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Internal Carotid Artery Dissection Presenting With Dysgeusia, Horner Syndrome, and Hypesthesia of the Fifth Cranial Nerve: A Case Report
The Journal of Emergency Medicine, Vol. -, No. -, pp. 1–3, 2019 Ó 2019 Elsevier Inc. All rights reserved. 0736-4679/$ - see front matter
https://doi.org/10.1016/j.jemermed.2019.09.035
Clinical Communications: Adult INTERNAL CAROTID ARTERY DISSECTION PRESENTING WITH DYSGEUSIA, HORNER SYNDROME, AND HYPESTHESIA OF THE FIFTH CRANIAL NERVE: A CASE REPORT Kirsten V. M. van der Zwet, MSC,* A. Vanessa Brown, MD,* and Stef L. M. Bakker, MD† *Department of Emergency Medicine, Franciscus Gasthuis & Vlietland, Rotterdam, the Netherlands and †Department of Neurology, Franciscus Gasthuis & Vlietland, Rotterdam, the Netherlands Corresponding Address: Kirsten V.M. van der Zwet, MSC, Department of Emergency Medicine, Franciscus Gasthuis & Vlietland, Kleiweg 500, Rotterdam 3045 PM, the Netherlands.
, Abstract—Background: Carotid artery dissection (CAD) is a significant cause of ischemic stroke. Early recognition and treatment of CAD is important to prevent accompanying cerebral ischemia. This case report presents an atypical clinical presentation of CAD and emphasizes the diagnostic challenge for emergency physicians. Case Report: A 54-year-old woman presented to the emergency department with a bilateral headache of 4 days’ duration, hypesthesia of the left fifth cranial nerve, dysgeusia, and partial Horner syndrome on the left side. Magnetic resonance angiography showed a left-sided CAD without any signs of cerebral ischemic events. Antiplatelet therapy with clopidogrel was started, and the patient did not show any deterioration in the weeks thereafter. Why Should an Emergency Physician be Aware of This?: CAD can present with different combinations of cranial nerve palsies and should be in the differential diagnosis of dysgeusia and hypesthesia of the trigeminal nerve. Early recognition of CAD can be challenging in patients with rare cranial nerve involvement, but early treatment is crucial to prevent cerebral ischemic events. Ó 2019 Elsevier Inc. All rights reserved.
INTRODUCTION Carotid artery dissection (CAD) is a significant cause of stroke, accounting for 2–3% of all strokes and #25% of strokes in young adults (1,2). The early recognition and treatment of CAD is important to prevent cerebral ischemia (3,4). Only 33% of all cases present with the classical triad of (unilateral) headache, Horner syndrome, and ischemic events, and therefore early recognition can be challenging (1). We present an atypical clinical presentation of CAD and emphasize the diagnostic challenge for emergency physicians. CASE REPORT A 54-year-old woman visited her general practitioner because of a headache of 4 days’ duration and an asymmetrical appearance of her face that she noticed a couple of hours before presentation. She also complained about a change in the taste of food. She had a medical history of migraines. The general practitioner noticed left-sided ptosis and referred her to the emergency department for further analysis. Upon admission she told us that her headache was bilateral and resembled the migraine with photophobia and phonophobia; however, this time the headache was worse and her normal medication (rizatriptan) did not
, Keywords—carotid artery dissection; cerebral ischemia; dysgeusia; Horner syndrome; trigeminal neuropathy
Reprints are not available from the authors.
RECEIVED: 30 June 2019; FINAL SUBMISSION RECEIVED: 9 September 2019; ACCEPTED: 20 September 2019 1
2
have any effect on the pain. Three days before the headache started, she experienced nausea and vomited twice. She confirmed having dysgeusia. We saw an alert and oriented woman with a pulse rate of 80 beats/min and blood pressure of 155/105 mm Hg. She rated the headache 3 of 10 on the visual analog scale. A neurologic examination revealed left-sided ptosis and mild miosis (3+/2+) without facial anhidrosis (partial left-sided Horner syndrome) and hypesthesia of the fifth cranial nerve on the left side. The remainder of her physical and neurologic examinations was unremarkable. Routine laboratory results and electrocardiogram did not show any abnormalities. Because of the headache and the partial Horner syndrome we considered a CAD despite hypesthesia of the trigeminal nerve and dysgeusia being atypical symptoms. We decided to perform magnetic resonance angiography, which showed loss of normal signal void in the left extracranial internal carotid artery (ICA) continuing into the carotid canal and the proximal intracranial ICA (Figure 1). Magnetic resonance angiography also showed an intimal flap in the left ICA (Figure 2). There were no signs of accompanying cerebral ischemic events.
Figure 1. Brain magnetic resonance angiography showing decreased contrast enhancement of the left internal carotid artery (arrow).
K. V. M. van der Zwet et al.
A diagnosis of left internal CAD was made, possibly provoked by vomiting a couple of days before the symptoms started. Antiplatelet therapy with clopidogrel was started. The patient was stable over the next 2 days without any neurologic deterioration, and therefore she was discharged home. She still had a partial Horner syndrome at the 6-week follow-up visit, but she did not have any other symptoms. DISCUSSION CAD is a serious risk factor for cerebral infarction, with 50–90% of these patients presenting with ischemic manifestations (1,2). In 90% of cases, ischemia is the result of a thromboembolic process arising from the site of the dissection, and in the other 10% is a consequence of vessel lumen compromise resulting in cerebral hypoperfusion (1,5–7). Most cerebral infarctions occur during the first week after local symptom onset, which emphasizes the importance of early treatment (1,6,7). Treatment is focused on prevention of thrombus formation and therefore infarction and consists of antiplatelet or anticoagulant therapy (6,7). When treatment is started, stroke recurrence is about 2% (6,7). Diagnosing spontaneous CAD can be difficult because of a large variability in symptoms. This variability can be explained by the exact anatomic location of the dissection area. As mentioned before, the classic triad is only present in less than one third of the cases (1). Partial Horner
Figure 2. Left carotid artery with intimal flap on magnetic resonance angiography.
Internal CAD With Dysgeusia, Horner Syndrome, and Hypesthesia
3
unclear (10). The presence of any connective tissue disease or a family member with connective tissue disease also increases the risk of spontaneous CAD. In addition, although the term spontaneous refers to a dissection without any preliminary significant trauma, a history of a precipitating trivial event such as vomiting (as in this case), coughing, a sudden head movement, or massage is present in 12–34% of cases (1,2). An infectious disease may also trigger a spontaneous CAD, which is supported by the peak incidence in autumn (1). A thorough historytaking is important to identify any precipitating events. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?
Figure 3. Drawing of the anatomic localization of the carotid artery dissection in relation to the cranial nerves. Drawing made by Noortje W. Haenen, MSc.
syndrome is seen in 28–58% of cases with CAD and is caused by compression of the pericarotid sympathetic fibers in the wall of the ICA (2). Facial anhidrosis is usually not present because the facial sweat glands are innervated by the sympathetic plexus surrounding the external carotid artery (1,2). In 8–16% of cases there are symptoms of cranial nerve involvement, mostly concerning the lower cranial nerves (IX–XII) (8). An explanation for cranial nerve palsies is compression or stretching of the nerve by the expanded artery. An alternative explanation is interruption of the nutrient vessels supplying the nerve (9). Dysgeusia associated with CAD is probably caused by local compression of the chorda tympani of the facial cranial nerve or compression of the glossopharyngeal nerve, and is an uncommon finding that is only present in 7% of cases (1). Even more atypical for CAD is involvement of the trigeminal nerve in only 3.7% of the cases (9). The CAD in our case was in the extracranial ICA and the proximal intracranial ICA, which can explain compression of the trigeminal ganglion, the chorda tympani of the facial cranial nerve, and the pericarotid sympathetic plexus surrounding the ICA (Figure 3). This patient’s clinical history included migraine, which is thought to be a risk factor for CAD (10). Pathophysiologic mechanisms behind this association are
CAD can present with different combinations of cranial nerve palsies and should be included in the differential diagnosis of dysgeusia and hypesthesia of the trigeminal nerve. The early recognition of CAD can be challenging in patients with rare cranial nerves involvement, but early treatment is crucial to prevent cerebral ischemic events. REFERENCES 1. Schelfaut D, Dhondt E, De Raedt S, Nieboer K, Hubloue I. Carotid artery dissection: three cases and a review of the literature. Eur J Emerg Med 2012;19:181–7. 2. Fusco MR, Harrigan MR. Cerebrovasculair dissections – a review part 1: spontaneous dissections. Neurosurgery 2010;68:242–7. 3. Beletsky V, Nadareishvili Z, Lynch J, et al. Cervical aterial dissection. Time for a therapeutic trial? Stroke 2003;34:2856–60. 4. Arauz A, Hoyos L, Espinoza C, Cantu´ C, Barinagarrementeria F, Roma´n G. Dissection of cervical arteries: long-term follow-up study of 130 consecutive cases. Cerebrovasc Dis 2006;22:150–4. 5. Bhatti MT, Schmalfuss I. Dysgeusia and painful Horner’s syndrome from an internal carotid artery dissections: radiological-anatomical correlation. Acta Ophtalmol Scand 2002;80:562–4. 6. Markus HS, Hayter E, Levi C, et al. Antiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): a randomised trial. Lancet Neurol 2015;4:361–7. 7. Kernan WN, Ovbiagele B, Black HR, et al. Guidelines for the prevention of stroke in patients with stroke and transient ischemic attack. Stroke 2014;45:2160–236. 8. Majeed A, Lobato Ribeiro NP, Ali A, Hijazi M, Farook H. A rare presentation of spontaneous internal carotid artery dissection with Horner’s syndrome, VIIth, Xth and XIIth nerve palsies. Oxf Med Case Rep 2016;10:255–8. 9. Mokri B, Silbert PL, Schievink WI, Piepgras DG. Cranial nerve palsy in spontaneous dissection of the extracranial internal carotid artery. Neurology 1996;46:356–9. 10. Rist PM, Diener H, Kurh T, Schu¨rks M. Migraine, migraine aura, and cervical artery dissection: a systematic review and meta-analysis. Cephalalgia 2011;31:886–96.
https://doi.org/10.1016/j.jemermed.2019.09.035
Clinical Communications: Adult INTERNAL CAROTID ARTERY DISSECTION PRESENTING WITH DYSGEUSIA, HORNER SYNDROME, AND HYPESTHESIA OF THE FIFTH CRANIAL NERVE: A CASE REPORT Kirsten V. M. van der Zwet, MSC,* A. Vanessa Brown, MD,* and Stef L. M. Bakker, MD† *Department of Emergency Medicine, Franciscus Gasthuis & Vlietland, Rotterdam, the Netherlands and †Department of Neurology, Franciscus Gasthuis & Vlietland, Rotterdam, the Netherlands Corresponding Address: Kirsten V.M. van der Zwet, MSC, Department of Emergency Medicine, Franciscus Gasthuis & Vlietland, Kleiweg 500, Rotterdam 3045 PM, the Netherlands.
, Abstract—Background: Carotid artery dissection (CAD) is a significant cause of ischemic stroke. Early recognition and treatment of CAD is important to prevent accompanying cerebral ischemia. This case report presents an atypical clinical presentation of CAD and emphasizes the diagnostic challenge for emergency physicians. Case Report: A 54-year-old woman presented to the emergency department with a bilateral headache of 4 days’ duration, hypesthesia of the left fifth cranial nerve, dysgeusia, and partial Horner syndrome on the left side. Magnetic resonance angiography showed a left-sided CAD without any signs of cerebral ischemic events. Antiplatelet therapy with clopidogrel was started, and the patient did not show any deterioration in the weeks thereafter. Why Should an Emergency Physician be Aware of This?: CAD can present with different combinations of cranial nerve palsies and should be in the differential diagnosis of dysgeusia and hypesthesia of the trigeminal nerve. Early recognition of CAD can be challenging in patients with rare cranial nerve involvement, but early treatment is crucial to prevent cerebral ischemic events. Ó 2019 Elsevier Inc. All rights reserved.
INTRODUCTION Carotid artery dissection (CAD) is a significant cause of stroke, accounting for 2–3% of all strokes and #25% of strokes in young adults (1,2). The early recognition and treatment of CAD is important to prevent cerebral ischemia (3,4). Only 33% of all cases present with the classical triad of (unilateral) headache, Horner syndrome, and ischemic events, and therefore early recognition can be challenging (1). We present an atypical clinical presentation of CAD and emphasize the diagnostic challenge for emergency physicians. CASE REPORT A 54-year-old woman visited her general practitioner because of a headache of 4 days’ duration and an asymmetrical appearance of her face that she noticed a couple of hours before presentation. She also complained about a change in the taste of food. She had a medical history of migraines. The general practitioner noticed left-sided ptosis and referred her to the emergency department for further analysis. Upon admission she told us that her headache was bilateral and resembled the migraine with photophobia and phonophobia; however, this time the headache was worse and her normal medication (rizatriptan) did not
, Keywords—carotid artery dissection; cerebral ischemia; dysgeusia; Horner syndrome; trigeminal neuropathy
Reprints are not available from the authors.
RECEIVED: 30 June 2019; FINAL SUBMISSION RECEIVED: 9 September 2019; ACCEPTED: 20 September 2019 1
2
have any effect on the pain. Three days before the headache started, she experienced nausea and vomited twice. She confirmed having dysgeusia. We saw an alert and oriented woman with a pulse rate of 80 beats/min and blood pressure of 155/105 mm Hg. She rated the headache 3 of 10 on the visual analog scale. A neurologic examination revealed left-sided ptosis and mild miosis (3+/2+) without facial anhidrosis (partial left-sided Horner syndrome) and hypesthesia of the fifth cranial nerve on the left side. The remainder of her physical and neurologic examinations was unremarkable. Routine laboratory results and electrocardiogram did not show any abnormalities. Because of the headache and the partial Horner syndrome we considered a CAD despite hypesthesia of the trigeminal nerve and dysgeusia being atypical symptoms. We decided to perform magnetic resonance angiography, which showed loss of normal signal void in the left extracranial internal carotid artery (ICA) continuing into the carotid canal and the proximal intracranial ICA (Figure 1). Magnetic resonance angiography also showed an intimal flap in the left ICA (Figure 2). There were no signs of accompanying cerebral ischemic events.
Figure 1. Brain magnetic resonance angiography showing decreased contrast enhancement of the left internal carotid artery (arrow).
K. V. M. van der Zwet et al.
A diagnosis of left internal CAD was made, possibly provoked by vomiting a couple of days before the symptoms started. Antiplatelet therapy with clopidogrel was started. The patient was stable over the next 2 days without any neurologic deterioration, and therefore she was discharged home. She still had a partial Horner syndrome at the 6-week follow-up visit, but she did not have any other symptoms. DISCUSSION CAD is a serious risk factor for cerebral infarction, with 50–90% of these patients presenting with ischemic manifestations (1,2). In 90% of cases, ischemia is the result of a thromboembolic process arising from the site of the dissection, and in the other 10% is a consequence of vessel lumen compromise resulting in cerebral hypoperfusion (1,5–7). Most cerebral infarctions occur during the first week after local symptom onset, which emphasizes the importance of early treatment (1,6,7). Treatment is focused on prevention of thrombus formation and therefore infarction and consists of antiplatelet or anticoagulant therapy (6,7). When treatment is started, stroke recurrence is about 2% (6,7). Diagnosing spontaneous CAD can be difficult because of a large variability in symptoms. This variability can be explained by the exact anatomic location of the dissection area. As mentioned before, the classic triad is only present in less than one third of the cases (1). Partial Horner
Figure 2. Left carotid artery with intimal flap on magnetic resonance angiography.
Internal CAD With Dysgeusia, Horner Syndrome, and Hypesthesia
3
unclear (10). The presence of any connective tissue disease or a family member with connective tissue disease also increases the risk of spontaneous CAD. In addition, although the term spontaneous refers to a dissection without any preliminary significant trauma, a history of a precipitating trivial event such as vomiting (as in this case), coughing, a sudden head movement, or massage is present in 12–34% of cases (1,2). An infectious disease may also trigger a spontaneous CAD, which is supported by the peak incidence in autumn (1). A thorough historytaking is important to identify any precipitating events. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?
Figure 3. Drawing of the anatomic localization of the carotid artery dissection in relation to the cranial nerves. Drawing made by Noortje W. Haenen, MSc.
syndrome is seen in 28–58% of cases with CAD and is caused by compression of the pericarotid sympathetic fibers in the wall of the ICA (2). Facial anhidrosis is usually not present because the facial sweat glands are innervated by the sympathetic plexus surrounding the external carotid artery (1,2). In 8–16% of cases there are symptoms of cranial nerve involvement, mostly concerning the lower cranial nerves (IX–XII) (8). An explanation for cranial nerve palsies is compression or stretching of the nerve by the expanded artery. An alternative explanation is interruption of the nutrient vessels supplying the nerve (9). Dysgeusia associated with CAD is probably caused by local compression of the chorda tympani of the facial cranial nerve or compression of the glossopharyngeal nerve, and is an uncommon finding that is only present in 7% of cases (1). Even more atypical for CAD is involvement of the trigeminal nerve in only 3.7% of the cases (9). The CAD in our case was in the extracranial ICA and the proximal intracranial ICA, which can explain compression of the trigeminal ganglion, the chorda tympani of the facial cranial nerve, and the pericarotid sympathetic plexus surrounding the ICA (Figure 3). This patient’s clinical history included migraine, which is thought to be a risk factor for CAD (10). Pathophysiologic mechanisms behind this association are
CAD can present with different combinations of cranial nerve palsies and should be included in the differential diagnosis of dysgeusia and hypesthesia of the trigeminal nerve. The early recognition of CAD can be challenging in patients with rare cranial nerves involvement, but early treatment is crucial to prevent cerebral ischemic events. REFERENCES 1. Schelfaut D, Dhondt E, De Raedt S, Nieboer K, Hubloue I. Carotid artery dissection: three cases and a review of the literature. Eur J Emerg Med 2012;19:181–7. 2. Fusco MR, Harrigan MR. Cerebrovasculair dissections – a review part 1: spontaneous dissections. Neurosurgery 2010;68:242–7. 3. Beletsky V, Nadareishvili Z, Lynch J, et al. Cervical aterial dissection. Time for a therapeutic trial? Stroke 2003;34:2856–60. 4. Arauz A, Hoyos L, Espinoza C, Cantu´ C, Barinagarrementeria F, Roma´n G. Dissection of cervical arteries: long-term follow-up study of 130 consecutive cases. Cerebrovasc Dis 2006;22:150–4. 5. Bhatti MT, Schmalfuss I. Dysgeusia and painful Horner’s syndrome from an internal carotid artery dissections: radiological-anatomical correlation. Acta Ophtalmol Scand 2002;80:562–4. 6. Markus HS, Hayter E, Levi C, et al. Antiplatelet treatment compared with anticoagulation treatment for cervical artery dissection (CADISS): a randomised trial. Lancet Neurol 2015;4:361–7. 7. Kernan WN, Ovbiagele B, Black HR, et al. Guidelines for the prevention of stroke in patients with stroke and transient ischemic attack. Stroke 2014;45:2160–236. 8. Majeed A, Lobato Ribeiro NP, Ali A, Hijazi M, Farook H. A rare presentation of spontaneous internal carotid artery dissection with Horner’s syndrome, VIIth, Xth and XIIth nerve palsies. Oxf Med Case Rep 2016;10:255–8. 9. Mokri B, Silbert PL, Schievink WI, Piepgras DG. Cranial nerve palsy in spontaneous dissection of the extracranial internal carotid artery. Neurology 1996;46:356–9. 10. Rist PM, Diener H, Kurh T, Schu¨rks M. Migraine, migraine aura, and cervical artery dissection: a systematic review and meta-analysis. Cephalalgia 2011;31:886–96.