Acute aortic dissection (AAD) is a serious life-threatening condition often resulting in death due to cardiac tamponade and aortic rupture (1, 2). Clinical manifestations of aortic dissection are highly variable, which can challenge the diagnosis (2, 3). Untreated, it has a mortality rate of 50% in the first 3 days and 80% during the first two weeks (4). It usually manifests as a sudden excruciating pain anterior in the chest, which can radiate to the back (5). 17%–40% of all cases of aortic dissection present with neurological symptoms despite being a rare cause of ischemic stroke (6, 7). This is especially important for neurologists, as patients with focal neurologic symptoms due to acute aortic dissections who receive thrombolysis with recombinant tissue plasminogen activator (r-TPA) have a 71% mortality rate (7, 8). Furthermore, it has been estimated that aortic dissections are unsuspected in approximately 15%-43% of cases upon admission to hospitals, which underlines the importance of a high level of suspicion in a clinical setting (7, 9).

In this study, we present the case of a 54-year-old man who presented with thunderclap headache and focal neurological deficits as the presenting symptoms of acute aortic dissection.

A 54-year-old man with no prior medical history, no use of medication, non-smoker, and no prior traumatic injuries in the days leading up to admission presented to the emergency department with a sudden thunderclap headache that originated in the neck and radiated to the top of the head. Within seconds, the headache became holocranial and was rated as 10 out of 10 on a numeric rating scale (NRS). This was followed by a sensation of pins and needles in his right arm, which spread to both legs, followed by weakness in both legs and his left arm. After a few minutes, the sensory-motor deficits on the right-sided extremities subsided, while symptoms on the left-sided extremities subsided after approximately 30 min. Before admission, the patient had regained normal strength in all four extremities. Upon admission, the patient was still complaining of holocranial headache, which was reduced to 3 out of 10 on a NRS, nausea without vomiting, nautical dizziness, photophobia, and slight discomfort in the abdomen. There were no visual symptoms, complaints of chest pain, back pain, or cardiopulmonary symptoms.

The neurological exam was unremarkable except for slight unsteadiness in the Romberg Test, and the patient had a National Institutes of Health Stroke Scale score of 0. The patient had no cranial nerve dysfunction, did not have neck stiffness, and had a Glasgow coma scale score of 15 points. There were no clinical findings suggestive of connective tissue disease, such as a marfanoid habitus.

Upon arrival, his blood pressure was 127/56 mmHg, pulse rate 80 beats per minute, oxygen saturation 97%, and temperature 36.6 degrees Celsius. Blood tests revealed normal white blood cell counts, C-reactive protein levels, and liver and kidney parameters. Electrocardiogram demonstrated sinus rhythm with premature atrial complexes and without ST-segment deviations.

Non-contrast-enhanced computed tomography (CT) of the cerebrum revealed no intracranial abnormalities, including hemorrhage or ischemic lesions. Subarachnoidal hemorrhage was still suspected due to the thunderclap nature of the headache despite the normal CT scan of the cerebrum, while transitory cerebral ischemia was also considered a differential diagnosis. CT angiography of the cerebrum did not reveal any vascular abnormalities. Due to complaints of slight abdominal discomfort shortly after admission alongside the severe headache, CT angiography of the thorax and abdomen in the context of differential diagnostic purposes on the suspicion of aortic dissection was done, revealing a Stanford type A aortic dissection without concomitant affection of the carotid or vertebral arteries (Figure 1). Subsequent transthoracic echocardiography also visualized the extent of the aortic dissection from the aortic annulus to the femoral artery.

Subsequently, the patient was transferred to the department of thoracic surgery, undergoing a surgical insertion of a freestyle prosthesis at the aortic root and arch. The postoperative phase was complicated with atrial fibrillation and pericardial exudate, treated with amiodarone and subxiphoid pericardiotomy, respectively. The patient was discharged after a few weeks with follow-up in the cardiology department.

Aortic dissection occurs because of a breach in the intima, penetrating the intima due to high blood pressure, causing blood to enter this space with further dissection propagation (10). Hypertension and atherosclerosis are the most common predisposing factors, while Marfan's syndrome, prior cardiac surgery, and aortic aneurysm are some of the other risk factors involved in developing aortic dissection (2, 10). The incidence is approximately 3 per 100.000 each year (11).

Aortic dissections are classified based on their anatomic location and time from onset, where Stanford type A dissections involve the ascending aorta and Stanford type B dissections occur distal to the left subclavian artery without involvement of the ascending aorta (2).

A systematic search of the literature in the PubMed database was performed in March 2025. We aimed to include all case reports with aortic dissection with headache as a presenting symptom, where the search term “aortic dissection AND headache” was applied. 405 studies were reviewed, of which 22 case reports were included (Table 1). One study was not included due to unavailable full text. Included cases were divided into Stanford type A (n = 19 case reports) (1, 7, 8, 12–25), Stanford type B aortic dissection (n = 2 case reports) (26, 27), and one case with a patient presenting with a Stanford type A dissection progressing into a Stanford type B (Figure 2) (28). The major risk factor (n = 11) was hypertension, and one case reports the underlying cause as Marfan syndrome (25), one case reported paraganglioma on the right side of the aorta on the level of the celiac trunk (22), and one case reported giant cell arteritis with the focus of inflammation being the aortic wall (20). Alongside headache as the initial symptom, nausea/vomiting (n = 9 case reports) and loss of consciousness (n = 6 case reports) were the most prevalent initial symptoms. CT of the cerebrum was done in 10 studies, of which two were abnormal; one case was with dissection of extracranial arteries (17) and one case with thrombus in the right middle cerebral artery (7). Two cases of Stanford type A aortic dissections were with feeble or not palpable pulses in the brachial or radial arteries in one arm (21, 24), and 3 cases (out of 11 reporting blood pressure) reported lateralized blood pressure difference of 20–66 mmHg (12, 19, 21), with one case reporting an unrecordable blood pressure in one arm (24).

Patients with aortic dissections typically present with acute onset severe chest pain (72.7%) or back pain (53.2%) (2). Anterior chest pain is usually observed in patients with Stanford type A aortic dissections, while type B dissection typically presents with pain in the back and abdomen (2). In this case report, the patient complained of mild abdominal pain. However, neurological symptoms such as headache, paraplegia, transitory cerebral ischemia, or stroke are seen in about a third of patients with aortic dissection (29). Headache as the initial manifestation of aortic dissection may be due to vessel wall distension, ischemia of the pericarotid plexus, or decreased cerebral perfusion due to antegrade flow and subclavian steal (1, 17, 19). A disruption of sympathetic projections could also be a cause of pain sensations due to the affection of the ciliary ganglion and pterygopalatine ganglion by mechanical irritation of or occlusion of the vasa nervorum of the cardiac plexus and the internal carotid plexus (17).

The abovementioned neurological manifestations can divert attention away from an underlying aortic dissection (3), contributing to the substantial percentage of up to 43% of cases missed upon initial clinical evaluation (7, 9). This can ultimately have devastating consequences for the patient as treatment with r-TPA due to the suspicion of stroke has shown to have a 71% mortality rate (7, 8).

Aortic dissection can diagnosed using CT angiography, magnetic resonance imaging, and transoesophageal echocardiography (2, 12), while chest x-ray showing a widening of the mediastinum can support the diagnosis (15). Normal echocardiography or chest x-ray findings do not exclude the possibility of aortic dissection (15).

This case report demonstrates a 54-year-old man presenting with thunderclap headache and focal neurological deficits as the initial manifestations of acute aortic dissection. These atypical manifestations can sometimes mimic neurological conditions, thus increasing the possibility of overlooking an aortic dissection.

Therefore, a high level of clinical suspicion for aortic dissection is warranted when patients present with a sudden severe headache accompanied by mild complaints of pain in the abdomen, chest, or back, nausea, and loss of consciousness. At the same time, asymmetry of pulse and blood pressure asymmetry of the arms should also raise suspicion. Furthermore, CT-angiography of the thorax and cerebrum is necessary to establish a timely diagnosis and subsequent treatment of aortic dissection.