Majority Of Seizures Research Articles

Dispatches

Dispatches

Dravet syndrome: The long‐term outcome

Few studies focused on the long-term outcome of Dravet syndrome in adulthood are available in the literature, but all are concordant. In this article, we consider the outcomes of 24 patients followed at the Centre Saint-Paul, Marseille, up to the age of 50, and compare them to the patients reported in the literature. Five patients (20.8%) died, at a mean age of 24.8 years, one by status epilepticus, three by sudden unexpected death in epilepsy (SUDEP), and one of unknown cause. Epileptic seizures tend to become less frequent and less severe after childhood. Fever sensitivity (temperature variations) persists throughout the clinical course of DS, but its impact on seizure frequency and severity is milder than in infancy. Generalized convulsive seizures, mostly reported as generalized tonic-clonic seizures (GTCS), were the only seizure type observed in almost all of the patients, often with a focal onset. They are less frequent than in childhood and mostly nocturnal. Some of these major convulsive seizures have less typical aspects, for example, bilateral or asymmetric tonic posturing, followed in some cases by a tonic vibratory state or clonic movements (Oguni et al., Brain Dev 2001;23:736-748; Akiyama et al., Epilepsia 2010;51:1043-1052). Other seizures like myoclonic seizures, atypical absences, and complex partial seizures (CPS) are less common in adulthood: Among our 24 patients, only 6 had atypical absences, and one myoclonic and one complex focal seizures. Electroencephalography (EEG) also changes with age but is still multiple and heterogenous, interictally and ictally. Photosensitivity and pattern sensitivity also showed a tendency to disappear before the age of 20. Motor abnormalities are common. Cerebellar features, including ataxia, dysarthria, intention tremor, and eye movement disorder, become more prominent. Walking is markedly impaired, often due to orthopedic signs such as kyphosis, kyphoscoliosis, flat feet, or claw feet. This symptomatology was minor during childhood and worsened during and after adolescence, despite physiotherapy. Mental retardation ranged from moderate to severe, with predominance of language impairment, and some patients had a major personality disorder, labeled autistic or psychotic. Dependency in adulthood is nearly constant: Only 3 of our 24 adult patients lived independently.

Retrospective study of seizure-related injuries in older people: A 10-year observation

Objective The goal of this study was to assess the characteristics and risk factors for injuries caused by seizures in older persons. Methods All patients aged 65 years or older having injuries secondary to seizures between July 1, 1999, and June 30, 2009, were identified through the diagnostic coding system. Results Over the assessment period, a total of 615 patient episodes of seizures were recorded. Nineteen seizure-related injury events occurred in 18 persons with a total of 31 injuries. In the control group, 34 non-seizure-related injuries that were not seizure related occurred in 27 patients (28 patient episodes) ( P = 0.21). The majority of injuries in both groups resulted from falls. Fifty-three percent of seizures occurred indoors, and the majority of seizures were generalized tonic–clonic seizures. Of the seizure-related injury events, 8 (42%) were single injuries, and 11 (61%) were multiple injuries. The predominant injuries were soft tissue injuries (55%), fractures (35%), and head injuries (10%). The groups did not differ significantly with respect to fractures ( P = 0.06) or soft tissue injuries with lacerations ( P = 0.41), or injury severity ( P = 0.16), or treatment of osteoporosis ( P = 0.56). Conclusion These findings suggest that falls rather than seizures per se are the dominant influence in the pathogenesis of fractures in older patients with epilepsy.

Rufinamide in children and adults with Lennox–Gastaut syndrome: First Italian multicenter experience

This is the first multicenter Italian experience with rufinamide as an adjunctive drug in children, adolescents and adults with Lennox-Gastaut syndrome. The patients were enrolled in a prospective, add-on, open-label treatment study from 11 Italian centers for children and adolescent epilepsy care. Forty-three patients (26 males, 17 females), aged between 4 and 34 years (mean 15.9 ± 7.3, median 15.0), were treated with rufinamide for a mean period of 12.3 months (range 3-21 months). Twenty patients were diagnosed as cryptogenic and 23 as symptomatic. Rufinamide was added to the baseline therapy at the starting dose of 10mg/kg body weight, evenly divided in two daily doses and then increased by 10mg/kg approximately every 3 days up to a maximum of 1000 mg/day in children aged ≥4 years with a body weight less than 30 kg. In patients more than 30 kg body weight, rufinamide could be titrated up to 3200 mg/day. After a mean follow-up period of 12.3 months (range 3-21 months), the final mean dose of rufinamide was 33.5mg/kg/24h (range 11.5-60) if combined to valproic acid, and of 54.5mg/kg/24h (range 21.8-85.6) without valproic acid. The response rate (≥50% decrease in countable seizures) was 60.5% (26 of 45 patients) in total; 51.1% experienced a 50-99% reduction in seizure frequency and complete seizure control was achieved in the last 4 weeks follow-up by 9.3% of patients. Two patients (4.7%) had a 25-50% seizure reduction, while seizure frequency remained unchanged in 13 (30.2%) and increased in 2 (4.7%). Reliable data for atypical absence seizures and myoclonic seizures were not available, as these are usually impossible to count. Ten patients (23.2%) reported adverse side effects, while taking rufinamide. They were generally mild and transient and most frequently included vomiting, drowsiness, irritability and loss of appetite. In conclusion, rufinamide as an adjunctive therapy reduced the number of drop attacks and major motor seizures in about 60% of patients with Lennox-Gastaut syndrome and produced only mild or moderate adverse side effects.

Efficacy of Pyridoxine in Early-Onset Idiopathic Intractable Seizures in Children

To identify pyridoxine responsive seizures among children with early onset intractable seizures, and to identify pyridoxine-dependency as a subset in this group. Patients with neonatal onset idiopathic, intractable seizures were identified over a 6-month period and subjected to a 'pyridoxine trial', at the Pediatric Neurology Clinic of a tertiary-care teaching hospital in New Delhi, India. This consisted of an intravenous infusion of 100 mg of pyridoxine over 10-min with a simultaneous EEG monitoring. This procedure was carried out in the EEG laboratory with all appropriate precautions (including availability of resuscitation equipment and trained personnel). Continuous EEG monitoring was done throughout the infusion and till 20 min later, to look for correction of EEG abnormalities. All patients were then prescribed oral pyridoxine, 10-15 mg/kg/day divided TDS for 6 weeks, in addition to their current anticonvulsant therapy. Patients were reviewed every 15 days regarding compliance and change in seizure frequency. A reduction in seizure frequency by 50% of the baseline was considered as 'response' (significant change), meriting further continuation of pyridoxine therapy. In patients who remained seizure free on pyridoxine therapy, previous anti-epileptics were gradually tapered one by one. 621 children with active epilepsy were seen at the PNC, of which 48 had early-onset, medical intractable epilepsy, and 21 children (13 males and 8 females), aged between 11 month and 38 month were enrolled. The median age at onset of seizure was 5.1 months. The major seizure type was focal in 3 and generalized in 18 (including infantile spasm in 11). No patient had normalization of EEG during the 'trial'. Two patients (9.5%) had a response during the 2 weeks of oral treatment and oral therapy was continued. No toxicity or side-effects of pyridoxine were observed in these two patients over a follow-up of more than 18 months. Pyridoxine responsive seizures contribute a significant proportion to early-onset idiopathic intractable epilepsy in childhood. Routine use of pyridoxine in the management of early onset resistant seizures would go a long way in identifying these patients early.

Lennox–Gastaut syndrome in adulthood: Clinical and EEG features

We performed a retrospective study to investigate seizure, EEG, social and cognitive outcome in adult LGS subjects. We retrospectively evaluated 27 LGS patients aged 40-59 years. We assessed in particular the evolution of different seizure types and EEG findings, as well as cognitive and social outcome. During the early stages of the disease, all patients presented tonic seizures (TS) during wakefulness and sleep, 20/27 had atypical absences (AA), more rarely other seizure types. EEG showed slow background activity in 21/27 patients, diffuse slow spike-wave discharges (DSSW) during wakefulness in 22/27, and bursts of diffuse fast rhythms (DFR) in sleep in all patients. At last observation, 11 patients only had TS during wakefulness, but all still presented TS during sleep; AA persisted in 6 patients. EEG showed normal BA in 12/27 patients; only 7/27 still presented DSSW. On the contrary, sleep EEG showed the persistence of DFR in all. A moderate to severe cognitive impairment was observed in 26/27 patients. In adult LGS patients TS during sleep remain the major seizure type; moreover, a standard waking EEG may be normal. Thus, polysomnography represents the most important mean of investigation also in adult LGS patients.

Clinical features of psychogenic nonepileptic seizures: A study of 64 cases in southwest China

This article describes the clinical features of psychogenic nonepileptic seizures (PNES) in people in southwest China. Patients with a confirmed diagnosis of pure PNES by video/EEG monitoring were retrospectively reviewed. A total of 64 patients with PNES were included, 32 (50%) of whom were male. Twenty (31.3%) patients had previously been misdiagnosed and treated for epilepsy. Psychological trauma and head injuries were considered antecedent traumatic factors. A history of abuse was rare. The PNES cases were divided into three subtypes: psychogenic minor motor seizures, psychogenic major motor seizures, and unresponsive seizures. Age at onset was identified as a predictor of prognosis. The results of this study demonstrated a higher prevalence of PNES in males compared with previous studies. The semiology of PNES in China is similar to that in Western countries. Classification of semiology may be helpful in the differential diagnosis of PNES.

Prospective study of new-onset seizures presenting as status epilepticus in childhood

To characterize children with new-onset seizures presenting as status epilepticus at a tertiary care children's hospital. Prospectively collected data were reviewed from a database derived from a mandated critical care pathway. A total of 1,382 patients presented with new-onset seizures between 2001 and 2007. A total of 144 patients presented in status epilepticus. The average age was 3.4 years. The majority of seizures (72%) lasted between 21 and 60 minutes. The majority of patients had no significant past medical history; one-fourth had a family history of epilepsy. Five (4%) patients with EEGs had electrographic seizures during the study, captured only with prolonged monitoring. The most common etiology was febrile convulsion, followed by cryptogenic. The most common acute symptomatic cause was CNS infection; the most common remote symptomatic cause was cerebral dysgenesis. Combined CT and MRI provided a diagnosis in 30%. CT was helpful in identifying acute vascular lesions and acute edema, whereas MRI was superior in identifying subtle abnormalities and remote symptomatic etiologies such as dysplasia and mesial temporal sclerosis. Children who present in status epilepticus that is not a prolonged febrile convulsion should undergo neuroimaging in the initial evaluation. For any child who presents in status epilepticus and has not yet returned to baseline, the possibility of nonconvulsive status epilepticus should be considered. Although CT is often more widely accepted, especially in the urgent setting, strong consideration for MRI should be given when available, due to the superior yield.

Linear clinical progression, independent of age of onset, in Niemann–Pick disease, type C

Niemann-Pick disease, type C is a neurodegenerative, lysosomal storage disorder with a broad clinical spectrum and a variable age of onset. The absence of a universally accepted clinical outcome measure is an impediment to the design of a therapeutic trial for NPC. Thus, we developed a clinical severity scale to characterize and quantify disease progression. Clinical signs and symptoms in nine major (ambulation, cognition, eye movement, fine motor, hearing, memory, seizures, speech, and swallowing) and eight minor (auditory brainstem response, behavior, gelastic cataplexy, hyperreflexia, incontinence, narcolepsy, psychiatric, and respiratory problems) domains were scored. Data were collected from 18 current NPC patients and were extracted from records of 19 patients. Both patient cohorts showed a linear increase in severity scores over time. Cross-sectional evaluation of current patients showed a linear increase in the severity score. Longitudinal chart review of historical data demonstrated that although age of onset varied significantly, the rate of progression appeared linear, independent of age of onset, and similar in all patients. Combining the data from both cohorts, disease progression could be modeled by the following equation: ŝ(t0+x) = ŝ(t0) + 1.87x; where ŝ(t0) is the initial score and ŝ(t0+x) is the predicted future score after x years. Our observation that disease progression is similar across patients and independent of age of onset is consistent with a biphasic pathological model for NPC. This scale may prove useful in the characterization of potential biomarkers, and as an outcome measure to monitor disease progression in NPC patients.

Continuous Electroencephalogram Monitoring in the Intensive Care Unit

Because of recent technical advances, it is now possible to record and monitor the continuous digital electroencephalogram (EEG) of many critically ill patients simultaneously. Continuous EEG monitoring (cEEG) provides dynamic information about brain function that permits early detection of changes in neurologic status, which is especially useful when the clinical examination is limited. Nonconvulsive seizures are common in comatose critically ill patients and can have multiple negative effects on the injured brain. The majority of seizures in these patients cannot be detected without cEEG. cEEG monitoring is most commonly used to detect and guide treatment of nonconvulsive seizures, including after convulsive status epilepticus. In addition, cEEG is used to guide management of pharmacological coma for treatment of increased intracranial pressure. An emerging application for cEEG is to detect new or worsening brain ischemia in patients at high risk, especially those with subarachnoid hemorrhage. Improving quantitative EEG software is helping to make it feasible for cEEG (using full scalp coverage) to provide continuous information about changes in brain function in real time at the bedside and to alert clinicians to any acute brain event, including seizures, ischemia, increasing intracranial pressure, hemorrhage, and even systemic abnormalities affecting the brain, such as hypoxia, hypotension, acidosis, and others. Monitoring using only a few electrodes or using full scalp coverage, but without expert review of the raw EEG, must be done with extreme caution as false positives and false negatives are common. Intracranial EEG recording is being performed in a few centers to better detect seizures, ischemia, and peri-injury depolarizations, all of which may contribute to secondary injury. When cEEG is combined with individualized, physiologically driven decision making via multimodality brain monitoring, intensivists can identify when the brain is at risk for injury or when neuronal injury is already occurring and intervene before there is permanent damage. The exact role and cost-effectiveness of cEEG at the current time remains unclear, but we believe it has significant potential to improve neurologic outcomes in a variety of settings.

Stressors at the onset of adult epilepsy: implications for practice

The association between major life events and seizure frequency in patients with chronic epilepsy has previously been suggested in the literature. However, significant life events as precipitating factors for the occurrence of the first seizure have been considered but not documented. Recognition of such triggers may lead to a better understanding of the cause and mechanism of the epilepsy. Using a phenomenological approach, 19 participants were interviewed and recalled the occurrence of significant life events in the year prior to a diagnosis of generalized or focal epilepsy. There were gender and age-related differences in the types of triggering events, e.g. men tended to specify work related stressors while women generally cited relationship issues. None of the participants reported constraining beliefs about the cause of their epilepsy. Most respondents incorporated their knowledge of seizure triggers into strategies to achieve control of their epilepsy. This study highlights the potential value of questioning possible life stressors as triggers for the onset of epilepsy. Early awareness of high risk factors for seizures may lead to strategies of seizure self control by avoiding situations associated with high risks, improving lives disrupted by the uncertainty of epilepsy.

Epileptic negative myoclonus: SPECT, PET, and video/EEG studies and the dramatic effects of levetiracetam

An 18-year-old woman presented with epileptic negative myoclonus (ENM) as her major seizure pattern for 4years. Her seizures were characterized by intermittent postural lapse of the right limbs for a period of hours to 2 days. Ictal electroencephalography (EEG)-electromyography showed a silent period that was time-locked to generalized spike-wave discharges. Video/EEG monitoring demonstrated marked improvement of ENM after oral administration of levetiracetam. Cranial magnetic resonance imaging was normal, but 2-deoxy-2-[(18)F]fluoro-d-glucose positron emission tomography during frequent ENM showed hypometabolism in the left frontoparietal cortex. Technetium-99m-ethyl cysteinate dimer single-photon emission computed tomography revealed hyperperfusion over the left parietal cortex and putamen. Here, we document the short-term effects of levetiracetam in this subject with nearly isolated ENM and the neuroimaging results during ENM. Long-term follow-up is in progress to evaluate the clinical evolution and long-term effects of levetiracetam on ENM.

Status Epilepticus: A Review

Objective Status Epilepticus (SE) has been described as a series of major motor seizure without recovery of consciousness between seizures. SE is a medical emergency that requires prompt diagnosis and appropriate treatment. In this article we shall conclude history, epidemiology, etiology, risk factors, the best management as well as the prognosis of the condition.

Ictal Scalp EEG Findings in Patients with Mesial Temporal Lobe Epilepsy

The syndrome of mesial temporal lobe epilepsy (MTLE) is a well-defined clinical entity that responds to surgical treatment in a considerable number of patients. Although it has been subjected to intensive clinical research, few investigators have published the ictal scalp EEG findings and looked for specific features that might predict postoperative outcome. This study was designed to examine ictal scalp EEG characteristics in detail, in a group of patients with pathologically confirmed hippocampal sclerosis (HS). Patients who underwent long-term video-EEG monitoring at our center during a 3-year period and were diagnosed to have MTLE and pathologically proven HS were included in this retrospective study. All ictal scalp EEGs were investigated in a common reference montage, paying attention to the localization, morphology and frequency of ictal discharges that were accepted to represent a specific phase if the findings were sustained for at least 3 seconds. Any significant change in localization, morphology or frequency of discharges was said to represent a different phase. The ictal EEG patterns in different phases were later compared among seizures of different patients. In addition, the ictal EEG characteristics of the patients in Group I (Engel's classification) were compared with the ictal EEG findings in patients who were included in another group. All the patients have been followed for more than 5 years. Seventy-one ictal EEGs were investigated in 25 adult patients (11 M, 14 F). Onset patterns were lateralized in 81.7% and localized in 76% of the seizures. Thirteen different patterns of onset were detected, the most common of which was the cessation of interictal discharges (35.2%). The most common ictal pattern following the initial changes was ipsilateral temporal rhythmic theta-delta activity (85.2%) that occurred on the average 13.4 seconds after onset. Nonlocalized/lateralized seizure onset of all the seizures or bilateral independent onset was present in 75% of the patients in Groups II-III, whereas this ratio was 14.3% in the patients in Group I (p=0.031). In conclusion, ictal scalp EEG in MTLE allows correct lateralization and localization in most of the seizures. Onset patterns may vary considerably; however, a later significant pattern consisting of rhythmic ipsilateral temporal build-up develops in the majority of seizures. Some ictal EEG characteristics may be related to post-operative outcome.

Adverse events during rotary-wing transport of mechanically ventilated patients: a retrospective cohort study

IntroductionPatients triaged to tertiary care centers frequently undergo rotary-wing transport and may be exposed to additional risk for adverse events. The incidence of physiologic adverse events and their predisposing factors in mechanically ventilated patients undergoing aeromedical transport are unknown.MethodsWe performed a retrospective review of flight records of all interfacility, rotary-wing transports to a tertiary care, university hospital during 2001 to 2003. All patients receiving mechanical ventilation via endotracheal tube or tracheostomy were included; trauma, scene flights, and fixed transports were excluded. Data were abstracted from patient flight and hospital records. Adverse events were classified as either major (death, arrest, pneumothorax, or seizure) or minor (physiologic decompensation, new arrhythmia, or requirement for new sedation/paralysis). Bivariate associations between hospital and flight characteristics and the presence of adverse events were examined.ResultsSix hundred eighty-two interfacility flights occurred during the period of review, with 191 patients receiving mechanical ventilation. Fifty-eight different hospitals transferred patients, with diagnoses that were primarily cardiopulmonary (45%) and neurologic (37%). Median flight distance and time were 42 (31 to 83) km and 13 (8 to 22) minutes, respectively. No major adverse events occurred during flight. Forty patients (22%) experienced a minor physiologic adverse event. Vasopressor requirement prior to flight and flight distance were associated with the presence of adverse events in-flight (P < 0.05). Patient demographics, time of day, season, transferring hospital characteristics, and ventilator settings before and during flight were not associated with adverse events.ConclusionMajor adverse events are rare during interfacility, rotary-wing transfer of critically ill, mechanically ventilated patients. Patients transferred over a longer distance or transferred on vasopressors may be at greater risk for minor adverse events during flight.

Transcallosal resection of hypothalamic hamartoma for gelastic epilepsy

Hypothalamic hamartomas (HHs) are commonly associated with severe epilepsy resistant to anticonvulsant therapy. Historically, surgical resection of HHs resulted in considerable morbidity. Two series of patients who successfully underwent resection using a transcallosal approach have now been published; we report the first UK experience of this technique in a series of five patients with HHs and gelastic epilepsy resistant to anticonvulsant therapy. Patients were assessed pre- and postoperatively for seizure activity, endocrine function, ophthalmology, and neurocognitive function. Two patients had precocious puberty and all had evidence of developmental delay and behavioral problems. Postoperatively, all children experienced at least a 50% reduction in seizure frequency with abolition of major seizure types; one child remains seizure-free. One child developed a mild postoperative right hemiparesis and one developed transient diabetes insipidus. There were no adverse developmental effects of surgery. Transcallosal resection of HHs ameliorates resistant epilepsy syndromes associated with HH.

Examining a community model of epilepsy care for people with learning disabilities

To assess the use of specialised medical epilepsy services by people with learning disabilities (LD) and epilepsy in a community healthcare setting, to compare medical epilepsy care in this group to current management guidelines, and to contrast important outcomes with those achieved in different healthcare settings. Postal survey with a carer completed questionnaire addressed to all adults with epilepsy registered on an LD register in Sheffield, UK (n=442). An analysis based on 225 returned questionnaires revealed that 22.7% of individuals with LD and epilepsy had been free of seizures for over 1 year. 95.1% were taking antiepileptic drugs (AEDs), 46.2% had had an EEG, and 41.3% a brain scan. 53.3% of diagnoses had been made by epilepsy experts, 38.7% of individuals with LD and epilepsy were under specialist review. Although patients with more severe epilepsy were more likely to be under specialist care, 60.6% of patients with ongoing seizures, 57.9% with major seizures and 68.7% of individuals taken to hospital with prolonged had no access to specialist advice. The proportion of people with LD who achieved seizure-control in the described population was lower than in all previously reported studies of LD patient groups. The poor outcome in terms of seizure-control, the lack of access to the epilepsy specialist service, and the apparent under-utilisation of investigations indicate that there are grounds for serious concern about this community model of medical epilepsy care for people with LD.

Takotsubo cardiomyopathy in a Caucasian Italian woman: Case report

BackgroundTakotsubo cardiomyopathy is an acute cardiac syndrome characterized by transient LV regional wall motion abnormalities (with peculiar apical ballooning appearance), chest pain or dyspnea, ST-segment elevation and minor elevations of cardiac enzyme levelsCase presentationA 68-year-old woman was admitted to the Emergency Department because of sudden onset chest pain occurred while transferring her daughter, who had earlier suffered a major seizure, to the hospital. The EKG showed sinus tachycardia with ST-segment elevation in leads V2–V3 and ST-segment depression in leads V5–V6, she was, thus, referred for emergency coronary angiography. A pre-procedural transthoracic echocardiogram revealed regional systolic dysfunction of the LV walls with hypokinesis of the mid-apical segments and hyperkinesis of the basal segments. Coronary angiography showed patent epicardial coronary arteries; LV angiography demonstrated the characteristic morphology of apical ballooning with hyperkinesis of the basal segments and hypokinesis of the mid-apical segments. The post-procedural course was uneventful; on day 5 after admission the echocardiogram revealed full recovery of apical and mid-ventricular regional wall-motion abnormalities.ConclusionTakotsubo cardiomyopathy is a relatively rare, unique entity that has only recently been widely appreciated. Acute stress has been indicated as a common trigger for the transient LV apical ballooning syndrome, especially in postmenopausal women. The present report is a typical example of stress-induced takotsubo cardiomyopathy in a Caucasian Italian postmenopausal woman.

Usefulness of the eeg investigation to diagnose TIC disorders in children and adolescents.

The convulsant action of 3-mercaptopropionic acid (3-MPA), a known inhibitor of glutamate decarboxylase activity, was studied in 7-, 12-, 18- and 25-day-old rats and in adult animals. 3-MPA elicited predominantly clonic, minimal seizures as well as generalized tonicclonic (major) seizures at all developmental stages studied. The CD50 for major seizures did not change during development; CD50 for minimal seizures was significantly lower in 18-day-old rats than in older animals. Latency to the onset of seizures was shortest in 18-dayold rats and extremely long in 12- and, especially, in 7-day-old rats. This long latency might signify either changing molecular properties of glutamate decarboxylase during development or slow turnover of GABA at early postnatal stages. Electrocorticographic recordings demonstrated sharp EEG components in the frontal region as a first sign of 3-MPA action, and seizure patterns exhibited similar developmental changes as found with other seizure models (a decrease in duration of individual graphoelements and an increase in synchronization among various cortical regions). This indicates the primary importance of brain maturation in the expression of epileptic EEG phenomena. The correlation between EEG and motor phenomena was poor in the youngest animals and it ameliorated with age, but it never became perfectly coincidental.

Eyelid myoclonia with absences: Routine EEG is sufficient to make a diagnosis

To identify the prevalence, clinical characteristics and routine EEG features of the syndrome of eyelid myoclonia with absences (EMA) using a retrospective case control study design. EEGs from 1996 to 2005 were searched using the following keywords: eyelid flutter, eyelid blinking, tics, idiopathic generalized epilepsy, clinical absence, atypical absence and photoparoxysmal response. During the same period, patients with a diagnosis of idiopathic generalized epilepsy were identified. Patients with mainly eyelid fluttering/eyelid blinking as their seizure semiology were divided into EMA and non-EMA groups using previously published criteria and compared using parametric (Student's t-test) and non-parametric tests (Chi square) where appropriate. A p-value of <0.05 was considered significant. The keywords identified 997 patients, 288 patients were diagnosed with idiopathic generalized epilepsy; 126 had eyelid fluttering/blinking as their major seizure semiology. After excluding 51 patients due to incomplete data, of 75 remaining patients, 26 (9.03%) had EMA. Patients with EMA were (1) older at time of first EEG (OR=2.86; 95% CI=7.00-10.23; p=0.005) (2) more likely to have an event on routine EEG (OR=3.62; 95% CI=1.28-10.19; p=0.01) (3) had >3 events per day (OR=9.73; 95% CI=2.06-45.96; p=0.0012) (4) had higher prevalence of developmental delay (OR=4.46; 95% CI=1.36-14.67; p=0.01) and (5) had normal EEG background compared to the non-EMA group. EMA is not uncommon; diagnosis can be made with good clinical history and routine EEG. As developmental delay is a common association with EMA in this study, early identification and treatment are important.