Hemicrania continua in carotid artery dissection – symptomatic cases or linked pathophysiology?

Abstract

Background

Hemicrania continua (HC) -like headaches have been rarely reported as symptomatic headaches, including cases secondary to cervical artery dissection.

Case series

We present five cases of HC-like headaches following cervical artery dissection, in three cases with specific indomethacin response. In two cases, comorbidity of fibromuscular dysplasia (FMD) was noted.

Conclusion

Carotid artery dissection may result in an HC-like headache syndrome. A specific response to indomethacin does not rule out dissection as underlying pathology. Screening for extracranial manifestations of FMD should be considered, especially in middle-aged females.

Introduction

A clinical picture resembling hemicrania continua occurring after cervical/carotid artery dissection has rarely been reported in the literature (1,2). Here, we report a case series of patients who developed typical hemicrania continua-like headaches after carotid artery dissection and discuss the possible pathophysiological link. We also discuss the comorbidity of fibromuscular dysplasia (FMD) that is present in two cases.

Case reports

Case 1

A 50-year-old Caucasian female without a particular headache or other medical history experienced subacute onset of headaches over the span of a day, initially in the left ear and occiput, then behind her left eye. Her blood pressure was elevated to 162/95 mmHg. She was seen in an urgent care clinic, and a few days later by her internist; pain was attributed to otitis and hypertension, respectively. The headaches progressed but fluctuated in intensity. She underwent a magnetic resonance imaging (MRI) scan of the head, suggesting carotid artery dissection, and an MR angiography (MRA) of head and neck showed a left internal carotid artery (ICA) dissection from the bifurcation to the petrous segment (but otherwise normal vasculature); she was started on acetylsalicylic acid (ASA) 325 mg daily. About one month after headache onset she was seen by a neurologist; she had a normal neurological examination, and headaches were under fair control with gabapentin and non-steroidal anti-inflammatory drugs (NSAIDs) (opioids were neither effective nor well tolerated). She returned seven weeks after symptoms onset because her constant left sided headaches had worsened, and she complained of hypersensitivity of the scalp in the temporoparietal area. There was mild swelling of the inferior periorbital area and mild ptosis (increased lacrimation, rhinorrhea or other autonomic symptoms were denied). Her MRA seven weeks later showed interval healing of the left ICA dissection, with a minimal rim of residual hyperintensity but without compromise of the carotid lumen (and this finding was no longer present at a follow-up study in week 11). She was started on indomethacin up to a daily dose of 225 mg; she had complete relief with that dose when extended and fast release preparations were combined.

Due to her new diagnosis of arterial hypertension and the patient’s concern about having FMD (after speaking to a physician friend), she underwent a CT angiography of the abdomen and pelvis. This study was suggestive of FMD involving both renal arteries. Indomethacin was discontinued to minimize use of NSAIDs given the renal involvement, and ipsilateral headaches recurred. At one-year follow-up, the patient complained of less intense persisting headaches and fatigue, and she no longer plays tennis as she used to prior to her dissection.

Case 2

This case has already been published elsewhere (1). A 38-year-old man was referred by his general practitioner with a four-day history of right-sided temporo-orbital headache of moderate to severe intensity. He had no history of headache or other medical history. He had been treated with several NSAIDs without relief. During excruciating short severe pain attacks, the headache was accompanied by eyelid and temporal edema, ptosis, and miosis; neurological examination was otherwise normal. The headache responded completely to indomethacin 50 mg three times daily.

Ultrasound examination of the brain-supplying arteries revealed a distal dissection of the right ICA. MRI and MRA confirmed the diagnosis of ICA dissection with a string sign 1 cm above the carotid bifurcation. No infarction was detected on brain MRI. Transoesophageal echocardiography was normal. The patient was treated with full dose warfarin for half a year. At eight-week follow-up, indomethacin was slowly tapered and the patient remained headache free. Ultrasound demonstrated a patent ICA.

Case 3

A 44-year-old physician one morning noted Horner’s syndrome in the mirror. He at once presented to the neurological outpatient clinic, where an ultrasound examination of the brain-supplying arteries was performed, indicating an extracranial right carotid artery dissection without occlusion or stenosis of the vessel. MRI and MRA of head and neck confirmed the ultrasound diagnosis and did not reveal other vascular pathology. The patient was treated with warfarin and discharged home.

Two days later, the patient returned to the neurology outpatient clinic and complained of a continuous right-sided periorbital headache. The headache was dull and of moderate intensity, but at times the patient experienced episodic worsening of headaches accompanied by lacrimation and more pronounced signs of Horner’s syndrome, and a feeling of sweating in the right face. The clinical picture fulfilled the criteria for hemicrania continua (3). There was complete relief with indomethacin 200 mg per day; there was also improvement of the accompanying autonomic symptoms, including Horner’s syndrome. After three months on the same dose of indomethacin, the patient returned to clinic and was headache free; indomethacin was discontinued and the headache did not recur. MRI/MRA no longer showed evidence of carotid artery dissection.

Case 4

A 47-year-old right-handed female with a history of episodic migraine headaches without aura was noted to have a small pupil on the right side; she later also noted intermittent ipsilateral ptosis, especially when tired. The next day, she developed a sudden onset frontotemporal headache that felt very different from her typical migraine headaches. She went to an ophthalmologist who sent her to the emergency room, where she was found to have an extensive dissection of the right ICA extending from the C2/3 level into the skull base; further findings included irregularities of the residual lumen on both sides (Figure 1) and a 2 mm anterior communicating artery aneurysm. Based on these findings, a diagnosis of FMD was made. She underwent immediate successful angioplasty with four stents. The headaches were exclusively right-sided, either pressure-like or stabbing, worsening with exercise and accompanied by ptosis. They were constant for five weeks, and then became gradually intermittent and holocephalic; they had nearly resolved when she was first seen by a neurologist about six months after the onset of symptoms. Medications that were tried with modest relief included ibuprofen, acetaminophen and oxycodone. She later underwent, more than seven months after presentation, successful clipping of the 2 mm anterior communicating artery aneurysm.

Figure 1.

CT angiogram of both carotid arteries in the coronal plane (case 4). On the right, there is an internal carotid artery dissection beginning in the distal cervical segment (full arrow) with extension into the petrous segment. Also note the irregularities of the right and left internal carotid arteries (arrowheads).

Case 5

A 42-year-old male patient presented to the emergency room, complaining of left retroorbital headaches occurring for three weeks since he lifted a heavy weight. There was an underlying continuous headache radiating to the left temporal and parietal area with superimposed attacks that were stabbing in nature, moderate in intensity and alleviated by movement. The frequency of these attacks was about five to 10 times per day, with a duration of 30 seconds, and they could be triggered by forced looking to the left and could be stopped by intense squinting of the left eye. There was increased lacrimation during the attacks (but nausea, rhinorrhea, and conjunctival injection were denied). Neurological examination was normal, in particular without suggestion of Horner’s syndrome. CT angiography and MRI/MRA of the head revealed occlusion of the left ICA, with intramural hematoma indicating dissection (no abnormalities of the brain parenchyma were noted). The headache was relieved by steroids (intravenous methylprednisolone 250 mg per day for three days), with the occasional addition of acetaminophen. There was further improvement over the next four weeks, and subsequently the patient reported being headache free.

Discussion

We present five cases of hemicrania continua-like headaches, with three cases fulfilling all IHS criteria (3) apart from the time criterion (A), which is unlikely to be met in patients who are treated in a timely way and have an indomethacin response. Patients 4 and 5 were not exposed to indomethacin, as the headaches resolved either before being seen by a neurologist or because steroids were effective, respectively.

In a larger case series (4), it was noted that 68% of patients with dissection of the internal carotid artery had headaches related to dissection, although in 90% these lasted less than one and in 97% less than six weeks, while the cases presented here were, with the exception of cases 2 and 5, longer lasting; moreover, a specific response to indomethacin was noted in three of these cases.

Trigeminoautonomic cephalgias associated with cervical artery dissection have been reported in the literature previously, mostly cluster headache (5–9) and HC-like headaches in two individuals (1,2). These co-occurences, as well as the anatomy of the sympathetic supply to the pterygopalataine fossa (10) suggest that ICA dissection might contribute to trigeminal pain associated with autonomic features via the lesion of periarterial sympathetic fibres. This pathophysiology may also apply to the symptomatic HC reported in the context of an ICA aneurysm (11).

Another possible link could be the presence of FMD. Two of the presented patients were diagnosed with FMD after initial presentation, one due to abnormalities on non-invasive imaging of brain vessels (in addition to the symptomatic dissection) and the other due to subsequent screening of abdominal and renal arteries. FMD is known to be associated with both cervical artery dissection and with headaches, and headaches may be present even if there is no known involvement of brain-supplying arteries (12). FMD used to be considered a rare disorder, but imaging studies suggest incidental findings of FMD close to 6% in the renal and up to 3.2% in brain-supplying arteries, with many subjects being asymptomatic (12). The majority of headaches in FMD are rather of the recurrent type (13); in the context of carotid artery dissection associated with FMD, it may be necessary to separate new onset acute headaches from patients with longer histories of recurrent headaches. Given the observation that most patients with FMD are female (91%) and middle-aged (mean 52 years) (12), it may be rational to screen middle-aged women with cervical artery dissection for other manifestations of FMD, in addition to imaging of brain supplying arteries.

Clinical implications

A hemicrania continua-like syndrome can be a consequence of carotid artery dissection.

This may be the case even there is a specific response to indomethacin.

Fibromuscular dysplasia may be present even if the brain vasculature is normal (except for the dissection).