الفهرس 
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Abstract
Neurocutaneous syndromes (NCS) are progressive multisystem disorders characterized by both neurological and skin involvement in which the dermatological findings may be apparent before the neurological signs and symptoms appear.
Neurocutaneous disorders are relatively common and could be seen in different societies with different incidence for example, NF type1 affects one every 4000 births while NF type2 affects one every 60,000 births in United Kingdom. In tuberous sclerosis one every 6000-9000 births could be affected and nearly one million people worldwide are known to have tuberous sclerosis, about 50,000 in United States. Sturge Weber syndrome, Ataxia telangectasia and Von Hippel Lindau disease are rare disorders.
This is a prospective study done in Sohag university hospital in period between 1st of December 2012 to 30th of November 2013 on children from birth to 15 years old who were suspected to have NCS by clinical examination (neurological, dermatological), neuroradiological studies (CT brain), EEG, IQ assessment, other investigations e.g. (ophthalmological examination, echocardiography) presented to the neuropediatric and dermatology outpatient clinics during the period of the study.
Our study included the following NCS: Neurofibromatosis type 1(NF1), Tuberous sclerosis (TS), Sturge Weber Syndrome (SWS), Xeroderma Pigmentosa (XP), Incontenintia Pigmenti (IP) and Hypo melanosis of Ito (HI).
We performed a clinical analysis of 27 children suspected to have NCS considering the type of NCS by characteristic skin lesions, neuroradiological findings (CT brain) with special consideration for the presence of epilepsy and its characteristics in EEG e.g. infantile spasms in TS.
We found that the most common NCS was (TS) followed by (NF type1), (XP), (SWS) and less common types were (IP) and (HI) with the most common clinical features of NCS in our community were characteristic skin lesions, seizures and global developmental delay, and the most valuable tools in diagnosis were brain CT, EEG, together with clinical examination of skin. These findings are very important in the diagnosis; management and counseling of patients with NCS.