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Idiopathic hypertrophic pachymeningitis is a fibroinflammatory immune-mediated disease of the dura mater. Its diagnosis requires the preclusion of infectious, tumoral and other inflammatory diseases. In recent years new entities have been reported that can present with hypertrophic pachymeningitis, such as IgG4-associated disease and MPO-ANCA+ pachymeningitis, as a form of vasculitis limited to the central nervous system.We describe the case of a 64 years-old male with headaches and cervicalgia, predominantly at night, and clinical signs and symptoms of spinal cord compression. Following the diagnosis of craniocervical hypertrophic pachymeningitis provided by the magnetic resonance imaging study, an aetiological study was conducted. Infectious and tumoral diseases were precluded. The clinical features did not show any systemic involvement and high levels of IgG4 and MPO-ANCA+ were found in the results of the analyses. The clinical signs and symptoms quickly improved following treatment with corticoids.IgG4-related disease and MPO-ANCA-associated vasculitis limited to the central nervous system can account for a high percentage of the cases of hypertrophic pachymeningitis that were considered idiopathic, and their diagnosis requires a biopsy and a histological study.Paquimeningitis hipertrofica relacionada con IgG4 y MPO-ANCA.Introduccion. La paquimeningitis hipertrofica idiopatica es una enfermedad fibroinflamatoria de la duramadre. Su diagnostico requiere la exclusion de enfermedades infecciosas, tumorales y otras enfermedades inflamatorias. En los ultimos años se han descrito nuevas entidades que pueden presentarse con paquimeningitis hipertrofica: la enfermedad relacionada con IgG4 y la paquimeningitis MPO-ANCA+ como forma de vasculitis limitada al sistema nervioso central. Caso clinico. Varon de 64 años con cefalea y cervicalgia de predominio nocturno y clinica de compresion medular. Tras el diagnostico de paquimeningitis hipertrofica craneocervical facilitado por el estudio de resonancia magnetica, se realizo un estudio etiologico. Se descartaron enfermedades infecciosas y tumorales. La clinica no mostraba afectacion sistemica y en la analitica presentaba IgG4 elevada y MPO-ANCA+. Tras tratamiento con corticoides presento una rapida mejoria de la clinica. Conclusiones. La enfermedad relacionada con IgG4 y la vasculitis asociada a MPO-ANCA limitada al sistema nervioso central pueden representar un alto porcentaje de las paquimeningitis hipertroficas que se consideraban idiopaticas, y su diagnostico requiere biopsia y estudio histologico.

Introduccion. La paquimeningitis hipertrofica idiopatica es una enfermedad fibroinflamatoria de la duramadre. Su diagnostico requiere la exclusion de enfermedades infecciosas, tumorales y otras enfermedades inflamatorias. En los ultimos anos se han descrito nuevas entidades que pueden presentarse con paquimeningitis hipertrofica: la enfermedad relacionada con IgG4 y la paquimeningitis MPO-ANCA+ como forma de vasculitis limitada al sistema nervioso central. Caso clinico. Varon de 64 anos con cefalea y cervicalgia de predominio nocturno y clinica de compresion medular. Tras el diagnostico de paquimeningitis hipertrofica craneocervical facilitado por el estudio de resonancia magnetica, se realizo un estudio etiologico. Se descartaron enfermedades infecciosas y tumorales. La clinica no mostraba afectacion sistemica y en la analitica presentaba IgG4 elevada y MPO-ANCA+. Tras tratamiento con corticoides presento una rapida mejoria de la clinica. Conclusiones. La enfermedad relacionada con IgG4 y la vasculitis asociada a MPO-ANCA limitada al sistema nervioso central pueden representar un alto porcentaje de las paquimeningitis hipertroficas que se consideraban idiopaticas, y su diagnostico requiere biopsia y estudio histologico.

The disorders of the autistic spectrum form a collection of symptoms due to dysfunction of the central nervous system with great variations in the degree of severity. Autism is considered to be a generalized disorder of development (DSM-IV). Autism is not defined as a specific disease, since it does not have a specific aetiology.There are many syndromes related to autism, but most of these disorders are not selective and show a combination of autistic symptoms together with symptoms of neurological dysfunction. There is no specific aetiology, although in recent years genetics have been shown to be important. The prevalence varies between 1 and 1.2/1,000. Boys are more often affected than girls, in a proportion of 3-4 to 1. Diagnosis is clinical and is based on alterations of social interaction, problems of communication and also a restricted range of activities and interests (DSM-IV). There are anomalies associated with behaviour problems, such as delay in speaking, mental retardation, sensorial defects and motor difficulties.Over 75% of autistic children have mental retardation, and this proportion is higher in severe cases, especially when the children have attention deficit with hyperactivity. These children have many of the typical signs of autism: stereotyped movements, inappropriate language, obsessive behaviour with little mental flexibility, naivety and little skill in social interaction. In these cases it is difficult to draw the line between mental retardation and autism.

Reflex epileptic seizures are caused by a specific sensorial stimulus which determines their classification. Photosensitive epilepsies are the commonest forms and are included with the idiopathic generalized epilepsies.We analyze the different responses to intermittent light stimulation, in both normal and epileptic persons, and study the various epileptic syndromes in which photosensitivity is seen. The purely photosensitive (photogenic) epilepsies are characterized by seizures caused only by light as compared with the epilepsies with photosensitivity which also present spontaneous seizures. Special mention is made of seizures induced by the television screen, computer screen and video-games. The palpebral myoclonias with absences may be considered to be reflex seizures, since they are induced by eyelid closure, and include the vary rare self-induced epileptic seizures. Finally we study the epileptic seizures induced by pattern and exclusively due to intermittent light stimulation.

The idiopathic generalized epilepsies of childhood form a heterogeneous group of epileptic syndromes, with certain clinical and electroencephalographic characteristics in common. From the onset, the seizures affect both cerebral hemispheres with generalized clinical expression and from the start there are bilateral electroencephalographic patterns during seizures. These epilepsies have no known causes, there is a genetic predisposition and relation to the age of onset. The electroencephalographic characteristics during and between seizures have points in common: the basal electroencephalographic activity is normal in all the epilepsies of this group, the paroxystic anomalies are generalized, synchronic and symmetrical, and are formed of bilateral discharges of spikes, multiple spikes, spikes and waves and multiple spikes and waves, with discharges at a frequency of 3 Hz or more.In this article we review the electroencephalographic characteristics during and between seizures of the idiopathic generalized epilepsies and epileptic syndromes of childhood, with particular emphasis on childhood absences, since we consider this to be the commonest and most representative of the idiopathic generalized epilepsies of infancy.We consider that the electroencephalographic aspects of epilepsies and the epileptic syndromes make up a factor of great importance as a help in confirmation in diagnosis.

Rett syndrome was described in 1966 and became known through the English medical literature in 1983. There are typical and atypical forms. The objective of this study was to record the cases diagnosed in Spain and discover their clinical characteristics in order to describe its phenotype and geographical distribution.We know of 207 cases and have obtained the records of 168 of these patients. A protocol and data collection programme has been developed giving the criteria for inclusion, and data which support or exclude this. Data collection was by post and the data for identification were the date of birth and the initials of the name and two surnames. With these variables, double-registering of patients was almost impossible. A statistical study with descriptive analysis and a study of continuous and alternating variables was immediately done.The results gave the main characteristics, the differences between typical and atypical cases and a comparative study of variables. It has given clinical data which may be useful for prognosis of the condition in the future.

The Adie tonic pupil is an intrinsic, isolated and benign ophthalmoplegia producing defective accommodation and anisocoria. The affectation is unilateral in the 90% of cases and can appear at any age. If associated to patellar tendinous reflexes abolition, constitutes Holmes-Adie syndrome.We present a 6 years old girl. She suffered a right temporal trauma with cephalea and vomiting for a few hours. After two months, she presented right mydriasis. There were no familiar antecedents. After a year evolution, she has not presented clinical changes.We comment the process's physiopathology.

We present a male patient which presented distal muscular stiffness, from the first hours of the life. At this time, he also presented episodes of generalized muscular hypertonia with cyanosis and apnoea, started by somato-sensorial stimuli. Those episodes were terminated by passive flexion of the extremities and the head. Later, dream myoclonic jolts appeared. EEG tracings during hypertonic episodes showed an initial artefact potential followed by bursts of rhythmic and repetitive acute potentials in the bilateral fronto-central regions, at a 20-22 Hz frequency, similar to an epileptiform spike-wave burst. The EMG showed a continuous muscular activity, suppressed by rest and the administration of diazepam. Treatment with oral diazepam has been very effective. Now, the patient is four years old, is asymptomatic and continues treatment with oral diazepam. If the dose is decreased, the child starts unstable march, startle response on somatosensory stimuli with falls, and above all nocturnal myoclonia. The patient does not have familiar antecedents of hyperekplexia, even in its minor form.Clinical picture suggests us a not familiar form of hyperekplexia.

The childhood opsoclonus-myoclonus or Kinsbourne syndrome, is a uncommon process, of acute or subacute beginning, which affects infant and children. It's course is characterized by opsoclonus, polimyoclonias and cerebellar ataxia. The disease is frequently associated to neuroblastoma (46%).We present a retrospective study on 9 patients, emphasizing the clinical presentation and the evolution aspects.We found changes in the EEG in three cases. Most surprising is the scarce incidence of neuroblastoma, which has been found only in a one out of nine patients. We found three cases with relapse during the treatment or on withdrawal and one of them relapsed twice again. The evolution has been variable, since 5/9 patient have presented some type of mild or moderate neuro-psychological sequelae. Out of three patient with relapses, two presented permanent neurological sequelae. A patient which suffered three relapses, is also the one which presents more serious sequelae.

Symptomatic epilepsy secondary to hereditary metabolic or degenerative disorders, is usually associated to neurological deterioration. Though epilepsy by itself does not induce neurological deterioration, we should remind that some epileptics encephalopathies, such as the West or Lennox-Gastaut syndromes, do actually induce limited neurological deterioration. Furthermore, in some forms of complex partial epilepsy, motor problems and behavior disorders can be observed, specially in adolescents with temporary lobe epilepsy. Other forms of epilepsy, such as the atypical benign partial epilepsy or the Landau-Kleffner syndrome, can present a certain degree of cognitive deterioration in the evolution, although they can recover later lost functions, totally or partially. The evolution of some refractory epilepsy, as patients are submitted to a multiple treatments, can make us suspect a degenerative disease. In some cases, the diagnosis of the hereditary metabolic and heredodegeneratives diseases can be made by the characteristics of the seizures but in most cases the diagnosis will be established by the symptoms of the basic disease and the lab data.

Between 369 cases of benign partial epilepsy of the childhood with Rolandic spikes (BECRS) diagnosed in our hospital, we have been able to study three patients that have presented a neurophysiological and clinical presentation that was compatible with the form described by Aicardi and Chevrie in 1982.None of our patients had familial epilepsy precedents. There were no pathological birth or previous neurological disorders. The neurophysiological development was normal until the beginning of the clinical picture. The seizures began when the patients were 3 years and 4 months, and 5 years, with partial seizures of the same characteristic that present patients with BECRS. Two of them had generalized tonic-clonic seizures during sleep. All the patients presented throughout the evolution absences myoclonic and/or atonic seizures. The awake EEG patterns showed normal background activity and paroxysms of focal spike were complexes with diffusion to central areas. During the slow wave sleep, every cases showed paroxysms of diffuse and generalized slow spike and wave complexes practically continuous. The partial seizures were scarce, but the absences and the atonicas seizures presented several times every day, and in one case appeared in the form of a grand mal state.The evolution of the patients was favourable, as they were free of seizures, with a normal neurophysiological development. At the beginning, the seizures were resistant to treatment with several antiepileptics drugs (AED). The treatment with valproic acid (VPA) and clonazepam (CZP), has been very effective.

Introduction The idiopathic generalized epilepsies of childhood form a heterogeneous group of epileptic syndromes, with certain clinical and electroencephalographic characteristics in common. From the onset, the seizures affect both cerebral hemispheres with generalized clinical expression and from the start there are bilateral electroencephalographic patterns during seizures. These epilepsies have no known causes, there is a genetic predisposition and relation to the age of onset. The electroencephalographic characteristics during and between seizures have points in common: the basal electroencephalographic activity is normal in all the epilepsies of this group, the paroxystic anomalies are generalized, synchronic and symmetrical, and are formed of bilateral discharges of spikes, multiple spikes, spikes and waves and multiple spikes and waves, with discharges at a frequency of 3 Hz or more. Development In this article we review the electroencephalographic characteristics during and between seizures of the idiopathic generalized epilepsies and epileptic syndromes of childhood, with particular emphasis on childhood absences, since we consider this to be the commonest and most representative of the idiopathic generalized epilepsies of infancy. Conclusions We consider that the electroencephalographic aspects of epilepsies and the epileptic syndromes make up a factor of great importance as a help in confirmation in diagnosis.

Introduction The Adie tonic pupil is an intrinsic, isolated and benign ophthalmoplegia producing defective accommodation and anisocoria. The affectation is unilateral in the 90% of cases and can appear at any age. If associated to patellar tendinous reflexes abolition, constitutes Holmes-Adie syndrome. Clinical case We present a 6 years old girl. She suffered a right temporal trauma with cephalea and vomiting for a few hours. After two months, she presented right mydriasis. There were no familiar antecedents. After a year evolution, she has not presented clinical changes. Conclusion We comment the process's physiopathology.

INTRODUCTION AND MATERIAL Between 369 cases of benign partial epilepsy of the childhood with Rolandic spikes (BECRS) diagnosed in our hospital, we have been able to study three patients that have presented a neurophysiological and clinical presentation that was compatible with the form described by Aicardi and Chevrie in 1982. RESULTS None of our patients had familial epilepsy precedents. There were no pathological birth or previous neurological disorders. The neurophysiological development was normal until the beginning of the clinical picture. The seizures began when the patients were 3 years and 4 months, and 5 years, with partial seizures of the same characteristic that present patients with BECRS. Two of them had generalized tonic-clonic seizures during sleep. All the patients presented throughout the evolution absences myoclonic and/or atonic seizures. The awake EEG patterns showed normal background activity and paroxysms of focal spike were complexes with diffusion to central areas. During the slow wave sleep, every cases showed paroxysms of diffuse and generalized slow spike and wave complexes practically continuous. The partial seizures were scarce, but the absences and the atonicas seizures presented several times every day, and in one case appeared in the form of a grand mal state. CONCLUSIONS The evolution of the patients was favourable, as they were free of seizures, with a normal neurophysiological development. At the beginning, the seizures were resistant to treatment with several antiepileptics drugs (AED). The treatment with valproic acid (VPA) and clonazepam (CZP), has been very effective.

INTRODUCTION The childhood opsoclonus-myoclonus or Kinsbourne syndrome, is a uncommon process, of acute or subacute beginning, which affects infant and children. It's course is characterized by opsoclonus, polimyoclonias and cerebellar ataxia. The disease is frequently associated to neuroblastoma (46%). MATERIAL AND METHODS We present a retrospective study on 9 patients, emphasizing the clinical presentation and the evolution aspects. RESULTS AND CONCLUSIONS We found changes in the EEG in three cases. Most surprising is the scarce incidence of neuroblastoma, which has been found only in a one out of nine patients. We found three cases with relapse during the treatment or on withdrawal and one of them relapsed twice again. The evolution has been variable, since 5/9 patient have presented some type of mild or moderate neuro-psychological sequelae. Out of three patient with relapses, two presented permanent neurological sequelae. A patient which suffered three relapses, is also the one which presents more serious sequelae.

Introduction and clinical case We present a male patient which presented distal muscular stiffness, from the first hours of the life. At this time, he also presented episodes of generalized muscular hypertonia with cyanosis and apnoea, started by somato-sensorial stimuli. Those episodes were terminated by passive flexion of the extremities and the head. Later, dream myoclonic jolts appeared. EEG tracings during hypertonic episodes showed an initial artefact potential followed by bursts of rhythmic and repetitive acute potentials in the bilateral fronto-central regions, at a 20-22 Hz frequency, similar to an epileptiform spike-wave burst. The EMG showed a continuous muscular activity, suppressed by rest and the administration of diazepam. Treatment with oral diazepam has been very effective. Now, the patient is four years old, is asymptomatic and continues treatment with oral diazepam. If the dose is decreased, the child starts unstable march, startle response on somatosensory stimuli with falls, and above all nocturnal myoclonia. The patient does not have familiar antecedents of hyperekplexia, even in its minor form. Conclusion Clinical picture suggests us a not familiar form of hyperekplexia.

Symptomatic epilepsy secondary to hereditary metabolic or degenerative disorders, is usually associated to neurological deterioration. Though epilepsy by itself does not induce neurological deterioration, we should remind that some epileptics encephalopathies, such as the West or Lennox-Gastaut syndromes, do actually induce limited neurological deterioration. Furthermore, in some forms of complex partial epilepsy, motor problems and behavior disorders can be observed, specially in adolescents with temporary lobe epilepsy. Other forms of epilepsy, such as the atypical benign partial epilepsy or the Landau-Kleffner syndrome, can present a certain degree of cognitive deterioration in the evolution, although they can recover later lost functions, totally or partially. The evolution of some refractory epilepsy, as patients are submitted to a multiple treatments, can make us suspect a degenerative disease. In some cases, the diagnosis of the hereditary metabolic and heredodegeneratives diseases can be made by the characteristics of the seizures but in most cases the diagnosis will be established by the symptoms of the basic disease and the lab data.