Acute neurological deficits in a young adult with cystic fibrosis

An abrupt onset of a neurological deficit is a rare occurrence in patients with cystic fibrosis (CF). As many CF patients have indwelling intravenous catheters, one of the complications may be deep venous thrombosis. Cerebral thromboembolism through an intracardiac shunt should be considered in CF patients who develop unexplained acute neurological deficits. We report on the case of a 19-year-old CF patient with insulin-dependent diabetes mellitus who was on oral contraceptives and had a Port-A-Cath® in place. The patient developed an acute neurological deficit after pulmonary function testing. Radiologic investigations of her head and neck were unremarkable, except for bilateral maxillary and ethmoid sinusitis. An electroencephalogram showed epileptiform discharges primarily from the right hemisphere. A transthoracic echocardiogram (TTE) revealed a small thrombus in the right atrium. A transesophageal echocardiogram (TEE) demonstrated a left-to-right shunt through a patent foramen ovale (PFO) that was not found by TTE. Extensive investigation to rule out congenital and acquired thrombophilia was negative. Treatment consisted of aspirin and discontinuation of oral contraceptives and vitamin K supplementation. Spontaneous complete recovery of the neurological deficits occurred within 24 hr after onset of symptoms. We conclude that paradoxical embolism should be in the differential diagnoses of CF patients who have indwelling intravenous catheters and who develop an unexplained stroke. An extensive investigation to rule out intracardiac abnormalities and thrombophilia should be considered. The risks and benefits of PFO closure vs. prophylactic anticoagulant and antiplatelet aggregation treatment in this group of patients should be carefully weighed.