Rasmussen syndrome (RS) is a rare encephalitis characterized by inflammation of one cerebral hemisphere that causes atrophy, neurological and cognitive impairment. We present a 42-year-old female patient, with no significant previous history and diagnosed with epilepsy since she was 24 years old, epileptic seizures are reported in different time windows with temporary cessation, but with constant reinstallations of acute and drug-resistant episodes. Neurological examination revealed hyperkinesia of the extremities, with persistent flexion and extension movements. The neuropsychological evaluation carried out with the MoCA test, Rey’s complex figure and Token test, shows severe cognitive impairment with psychiatric symptoms. In addition, electroencephalogram results show unilateral hemispheric slowing with unilateral focal onset and MRI evidence of generalized atrophy of the left hemisphere, elements supporting the diagnosis of late-onset RS. Finally, information is offered about the associated neuropsychological manifestations.
Epilepsy
Rehabilitación neuropsicológica en memoria declarativa y la funcionalidad en un adulto con epilepsia y lobectomía temporal izquierda. Neuropsychological rehabilitation program on declarative memory and functionality in an adult with epilepsy and left temporary lobectomy
Introduction: Surgical intervention is a treatment option for refractory epilepsy, and after this procedure cognitive alterations may occur. Software-based intervention approaches represent an alternative to traditional approaches.
Objective: To identify the effect of a neuropsychological rehabilitation plan for declarative memory in an adult with left temporal lobectomy, on their functional abilities.
Methodology: A single-case quasi-experimental design was used; the participant was a 45-year-old woman with memory problems and difficulties in performing daily activities. The intervention was carried out using the CogniFit rehabilitation software, and the Functioning Classification Scale and the Quality of Life Questionnaire in Epilepsy were used for the measurement.
Results: A large effect (NAP; 95%) was found in both indices.
Conclusions: The implementation of a software-based program allows a comprehensive rehabilitation.
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Abordaje prequirúrgico en epilepsia de difícil control. Presurgical approach in drug-resistant epilepsy
Epilepsy is one of the main reasons for consultation in general neurology. It is a highly prevalent pathology, with a high impact on the quality of life of these patients. There is a percentage of drug resistance between 30% and 40% of epilepsy cases, and therefore it is very important to know the surgical alternative, as well as the importance of a timely and prompt referral to a specialized surgery center of epilepsy given the high possibility of seizure remission or improvement towards less disabling seizures, with a notable improvement in quality of life. The evaluation process of a patient with drug-resistant focal epilepsy who is a candidate for epilepsy surgery is based on a set of non-invasive diagnostic techniques. The evaluation process is based on the seizure locating semiology, an adequate protocol for the imaging study, electroencephalogram, and interictal and ictal video-monitoring, and neuropsychological evaluation in all cases, and in other functional studies such as computed tomography are necessary, ictal and interictal single-photon emission, positron emission tomography, and also invasive monitoring techniques, through which it is possible to proceed to surgery. A review of the literature is made.
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Canalopatía de calcio CACNA1E: Nueva encefalopatía epiléptica en un paciente pediátrico. CACNA1E calcium channelopathy: New epileptic encephalopathy in a pediatric patient
Introduction: Epileptic and developmental encephalopathies, related to voltage-associated calcium channel mutations, are heterogeneous entities recently described in pediatric population.
Objective: To describe the case of pediatric patient with epileptic and developmental encephalopathy caused by a mutation in the CACNA1E gene that codes for a calcium channel.
Clinical case: Preschool male patient with refractory epilepsy starting at two months of life, associated with global developmental delay, behavioral disorder, hyperkinetic movements (dystonia) and hypotonia, with multifocal interictal activity and normal brain resonance, with pathogenic mutation in the gene CACNA1E.
Conclusion: Mutations in the CACNA1E gene cause functional alteration of the calcium channel CaV2.3, originating a clinical picture of a recently described phenotype.
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Epilepsia Como Factor Pronóstico de Refractariedad y Funcionalidad en Estado Epiléptico en Pacientes Mexicanos. Epilepsy As Prognostic Factor Of Refractoriness And Functionality In Status Epilepticus In Mexican Patients.
Introduction: Status epilepticus is a medical condition which could lead to a great disability and be mortal. Background of epilepsy could prevent a progression to a refractory status epilepticus and predict a good functional prognosis. Nevertheless, in Mexican population there are no studies about that.
Materials and methods: An observational, analytic, retrospective, cohort study was performed with patients with status epilepticus in Specialities Hospital of “Siglo XXI” National Medical Center, in Mexico. It was evaluated the probability of presenting refractory status epilepticus and a good functional outcome in patients with a background of epilepsy, performing Pearson Chi-square.
Results: 40 patients with status epilepticus were included, 32 with refractory status epilepticus and 14 with a background of epilepsy. The probability of presenting a refractory status epilepticus with a background of epilepsy was OR 1.29 (p 0.53), and for a good functional outcome was OR 3.63 (p 0.10).
Conclusions: Background of epilepsy is not a protective factor for preventing the progression to a refractory status epilepticus, neither a factor for predicting a good functional outcome in our study with Mexican population. Nevertheless, further studies with a bigger sample are needed in order to prove these results.
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Etiología del Daño Cerebral: Un Aporte Neuropsicológico en su Construcción Teórica (Primera Parte). Etiology Of Brain Damage: A Neuropsychological Contribution In Its Theoretical Construction (First Part)
The brain is the organ responsible for all the brilliant activities that human beings perform, such as thinking, acting, speaking, solving problems, making decisions, regulating emotions and other fascinating mental abilities. Any of these brain functions can be damaged at the least expected moment, generating cognitive and behavioral problems in the patient who suffers from acquired brain damage. This article proposes a theoretical review of various etiological factors of brain damage: (a) the most frequent of them, traumatic brain injury (TBI), a condition that causes a range of cognitive and behavioral deficiencies, in addition to being the main cause of prolonged dysfunction in industrialized countries; (b) brain tumors are also an important etiological factor, since any area of the Central Nervous System (CNS) can be affected and the effects are very varied according to the location of the tumor, its level of severity, its nature and whether or not surgery was performed; (c) thirdly, epilepsy is presented as a neurological disease present in 50 million people around the world and whose consequences on the CNS are related to seizures and anti-seizure drugs; finally, (d) the neurobiology of child abuse is exposed, which can generate alterations in the brain configuration of a minor. In each of these factors, its definition, classification, associated risk factors and, finally, what is its effect on the brain and the nature of the damage will be presented.
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Malformación Arteriovenosa y Epilepsia. Arteriovenous Malformation And Epilepsy.
Perspectivas en el Abordaje Farmacológico de la Epilepsia: Una Revisión Extensa de la Farmacoterapia Perspectives On The Pharmacologic Management Of Epilepsy: A Comprehensive Review
Background. Epilepsy represents the third most common reported neurologic disorder, surpassed only by cerebrovascular accidents and Alzheimer’s disease. Iyt is believed that around 70% to 80% of all convulsive disorders can be successfully controlled with monotherapy alone and an additional 10% to 15% with combination therapy. Around 10% of cases never achieve remission through pharmacological therapy alone. Objectives. Through this comprehensive review of the literature describing the current available pharmacologic therapies for the management of epilepsy and their recognized indications, the authors intend to provide an educational tool that could assist the general practitioner to make decisions when selecting a suitable treatment strategy according to a specific clinical scenario (and as guided by their own professional judgment, circumstances and clinical reality). Methods. We used a wide variety of medical search engines, articles and abstracts for the purpose of data collection and interpretation. Conclusions. Through this review the authors managed to present all current and innovative approaches regarding the pharmacologic management of epilepsy encompassing the scope of current knowledge.
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Primera Crisis Epiléptica en Adultos: ¿Epilepsia o no? First Seizure In Adults: Epilepsy Or Not?
First epileptic seizure is a fact that impacts the life of who suffers and its environment as a relationship with the possibility of a manifestation of epilepsy. It is one of the main causes of consultation in the emergency service, so neurologists and general doctors must have a basic and global knowledge of this. However, there are factors (provoked or unprovoked seizures, risk of recurrence) that it will allow to diagnose of epilepsy with the aim of establishing a timely pharmacological treatment that reduces the possibilities of a new crisis also to resolve the doubts that patients have regarding this disease.
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Adherencia al Tratamiento Antiepiléptico en Pacientes Pediátricos del Hospital Roberto Gilbert Elizalde en el Año 2014. Adherence To Antiepileptic Treatment In Pediatric Patients At Hospital Roberto Gilbert Elizalde During 2014.
Introduction: Epilepsy in the pediatric population is an entity that mostly affects underdeveloped countries. In Ecuador, hospital admissions due to poor control are increasing and the main cause is poor adherence to antiepileptic treatment.
Objective: To demonstrate the incidence of nonadherence to the antiepileptic regimen and determine the factors that contributes to it.
Materials and methods: A cross-sectional study was conducted in which 119 patients were included with ages between 6 months and 17 years. The parents or legal guardians were questioned regarding the degree of adherence to antiepileptic treatment using the Morisky questionnaire. A logistic regression model was used to measure the strength of association between variables.
Results: An incidence of non-adherence to antiepileptic treatment of 36.97% was found. The significantly associated variables were the mother’s schooling (x2=11.83, IC= 10.13 – 13.53, p=0.018), previous medical information (x2=9.35, IC= 7.95 – 10.75, p=0.02) and the failure to obtain medication due to lack of money (x2=5.98, IC= 5.29 – 6.67, p=0.01).
Conclusion: The incidence of nonadherence in this study was high; control over sociodemographic factors and related to treatment can have a great impact on these patients.
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Neurocisticercosis, Epilepsia y Uso de Drogas Antiparasitarias. Neurocysticercosis, Epilepsy And The Use Of Antiparasitic Drugs.
Cysticidal drugs have been used for more than three decades. However, its efficacy has been questioned on the assumption that cysts would die spontaneously, and thus, inflammatory reactions related to therapy are unnecessary. In addition, isolated reports have also questioned whether neurocysticercosis (NCC) causes epilepsy. A large body of evidence is currently available and little – if any – doubt exists on NCC as a cause of unprovoked seizures. NCC is consistently associated with seizures when suitable groups are compared, and in a sizable subset of patients, the semiology of seizures correlates with the anatomical location of parasites. Cyst degeneration and the subsequent inflammatory reaction related to therapy may transiently increase seizure expression, and this can be anticipated and managed with the additional use of corticosteroids. Several controlled trial support the concept that cysticidal drug efficacy, safety, and the impact of cyst destruction in decreasing seizures largely overcome the potential risks of therapy, and have provided evidence of the role of NCC as a cause of r ecurrent unprovoked seizures (epilepsy).
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Complejo Esclerosis Tuberosa Atípico Con Espasmos Epilépticos de Inicio Tardío: Diagnóstico Complejo Por Telemedicina En Una Comunidad Rural de Honduras. Atypical Tuberous Sclerosis Complex With Late Onset Epileptic Spasms: Complex Diagnosis by Telemedicine in a Rural Community of Honduras.
Tuberous sclerosis complex (TSC) is an uncommon neurocutaneous disease often underdiagnosed in remote areas where specialists in neurology are usually not available. A 22 month-old girl was taken to a clinic in a rural village presenting refractory epilepsy of undetermined cause that started at 18 months of age with upward eye deviation episodes and epileptic extensor spasms. She also presented perinasal angiofibromas and hypomelanotic skin lesions in trunk. The interictal encephalogram done in the community showed multifocal and generalized epileptic activity. The girl had been taken to the city for a brain magnetic resonance, which revealed multifocal subcortical hyperintensities and subependymal nodules. By teleconsult between community doctors and national and international specialists, atypical TSC with late onset epileptic spasms was diagnosed. The recommended treatment resulted in controlled seizures, and a follow-up plan was established. This case illustrates that telemedicine units in rural areas are a technological option to provi de access to specialized epilepsy care.
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Estatus Epiléptico. Factores Asociados a Una Evolución Desfavorable en un Centro Terciario. Status Epilepticus. Prognostic Variables For An Unfavorable Outcome In A Tertiary Center Of Care.
Introduction. Status Epilepticus (SE) is a frequent neurologic emergency. Little research has been done in South America to evaluate the prognostic variables of mortality and disability in patients with SE. Objective. To determine the variables associated to an unfavorable outcome at hospital discharge in the patients who were treated for SE. Methods. A retrospective study was performed during the period of January 2016–June 2017. A total of 26 patients were diagnosed of SE and its different variants. The effects of clinical, radiological, and electroencephalographic features on hospital outcome according Rankin scale were evaluated Results. Twelve (46.2%) patients had an unfavorable outcome at hospital discharge, while the mortality rate reached 23.1%. There was a predominance of males with a 76.9% of all the patients. The independent variables associated with an unfavorable outcome were the number of comorbidities (p=0.01, OR: 4.27-95%CI1.33-13.6), structural lesions on the Magnetic Resonance Image (MRI) (p=0.04, OR: 3.92-95%CI1.05-14.61) and refractory SE (p=0.01, OR: 12.52-95%CI1.85-84.44). There was also a trend for age (p=0.07 OR: 1.03-95%CI0.99-1.07). While an initial good clinical condition, according to the Glasgow Scale represent a protective factor (p=0.00 OR: 0.49-IC95% 0.29-0.84) of an unfavorable outcome. Conclusions. The unfavorable outcome was marginally associated with patient age, clinical status at the onset of SE according to the Glasgow Coma Scale, as well as brain lesions on brain MRI. Refractory SE and more than 4 comorbidities are predictors of an unfavorable outcome at hospital discharge.
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Utilidad del Video EEG en un Hospital Pediátrico de Nivel Terciario Durante el Año 2015. Utility Of Video EEG In A Pediatric Tertiary Hospital During 2015.
The aim was to evaluate the V-EEG usefulness in the differential diagnosis of epilepsy in a Third Level Children’s Hospital during 2015. Materials and Methods: A descriptive study was performed over 90 patients in this unit during 2015. The data was obtained from variables related to indications and results of V-EEG, which were analyzed using descriptive statistics. Results: Fifty three percent of the patients were male. The mean age was 7.7 years (SD ± 4.7 years). The time measured between the first seizure and the V-EEG recording was 4,3 years. Seventy two patients (80%) had epileptic seizures, 12 patients (13,3%) had nonepileptic seizures, while six children (6.7%) had no seizures during the V-EEG monitoring. Ninety three percent of all recordings were successful. Conclusions: It was demonstrated the usefulness of V-EEG monitoring for the differential diagnosis of epilepsy.
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Estatus Epiléptico en Colombia: Análisis Descriptivo de Serie de Casos. Status Epilepticus In Colombia: A Case Series Descriptive Analysis.
Status Epilepticus (SE) is a neurological emergency that is by itself a syndrome defined according its clinical presentation and electroencephalographical manifestations, both highly variable. There aren’t any case series about this relatively common neurological emergency in Colombian indexed journals, for which a search for cases was performed in two neurological critical care institutions in the Colombian capital city in the period between 2011-2015. We describe demographical, diagnostic and therapeutic characteristics emphasizing in its clinical and paraclinical presentation, as the treatment applied according international guidelines. We found convulsive SE was three times more common that the non-convulsing time, 1.4 times more common in females that in males, more common in the pediatric group and less common in the geriatric group, the majority of this cases presented SE secondary to a congenital structural injury while adult and elder patients presented SE as a consequence of non-voluntary discontinuation of the therapy, and in 66% of the cases the SE described was not the first of its kind. The therapeutic regime applied corresponded to the stipulated in international guidelines in only 41.67%.
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Prevalencia de Enfermedades Neurológicas no Transmisibles en una Población Rural del Estado de Chiapas, México: Protocolo y Resultados Basales. Estudio Pro-Mas (Proyecto Comunitario La Soledad)
Objective: We aimed to assess cardiovascular health and neurological status of community-dwelling adults living in “La Soledad,” Chiapas, México, to calculate prevalence and incidence of cerebrovascular diseases, epilepsy and cognitive decline. Among others, these conditions are currently considered as the new epidemics in rural.
Design: Following a protocol similar to that used for the Atahualpa Project, we conducted a two-phase epidemiological study. During phase I, La Soledad residents were interviewed with validated field instruments to assess cardiovascular health status and well as to detect suspected stroke, epilepsy and cognitive impairment patients. During phase II, trained physicians examined suspected individuals as well as a random sample of non-suspected individuals to assess prevalence of diseases of interest.
Comment: Public health strategies must be based on the study of region-specific risk factors. Studies such the Atahualpa Project were proven to be effective for the assessment of public health problems in a rural Ecuadorian village. PROMAS will likely be cost effective to increase current knowledge on these conditions in Mexican rural villages and to promote a better cardiovascular health status among their inhabitants.
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Epilepsia Benigna Occipital tipo Panayiotopoulos y Epilepsia Benigna con espigas centrotemporales concomitantemente en el mismo paciente.
The Panayiotopoulos type benign childhood occipital epilepsy, (PBCOE) and the benign childhood occipital epilepsy with centro-temporal spikes (BCECT) are two forms of focal idiopathic epilepsy recognized by the International League Against Epilepsy. Several authors have reported that some patients with PBCOE evolve later in life toward a BCECT, remarking the fact of the morphological similarities of their paroxysms and the spikes migration phenomena. However, few cases of patients with PBCOE have been described concomitantly clinical characteristics of a BCECT in the same evolving process. One clinical case with the mentioned concomitant manifestation is shown, analyzing its clinical characteristics, differential diagnosis and treatment. Finally it is postulated that the PBCOE could be an early form of the BCECT
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Una propuesta para la evaluación de la Prueba de Wada en pacientes analfabetos: Presentación de un caso.
In the preoperative neuropsychological evaluation of certain patients with epilepsy it is important to determine the hemispheric dominance; particularly in those of doubtful laterality, due to the high postoperative risks of affectation of the language and the memory. The test of Wada allows guiding the neurosurgeon about the possible impact of the surgical procedure in the hemispheric areas related to the language and the memory. Although it is well-known that the execution in diagnostic neuropsychological tests is under the influence of an important series of variables, as the culture and the educational level, until now there are not specific protocols for this kind of exploration in illiterate patients. In this study we describe the experience in the test of WADA of an illiterate patient with epilepsy diagnosis and cerebral congenital left hemiatrophy, of left-handed manual laterality. With base in the protocol of Trenerry and Loring the language tasks and memory were implemented according to the patient’s cultural context. It was found a right hemispheric representation for the language and the memory. These results confirm the effects of the cerebral plasticity in the reorganization and /or compensation of the cognitive functions, as the language and the memory in the cerebral hemisphere not affected.
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Hamartoma Hipotalámico.
The association between hypothalamic hamartoma and gelastic epilepsy has been described several years ago. This type of tumor, usually asymptomatic, when it is related to epilepsy, can cause intractable seizures and a particular type of laughter crises. A brief analysis of the clinical features and treatment is performed.
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Principales factores multicausales en la epilepsia que inducen al deterioro de funciones cognitivas.
By studying a group of epileptic patients we try to know the low levels of intelligence quotient (IQ) and positive cognitive deterioration index (DI) and its relation to a group of multietiologic factors. Wechsler’s IQ and DI were used in the study with 130 epilepsy diagnosed patients that were divided into two groups: in the first one were those with CI < 89 and > 90, and in the other those that had either DI positive or not. These variables were analyzed according to biological, psychosocial and drug factors. The data was processed with statistigraphs as Fisher, Odds Ratio, and ANOVA. Out of the total of studied patients 52.31% had a CI <89 and 47.69% had CI >90. 52.3% of the patients had no deterioration and 47.7% showed deterioration index. The factors related to patients with CI < 89 were: past history of alcoholism, first seizure < of 15 years, seizures presented for more than 10 years, high frequency of the seizures, partial seizures with secondary generalization and phenytoin treatment.
Factors related to the DI were: alcoholism, high frequency of seizures, partial seizures with secondary generalization, association with chronic psychosis, treatment with phenytoin and a mixture of phenytoin and carbamazepine. Half of the patients had IQ < 89, and more than one fourth of them showed deterioration. It´s considered that although the deterioration, detected by Weschler Adults Intelligence Scale (WAIS), may not be visible, it is important to know its existence to diminish the risk factors.
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Diagnóstico de un paciente con crisis porfírica hepática aguda.
We present a 21 year old woman treated with carbamazepine because of epileptic seizures. She was evaluated at the epilepsy section for possible seizures associated with abdominal pain, psychosis, dysautonomic features and elevated hepatic enzymes. Diagnosis was based on clinical features and biochemical determination of urinary porphobilinogen and absence of fluorescence for porphyrin in blood sample and hepatic tissue. Conclusions: Acute Intermittent Porphyria constitutes a rare cause of symptomatic seizures but the association of seizures with abdominal pain, dysautonomic features, and psychosis suggest the disease and its recognition is very important because it is potentially mortal but suitable to improve with adequate treatment.
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Cirugía de Epilepsia en Ecuador 2010.
Epilepsy is one of the most common chronic neurological conditions in children and adults. The lifetime risk of developing epilepsy is 3.2%. In this review article we suggest how to apply a safety protocol for the surgical treatment of antiepileptic drugs resistant patients. We review who the ideal candidate for a pre-surgical evaluation is and when to do it; how to do it, using clinical, physiological, imaging and neuro-cognitive biomarkers in order to achieve medical benefit from epilepsy surgery; why patients should go and receive a surgical evaluation. Finally we review the current concepts of drug resistant epilepsy and the surgical intervention benefit/risk relationship.
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Epilepsia Parcial Benigna Atípica de la Infancia: Presentación de un Caso Peculiar y Revisión de la Bibliografía.
Introduction: Atypical benign partial epilepsy of childhood is a rare form of epilepsy characterized by a combination of partial seizures, as observed in Partial Benign Epilepsy of Childhood, as well as with centre-temporal paroxysms and generalized seizures; showing furthermore, a continuos peak wake electroencephalographic pattern characteristic of NREM sleep.
Clinical Case: Patient presenting with partial seizures is admitted to the hospital with EEG findings compatible with continuos peak wave electric status of NREM sleep.
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Aspectos Históricos del Tratamiento Farmacológico de la Epilepsia
In the present work, we make an historical revision on the different treatments that had been employed for epilepsy since ancient times through the present. This article reviews different periods such as: the believe between the Incas and the Aztecas that the cause of epilepsy was the action of malignant spirits and the basements of the treatments in magical and religious methods; the starting of pharmacological treatment in the XIX century with the use of bromides, the discovery of the antiepileptic drug phenobarbital by Alfred Hauptmann in 1912, phenytoin by H. Houston Merrit and Tracy Jackson Putnam in 1937, through the introduction of new antiepileptic drugs such as lamotrigine, tiagabine, vigabatrine, levetiracetam, etc. in the nineties.
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Perfil de la Epilepsia en el Ecuador
The point prevalence of active epilepsy in Ecuador is between 7/1000 and 12/1000, which is similar to that reported in developed countries; however, this finding does not necessarily indicate that the risk to develop epilepsy is the same in these two populations. The incidence of epilepsy in Ecuador is between 120/100,000 and 172/100,000, which is two to threefold higher than that reported in developed countries. These results should be confirmed by further studies. In Ecuador, the frequency of epilepsy is highest during adolescence and adulthood, probably due to the high prevalence of infectious and parasitic diseases in these age groups. Regarding the prognosis, seizures recur in 43% of patients with epilepsy. The risk of recurrence by the Kaplan-Meier actuarial analysis is 30% at 12 months, 51% at 24 months, and 79% at 45 months of follow-up. Statistically significant risk factors for recurrence are the etiology (52% recurrence risk in patients with symptomatic epilepsy) and the presence of an abnormal CT scan (51% recurrence risk in patients with abnormal CT scans). Mortality in patients with epilepsy is six-fold higher than in the general population of Ecuador (standardized mortality ratio SMR of 6.3). The SMR for sudden death in patients with epilepsy (3.9) is also higher than that reported in developed countries. These greater mortality ratios probably explain the relatively low prevalence rates in our country. In 1995, Ecuador spent 4.6% of the gross national product on the health system. The Ministry of Health, which funds the public health system, contributed only 1.5% of this amount. This government contribution is one of the lowest in South America. There are no government programs in Ecuador working to manage and prevent chronic diseases, such as epilepsy. There are also no laws to protect patients with epilepsy. This complex background obligates the health authorities, scientific community, and the society in general, to join together in the fight against epilepsy, as a bio-psycho- social problem.
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Epilepsias Mioclónicas
Objective: To analyze the frequency of myoclonic epilepsies (ME) according to the classification of the International League Epilepsy (ILAF) and workshop of the Comission on Pediatric Epilepsy (Royaumont-France 1997), considering the possibility to include or modifi myoclonic epileptic syndromes. Patients and Methods:Clinic histories of 113 patients (56 men and 57 women) with diagnostic of ME evaluated between January 1993 and July 1998 were reviewed. Early epileptic encephalopathy, progressive and photosensitive epilepsies, as well as other epilepsies presenting with myoclonic seizures during their evolutive course, were excluded. Results: We recognized the following syndromes: a) Idiophatic: 1) Bening ME of Infancy, 10 cases (8.8%), 2) Reflex ME of Infancy, 2 cases (1.8%), 3) Eyelid myoclinia with absence (EMA), 3 cases (2.6%), and 4) Juvenile ME, 29 cases (25.6%); b) Cryptogenic: 1) Myoclonic-Astatic Epilepsy (MAE) of favorable course, 21 cases (18.5%) and unfavorable course, 10 cases (8.8%), 2) Severe ME of infancy, 25 cases (22.1%), and 3) Myoclonic absence epilepsy, 2 cases (1.8%); and c) Symptomatic: 1) MAE, 2 cases (1.8%), 2) Severe ME of Infancy, 2 cases (1.8%), 3) Myoclonic Status in non-progressive encephalopathies (MSnPE), 4 cases (3.5%), and 4) others, 3 cases (2.6%). Conclusion: Cryptogenic (51.3%) and idiopathic (38.9%) seizures were the most common types of ME in our study. In the idiopathic group, the most frequent syndrome was juvenile ME, while in the cryptogenic group, was the Myoclonic-Astatic epilepsy. We consider that EMA should be included in the new classification of epilepsies as an idiopathic syndrome. We also suggest that Reflex ME of Infancy should be discussed as a new syndrome of ME or as a variant of benign ME of Infancy. Finally, whether MsnPE is a new syndrome or a peculiar evolution of symptomatic epilepsies needs further discussion.
