====== Neurocutaneous disorders ======

Formerly called [[phakomatoses]]. [[Neurocutaneous]] disorders (NCD) are a group of conditions, each with unique neurologic findings and benign cutaneous lesions (NB: both skin and the CNS derive embryologically from ectoderm), usually with [[dysplasia]] of other organ systems (often including the eyes). 

★ With the exception of Sturge-Weber and ataxia-telangiectasia, all exhibit [[autosomal dominant]] inheritance. There is also a high rate of spontaneous [[mutation]]s. These [[syndrome]]s should be kept in mind in a pediatric patient with a tumor, and other stigmata of these syndromes should be sought.

"Neurocutaneous [[disorder]]s" is a catch-all phrase that includes all disorders involving both the [[nervous system]]s and the [[skin]]. These may range from disorders in which cutaneous findings are essential to diagnosis to those with less significant involvement of the skin. Because of the variety of disorders that involve the skin and the nervous system
((Barbagallo JS, Kolodzieh MS, Silverberg NB, Weinberg JM. Neurocutaneous
disorders. Dermatol Clin. 2002 Jul;20(3):547-60, viii. Review. PubMed PMID:
12170887.
)).

NCDs that are more likely to come to the attention of the neurosurgeon:

1. [[neurofibromatosis]].

2. [[tuberous sclerosis]]

3. [[Von Hippel-Lindau disease]] 

4. [[Sturge-Weber syndrome]] 

5. racemose angioma ([[Wyburn-Mason syndrome]]): midbrain and retinal AVMs