Spot diagnosis - Identify the syndrome

Discussion in 'Spot Diagnosis' started by neo_star, Dec 30, 2012.

  1. neo_star

    neo_star Moderator

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    identifysynd_zps985b7f3e.jpg

    Identify the syndrome

    Hint : the MRI shows a tumor in the hypothalamic area and this syndrome is more common in boys.
    : Also frequently associated with Slipped Capital Femoral Epiphysis
     

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    Last edited: Dec 30, 2012

  2. neo_star

    neo_star Moderator

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    Ans: Adiposogenital dystrophy aka Babinski-Fröhlich syndrome aka Frölich's Syndrome aka Hypothalamic Infantilism-Obesity

    Discussion

    Adiposogenital dystrophy is a condition which may be caused by secondary hypogonadism originating from decreased levels in GnRH. Low levels of GnRH has been associated with defects of the feeding centers of the hypothalamus, leading to an increase consumption of food and thus caloric intake.

    Presentation


    It is characterized by:
    1) Feminine obesit
    2) Growth retardation and retarded sexual development, atrophy or hypoplasia of the gonads, and altered secondary sex characteristics,
    3) headaches
    4) Problems with vision
    5) polyuria, polydipsia


    It is usually associated with tumours of the hypothalamus, causing increased appetite and depressed secretion of gonadotropin. It seems to affect males mostly.

    Many overweight children may appear to have the disorder because of the concurrence of obesity and retarded sexual development; these children have no endocrine disturbances, however, and they mature normally after delayed puberty.

    ref - http://en.wikipedia.org/wiki/Adiposogenital_dystrophy
     

    Last edited: Jan 8, 2013

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