediately before a tonic phase, associated with some clinical symptoms. EEG patterns of the “preconvulsive phase” were divided into six types with different clinical features. In most all cases of bamegride activation, poly-spike and wave discharges preceded on seizure activities and some of them related with absence or psychomotor epilepsies. In all cases of sleep activation and photic stimulation, high voltage slow bursts or poly spikes preceded. Seizure patterns recorded at awake-rest were indefinite. A few cases didn't show any preconvulsive EEG changes, and were closely related with grand mal epilepsies. While the grand mal seizure during sleep had a long tonic-clonic convulsive phase and a short pre- and postconvulsive phase, that induced by bemegride activation showed the opposite findings. The grand mal seizure at awake-rest had the middle tendency between the two. The differences among the effects of various recording conditions on seizure patterns present some suggestions as to the patho-physiological mechanism of epileptic seizures.     

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.     

Long-Term Prognosis of Epilepsy in Children—A Follow-up Report beyond 18 Years of Age

The long-term prognosis of 185 children with epilepsy, who continued to attend the Clinic for Epileptic Children, the Department of Pediatrics, the University of Tokyo, beyond the age of 18 years, was reported. The length of follow-up varied from three to 20 years, but most of them were followed longer than 10 years. The presumed etiology in these children was divided into a cryptogenic group (124, 67.0%) and a symptomatic group (61). The types of seizures were classified into grand mal (86 cases), focal seizure (27), petit mal absence (4), psychomotor seizure (S), infantile spasms (7), and so on. It may be noted that the highest frequency of grand rnal was demonstrated, while the incidences of infantile spasms, myoclonic seizure, and akinetic seizure were low in the series. Only 28 children (15.1%) had complications of physical and/or mental handicaps. The follow-up study revealed that 140 patients (75.7%) had been seizure-free in the last 12 months. One hundred and fifteen of them had no seizures for five years or longer. On the other hand, electroencepha-lographic abnormalities generally continued for a long time after disappearance of seizures, Eighty-one of well-controlled patients were gradually decreasing the doses of anti-convulsants. As for seizure types, it is noted that focal seizure, psychomotor seizure, and infantile spasms were relatively difficult to be controlled. Except for 27 patients, most of them attended normal schools, including junior colleges or universities, and engaged in various occupations. Fifteen female patients had already married, and out of 13 babies who were born from these patients, there were one with ventricular septal defect, one with mental deficiency, and one with anencephaly, while the rest were entirely normal. Additional problems on withdrawal of anticonvulsants after a long-term seizure-free period, and what a medical system should be for treatment of epilepsy in children up to their adulthood were discussed.     

Uncontrollable Cases of Absence

Abstract: Forty-three cases of absence seizure were studied from the viewpoint of prognosis. Thirty-three had been seizure-free for at least one year until the time of follow-up. The remaining 10 cases were uncontrollable and their plasma levels of the drugs were measured. The following characteristics were more significantly found in the uncontrollable group: (1) late onset (14 years old or later) (2) preceding grand mal seizures (3) accompanying automatisms. The above three factors were found to be interrelated. (4) concomitant grand mal seizures of sleep or diffuse (other than awakening) type (5) psychological disturbances (low intelligence, circuitous character) and (6) chronological shift to the Lennox-Gastaut syndrome.     

Generalized Tonic-Clonic Seizures in Patients with Complex-Partial Seizures: Natural History and Prognostic Relevance

Clinical course and long-term seizure prognosis were studied in 155 patients with complex-partial seizures during a follow-up of 10.1 +/- 1 (SD) years. In 79% of the patients generalized tonic-clonic seizures were recorded, mostly within the first 3 years of epilepsy but occurring as late as 20 years after the onset of epilepsy. Seizure control was defined as complete absence of all seizures, including auras, for a minimum of 2 years. Seizure control occurred in 20 of 32 patients (63%) with complex-partial seizures only and in 76 of 123 patients (62%) with complex-partial seizures and generalized tonic-clonic seizures. The onset of the epilepsy with generalized tonic-clonic seizures or complex-partial seizures did not influence the therapeutic outcome despite differences in their natural history. A family history of epilepsy and other generalized seizures (e.g., absence) were more frequent in patients with generalized tonic-clonic seizures at the onset of epilepsy. Seizure control was significantly lower (44%) in patients with a history of a maximum frequency of one or more generalized tonic-clonic seizures per month when compared to patients (79%) with a total of less than six generalized tonic-clonic seizures (p less than 0.05). The frequency of generalized tonic-clonic seizures is of predictive value for the seizure prognosis of patients with complex-partial seizures.     

Idiopathic generalized epilepsies with versive or circling seizures

OBJECTIVES: To describe the electroclinical features of the idiopathic generalized epilepsies (IGEs) with versive or circling seizures. METHODS: Sixteen patients with versive or circling seizures and interictal electroclinical features of IGE were studied. Patients with insufficient clinical or imaging data, with a follow-up period less than 1 year or with partial seizures in addition to the versive or circling ones were excluded from the study. All patients underwent full interictal clinical and neurophysiological studies. The EEG patterns of 13 versive or circling seizures from 4 patients were also analyzed. RESULTS: A specific IGE syndrome was recognized in 9 out of the 16 patients (56%). More specific, 1 patient had childhood absence epilepsy (CAE), 4 had juvenile absence epilepsy (JAE), and 4 had juvenile myoclonic epilepsy (JME). No specific IGE syndrome was recognizable in the remaining 7 patients (44%). These 7 patients had a juvenile epileptic syndrome (mean age at onset of seizures was 15.7 years) characterized by versive or circling seizures followed or not by generalized tonic-clonic fits. Three main EEG patterns were identified during versive or circling seizures: 1) generalized spike-and-wave discharges at 3-4 cps; 2) generalized polyspike-and-wave discharges at 1 to 2.5 cps beginning with generalized fast activity at 12-14 cps, and 3) generalized spike-and-wave discharges at 3-4 cps intermingled with fast activity at 12-14 cps. Most patients had good response to treatment on a single drug regimen (mainly valproic acid). CONCLUSIONS: Versive or circling seizures may occur in the context of an IGE. Although many individuals share the features of different IGE syndromes including CAE, JAE and JME, a consistent number of patients, who show circling or versive seizures solely, remain without a specific syndromic diagnosis. When occurring in the context of IGE, circling or versive seizures do not worsen the prognosis.     

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.     

Myoclonic astatic epilepsy: an age-dependent epileptic syndrome with favorable seizure outcome but variable cognitive evolution

The objective of the study was to explore clinical, electroencephalography (EEG), neuropsychological features and prognosis of myoclonic-astatic epilepsy (MAE). Of 327 children aged between 1 and 9 years with a diagnosis of generalized epilepsy followed between 2000 and 2008, 18 (5.5%) had MAE. Male significantly predominated (88.9%). Age at onset ranged from 2.3 to 4.9 years (mean 3.6 years). Median follow-up period was 6.3 years. In addition to myoclonic-astatic seizures patients had myoclonic seizures (66.7%), drop attacks (72.2%), head drops (77.8%) absences (88.9%), tonic-clonic generalized seizure (77.8%), tonic seizures (38.9%), non-convulsive status epilepticus (16.7%). Seven patients (38.9%) had an epileptic encephalopathy. At onset, interictal epileptiform and slow abnormalities were recorded, respectively, in 100% and 77.8% of patients. EEG abnormalities disappeared in all patients within 4 years since the onset. At long-term follow-up, two patients developed focal abnormalities typical of rolandic epilepsy and two patients photosensitivity. On neuropsychological testing 66.7% of patients had a normal IQ (mean 81.2±17.0, range 47-105, median 84.5) after a mean period of 4.4 years since the last seizure. Sixteen out of 18 patients remitted within 3.5 years since the onset and in two patients tonic seizures persisted. MAE is generalized childhood epilepsy: although cognitive functions might deteriorate, outcome is good regarding seizures.     

Neonatal Seizures: Eyes Open or Closed?

PURPOSE: It has been shown that persistent eye closure during paroxysmal events in infants makes seizures unlikely. Our study aims to assess whether this is also true in neonates. METHODS: We reviewed and classified all archived neonatal seizures in our video database, considering electroclinical seizures only and excluding electrographic seizures and clinical seizures without ictal change in EEG. We assessed whether eyes were open during the seizure. One hundred and thirty-one electroclinical seizures (clonic, focal and generalized tonic, tonic-clonic, generalized myoclonic, subtle and spasms) in 46 neonates were included. RESULTS: In 115 (88%) seizures, eyes were open; in 10 seizures, they were closed; and in six seizures, eye opening could not be evaluated. All 10 seizures with persistent eye closure were clonic seizures. CONCLUSIONS: Our data demonstrate that persistent eye closure during an event suggestive of a seizure in a newborn makes an electroclinical seizure unlikely.     

Idiopathic generalised epilepsy of adult onset: clinical syndromes and genetics

OBJECTIVE: To study the clinical features and genetics of idiopathic generalised epilepsy (IGE) beginning in adult life. METHODS: Consecutive patients with IGE, defined as generalised seizures with spike or polyspike and wave on EEG, were studied in the setting of a first seizure clinic where an early postictal EEG record is part of the protocol. Patients were divided into two groups: "classical IGE" with onset before 20 years and inclusive of all the IGE subsyndromes recognised by the international classification; and "adult onset IGE", when seizure onset was at age 20 years or later. Seizure patterns, clinical features, and genetics of the adult onset group were examined. RESULTS: Of 121 patients with an electro-clinical diagnosis of IGE, 34 (28%) were diagnosed as adult onset IGE. The seizure patterns in these 34 cases were tonic-clonic seizures + absences (3), tonic-clonic seizures + myoclonus (6), and tonic-clonic seizures alone (25). Tonic-clonic seizures were often precipitated by alcohol or sleep deprivation. The proportion of affected first and second degree relatives did not differ between the classical and adult onset IGE groups. Twenty adult onset cases were treated with sodium valproate, four with other antiepileptic drugs, and 10 were untreated. Follow up of 32 of the 34 cases (for 31 (22) months (mean (SD)) showed that tonic-clonic seizures recurred in eight patients: five with identified provocative factors and three without. CONCLUSIONS: Adult onset IGE is a relatively frequent and benign disorder. Seizures are usually provoked and are easy to control. Patients in this age group may often be misdiagnosed as having non-lesional partial epilepsy. Early postictal EEG and sleep deprivation studies may improve the detection of these patients. Pedigree analysis suggests that adult onset IGE, like classical IGE, has a genetic aetiology.     

Long-term seizure outcome in 74 patients with Lennox-Gastaut syndrome: effects of incorporating MRI head imaging in defining the cryptogenic subgroup

PURPOSE: To determine if using more stringent criteria for cryptogenic Lennox-Gastaut syndrome (LGS) would result in an improved prognosis for that group. Cryptogenic, symptomatic, and non-cryptogenic LGS patients without etiology (indeterminate) were compared with respect to seizure and cognitive outcome. METHODS: Retrospective chart review was performed on 245 patients seen at the Mayo Clinic Rochester from 1976 to 1997, with a diagnosis of either LGS or slow spike wave on EEG. LGS was confirmed in 107 (64 male, 43 female) patients. This group was divided into cryptogenic, symptomatic, and indeterminate groups containing 23, 47, and 37 patients, respectively. In this study, cryptogenic patients all had normal development before onset of LGS, absence of dysmorphic features, normal neurologic examination, and normal magnetic resonance (MRI) brain imaging. Of the 107 patients, 74 had >/=3 years of follow-up. RESULTS: LGS onset in the 107 patients occurred at a median age of 4.0 years (range, 0.6-28.9 years). When last seen, 63% of those with symptomatic LGS had more than three seizures a day compared with 50% of cryptogenic and 34% of indeterminate patients. The most common seizure types were tonic (77%), atypical absence (61%), and generalized tonic-clonic (56%). Only three patients, all part of the indeterminate group, were seizure free at last follow-up. CONCLUSIONS: Using stringent criteria in defining the cryptogenic subgroup resulted in no significant difference in seizure outcome. Individuals with a normal cognitive outcome did not segregate into one etiologic subgroup, but did have LGS onset at an older age.     

Double-blind, randomized controlled pilot study of bilateral cerebellar stimulation for treatment of intractable motor seizures

PURPOSE: The efficacy and safety of cerebellar stimulation (CS) was reevaluated in a double-blind, randomized controlled pilot study on five patients with medically refractory motor seizures, and especially generalized tonic-clonic seizures. METHODS: Bilateral modified four-contact plate electrodes were placed on the cerebellar superomedial surface through two suboccipital burr holes. The implanted programmable, battery-operated stimulator was adjusted to 2.0 microC/cm(2)/phase with the stimulator case as the anode; at this level, no patient experienced the stimulation. Patients served as their own controls, comparing their seizure frequency in preimplant basal phase (BL) of 3 months with the postimplant phases from 10 months to 4 years (average, eight epochs of 3 months each). During the month after implantation, the stimulators were not activated. The patient and the evaluator were blinded as to the next 3-month epoch, as to whether stimulation was used. The patients were randomized into two groups: three with the stimulator ON and two with the stimulator OFF. After a 4-month postimplantation period, all patients had their stimulator ON until the end of the study and beyond. Medication was maintained unchanged throughout the study. EEG paroxysmal discharges also were measured. RESULTS: Generalized tonic-clonic seizures: in the initial 3-month double-blind phase, two patients were monitored with the stimulation OFF; no change was found in the mean seizure rate (patient 1, 100%, and patient 5, 85%; mean, 93%), whereas the three patients with the stimulation initially ON had a reduction of seizures to 33% (patient 2, 21%; patient 3, 46%; patient 4, 32%) with a statistically significant difference between OFF and ON phase of p = 0.023. All five patients then were stimulated and monitored. At the end of the next 6 months of stimulation, the five patients had a mean seizure rate of 41% (14-75%) of the BL. The second patient developed an infection in the implanted system, which had to be removed after 11 months of stimulation; the seizures were being reduced with stimulation to a mean of one per month from a mean of 4.7 per month (BL level) before stimulation. At the end of 24 months, three patients were monitored with stimulation, resulting in a further reduction of seizures to 24% (11-38%). Tonic seizures: four patients had these seizures, which at 24 months were reduced to 43% (10-76%). Follow-up surgery was necessary in four patients because of infection in one patient and lead/electrode displacement needing repositioning in three patients. The statistical analysis showed a significant reduction in tonic-clonic seizures (p < 0.001) and tonic seizures (p < 0.05). CONCLUSIONS: The superomedial cerebellar cortex appears to be a significantly effective and safe target for electrical stimulation for decreasing motor seizures over the long term. The effect shows generalized tonic-clonic seizure reduction after 1-2 months and continues to decrease over the first 6 months and then maintains this effectiveness over the study period of 2 years and beyond.     

Outcome of electroclinical, electrographic, and clinical seizures in the newborn infant

Three seizure types have been described in the neonate: electroclinical, electrographic, and clinical only. Controversy still exists about whether the episodic abnormal movements seen in some infants, which are not accompanied by simultaneous ictal discharges on the EEG, are true seizures. Twenty-four infants with seizures were studied, 17 had purely electrographic and/or electroclinical seizures, seven had clinical-only seizures; six of these seven had clonic seizures, without facial manifestations or autonomic change. The three seizure types were investigated using video-EEG and a Griffiths neurodevelopmental assessment was performed in each seizure group. Of the seven infants with clinical-only seizures, six had clonic seizures with a normal background EEG, neuroimaging studies and neurodevelopmental follow-up assessment were normal in five. In the remaining 17 infants with electrographic and/or electroclinical seizures, seizure discharges were often associated with ocular phenomena, apnoea, or tonic posturing, and the background EEG was abnormal in all but one subject. Neurodevelopmental follow-up assessments revealed a poor outcome (14 of 17) in this group. In otherwise healthy infants, purely clonic seizures involving only the limbs may be a benign phenomenon and an EEG should be obtained to avoid unnecessary treatment. Infants with seizures superimposed on an abnormal background EEG pattern had a poor outcome.     

Epilepsy in patients with Cornelia de Lange syndrome: A clinical series

PurposeCornelia de Lange (CdLS) syndrome is characterized by multiple congenital anomalies and mental retardation. Epilepsy is a clinical feature found in about 20% of cases, but there are no data about its electroclinical features and long-term outcome.Methodswe describe a clinical series of fourteen Caucasian CdLS paediatric patients who developed epilepsy, with special reference to the long term prognosis.ResultsEpilepsy manifested between age 0.6 and 16.3 years. The majority of patients (64.3%) presented with partial seizures and interictal EEGs mainly revealed focal epileptic paroxysms involving temporal and parietal areas. Thirteen of 14 children became seizure-free with treatment. Valproate monotherapy was used in eight patients (57.1%), controlling seizures in seven. Otherwise monotherapy with topiramate, levetiracetam, carbamazepine and oxcarbazepine appeared to be effective in controlling seizures in four cases. At the end of the follow-up (age range, 7.3–24.2 years; follow-up, 8.2 ± 3.9 years), thirteen patients were seizure free (three still in therapy), while in one patient seizures were not controlled.ConclusionsPartial epilepsy is the most common type of epilepsy in CdLS patients. In the majority of cases the prognosis of this epilepsy is favourable and therapy can be withdrawn after few years of complete seizure control.