Plasma prostacyclin from birth to adolescence.

摘要

<h3>Objective:</h3> Illustrate a case of pheochromocytoma presenting with recurrent thunderclap headache and segmental cerebral vasoconstriction that resolves after surgical resection. <h3>Background:</h3> Pheochromocytoma is a rare but curable cause for recurrent thunderclap headache and cerebral vasoconstriction. There are limited cases in the literature highlighting the severe neurologic complications associated with pheochromocytoma. <h3>Design/Methods:</h3> NA <h3>Results:</h3> <h3>CASE:</h3> A 31-year-old postpartum woman (G1P1) with history of gestational diabetes and preeclampsia developed recurrent thunderclap headaches in the peripartum period. Vital signs were notable for systolic blood pressures in the 140s. Neurologic examination was nonfocal. Initial CT head, CT angiography, and CT venography was unremarkable. Her symptoms were controlled with oral and intravenous analgesics. Unfortunately, her headaches intensified following discharge. Therefore, she underwent brain MRI and MR angiography, which revealed multiple foci of intracranial vascular narrowing. Lumbar puncture was pursued, which demonstrated: total nucleated cells, 1 cell/mcL; glucose, 65 mg/dL; protein, 85 mg/dL; and 0 oligoclonal bands. Upon further questioning, the patient endorsed similar paroxysmal headaches one year earlier. Therefore, a 24-hour urine metanephrine study was obtained, revealing markedly elevated urine metanephrines. Subsequent CT abdomen/pelvis exposed a 3.5 cm complex solid and cystic mass in the left adrenal gland, consistent with a pheochromocytoma. She was referred to General Surgery and underwent a robotic-assisted left adrenalectomy. Pathology confirmed diagnosis of pheochromocytoma. Patient exhibited complete headache resolution following surgery. Follow-up MR angiography one-week later revealed interval resolution of the vascular narrowing. <h3>Conclusions:</h3> Recurrent thunderclap headache in a young person should prompt consideration of reversible cerebral vasoconstriction syndrome; however, this diagnosis implies a monophasic course with complete or substantial normalization of cerebral arteries on angiography within 12 weeks of symptom-onset. This case highlights a potential neurologic complication associated with pheochromocytoma and should serve to instruct clinicians to search for other mechanisms of cerebral vasoconstriction in atypical cases. <b>Disclosure:</b> Dr. English has nothing to disclose. Dr. Nasr has nothing to disclose.