[Hair with a double crown and dilatation of Virchow-Robin spaces: a proposal for a new neurocutaneous association]. / Implantación del cabello en doble remolino y dilatación de los espacios de Virchow-Robin: propuesta de nueva asociación neurocutánea.

INTRODUCTION: The primitive ectoderm is the common origin of the nervous system, the skin and related organs. These structures can therefore be affected simultaneously by aggressions that take place during embryogenesis involving the ectodermal layer. To date multiple neurocutaneous associations have been reported, some of which were catalogued as genetically determined syndromes or diseases. Thus, it becomes possible to recognise these entities at an early stage, to carry out a better therapeutic approach to the neurological pathology and even, in some cases, to establish a prognosis. CASE REPORTS: We present five male patients between 2 and 7 years of age in whom we found a common pattern of deviated psychomotor and behavioural development consisting of a hyperactivity and impulsivity disorder associated to retarded language acquisition. Common to all of them was the hair growing with a double crown and dilatation of the Virchow-Robin spaces seen in the neuroimaging studies. CONCLUSIONS: We propose a clinical-radiological association that, to our knowledge, has no equivalent in the literature, and we highlight the importance of knowing how to recognise both the neuropsychiatric symptoms and the skin features, as well as the characteristic neuroimaging findings of this group of patients.

Implantación del cabello en doble remolino y dilatación de los espacios de Virchow-Robin: propuesta de una nueva asociación neurocutánea / Hair with a double crown and dilatation of virchow-robin spaces: a proposal for a new neurocutaneous association

Introducción. El ectodermo primitivo es el origen común del sistema nervioso, la piel y sus anexos. Estas estructuras, por tanto, pueden afectarse de forma simultánea ante agresiones producidas durante la embriogénesis sobre la lámina ectodérmica. Hasta ahora se han descrito múltiples asociaciones neurocutáneas, algunas de ellas catalogadas como síndromes o enfermedades, la mayoría determinadas genéticamente. De esta forma, podemos reconocer precozmente dichas entidades, realizar un mejor abordaje terapéutico de la patología neurológica e incluso, en algunos casos, establecer un pronóstico. Casos clínicos. Presentamos cinco pacientes varones entre los 2 y los 7 años de edad en los que hemos encontrado un patrón común de desviación del desarrollo psicomotor y conductual consistente en un trastorno de hiperactividad e impulsividad asociado a un retraso en la adquisición del lenguaje. Todos ellos tenían en común tanto la implantación del cabello en doble remolino como la dilatación de los espacios de Virchow-Robin en el estudio de neuroimagen. Conclusiones. Proponemos una asociación clínicorradiológica de la cual no hemos encontrado equivalente en la literatura, y destacamos la importancia de saber reconocer tanto los síntomas neuropsiquiátricos como los rasgos cutáneos y los hallazgos de la neuroimagen característicos de este grupo de pacientes (AU)

Introduction. The primitive ectoderm is the common origin of the nervous system, the skin and related organs. These structures can therefore be affected simultaneously by aggressions that take place during embryogenesis involving the ectodermal layer. To date multiple neurocutaneous associations have been reported, some of which were catalogued as genetically determined syndromes or diseases. Thus, it becomes possible to recognise these entities at an early stage, to carry out a better therapeutic approach to the neurological pathology and even, in some cases, to establish a prognosis. Case reports. We present five male patients between 2 and 7 years of age in whom we found a common pattern of deviated psychomotor and behavioural development consisting of a hyperactivity and impulsivity disorder associated to retarded language acquisition. Common to all of them was the hair growing with a double crown and dilatation of the Virchow-Robin spaces seen in the neuroimaging studies. Conclusions. We propose a clinical-radiological association that, to our knowledge, has no equivalent in the literature, and we highlight the importance of knowing how to recognise both the neuropsychiatric symptoms and the skin features, as well as the characteristic neuroimaging findings of this group of patients (AU)

Diagnóstico del síndrome 9p- al nacimiento. Una nueva observación / Diagnosis of 9p- syndrome at birth. A new case

No disponible