Summary:
A young boy of 18 years was admitted at department of
Neurology, Dhaka Medical College Hospital with the
complaints of progressive generalized hyper-pigmentation,
gradual loss of vision, hearing impairment, abnormal
behaviors and one episode of seizure. Examination finding
revealed, abnormal behaviors, generalized hyper
pigmentation of skin, oral mucosa, gum, tongue and palmer
creases. He has diffuse hair loss, bilateral primary optic
atrophy, bilateral sensoryneural deafness. All routine
investigations revealed normal findings except, CSF protein
were elevated, biochemical features (very high ACTH, low
basal cortisol) of primary adrenal failure, Magnetic resonance
imaging (MRI) of the head showed bilateral symmetrical