Recurrent Episodes of Adrenal Crises in an Infant during ACTH Treatment for Infantile Spasms

Background: West syndrome is an infantile epileptic disorder characterized by the triad of infantile spasms, neurodevelopmental delay, and hypsarrhythmia, which responds well to ACTH treatment.

Case presentation: A 5-month-old infant with infantile spasms treated with adrenocorticotropic hormone (ACTH) during dose tapering presented with anorexia and restlessness 5 days following the last treatment
On admission, his vital signs were: BP of 107/68mmHg, HR 200bpm, temperature 37.6°c, weight loss of 7% within 5 days.
Initial examination was unremarkable apart from cushinoid appearance and minor signs of dehydration. Blood tests: Na-127 ,K-5.2 , glucose-159. Cortisol level was 2216 nmol/L.
A diagnosis of adrenal crisis was suspected and 25 mg hydrocortisone was given with rapid improvement. 2 hours later he started to eat, slept well and sodium increased to 131 mmol/L ,potassium 4.5 mmol/L , glucose 170 mg/dL.
One month later, he presented again with similar complaints 5 days after his ACTH treatment. Na-117, K-5.9, and rapid response to hydrocortisone with Na increase and improvement in well being.

Conclusion: Chronic ACTH therapy may cause adrenal suppression and cortisol levels may be falsely elevated given the stimulatory effect ACTH has on the adrenals. Nevertheless, the clinical and biochemical picture is consistent with adrenal crises, as is the response to hydrocortisone. Data on adrenal function during ACTH protocol for infantile spasm are limited and no reports of adrenal crises during this treatment were found. Given the seriousness of adrenal crisis, patients should be informed of this complication and careful monitoring is essential.