Describe a case of episodic lingual Raynaud’s phenomenon with presumed multifocal vasospasm and related focal neurologic deficits in a patient with longstanding cystic fibrosis.

Cystic fibrosis has widespread effects on multiple sites and organ systems, including transient neurologic dysfunction during coughing paroxysms; these are typically non-focal symptoms like dizziness, syncope, and headache. Raynaud’s phenomenon, in general or of the tongue, also does not have an established association with cystic fibrosis in the literature. In addition, there are no available case reports of lingual dysarthria associated with multifocal transient neurologic deficits.

Clinical and video findings are presented in a woman with cystic fibrosis demonstrating lingual Raynaud’s and episodic focal neurologic deficits.

The patient was a 33 year-old female with cystic fibrosis complicated by multidrug resistant Pseudomonas aeruginosa colonization. She experienced five years of episodes up to multiple times per week characterized as right arm greater than leg diffuse paresthesias. Episodes worsened in setting of recurrent pneumonia, which triggered associated right-sided tongue paresthesias and color change to white followed by purple-blue respecting the median. Other associated symptoms could include right arm weakness, “kaleidoscoping” of the vision, left leg numbness, and crushing substernal chest pressure. Events were often triggered by severe coughing fits during illness. Workup was unremarkable including magnetic resonance angiography, magnetic resonance imaging of the brain, electrocardiograms, and cardiac enzymes. Patient had a potential response to fluoxetine with resolution of symptoms while on medication. Less than two months after discontinuation of fluoxetine, she passed away from cardiac arrest.

This case demonstrates video and photographic evidence of a correlation between cystic fibrosis, lingual Raynaud's phenomenon, and likely multifocal vasospasm with a potential response to fluoxetine prophylaxis.