Thunderclap headache as the main symptom of an acute aortic dissection type A

Abstract

A 67-year-old Caucasian woman presented to our emergency department after the sudden onset of thunderclap headache. The pain was localized ubiquitously in the whole head with a maximum in the visceral cranium and described by the patient as of a knife-stabbing character. A loss of consciousness did not occur. The patient reported on a short-lasting episode of mainly motor aphasia immediately after the onset of the headache. The patient had no history of migraine or other forms of headache and was not taking any medication. The neurological and physical examination was unremarkable. A computer tomography (CT) of the brain showed no signs of intracerebral haemorrhage. A lumbar puncture was declined by the patient. We decided to conduct a CTangiography to detect a possible aneurysm of the intracranial vessels. The CT-examination even included a part of the ascending aorta and the aortic arch to detect a possible dissection of the proximal vertebral arteries. The CT showed a Stanford type A acute aortic dissection (Fig. 1). Furthermore the CT also showed an affection of the proximal part of the supra-aortic vessels (Fig. 2). Confronted with the radiological diagnosis, the patient reported on an aortic aneurysm which had been diagnosed approximately 6 months before. The patient did not show any of the typical symptoms or complications of an acute aortic dissection type A such as pain between the scapulae, the chest, the throat, the abdomen, dyspnoea, heart rate deficits, hypotension and shock, seizures or paresis in the clinical course at all. Subsequent cardiac surgery was conducted successfully. The patient survived without any impairment. Stanford type A acute aortic dissection presenting with headache has been reported before [1–3]. In contrast to the cases published before, headache was, apart from a doubtful short episode of motor aphasia, the only symptom of a Stanford type A acute aortic dissection in the present case. Differential diagnostic causes for the occurrence of thunderclap headache include subarachnoidal haemorrhage, intracerebral haemorrhage, reversible cerebral vasoconstriction syndrome, cerebral venous sinus thrombosis, and dissections of the extracranial vessels [4–6]. Though lacking the examination of cerebrospinal fluid, a subarachnoidal haemorrhage as the underlying cause of the thunderclap headache is not likely regarding the clinical course. Cerebral angiography as well as a doppler and duplex sonographical examination showed no signs of reversible cerebral vasoconstriction syndrome or cerebral venous sinus thrombosis. The clinical course with no other occurrence of thunderclap headache during almost a year after the present incident argues that a reversible cerebral vasoconstriction is also unlikely. A dissection of the proximal extracranial arteries accompanying the aortic dissection could be shown via CT-angiography. A possible explanation of the pain sensation might be the mechanical irritation or vasa nervorum occlusion of the cardiac plexus and the internal carotid plexus with its sympathetic nerve tract projection to the ciliary ganglion and pterygopalatine ganglion. The absence of any cardiovascular reflexes or cardiac pain during clinical observation might be due to the fact H. Rust (&) H. Kimmig Clinic of Neurology, Schwarzwald Baar Klinikum, Rontgenstr. 20, 78054 Villingen-Schwenningen, Germany e-mail: heiko.rust@googlemail.com