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A case report of carotid artery dissection presenting as cluster headache
BRIEF CLINICAL OBSERVATIONS
A Case Report of Carotid Artery Dissection Presenting as Cluster .Headache Curtis J. Rosebraugh, MD, DonnaJ. Griebel, MD, Donald J. DiPette, MD University of Texas Medical Branch-Galveston, Galveston, Texas
Pontaneous carotid artery dissection (SCAD) has b e e n associated with a n u m b e r of clinical synd r o m e s including transient ischemic attack (TIA), cerebral vascular accident (CVA), cranial neuropathies and migraine headaches. 1 This p a p e r reports an unusual p r e s e n t a t i o n of SCAD that included episodic h e a d a c h e s and H o m e r ' s s y n d r o m e consistent with acute cluster headaches.
S
CASE REPORT A 34-year-old white m a n presented with a chief complaint of daily h e a d a c h e for 2 weeks. The headache o c c u r r e d every morning as a sharp "ice-pick" pain initially involving the right h e m i c r a n i u m localizing to the right retrobulbular area over a 2-hour period. The h e a d a c h e was associated with rightsided nasal stuffiness, lacrimation, and an injected conjunctiva. The patient denied photophobia, nausea, vomiting, neurologic symptoms, or visual scintillations. Medications included a c e t a m i n o p h e n with codeine, which only partially relieved the pain. The patient was an accountant, s m o k e d two p a c k a g e s of cigarettes p e r day, and drank five beers dally. Physical examination revealed a blood pressure of 120/ 80 m m Hg with a pulse of 70. A right-sided H o m e r ' s w a s p r e s e n t with lid droop and miosis of the pupil. The r e m a i n d e r of the cranial nerves w e r e intact. The carotid arterial pulses w e r e normal, and no bruits were auscultated. The remainder of the physical and neurologic . examination was normal. Complete blood count, metabolic profile, and sedimentation rate were normal. A c o m p u t e d axial t o m o g r a p h y (CAT) scan w a s n o r m a l except for a possible rightsided venous pool. A neurological c o n s u l t a n t diagn o s e d cluster headaches. A magnetic r e s o n a n c e imaging scan (MRI) w a s obtained to further delineate the venous PO01 and revealed, as an u n e x p e c t e d finding, a dissection and occlusion of the right internal carotid artery ( F i g u r e 1). This was confirmed b y carotid angiogram ( F i g u r e 2).
DISCUSSION Spontaneous carotid artery dissection occurs w h e n there is no a n t e c e d e n t history of t r a u m a or t u m o r and was first described in 1959 b y Anderson. 2 418
April 1997 The American Journal of Medicine ® Volume 102
Figure 1. Magnetic resonance image demonstrating hematoma in internal carotid artery (closed arrow). Note normal internal carotid artery (open arrow).
B e t w e e n 1970 and 1989 there were a p p r o x i m a t e l y 265 case reports with an equal n u m b e r of case rep o r t s f r o m 1989 to the present. The dissection begins as a rent in a microscopically normal a r e a of the tunica m e d i a of the internal carotid artery. A hemat o m a f o r m s a n d causes a dissection of the arterial wall that results in c o m p r e s s i o n and collapse in the lumen of the artery. N e r v e damage occurs s e c o n d a r y to an interruption of blood flow and the c o m p r e s s i o n that results f r o m local inflammatory r e s p o n s e to the forming h e m a t o m a . Damage c o m m o n l y occurs to the superior cervical ganglion, which causes Horner's syndrome, and to any cranial nerves that are in close proximity to the carotid artery. C o m m o n l y recognized s y m p t o m s of SCAD are ipsilateral headache, miosis, ptosis, n e c k pain, bruit, dysgeusia, and visual scintillation. With increased usage of MRI, SCAD is n o w recognized as a cause of other cranial neuropathies including postcoital headache, scintillating scotomata, l ipsilateral hypoglossal nerve palsy, 3 multiple cranial neuropathies including cranial nerves 5, 7, 9, 10, and 9, 10, 11, 12, 4 and isolated abducens nerve palsy. 5 Originally, SCAD w a s felt to be a benign entity, but in patients with neurologic sequela the prognosis can be grave. In a large study of patients with stroke secondary to SCAD, 6 23% of patients died in the first
BRIEF CLINICAL OBSERVATIONS
Therapy includes short-term heparin followed b y long-term anticoagulation with coumadin until recanalization of the artery occurs. Patients should be monitored at regular intervals, and w h e n recanalization occurs, anticoagulation m a y b e discontinued. The majority of patients will have recanalization of the carotid artery with restoration of blood flow within 6 months. The unique features of this case included a presentation consistent with cluster headaches. Despite anticoagulation and d o c u m e n t e d recanalization, the patient's h e a d a c h e and intermittent H o m e r ' s syndrome persisted. Without the initial MRI, this patient would have b e e n diagnosed with cluster headaches. In patients with h e a d a c h e and postganglionic Horner's syndrome, evaluation for SCAD should be considered, and if present, appropriate medical t h e r a p y instituted.
REFERENCES
Figure 2. Arteriogram demonstrating characteristic tapering "funnel sign" of the internal carotid artery (arrow). Note normal external carotid artery (arrowhead),
w e e k and 48% of survivors w e r e unable to r e s u m e previous activities. Prospective studies have found SCAD to account for 2.5% to 10% of stroke in patients depending on the age of the group studied. 7's Magnetic r e s o n a n c e imaging has displaced carotid arteriogram as the gold standard test for diagnosis, s'9 It has t h e advantage of being less expensive, is noninvasive, and is able to d e m o n s t r a t e an early hemat o m a in the wall of the internal carotid artery that has not yet caused total occlusion. 4 Duplex ultrasound is useful in monitoring for recanalization.
1. Ramadan NM, Tietjen GE, Levine SR, Welch KM. Scintillating scotomata associated with internal carotid artery dissection: report of three cases. Neurology. 1991;41(7):1084-1087. 2. Anderson RM, Schechter MM. A case of spontaneous dissecting aneurysms of the internal carotid artery, d Neurosurg Psychiatry. 1959;22:195-201. 3. Vighetto A, Lisovoske F, Revol A, et al. Internal carotid artery dissection and ipsilateral hypoglossal nerve palsy. J Neurol Neurosurg Psychiatry. 1990;53(6):530-531. Letter. 4. Panisset M, Eidelman BH. Case report: multiple cranial neuropathy as a feature of internal carotid artery dissection. Stroke. i990;21(1):141-147., 5. Maitland CG, Black JL, Smith WA. Abducens nerve palsy due to spontaneous dissection of the internal carotid artery. Arch Neurol. 1983;40:448-449. 6. Bogousslavsky J, Despland PA, Regli F. Spontaneous carotid dissection with acute stroke. Arch Neurol. 1987;44:137-140. 7. Lisovoski F, Rousseaux P. Cerebral infarction in young people. A study of 148 patients with early cerebral angiography. J Neurosurg Psychiatry. 1991;54(7):576-579. 8. Nelson Jr, Boundy KL. Magnetic resonance imaging in carotid dissection. Australas Radiol. 1992;36(1):40-43. 9. Cox LK, Bertorini T, Laster RE. Headache due to spontaneous internal carotid artery dissection magnetic resonance imaging evaluation and follow up. Headache. 1991;31(1):12-61. Manuscript submitted November 15, 1996 and accepted November 22, 1996.
April 1997 The American Journal of Medicine ® Volume 102
419
A Case Report of Carotid Artery Dissection Presenting as Cluster .Headache Curtis J. Rosebraugh, MD, DonnaJ. Griebel, MD, Donald J. DiPette, MD University of Texas Medical Branch-Galveston, Galveston, Texas
Pontaneous carotid artery dissection (SCAD) has b e e n associated with a n u m b e r of clinical synd r o m e s including transient ischemic attack (TIA), cerebral vascular accident (CVA), cranial neuropathies and migraine headaches. 1 This p a p e r reports an unusual p r e s e n t a t i o n of SCAD that included episodic h e a d a c h e s and H o m e r ' s s y n d r o m e consistent with acute cluster headaches.
S
CASE REPORT A 34-year-old white m a n presented with a chief complaint of daily h e a d a c h e for 2 weeks. The headache o c c u r r e d every morning as a sharp "ice-pick" pain initially involving the right h e m i c r a n i u m localizing to the right retrobulbular area over a 2-hour period. The h e a d a c h e was associated with rightsided nasal stuffiness, lacrimation, and an injected conjunctiva. The patient denied photophobia, nausea, vomiting, neurologic symptoms, or visual scintillations. Medications included a c e t a m i n o p h e n with codeine, which only partially relieved the pain. The patient was an accountant, s m o k e d two p a c k a g e s of cigarettes p e r day, and drank five beers dally. Physical examination revealed a blood pressure of 120/ 80 m m Hg with a pulse of 70. A right-sided H o m e r ' s w a s p r e s e n t with lid droop and miosis of the pupil. The r e m a i n d e r of the cranial nerves w e r e intact. The carotid arterial pulses w e r e normal, and no bruits were auscultated. The remainder of the physical and neurologic . examination was normal. Complete blood count, metabolic profile, and sedimentation rate were normal. A c o m p u t e d axial t o m o g r a p h y (CAT) scan w a s n o r m a l except for a possible rightsided venous pool. A neurological c o n s u l t a n t diagn o s e d cluster headaches. A magnetic r e s o n a n c e imaging scan (MRI) w a s obtained to further delineate the venous PO01 and revealed, as an u n e x p e c t e d finding, a dissection and occlusion of the right internal carotid artery ( F i g u r e 1). This was confirmed b y carotid angiogram ( F i g u r e 2).
DISCUSSION Spontaneous carotid artery dissection occurs w h e n there is no a n t e c e d e n t history of t r a u m a or t u m o r and was first described in 1959 b y Anderson. 2 418
April 1997 The American Journal of Medicine ® Volume 102
Figure 1. Magnetic resonance image demonstrating hematoma in internal carotid artery (closed arrow). Note normal internal carotid artery (open arrow).
B e t w e e n 1970 and 1989 there were a p p r o x i m a t e l y 265 case reports with an equal n u m b e r of case rep o r t s f r o m 1989 to the present. The dissection begins as a rent in a microscopically normal a r e a of the tunica m e d i a of the internal carotid artery. A hemat o m a f o r m s a n d causes a dissection of the arterial wall that results in c o m p r e s s i o n and collapse in the lumen of the artery. N e r v e damage occurs s e c o n d a r y to an interruption of blood flow and the c o m p r e s s i o n that results f r o m local inflammatory r e s p o n s e to the forming h e m a t o m a . Damage c o m m o n l y occurs to the superior cervical ganglion, which causes Horner's syndrome, and to any cranial nerves that are in close proximity to the carotid artery. C o m m o n l y recognized s y m p t o m s of SCAD are ipsilateral headache, miosis, ptosis, n e c k pain, bruit, dysgeusia, and visual scintillation. With increased usage of MRI, SCAD is n o w recognized as a cause of other cranial neuropathies including postcoital headache, scintillating scotomata, l ipsilateral hypoglossal nerve palsy, 3 multiple cranial neuropathies including cranial nerves 5, 7, 9, 10, and 9, 10, 11, 12, 4 and isolated abducens nerve palsy. 5 Originally, SCAD w a s felt to be a benign entity, but in patients with neurologic sequela the prognosis can be grave. In a large study of patients with stroke secondary to SCAD, 6 23% of patients died in the first
BRIEF CLINICAL OBSERVATIONS
Therapy includes short-term heparin followed b y long-term anticoagulation with coumadin until recanalization of the artery occurs. Patients should be monitored at regular intervals, and w h e n recanalization occurs, anticoagulation m a y b e discontinued. The majority of patients will have recanalization of the carotid artery with restoration of blood flow within 6 months. The unique features of this case included a presentation consistent with cluster headaches. Despite anticoagulation and d o c u m e n t e d recanalization, the patient's h e a d a c h e and intermittent H o m e r ' s syndrome persisted. Without the initial MRI, this patient would have b e e n diagnosed with cluster headaches. In patients with h e a d a c h e and postganglionic Horner's syndrome, evaluation for SCAD should be considered, and if present, appropriate medical t h e r a p y instituted.
REFERENCES
Figure 2. Arteriogram demonstrating characteristic tapering "funnel sign" of the internal carotid artery (arrow). Note normal external carotid artery (arrowhead),
w e e k and 48% of survivors w e r e unable to r e s u m e previous activities. Prospective studies have found SCAD to account for 2.5% to 10% of stroke in patients depending on the age of the group studied. 7's Magnetic r e s o n a n c e imaging has displaced carotid arteriogram as the gold standard test for diagnosis, s'9 It has t h e advantage of being less expensive, is noninvasive, and is able to d e m o n s t r a t e an early hemat o m a in the wall of the internal carotid artery that has not yet caused total occlusion. 4 Duplex ultrasound is useful in monitoring for recanalization.
1. Ramadan NM, Tietjen GE, Levine SR, Welch KM. Scintillating scotomata associated with internal carotid artery dissection: report of three cases. Neurology. 1991;41(7):1084-1087. 2. Anderson RM, Schechter MM. A case of spontaneous dissecting aneurysms of the internal carotid artery, d Neurosurg Psychiatry. 1959;22:195-201. 3. Vighetto A, Lisovoske F, Revol A, et al. Internal carotid artery dissection and ipsilateral hypoglossal nerve palsy. J Neurol Neurosurg Psychiatry. 1990;53(6):530-531. Letter. 4. Panisset M, Eidelman BH. Case report: multiple cranial neuropathy as a feature of internal carotid artery dissection. Stroke. i990;21(1):141-147., 5. Maitland CG, Black JL, Smith WA. Abducens nerve palsy due to spontaneous dissection of the internal carotid artery. Arch Neurol. 1983;40:448-449. 6. Bogousslavsky J, Despland PA, Regli F. Spontaneous carotid dissection with acute stroke. Arch Neurol. 1987;44:137-140. 7. Lisovoski F, Rousseaux P. Cerebral infarction in young people. A study of 148 patients with early cerebral angiography. J Neurosurg Psychiatry. 1991;54(7):576-579. 8. Nelson Jr, Boundy KL. Magnetic resonance imaging in carotid dissection. Australas Radiol. 1992;36(1):40-43. 9. Cox LK, Bertorini T, Laster RE. Headache due to spontaneous internal carotid artery dissection magnetic resonance imaging evaluation and follow up. Headache. 1991;31(1):12-61. Manuscript submitted November 15, 1996 and accepted November 22, 1996.
April 1997 The American Journal of Medicine ® Volume 102
419