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.
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