TAY'S SYNDROME IN TWO SISTERS

Objectives: Tay's syndrome is a trichothiodystrophy associated with congenital ichthyosis, short stature, delayed physical and mental development and pyramidal tract signs with increase in muscular tone and brisk tendon reflexes. It is an autosomal recessive disorder characterised by sulphur-deficient brittle hair with disturbed synthesis of high- sulphur matrix proteins. Our patients represent with abnormalities of the nails and teeth, growth and mental redardation, congenital ichthyosis, brittle hair and progeria-like facies.

Methods: We report two sisters with Tay's syndrome whose are seven and three years old.

Results: Our patients represent with abnormalities of the nails and teeth, growth and mental redardation, congenital ichthyosis, brittle hair and progeria-like facies. We report two sisters of this rare syndrome with the finding on MRI.

Conclusion: T2-weighted images showed prominent diffuse confluent increase in signal symmetrically in all the corpus callosum and periventriculer white matter the findings on MRI.