Posterior Reversible Encephalopathy Syndrome in the Context of McCune-Albright Syndrome: A Case Report

Posterior reversible encephalopathy syndrome (PRES) is a clinical manifestation of various underlying causes, characterized by the combination of clinical and imaging findings associated with the posterior cerebral areas and relating to arterial hypertension and endothelial dysfunction. No association was made so far between PRES and McCune-Albright syndrome (MAS), a rare genetic disorder resulting in fibrous dysplasia. A 33-year-old female with MAS was presented to the emergency department of the 417 Army Share Fund Hospital in Athens (Greece) after seizure activity with two episodes of ocular upward deviation and transient facial palsy, each lasting a few minutes, followed by a postictal phase. On arrival, vital sign measurements revealed sinus tachycardia and elevated systolic and diastolic blood pressure (177/102 mm Hg). A neurologic examination demonstrated irritability and confusion, complete blindness from the left eye, and progressive visual loss from the right due to compression of optic nerves by fibro-osseous tissue bilaterally. Neuroimaging revealed the symmetrical presence of signs of vasogenic edema and dysfunction of the posterior parts of the cerebral hemispheres and, in conjunction with the clinical manifestations, advocated the diagnosis of PRES. The patient was treated with systematic administration of anticonvulsants, antihypertensive agents, and nebivolol. Laboratory examination indicated the presence of Cushing syndrome. The patient was discharged afebrile, hemodynamically stable, and clinically improved. MAS covers a spectrum of endocrine dysregulation, resulting in clinical manifestations of high variability. Even in cases of mild hypercortisolemia, it is vital to raise the clinical suspicion of CS, as its reverberations may occur with abrupt onset, like PRES.