Adrenal Dysfunction Presenting as Severe Fasting Hypoglycemia with Reversible Splenial Lesion Syndrome in a Young Iraqi Immigrant Woman

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: SUN 1-16-Adrenal Insufficiency
Clinical
Sunday, June 16, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board SUN-13
Tim Arakawa*, Navneet Mangat and Devjit Tripathy
University of Texas Health Science Center at San Antonio, San Antonio, TX
Introduction:  Severe hypoglycemia as a presenting feature is usually seen in newborns and young children with isolated adrenocorticotropic hormone (ACTH) deficiency (IAD). However, this condition is extremely rare in adults with IAD. We describe an uncommon presentation of IAD in a young adult woman with a reversible splenial lesion.

Clinical Case: A 23-year-old Iraqi female immigrating to the United States for political asylum began experiencing nausea, vomiting, hypotension, and altered mental status. Within 4 days, she presented to the emergency department with loss of consciousness and serum glucose of 16 mg/dL (60-100 mg/dL). On admission, she exhibited signs of severe sepsis but stabilized after aggressive fluid resuscitation. No vasopressors were required. Over the course of her hospital stay, she had persistent nausea and vomiting and was unable to tolerate a diet. By day 8, she began to have fasting hypoglycemia with serum glucose values as low as 35 mg/dL. A fasting insulin level drawn with a serum glucose of 63 mg/dL was <0.5 mU/I (3-28 mU/I) with C-peptide of <0.1 ng/mL (0.9-7.1 ng/mL) and proinsulin of 7.3 pmol/L (≤26.8 pmol/L). Work-up for other causes of hypoglycemia was negative. ACTH stimulation test showed an initial cortisol of 0.2 mcg/dL (4-25 mcg/dL) with 30 and 60 minute levels of 1.5 and 1.7 mcg/dL, respectively. ACTH level was 13 pg/mL (6-58 pg/mL) with a repeat level of 9 pg/mL. All other pituitary hormones were normal. Furthermore, she endorsed regular menstrual periods since menarche and had never been on chronic glucocorticoids. MRI imaging of her sella did not reveal any pituitary abnormalities. However, she did exhibit a lesion in the splenium of her corpus callosum. Supplementation with hydrocortisone was followed by rapid clinical improvement with weight gain and resolution of hypoglycemia. Repeat imaging 6 months later showed resolution of the splenial lesion consistent with reversible splenial lesion syndrome (RESLES). Her adrenal crisis was attributed to the acute stress of emigration in the context of long-standing IAD.

Conclusion: Long-standing IAD can present as hypoglycemia during acute stress in young adults without previous history of adrenal crisis. The severe hypoglycemia associated with adrenal insufficiency can lead to splenial lesions characteristic of RESLES which are reversible.

Nothing to Disclose: TA, NM, DT

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