Catecholamine-induced cerebral vasospasm and multifocal infarctions in pheochromocytoma

Summary: We report the case of a 76-year-old male with a remote history of papillary thyroid cancer who developed severe paroxysmal headaches in the setting of episodic hypertension. Brain imaging revealed multiple lesions, initially of inconclusive etiology, but suspicious for metastatic foci. A search for the primary malignancy revealed an adrenal tumor, and biochemical testing confirmed the diagnosis of a norepinephrine-secreting pheochromocytoma. Serial imaging demonstrated multiple cerebral infarctions of varying ages, evidence of vessel narrowing and irregularities in the anterior and posterior circulations, and hypoperfusion in watershed areas. An exhaustive work-up for other etiologies of stroke including thromboembolic causes or vasculitis was unremarkable. There was resolution of symptoms, absence of new infarctions, and improvement in vessel caliber after adequate alpha-adrenergic receptor blockade for the management of pheochromocytoma. This clinicoradiologic constellation of findings suggested that the etiology of the multiple infarctions was reversible cerebral vasoconstriction syndrome (RCVS). Pheochromocytoma remains a poorly recognized cause of RCVS. Unexplained multifocal cerebral infarctions in the setting of severe hypertension should prompt the consideration of a vasoactive tumor as the driver of cerebrovascular dysfunction. A missed or delayed diagnosis has the potential for serious neurologic morbidity for an otherwise treatable condition.

Learning points: The constellation of multifocal watershed cerebral infarctions of uncertain etiology in a patient with malignant hypertension should trigger the consideration of undiagnosed catecholamine secreting tumors, such as pheochromocytomas and paragangliomas. Reversible cerebral vasoconstriction syndrome is a serious but reversible cerebrovascular manifestation of pheochromocytomas that may lead to strokes (ischemic and hemorrhagic), seizures, and cerebral edema. Alpha-adrenergic receptor blockade can reverse cerebral vasoconstriction and prevent further cerebral ischemia and infarctions. Early diagnosis of catecholamine secreting tumors has the potential for reducing neurologic morbidity and mortality in patients presenting with cerebrovascular complications.

Keywords: 2020; ACTH; Adrenal; Adult; Alpha-blockers; Androstenedione; Angiography; August; Beta-blockers; Blood pressure; CT scan; Chromogranin A; Cortisol; Diltiazem; Echocardiogram; Epinephrine; Haemoglobin A1c; Headache; Hydrochlorothiazide; Hypertension; Lymph node dissection; MIBG scan; MRI; Male; Metoprolol; Nausea; Neurology; Noradrenaline; Norepinephrine; Normetanephrine; Papillary thyroid cancer; Phaeochromocytoma; Phenoxybenzamine; Radioiodine; Radiotherapy; Resection of tumour; Sleep hyperhidrosis; Syncope; Thyroidectomy; Triamterene; Troponin; Ultrasound scan; Unique/unexpected symptoms or presentations of a disease; United States; Vanillylmandelic acid (24-hour urine); Vomiting; White.