Volume 9, Number 1 (Scientific Journal of Hamadan University of Medical Sciences-Spring 2002)                   Sci J Hamadan Univ Med Sci 2002, 9(1): 0-0 | Back to browse issues page


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Taghdiri M M, Ghofrani M, Ashrafzadeh F. Report of 40 Cases of Tuberous Sclerosis in Tehran Mofid Children’s Hospital. Sci J Hamadan Univ Med Sci . 2002; 9 (1)
URL: http://sjh.umsha.ac.ir/article-1-760-en.html

Abstract:   (572 Views)

          Tuberous Sclerosis (T.S) , an autosomal dominant disorder is one of the 
   major neurocutanous diseases with the involvement of many other organs .

          In this retrospective cross sectional study, 40 cases of T.S  have been
   followed up in the department of pediatric neurology in Tehran Mofid
   children’s hospital between, 1997 to 2000 (20 males and 20 females). All
   patients suffered from seizure disorder and upon admission had extensive
   paraclinical investigations including biochemical, electorgraphic and
   neuroimaging, studies. In view of ophthalmic and cardiac involvement in a
   remarkable portion of T.S patients, we consulted cardiologists and
   ophthalmologists .

          In this report , neurologic, ophthalmic, cardiac and skin manifestations
   of T.S mental retardation and seizure disorder which are the most common
   neuroclinical symptomatologies have been described. Also in the present
   article, the varieties of seizures and EEG patterns in these patients have
   been discussed. All T.S patients had hypopigmentation areas. Of these 37
   had preventricular Calcification , 30 were diagnosed having subnormal
   mentation , 8 suffered from aterial rhabdomyoma , 7 adenoma sebaceus , 5
   shagreen patch , 5 retinal hammartoma and 3 had brain tumor.

          The combination of depigmented areas of skin, siezure disorder and
   delayed development are diagnostic for T.S.

     
Type of Study: Case Report | Subject: Special

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