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Grand Rounds and Teaching Files

Title :
Early morning seizures

Author Details :
Ira ShahMedical Sciences Department, Pediatric Oncall, Mumbai Address for Correspondence: Dr Ira Shah, Medical Sciences Department, Pediatric Oncall, 1, B Saguna, 271, B St. Francis Road, Vile Parle {W}, Mumbai 400056.

Clinical Problem :
A 3½ years old boy born of non consanguineous marriage presented with 3 episodes of hypoglycemia. First episode of hypoglycemia was at 2½ years of age with early morning seizure. Second episode was 20 days ago with early morning lethargy. Third episode was 10 days ago with early morning seizure. Parents have also noticed progressive darkening of skin since 1½ year. Child also has craving for extra salt in feeds.

Question :
What is the diagnosis__?

Expert Opinion :
This child has early morning seizures which is classical of hypoglycemia. Hypoglycemia in a 3½ years old boy can be due to a variety of reasons such as increased insulin secretion {insulinomas}, problems in glyconeogenesis {as in glycogen storage disease, fatty acid oxidation defects, galactosemia and liver disease} or problems in glucose formation {as with hormonal and endocrine disorders such as deficiency of cortisol and epinephrine}. In this child, there is progressive darkening of skin suggestive of adrenal insufficiency or Addison’s disease. Also, salt is craved for suggestive of mineralocorticoid deficiency also. Thus, the child has glucocorticoid as well as mineralocorticoid deficiency suggestive of decreased cortisol activity. This could be due to involvement of adrenals or the pituitary {decreased ACTH}. In this child serum cortisol was low and ACTH was very high suggestive of primary adrenal insufficiency. CT of adrenals showed calcification on the left side. E-published: August 2009 Vol 6 Issue 8 Art No. 45