[Hair with a double crown and dilatation of Virchow-Robin spaces: a proposal for a new neurocutaneous association].

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.