[Visuo-constructive disorders in periventricular leukomalacia]. / Trastornos visuoconstructivos en la leucomalacia periventricular.

INTRODUCTION: Cerebral palsy involves not only motor impairment but also the impairment of cognitive functions. Visuo spatial impairment has often been reported in preterm children with spastic diplegia due to periventricular leukomalacia. PATIENTS AND METHODS: Eight children with periventricular leukomalacia diagnosis. When examined their ages were between 3 and 13 years. RESULTS: We found visuo spatial anomalies in all subjects. These anomalies don't result in reading and writing learning difficulties. CONCLUSION: Constructional dyspraxia is frequently found in children with periventricular leukomalacia and correlates with reduction of peritrigonal white matter in parietal lobe.

[Magnetic Resonance in children with dyskinetic cerebral palsy secondary to perinatal injury]. / Resonancia magnética craneal en niños con parálisis cerebral discinética de probable origen perinatal.

INTRODUCTION: The incidence of perinatal factors in the dyskinetic cerebral palsy (DCP) is high. The introduction of the magnetic resonance (MR) has improved our understanding of the pathophysiology and early diagnosis of injury. OBJECTIVE: Review the clinical characteristics of a group of patients with DCP and the appearance of their MR. PATIENTS AND METHODS: Retrospective study of 20 patients with DCP from 37 weeks of gestational age and perinatal injury with MR done. RESULTS: The perinatal injury was: fetal distress (15 cases), severe hyperbilirubinaemia (1 case) and neurological symptoms of unknown origin (3 cases). From the 15 patients with fetal distress, 10 had clinical manifestations of neonatal hypoxia ischemia. The rest were clinical healthy. Seventeen patients had abnormal MR. Four of these had fetal distress without neonatal clinical manifestations. MR findings show high intensity areas on T2 weighted images in the nucleus thalamus in 6 cases, putamen in 4, thalamus and putamen in 1, thalamus and pallidus in 2, thalamus and lenticular in 3 and in the lenticular alone in one case. In 5 cases hipointensity areas on T1 weighted images at the same sites were identified and in 1 case prolongation of T1. In the child whose CDP was caused by severe hyperbilirubinaemia the MR was normal. CONCLUSIONS: The fetal distress occurring late in labour without neonatal hipoxic ischemic manifestations can precede the developmental of DCP. Our observations support that in CDP resulting from perinatal injury the MR shows characteristic focal abnormalities in the basal ganglia (putamen and thalamus).

Resonancia magnética craneal en niños con parálisis cerebraldiscinética de probable origen perinatal / Magnetic resonance in children with dyskinetic cerebral palsy secondary to perinatal injury

Introducción. La parálisis cerebral discinética (PCD) se relaciona con factores perinatales en un elevado porcentaje de casos. Los avances en técnicas de neuroimagen, especialmente la resonancia magnética (RM), han permitido un mejor conocimiento de la fisiopatología y diagnóstico precoz de las lesiones. Objetivo. Analizar los antecedentes de un grupo de pacientes con PCD de probable origen perinatal y correlacionar los hallazgos clínicos y la exploración con las lesiones en la RM. Pacientes y métodos. Estudio retrospectivo de 20 pacientes con PCD y nacidos a término, en los que se revisan sus antecedentes perinatales y resultados de la RM craneal. Resultados. Los antecedentes perinatales adversos fueron: sufrimiento fetal (15 casos), hiperbilirrubinemia grave (un caso), sepsis (un caso) y depresión neurológica de causa desconocida (tres casos). Del grupo de 15 pacientes con sufrimiento fetal, solamente 10 presentaron clínica neurológica en el período neonatal. La RM se altera en 17 casos, de los que cuatro corresponden a pacientes con antecedente de sufrimiento fetal sin clínica neonatal. Se encontraron áreas de hiperseñal en T2 en ambos núcleos talámicos en seis casos, putamen en cuatro, núcleo talámico y putamen en uno, núcleos talámicos y pálido en dos, núcleos talámicos y lenticular en tres, y núcleo lenticular en uno. En cinco casos se observa la misma lesión en T1 en forma de hiposeñal y en un caso en forma de hiperseñal (igual que en T2). La RM fue normal en el paciente que presentó la hiperbilirrubinemia. Conclusiones. El sufrimiento agudo al final del parto y la necesidad de reanimación profunda, incluso sin clínica neonatal, pueden dejar como secuela una PCD. La mayoría de los pacientes con PCD y antecedentes de sufrimiento fetal presentan en la RM un patrón típico de lesión cerebral, y destacan los núcleos talámico y putamen como las zonas más vulnerables (AU)

Casuística de epilepsia sintomática en una unidad de neuropediatría de referencia regional / Cases seen with symptomatic epilepsy in a regional reference neuropediatric unit

Objetivo. Analizar la casuística de epilepsia sintomática de la Sección de Neuropediatría del Hospital Miguel Servet de Zaragoza (España) desde el punto de vista etiológico. Pacientes y métodos. Se han estudiado los casos diagnosticados de epilepsia sintomática desde mayo de 1990 a noviembre de 1999. Resultados.De un total de 4.466 niños valorados durante el período de estudio, se ha establecido el diagnóstico de epilepsia en 461 niños (10,3 por ciento del total), que correspondió a epilepsias idiopáticas en 110 casos (23,9 por ciento), criptogénicas en 119 (25,8 por ciento) y sintomáticas en 232 casos (50,3 por ciento). La etiología de las epilepsias sintomáticas ha sido: encefalopatías prenatales en 137 casos (59 por ciento), perinatales en 33 (14,3 por ciento), posnatales (por accidentes, infecciones adquiridas y accidentes cerebrovasculares posnatales) en 20 (9 por ciento), patología tumoral (incluido período posquirúrgico) en 14 (6 por ciento), síndromes neurocutáneos en 13 (5,6 por ciento), enfermedades metabólicas y degenerativas en 13 (5,6 por ciento) y 1 caso de malformación vascular. Conclusiones. Las epilepsias sintomáticas representan la mitad de las epilepsias valoradas en la sección de neuropediatría, y en el 59 por ciento de ellas el origen es prenatal. Las demás causas de epilepsia sintomática han quedado representadas en la serie; su estudio detallado debe contribuir a profundizar en su conocimiento y mejorar su manejo.Consideramos de especial interés la aproximación etiológica en el estudio de la epilepsia, puesto que la etiología es uno de los factores pronósticos más importantes (AU)

[Cases of idiopathic and cryptogenic epilepsias in a regional referral neuropediatric unit]. / Casuística de epilepsias idiopáticas y criptogénicas en una unidad de neuropediatría de referencia regional.

INTRODUCTION AND OBJECTIVES: Advances in genetics, techniques for diagnosis and treatment, and increasing concern for the quality of life and neuropsychological aspects of epileptic children and the possible repercussions of treatment lead to changes in the approach to epilepsy and require continual adaptation. In this study we analysed a series of cases of idiopathic and cryptogenic epilepsy attended by the Neuropaediatric Department of the Hospital Miguel Servet in Zaragoza (Spain). PATIENTS AND METHODS: We studied the cases diagnosed as having idiopathic or cryptogenic epilepsy between May 1990 and December 1999. RESULTS: We assessed 4,507 children. In 1,794 (39.8%) consultation was for a paroxystic disorder. Epilepsy was diagnosed in 466 children (10.3%) and 103 cases were considered possibly or probably epilepsy but the diagnosis remains in doubt (2.3%). In 111 cases (23.8%) the epilepsy was considered idiopathic, in 122 (26.2%) cryptogenic and in 233 cases (50%) symptomatic. The following epileptic syndromes were identified: epilepsy-absence attacks in 28 cases (6%), benign myoclonic infantile epilepsy in 4 cases, other idiopathic generalized epilepsies in 61 cases (13%), benign Rolandic epilepsy in 18 cases (3.9%), 2 cases of cryptogenic Ohtahara syndrome, 6 cases of cryptogenic West syndrome, 1 severe case of infantile myoclonic epilepsy, 1 case of continuous wave epilepsy during slow eye movement sleep and 1 case of Landau-Kleffner syndrome. CONCLUSION: Current requirements and the rate of advances in epilepsy make it essential for neuropaediatric teams to include experts in epilepsy.

Otalgia como formade presentación de herniación uncal / Otalgia as a form of presentation of a hernia of the uncus

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Casuística de epilepsias idiopáticas y criptogénicasen una unidad de neuropediatría de referencia regional / A series of cases of idiopathic and cryptogenic epilepsy in a regional neuropediatric unit

Introducción y objetivos. Los avances producidos en genética, técnicas diagnósticas y tratamiento, la creciente preocupación por la calidad de vida y los aspectos neuropsicológicos del niño epiléptico, así como las posibles repercusiones terapéuticas, condicionan cambios en la orientación de la epilepsia que precisan una continua adaptación. Este trabajo analiza la casuística de epilepsias idiopáticas y criptogénicas de la Sección de Neuropediatría del Hospital Miguel Servet de Zaragoza (España). Pacientes y métodos. Se han estudiado los casos diagnosticados de epilepsia idiopática y criptogénica desde mayo de 1990 a diciembre de 1999. Resultados.Han sido valorados 4.507 niños. En 1.794 pacientes (39,8 por ciento) se ha dado el motivo de consulta de trastorno paroxístico. Se ha establecido el diagnóstico de epilepsia en 466 niños (10,3 por ciento) y han permanecido dudas en 103 casos considerados como posible o probable epilepsia (2,3 por ciento). Las epilepsias han sido consideradas idiopáticas en 111 casos (23,8 por ciento), criptogénicas en 122 pacientes (26,2 por ciento) y sintomáticas en 233 niños (50 por ciento). Se han identificado los siguientes síndromes epilépticos: epilepsia-ausencias en 28 casos (6 por ciento), epilepsia mioclónica benigna del lactante en cuatro casos, otras epilepsias generalizadas idiopáticas en 61 casos (13 por ciento), epilepsia rolándica benigna en 18 casos (3,9 por ciento), dos casos de síndromes de Ohtahara criptogénicos, seis casos de síndromes de West criptogénicos, un caso de epilepsia mioclónica grave del lactante, un caso de epilepsia con punta-onda continua durante el sueño lento y un caso de síndrome de Landau-Kleffner. Conclusión. Las necesidades actuales y el ritmo de avances en epileptología exigen equipos de neuropediatras con expertos en epilepsia (AU)

[Cerebral ischemia following varicella. A case report]. / Accidente cerebrovascular isquémico tras varicela. A propósito de un caso.

INTRODUCTION: Varicella in childhood is a self-limiting disease which usually follows a benign course. However, a considerable number of complications occur, particularly involving the nervous system. On rare occasions hemiparesis has been reported following an ischemic cerebrovascular accident related to varicella. CLINICAL CASE: We report the case of a 4 year old boy who presented with right hemiparesis eight days after the onset of varicella. CAT and MR showed a cerebral infarct involving the left caudate nucleus, putamen and internal capsule. Other possible causes of cerebral infarct were ruled out. Four years later he had slight residual right hemiparesis. The pathogenesis may have been due to vasculitis of the intracranial arteries caused by direct invasion of the vessels by virus from the nearby nerve tissue. Another theory suggest similarities with moyamoya disease, suggesting that lesions of the head and neck stimulate the superior cervical ganglion and this, together with the production of circulating immuno-complexes may be the cause of the vasculopathy of the intracranial blood vessels. CONCLUSIONS: In most cases of cerebrovascular accidents occurring in childhood the cause is unknown. However, it is important to discover the cause since the risk of recurrence often depends on the primary disease. Once other commoner causes have been excluded, one should ask whether there is a history of varicella, since the prognosis is generally good and recurrence uncommon.

Accidente cerebrovascular isquémico tras varicela. A propósito de un caso / Ischemic cerebrovascular accident following varicella. A case report

Introducción. La varicela en la infancia es una enfermedad autolimitada que generalmente tiene un curso benigno. Sin embargo, produce un número no desdeñable de complicaciones, muchas de ellas con afectación del sistema nervioso. En ocasiones esporádicas se ha descrito hemiparesia secundaria a accidente cerebrovascular isquémico en relación con la varicela. Caso clínico. Presentamos el caso de un niño de 4 años que presentó hemiparesia derecha, ocho días después del comienzo de la varicela. La TAC y la RM mostraron infarto cerebral que afectaba al caudado, putamen y cápsula interna izquierda. Se excluyeron otras posibles causas de infarto cerebral. Cuatro años más tarde presenta una discreta hemiparesia derecha residual. Una posible patogénesis es la vasculitis de arterias intracraneales debido a la invasión directa del vaso por el virus a partir de estructuras nerviosas próximas. Otra teoría encuentra similitudes con la enfermedad de moyamoya, y sugiere que las lesiones de cara y cuello estimularían al ganglio cervical superior, circunstancia que, unida a la producción de inmunocomplejos circulantes, sería la causa de la vasculopatía de los vasos intracraneales. Conclusiones. La causa de los accidentes cerebrovasculares en la infancia se desconoce en un alto porcentaje. Encontrar una causa es importante porque el riesgo de recurrencia viene dado en muchos casos por la enfermedad primaria. Una vez excluidas otras causas más frecuentes, debería preguntarse por una historia de varicela, ya que el pronóstico generalmente es bueno y no suele haber recurrencias (AU)

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[Torticollis as a cause of consultation in neuropediatrics]. / Tortícolis como motivo de consulta en neuropediatría.

INTRODUCTION AND OBJECTIVE: Torticollis is a very non-specific symptom occurring in different conditions and may therefore be the reason for consultation in many specialties including neuropaediatrics. Analysis of torticollis as a cause for consultation in neuropaediatrics may contribute to the establishment of a suitable strategy for diagnosis. PATIENTS AND METHODS: We review, from the diagnostic point of view, the clinical histories of cases in which the reasons for consultation included torticollis. These cases were included in the database of all the patients assessed by the neuropaediatric department of the Hospital Miguel Servet in Zaragoza between May 1990 and February 1999. RESULTS: Of the 4,138 new patients evaluated during the period studied, in 60 patients torticollis was either the sole symptom or was one of the symptoms leading to consultation. The diagnoses established were: 30 congenital torticollis (50%), 6 secondary to space-occupying intracranial lesions (10%), 5 benign paroxystic torticollis (8.3%), 4 post-traumatic, 3 secondary to ocular disorders, 3 Sandifer syndrome, 1 focal dystonia of the neck, 1 secondary to a submandibular adeno-phlegmon, 1 secondary to an epidural hematoma of the cervical spine, 1 to encephalomyelitis and 1 to spondylodiscitis, with 4 cases unclassified. CONCLUSIONS: The clinical history, physical examination and follow-up of the course of the disorder orientate or permit the diagnosis to be established in many cases of torticollis. The indications for complementary investigations, particularly neuroimaging, should be considered individually in each case.

[Ischemic stroke of middle cerebral artery territory after the traumatic epidural hematoma]. / Accidente cerebrovascular isquémico del territorio de la arteria cerebral media tras hematoma epidural traumático.

INTRODUCTION: Epidural haematoma is a complication which may occur after apparently minor head injury. It may lead to the sudden onset, after a variable lucid interval, of transtentorial uncal hernia with consequent risk to life. If drainage is done early, the prognosis is excellent. CLINICAL CASE: We present a case of right parietal epidural haematoma in a 5 year-old-boy following a minor head injury, with no fracture of the skull. After a clear interval of 40 hours his consciousness deteriorated, there was right mydriasis and extension hypertonia of arms and legs. Following surgical removal there was evidence of an extensive cerebrovascular accident in the territory of the middle cerebral artery. CONCLUSIONS: Although they are infrequent, infarcts have been described in relation to epidural haematoma in all cerebral territories, predominantly in the distribution of the posterior cerebral artery. In the territory of the middle cerebral artery this may be due to traction and stenosis or occlusion of the small perforating branches, secondary to the displacement of midline cerebral structures or the space-occupying effect of epidural haematoma. All head injuries, including minor injuries mean a risk of serious hemorrhagic complications, the prognosis of which depends on the rapidity with which they are treated. GUIDELINES: Adapted to each centre are necessary for a common problem, usually without complications, but which may have unpredictably fatal results. Head injury is a dynamic process and therefore requires close clinical observation, which cannot be substituted by any complementary test.

[Survival with no sequelae after near-drowning with very poor signs for prognosis including persistent bilateral non-reactive mydriasis]. / Supervivencia sin secuelas tras casi-ahogamiento con muy malos signos pronósticos, incluida midriasis arreactiva bilateral persistente.

INTRODUCTION: The outcome of cases of near-drowning and initially poor prognostic signs are usually discouraging because of the severity of the consequent encephalopathy in most survivors. However, good recovery has been described, in spite of bad prognostic factors initially. It is difficult to establish the predictors of poor outcome which would enable one to decide when to establish and maintain advanced cardio-pulmonary resuscitation measures (CPR), since each case of near-drowning is different. CLINICAL CASE: A four year old boy survived near-drowning in cold water without sequelas but with initial signs of very poor prognosis, including prolonged immersion time, coma, severe metabolic acidosis, hyperglycemia and persistent bilateral arreactive mydriasis. DISCUSSION: The beneficial effect of hypothermia is well known, and explains (at least partially) survival in cases of apparently irreversible near-drowning. Potential benefits are reduced metabolic demand which prevents the adverse effects of hypoxia and the 'diving reflex' which short-circuits the blood supply to vital organs such as the brain and heart. We consider that the persistently arreactive pupils were not due to hypoxia, but rather to bilateral uncal compression of the third cranial nerves due to cerebral edema secondary to initial hypoxia and water intoxication. CONCLUSION: This observation is yet another argument for the establishment and maintenance of aggressive manoeuvers of CPR and treatment in all children who have nearly-drowned, independently of the apparent seriousness or irreversibility.

[Hemimegalencephaly and pathological left handedness. A case report]. / Hemimegalencefalia y zurdería manual patológica. A propósito de un caso.

INTRODUCTION: Hemimegalencephaly is a disorder of neurone proliferation and migration, associated with excessive growth of all or part of one cerebral hemisphere, and variable degrees of pachygyria, polymicrogyria, neuronal heterotopy and gliosis of white matter. It is considered to be a rare malformation with severe encephalopathy, epilepsy resistant to treatment started early, contralateral hemiparesia and severe retardation of development. However, there may also be milder forms. The syndrome of pathological left handedness is associated with disorders of language, visuo-spatial changes and weakness and hypertrophy of the right limbs, with no family history of left handedness. It occurs in lesions predominantly of the left hemisphere which have occurred in the first six years of life. CLINICAL CASE: A 12 year old girl presented with a syndrome of pathological left-handedness and craniofacial asymmetry of the left hemisphere. MR showed the left ventricle and hemisphere to be of increased size and with focal hyperintensity in the white matter, and at the level of the left parietotemporoccipital region there was a thick cortex with little interdigitation of white and grey matter. CONCLUSIONS: Minor forms of hemimegalencephaly are probably more frequent than is generally believed. A high index of suspicion is necessary to identify them. They should be considered in cases of left handedness, craniofacial or bodily asymmetry, cutaneous hamartomas, persistent electroencephalographic asymmetry and asymmetry on neuroimaging with an enlarged ventricle and homolateral hemisphere. Study of this may give information which is useful for understanding plasticity and mechanisms of recovery and functional compensation of immature nerve tissue.