The coughing paroxysms of patients with
cystic fibrosis may occasion neurological symptoms. Although
cough syncope is well-known, and is associated with
headache and
paralysis, a migrainous mechanism has not been reported. We reviewed the medical records, autonomic testing results, and responses to treatment in two
cystic fibrosis patients with similar presentations of
cough-induced impairment of consciousness followed by
headache and
paralysis. A 24-year-old woman and an unrelated 38-year-old man, both with
cystic fibrosis, developed post-tussive
neurologic deficits. Both patients reported infrequent dramatic spells, always preceded by major
hemoptysis, and associated with left-sided
paralysis,
transient blindness,
nausea, and severe pulsating
headaches. Autonomic testing demonstrated only postural
tachycardia and a near-vasodepressor episode in the woman, and mild, generalized sympathetic dysfunction in the man. Treatment for presumptive
migraine with aura with
verapamil nearly eradicated symptoms in both patients. Discontinuation of
verapamil in the woman was associated with symptom recurrence and a
stroke, with significant persistent residual left
hemiparesis. In conclusion,
cough-induced
neurologic deficits were previously reported with
cystic fibrosis, without clear understanding of the mechanism of impairment of consciousness. Based on the
hemiparesis,
nausea, and
throbbing headache, which repeatedly followed the events in both patients, and based on the response to
verapamil, we hypothesize a migrainous mechanism in both of our patients. The pathophysiology that links the
hemoptysis to the spells deserves further investigation.