Coughing into the darkness

Abstract

In May, 2004, a 44-year-old man presented to his primarycare physician having had severe left-sided headaches for 4 weeks. The headaches lasted several hours and occurred two or three times a week. The patient was a physician and had no history of substance misuse or prolonged headaches. Several weeks before the onset of headaches he had had acute bronchitis with a severe non-productive cough. He also had a recent history of vertigo, hemianopsia, and scintillating scotoma. Physical examination was normal. His weight was 60 kg. He had a history of neurodermitis and allergic asthma, which required occasional inhaled β2-adrenergic receptor agonists. Further neurological examination including an electroencephalogram (EEG) was normal. The neuro logist’s diagnosis was migraine with aura. Our patient’s headaches persisted for 3 months and terminated spontaneously. In January, 2005, he presented to our department with severe headaches, amaurosis fugax in the left eye, scintillating scotoma, vertigo, and severe dizziness. Again, several weeks earlier, he had had acute bronchitis with a non-productive cough. His temperature, heart rate, and blood pressure were normal. Examination of the heart, lungs, and abdomen and results of laboratory tests were normal. Magnetic resonance angiography (MRA) of the brain was done, to exclude life-threatening diagnoses such as a brain aneurysm or haemorrhage, and showed an acute 2 cm long dissection of the right internal carotid artery starting at the skull base. The resulting stenosis was of high degree (>90%) and haemodynamically signifi cant (fi gure). The MRA also showed a fetal type of arterial supply of the right posterior cerebral artery. Fractionated heparin (body-weight-adapted: fraxiparin) twice daily for 14 days and overlapping therapy with phenprocoumon was started. The headaches stopped. A second MRA, 6 months later, showed complete resolution of the stenosis. Phenprocoumon was replaced by daily aspirin. 2 years later in April, 2007, our patient presented again to us with severe headaches, the same neurological symptoms, and a preceding history of severe bronchitis. Brain MRA showed a dissection of the left internal carotid artery at the craniocervical transition with 40% stenosis. Heparin followed by phenprocoumon was initiated. A control MRA 9 months later was normal, and his treatment was switched to daily aspirin. 24 h blood pressure recording and exercise ECG showed normal cardiovascular responses. Nuclear autoantibodies, C-reactive protein, and blood sedimentation rate were normal, excluding vasculitis or collagenosis. Morbus-Fabry disease could be excluded by normal activity of alpha-galactosidase A. We then learnt that our patient played the trumpet as a hobby. We advised him to refrain from this because blowing the trumpet and carotid dissections might be related. In August, 2010, at last follow-up, our patient continued to be asymptomatic and was fully integrated back into his professional and private life. Spontaneous dissections of cerebral arteries are rare events (2–3 per 100 000). The rate of re-dissections is even lower (<1%) and they typically aff ect the contralateral side. Remarkably, our patient reported severe bronchitis with coughing attacks preceding the dissections. Several studies have reported an association between cold, coughing, and carotid dissections. Furthermore, a relation between cerebrovascular events and playing wind instruments has been reported. Duplex sonography is a suitable method for the initial assessment and follow-up of dissections of carotid arteries. Our patient’s case should encourage the thorough investi gation of severe headaches associated with neurological symptoms, particularly in regard to the cerebral vasculature.