[Demyelinizing diseases in children. Acute disseminated encephalomyelitis and multiple sclerosis]. / Enfermedades desmielinizantes en niños.Encefalomielitis diseminada aguda y esclerosis múltiple.

The two main demyelinating diseases in children are reviewed. Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). For its physiopathological characteristics, probable etiologies, clinical manifestations, diagnosis, treatment, prognosis, evolution, as well as atypical alterations that complicate its diagnosis, the smaller the child is, more study is needed before reaching the diagnosis. The International Study Group of Multiple Pediatric Sclerosis, published the operating definitions for demyelinating diseases acquired from the central nervous system in children: the ADEM is monophasic, polysymptomatic and with encephalopathy. Its duration is up to 3 months, with fluctuating symptoms and magnetic resonance findings. MS is an isolated monofocal or polyfocal syndrome, without encephalopathy. Currently, two different and distinguishable diseases are considered from the onset of symptoms.

Se revisan dos de las principales enfermedades desmielinizantes en niños, la encefalomielitis aguda diseminada (EAD) y la esclerosis múltiple (EM). Por sus características fisiopatológicas, etiologías probables, manifestaciones clínicas, diagnóstico, tratamiento, pronóstico, evolución, así como alteraciones atípicas que complican su diagnóstico; cuanto más pequeño es el paciente se necesita estudiar más, antes de llegar al diagnóstico. El Grupo Internacional de Estudio de Esclerosis Múltiple Pediátrica publicó las definiciones operativas para enfermedades desmielinizantes adquiridas del sistema nervioso central: la EAD es monofásica, polisintomática y con encefalopatía. Su duración es de hasta 3 meses, con síntomas fluctuantes y hallazgos en resonancia magnética. La EM se define como síndrome aislado monofocal o polifocal, sin encefalopatía. Actualmente se consideran dos enfermedades diferentes y distinguibles desde el inicio de los síntomas.

Enfermedades desmielinizantes en niños: Encefalomielitis diseminada aguda y esclerosis múltiple / Demyelinizing diseases in children. Acute disseminated encephalomyelitis and multiple sclerosis

Se revisan dos de las principales enfermedades desmielinizantes en niños, la encefalomielitis aguda diseminada (EAD) y la esclerosis múltiple (EM). Por sus características fisiopatológicas, etiologías probables, manifestaciones clínicas, diagnóstico, tratamiento, pronóstico, evolución, así como alteraciones atípicas que complican su diagnóstico; cuanto más pequeño es el paciente se necesita estudiar más, antes de llegar al diagnóstico. El Grupo Internacional de Estudio de Esclerosis Múltiple Pediátrica publicó las definiciones operativas para enfermedades desmielinizantes adquiridas del sistema nervioso central: la EAD es monofásica, polisintomática y con encefalopatía. Su duración es de hasta 3 meses, con síntomas fluctuantes y hallazgos en resonancia magnética. La EM se define como síndrome aislado monofocal o polifocal, sin encefalopatía. Actualmente se consideran dos enfermedades diferentes y distinguibles desde el inicio de los síntomas.

The two main demyelinating diseases in children are reviewed. Acute disseminated encephalomyelitis (ADEM) and multiple sclerosis (MS). For its physiopathological characteristics, probable etiologies, clinical manifestations, diagnosis, treatment, prognosis, evolution, as well as atypical alterations that complicate its diagnosis, the smaller the child is, more study is needed before reaching the diagnosis. The International Study Group of Multiple Pediatric Sclerosis, published the operating definitions for demyelinating diseases acquired from the central nervous system in children: the ADEM is monophasic, polysymptomatic and with encephalopathy. Its duration is up to 3 months, with fluctuating symptoms and magnetic resonance findings. MS is an isolated monofocal or polyfocal syndrome, without encephalopathy. Currently, two different and distinguishable diseases are considered from the onset of symptoms.

[Transional syndrome: from West to Lennox-Gastaut syndromes]. / Síndrome transicional: del síndrome de West al síndrome de Lennox-Gastaut.

West syndrome or infantile spasms is an epileptic encephalopathy, classified as generalized epilepsies and syndromes. There are multiple reports of the evolution from West to Lennox-Gastaut syndrome of 25 up to 60%, without a specific cause is determined. It has been reported that they may be only an epileptic entity age dependent that it would be in relation to the degree of brain immaturity. In this retrospective review of 130 cases of West syndrome, only 14 (10.7%) evolved to Lennox-Gastaut. Having received in all cases vigabatrin as a treatment, makes us suppose that the low incidence could be related to the use of this drug. Given that vigabatrin has a gabaergic action and increased levels of ACTH, may explain this relationship but this must be confirmed with the best knowledge of the intimate mechanisms of these serious epileptic encephalopathies.

Síndrome transicional: del síndrome de West al síndrome de Lennox-Gastaut / Transional syndrome: from West to Lennox-Gastaut syndromes

El síndrome de West o espasmos infantiles, es una encefalopatía epiléptica clasificada como epilepsias y síndromes generalizados. Hay múltiples informes de la evolución de síndrome de West a síndrome de Lennox-Gastaut de un 25 hasta 60%, sin reconocerse una causa específica. Se ha comunicado que pueden ser solo una entidad epiléptica dependiente de la edad y que estaría en relación con el grado de inmadurez cerebral. En esta revisión retrospectiva de 130 casos de espasmos infantiles, solo 14 (10.7%) evolucionaron a Lennox-Gastaut. El haber recibido en todos los casos vigabatrina como tratamiento nos hace suponer que la baja incidencia podría estar relacionada con el uso de este fármaco. Dado que la vigabatrina tiene una acción gabaérgica y aumenta los niveles de ACTH podría explicar esta relación, pero esto deberá confirmarse con el mejor conocimiento de los mecanismos íntimos de estas graves encefalopatías.

West syndrome or infantile spasms is an epileptic encephalopathy, classified as generalized epilepsies and syndromes. There are multiple reports of the evolution from West to Lennox-Gastaut syndrome of 25 up to 60%, without a specific cause is determined. It has been reported that they may be only an epileptic entity age dependent that it would be in relation to the degree of brain immaturity. In this retrospective review of 130 cases of West syndrome, only 14 (10.7%) evolved to Lennox-Gastaut. Having received in all cases vigabatrin as a treatment, makes us suppose that the low incidence could be related to the use of this drug. Given that vigabatrin has a gabaergic action and increased levels of ACTH, may explain this relationship but this must be confirmed with the best knowledge of the intimate mechanisms of these serious epileptic encephalopathies.

Hipersomnias / Hypersomnia

Las hipersomnias son un grupo de trastornos caracterizados por una somnolencia excesiva durante al menos 1 mes, evidenciada tanto por episodios prolongados de sueño como por episodios de sueño diurno que se producen prácticamente cada día. Se dividen en primarios o centrales, (Hipersomnia idiopática, Narcolepsia y Síndrome de Klein-Levin) y secundarios (Privación del sueño crónica en niños). La somnolencia excesiva debe ser de su ciente gravedad como para provocar alteraciones clínicas significativas o deterioro social, escolar, laboral o de otras áreas importantes de la actividad del individuo; no aparece en el transcurso de otro trastorno del sueño o de otro trastorno mental ni se debe a los efectos fisiológicos directos de una sustancia o de una enfermedad médica. La somnolencia excesiva diurna (SDE) es una manifestación común, se presenta con una frecuencia variable; del 11% en niños hasta el 52,8% en adolescentes. La predominancia es igual en la narcolepsia con o sin cataplejía y en el Síndrome de Kleine-Levin. Su diagnóstico adecuado se basa en la historia clínica y estudios de polisomnografía. Y el tratamiento, ayudará al paciente a mejorar en sus actividades y a elevar su autoestima. La fisiopatología no es clara y su tratamiento va enfocado a disminuir el sueño diurno con fármacos como el Modafinil, Claritromicina o simpaticomiméticos y terapias de apoyo.

Abstract Hypersomnias are a group of disorders characterized by excessive drowsiness for at least 1 month, evidenced by both prolonged episodes of sleep and episodes of daytime sleep that occur almost every day. They are divided into primary or central, (idiopathic hypersomnia, Narcolepsy and Klein-Levin Syndrome) and secondary (Deprivation of chronic sleep in children). Excessive drowsiness should be of sufficient severity to cause significant cant clinical alterations or social, school, work or other important areas of the individual's activity; which does not appear in the course of another sleep disorder or other mental disorder, nor is it due to the direct physiological effects of substances or medical illness. Excessive daytime sleepiness (EDS) is a common manifestation; it occurs with a variable frequency; From 11% in children to 52.8% in adolescents. The predominance is the same in Narcolepsy with or without cataplexy and in Kleine-Levin Syndrome. The adequate diagnosis is based on clinical history and studies of polysomnography. The treatment will help the patient to improve their activities and raise their self-esteem. The pathophysiology is not clear and the treatment is focused on decreasing daytime sleep with drugs such as Modafinil, Clarithromycin or sympathomimetics and supportive therapies.

[Status epilepticus in childhood]. / Estado de mal epiléptico en pediatría.

Status epilepticus is a medical emergency which presents seizures by 30 minutes or more of continuous activity, or two or more consecutive crises without full recovery of consciousness between them. Currently, it is considered that a seizure convulsive or not, that last more than 5 minutes should be considered a status epilepticus. Different drugs for the treatment of this disease have been used. There is a general consensus in an aggressive handling should be done to reduce their morbidity and mortality, without forgetting that the cause of status is important for its management, control, and its aftermath.

Trastornos paroxísticos no epilépticos durante el sueño / Non-epileptic paroxysmal sleep disorders

Los trastornos paroxísticos no epilépticos durante el sueño son un gran reto para el clínico. Por ello, es importante conocer las diferentes manifestaciones clínicas que permitan llevar a cabo un diagnóstico diferencial adecuado, ya que las alteraciones, sobre todo motoras en el sueño, son parte de estos trastornos. En el presente trabajo se describen las fases del sueño normal y sus características electroencefalográficas, así como datos básicos de la polisomnografía. Las confusiones, sobre todo con la epilepsia nocturna del lóbulo frontal, son frecuentes y provocan que se administren fármacos innecesarios, así como una carga emocional en los padres o cuidadores del paciente, que resulta del diagnóstico de epilepsia. Se enuncian las posibles causas de los errores de diagnóstico (AU)

Non-epileptic paroxysmal disorders during sleep are a great challenge for the clinician. It is important to know the various clinical manifestations for appropriate differential diagnosis, since alterations in sleep, mostly motor, are part of these disorders. Our paper describes the normal sleep stages and electroencephalographic characteristics and polysomnography basic data. The confusions especially with nocturnal frontal lobe epilepsy are frequent and cause unnecessary drugs administered, the emotional burden of the parents or caretakers, which is the diagnosis of epilepsy. We discuss the possible causes of diagnostic errors (AU)

[Non-epileptic paroxysmal sleep disorders]. / Trastornos paroxísticos no epilépticos durante el sueño.

Non-epileptic paroxysmal disorders during sleep are a great challenge for the clinician. It is important to know the various clinical manifestations for appropriate differential diagnosis, since alterations in sleep, mostly motor, are part of these disorders. Our paper describes the normal sleep stages and electroencephalographic characteristics and polysomnography basic data. The confusions especially with nocturnal frontal lobe epilepsy are frequent and cause unnecessary drugs administered, the emotional burden of the parents or caretakers, which is the diagnosis of epilepsy. We discuss the possible causes of diagnostic errors.

TITLE: Trastornos paroxisticos no epilepticos durante el sueño.Los trastornos paroxisticos no epilepticos durante el sueño son un gran reto para el clinico. Por ello, es importante conocer las diferentes manifestaciones clinicas que permitan llevar a cabo un diagnostico diferencial adecuado, ya que las alteraciones, sobre todo motoras en el sueño, son parte de estos trastornos. En el presente trabajo se describen las fases del sueño normal y sus caracteristicas electroencefalograficas, asi como datos basicos de la polisomnografia. Las confusiones, sobre todo con la epilepsia nocturna del lobulo frontal, son frecuentes y provocan que se administren farmacos innecesarios, asi como una carga emocional en los padres o cuidadores del paciente, que resulta del diagnostico de epilepsia. Se enuncian las posibles causas de los errores de diagnostico.

Estado de mal epiléptico en pediatría / [Status epilepticus in childhood].

Status epilepticus is a medical emergency which presents seizures by 30 minutes or more of continuous activity, or two or more consecutive crises without full recovery of consciousness between them. Currently, it is considered that a seizure convulsive or not, that last more than 5 minutes should be considered a status epilepticus. Different drugs for the treatment of this disease have been used. There is a general consensus in an aggressive handling should be done to reduce their morbidity and mortality, without forgetting that the cause of status is important for its management, control, and its aftermath.

Estado de mal epiléptico en pediatría. / [Status epilepticus in childhood].

Status epilepticus is a medical emergency which presents seizures by 30 minutes or more of continuous activity, or two or more consecutive crises without full recovery of consciousness between them. Currently, it is considered that a seizure convulsive or not, that last more than 5 minutes should be considered a status epilepticus. Different drugs for the treatment of this disease have been used. There is a general consensus in an aggressive handling should be done to reduce their morbidity and mortality, without forgetting that the cause of status is important for its management, control, and its aftermath.

[Pediatric neurocysticercosis]. / Neurocisticercosis en pediatría.

Cysticercosis: parasitic disease which affects 3% of the population in Mexico. It is considered that there are more than 50 million infected people in the world, endemic in Mexico, Central and South America, Africa, Asia and India. It is considered the most important neurological infectious disease world-wide for its clinical manifestations. The causal agent in pigs and humans is the cysticercus of the Taenia solium, that can lodge in muscle, brain and ventricles. If pork meat contaminated with cysticercus is eaten, the tapeworm will live in the human intestine and create thousands of eggs that are excreted by the feces. When food contaminated with fecal matter is consumed by man or pig, the cisticercosis is disseminated in several parts of the organism, specially CNS. Man is the only host of the tapeworm and the pig is the only intermediary, reason why the prevalence of the teniasis-cisticercosis depends on this bond. It is diagnosed in endemic zones by the presence of convulsion crises, focal migraine, neurological symptoms, disorders of vision, endocraneal hypertension and CT scan with hypodense zones or cysts with a hyperdense ring. The antiparasitic treatment in children is controversial among pediatricians; it is suggested to use it only in the non calcified cystic phase and in cases associated with epilepsy. Few are the cases of hydrocephalic or intraventricular cysticercus that need surgical treatment.

Neurocisticercosis en pediatría / Pediatric neurocysticercosis

Cisticercosis: enfermedad parasitaria que afecta en México al 3% de la población. Se considera que hay más de 50 millones de infectados en el mundo, endémica en México, Centro y Sudamérica, Africa, Asia e India. Considerada la enfermedad infecciosa neurológica más importante mundialmente por sus manifestaciones clínicas. El agente causal en cerdos y humanos es el cisticerco de la Taenia solium, que puede alojarse en músculo, cerebro y ventrículos. Si se ingiere carne de cerdo contaminada con cisticercos, se adquiere la solitaria, que vive en los intestinos del ser humano y genera miles de huevecillos que se excretan por la materia fecal. Al ingerir alimentos contaminados con materia fecal por el humano y el cerdo, se adquiere la cisticercosis que compromete distintas partes del organismo, especialmente el SNC. El ser humano es el único hospedero de la solitaria y el cerdo es intermediario, por lo que la prevalencia de la teniasis-cisticercosis depende de este vínculo. Se diagnostica en zonas endémicas por la presencia de crisis convulsivas, cefalea, síntomas neurológicos focales, trastornos de visión, hipertensión endocraneal y tomografía cerebral con zonas hipodensas o quiste con un anillo hiperdenso. El tratamiento con antiparasitarios es controversial en pediatría, se sugiere administrarse en la fase quística no calcificada y en casos de epilepsia asociada. Pocos son los casos que ameritan tratamiento quirúrgico por hidrocefalia o por cisticerco intraventricular, que es muy raro.

Cysticercosis: parasitic disease which affects 3% of the population in Mexico. It is considered that there are more than 50 million infected people in the world, endemic in Mexico, Central and South America, Africa, Asia and India. It is considered the most important neurological infectious disease world-wide for its clinical manifestations. The causal agent in pigs and humans is the cysticercus of the Taenia solium, that can lodge in muscle, brain and ventricles. If pork meat contaminated with cysticercus is eaten, the tapeworm will live in the human intestine and create thousands of eggs that are excreted by the feces. When food contaminated with fecal matter is consumed by man or pig, the cisticercosis is disseminated in several parts of the organism, specially CNS. Man is the only host of the tapeworm and the pig is the only intermediary, reason why the prevalence of the teniasis-cisticercosis depends on this bond. It is diagnosed in endemic zones by the presence of convulsion crises, focal migraine, neurological symptoms, disorders of vision, endocraneal hypertension and CT scan with hypodense zones or cysts with a hyperdense ring. The antiparasitic treatment in children is controversial among pediatricians; it is suggested to use it only in the non calcified cystic phase and in cases associated with epilepsy. Few are the cases of hydrocephalic or intraventricular cysticercus that need surgical treatment.

[Cerebral palsy]. / Parálisis cerebral.

The term cerebral palsy (CP), is used for a great number of clinical neurological syndromes. The syndromes are characterized by having a common cause, motor defects. It is important, because they can cause a brain damage by presenting motor defects and some associated deficiencies, such as mental deficiency, epilepsy, language and visual defects and pseudobulbar paralysis, with the non-evolving fact. Some authors prefer using terms such as "non-evolving encephalopathies". In the treatment the utility of prevention programs of early stimulation and special rehabilitation methods, and treatment of associated deficiencies such as epilepsy, mental deficiency, language, audition and visual problems, and the attention deficit improve the prognosis in an important way. The prognosis depends on the severity of the disease and the associated manifestations.