P2.L234: Migrainosus Misadventure, A Crisis in Disguise

Introduction: Migraines are the most frequent primary headache disorder in adolescents. We present a case of refractory status migrainosus with electrolyte abnormalities and after steroid treatment precipitating the discovery of a unique chronic condition.

Case Description: A 10 year old boy with history of ocular coloboma, migraines with aura, and cyclic vomiting presented after 9 weeks of intractable headaches and periodic abdominal pain and vomiting. He was previously admitted 2 weeks prior with stable head imaging and Neurology consultation. After 5 days of abortive medications for migraines, Nubain, and IV fluids, symptoms improved and he was discharged. He was seen by his outpatient Neurologist who continued triptans as needed.

With ongoing symptoms, he was readmitted with persistent 10 out of 10 headache, dehydration, and hyponatremia. Vitals signs were within normal range, though later notable for intermittent tachycardia. No focal neurologic deficits or meningismus were present, although generalized abdominal tenderness and tan skin color were noted. Head imaging was again unremarkable. He trialed rizatriptan, pulse dose steroids, and a bupivacaine occipital nerve block all with limited improvement.

Throughout the patient’s admission, he developed worsening headaches and vomiting. He developed orthostatic tachycardia and hypotension, as well as postural dizziness. Metabolic panel demonstrated persistent hyponatremia, elevated creatinine, and transaminitis. Lipase was normal. Urine protein-to-creatinine ratio was unremarkable. Given electrolyte abnormalities unresponsive to IV fluid correction, further testing was pursued. TSH was 6.13 (range, 0.5-5.0) with normal thyroxine levels, Hemoglobin A1c was 5.9%, and fasting morning cortisol was low at 1.3 mcg/dL (range, 3.0 -27.0). Low and high-dose ACTH stimulation tests were performed, to which cortisol did not respond. Brain MRI was unremarkable for pituitary lesions.

Discussion: The lack of cortisol response to cosyntropin stimulation with 21-hydroxylase autoantibodies indicated primary adrenal insufficiency, and in conjunction with his other endocrine abnormalities, raised concern for Polyglandular Autoimmune Syndrome. Throughout his admission he developed hyponatremia, hyperkalemia, orthostatic vital sign instability, weight loss, and polydipsia and polyuria consistent with some presentations of adrenal crisis. It is important to note that this case did not fit a classic illness script for migraines or abdominal migraines and was refractory to routine management. His vital sign instability and electrolyte abnormalities called attention to the need for further testing.

Conclusion: We present this case to identify a unique and early pediatric presentation of Polyglandular Autoimmune Syndrome. While headache is a common presenting complaint for pediatric healthcare encounters, normal imaging, lack of symptomatic relief with routine abortive medications, systemic symptoms, and persistent electrolyte changes should prompt pediatric providers to broaden their differential to include adrenal and endocrine pathologies.