Cystic Fibrosis May Be a Risk Factor for Hemiplegic Migraine and Stroke

Cystic fibrosis CAT scan
Cystic fibrosis CAT scan
Due to brain excitability and hypoxia from coughing, patients with cystic fibrosis have a propensity for cortical spreading depression and hemiplegic migraine.

Cystic fibrosis may be a risk factor in the occurrence of cortical spreading depression, which is associated with auras and hemiplegic migraine attacks. Patients with both cystic fibrosis and hemiplegic migraine risk developing migrainous infarction, according to a case report published in Headache.

The investigators of this report sought to understand the relationship between hemiplegic migraine and cystic fibrosis presented in the case of a 32-year-old white man.

The patient was diagnosed with cystic fibrosis at birth, which progressed into chronic obstructive pulmonary disease (COPD) and pulmonary hypertension and later developed diabetes. In 2012, the patient began experiencing transient episodes triggered by bouts of coughing and characterized by symptoms (succession of phosphenes, one-sided paresthesia, motor aphasia, and homolateral hemiparesis) affecting only the right half of his body and concomitant migraine headaches.

In April 2017, the patient experienced a longer than usual bout of coughing from aggravated COPD accompanied by a migraine attack with aura. The patient experienced a 15-minute loss of consciousness and was treated with oxygen therapy. An MRI using diffusion-weighted imaging revealed a wide area of diffusion restriction around the left occipital-parietal frontal regions, which was reversed 10 days later except for a lesion in the primary motor area. The stroke workup results were negative.

The patient was diagnosed with hemiplegic migraine 2 months later with unremarkable diagnostic findings: neurological examination only revealed mild right-side hyperesthesia and mild weakness with increased homolateral reflexes, and genetic screening showed no familial hemiplegic migraine mutations. The discrepancy in volume between the altered area shown in the diffusion-weighted imaging and the final lesion led the patient to undergo brain perfusion single photon emission computed tomography (BP-SPECT).

Related Articles

The BP-SPECT revealed significant permanent hypoperfusion in the left occipital-parietal frontal regions involved in the prolonged aura, indicating neuronal damage greater than the visual lesion. This finding is compatible with cortical spreading depression and further supports the evidence that the stroke was due to migrainous infarction, which involves the brain regions responsible for aura symptoms. The investigators suggest that mild hypoxia, as a consequence of coughing bouts from COPD, facilitates cortical spreading depression and that extended periods of hypoxia may lead to migrainous infarction.

Patients with cystic fibrosis may be more vulnerable to cortical spreading depression and therefore may be considered at higher risk for experiencing hemiplegic migraine attacks with aura; patients with both cystic fibrosis and hemiplegic migraine have a greater chance of developing migrainous infarction.


Mancini V, Mastria G, Frantellizzi V, et al. Migrainous infarction in a patient with sporadic hemiplegic migraine and cystic fibrosis: A 99mTc-HMPAO brain SPECT study [published online January 8, 2019]. Headache. doi: 10.1111/head.13472