Frequency of Neuroectodermal Diseases (Tuberous Sclerosis Complex, Sturge-Weber Syndrom and Neurofibromatosis) and its Main Clinical Manifestations

Autor: MR Salehiomran, R Shahhoseini
Jazyk: English<br />Persian
Rok vydání: 2013
Předmět:
Zdroj: Majallah-i Dānishgāh-i ̒Ulūm-i Pizishkī-i Bābul, Vol 15, Iss 5, Pp 72-76 (2013)
Druh dokumentu: article
ISSN: 1561-4107
2251-7170
Popis: BACKGROUND AND OBJECTIVE: Neuroectodermal diseases are rare disorders which their main characteristic is cutaneous and neurologic symptoms but other organs may also be involved according the type of disease. One of the common neurologic problems of these diseases is seizure, which is the main leading factor for hospitalization. The aim of this study was to determine the frequency and main clinical manifestation of the tuberous sclerosis complex, sturge-weber syndrome and neurofibromatosis.METHODS: This cross-sectional study was performed on the hospital dossier of the patients admitted in clinic and ward of neurology of Amirkola children hospital of Babol between years of 2001-2007. After achieving of the number of total admitted patients, we studied patients hospital dossier and registered their information in a data sheet.FINDINGS: Of 2564 admitted patients, 23 patients (0.9%) had TSC disease. The age of these patients was between 1-17 years old and all of them suffer from seizure and also had hypopigmented patch in their skin, 3 patients (13.4%) had heart disease, 6 patients (26.8%) had kidney disease and 8 patients (34.8%) had retinal involvement. Of 2564 admitted patients, 11 patients (0.4%) had SWS disease. All of them suffer from seizure and also had Port Wine Stain nevus in the distribution of frontal branch of fifth cerebral nerve. Eye disease was seen in 4 patients (36.4%). From all admitted patients, 6 patients (0.23%) had NF disease. One of them suffers from seizure and all of them had Cafe-au-Iait spots in their skin. Two patients had lisch nodule in their iris and one patient had retinal hamartoma. CONCLUSION: The results of this study showed that one of the most common clinical manifestations of these diseases was seizure. Consequently the 100% prevalence of seizure can be due to a systematic error such as selection bias.
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