vasospasms of the intracranial arteries are a common and well - known complication of subarachnoid hemorrhage .
they are also encountered in other disorders such as cerebral vasculitis or reversible cerebral vasoconstriction syndrome ( rcvs ) .
in contrast , vasospasms of the extracranial circulation occur less frequently and have most often been associated with mechanical manipulations   or ergot poisoning .
recurrent spontaneous vasospasms occurring mainly within the intracranial but also within the extracranial arteries have also been reported in patients suffering from migraine headaches .
we present a patient with recurrent strokes due to transient vasospastic occlusions of the internal carotid artery ( ica ) without migrainous headaches .
our case supports the notion that recurrent non - migrainous vasospasms of the extracranial carotid artery likely reflect a distinct entity which can cause ischemic strokes .
in july 1999 , a previously healthy 34-year - old female patient was admitted to our hospital for the first time with an acute left - sided brachiofacial hemiparesis .
she reported that similar symptoms had occurred several times in the previous 10 years but had regressed spontaneously . aside from regular nicotine consumption , she had no other cardiovascular risk factors .
magnetic resonance imaging ( mri ) showed an internal middle cerebral artery ( mca ) territory watershed infarct , probably of hemodynamic origin .
duplex sonography on admission revealed proximal occlusions of both icas and retrograde flow in both supratrochlear arteries ( sta ) .
three days later , a digital subtraction angiography showed a recanalization of the left ica and a persistent occlusion of the right ica at the origin , which subsequently never recanalized .
on axial t1-weighted fat - suppressed images , the presence of intramural hematomas and thus arterial dissections as a cause of the bilateral ica occlusions could be ruled out .
the patient was treated with the calcium antagonist nimodipine ( 240 mg / day ) and was heparinized with an aim for a partial thromboplastin time of > 60 s. despite this treatment , a transient occlusion of the left ica , confirmed with repeated ultrasound and magnetic resonance angiography ( mra ) , occurred again during this first admission .
the patient was discharged receiving nimodipine , and an oral anticoagulation with phenprocoumon was begun . in february 2004
, she was readmitted due to a short episode with paresis of the right hand and aphasia . while mri including diffusion - weighted imaging showed no new infarction , an initial occlusion and subsequent reopening of the left ica
during the course of this admission , oral anticoagulation was discontinued and the patient was discharged with aspirin and nimodipine . in march 2010 , february 2011 and may 2011 , the patient experienced recurrent episodes with transient right - sided hemiparesis and aphasia .
in all three instances , mri revealed new small infarcts in the left mca territory .
extensive laboratory investigations were performed twice ( including virology , bacteriology and tests for thrombophilia and vasculitis ) , but revealed no pathological results .
likewise , 2 chest x - rays and an ultrasound of the abdomen were normal .
similar to the findings in 2004 , mra and ultrasound repeatedly revealed an initial occlusion and subsequent reopening of the left ica ( fig . 1 , fig
left ica occlusion , a reduced pulsatility in the left mca , a collateralization via the posterior communicating arteries and a retrograde flow within the ipsilateral sta were observed with ultrasound .
after recanalization , ultrasound showed an antegrade flow within the ipsilateral sta and a completely normalized mca flow . since may 2011
a coincidence with smoking was presumed during the first stays , but attacks appeared also after she quit smoking .
evidence is accumulating that recurrent vasospasms of the extracranial ica are a rare but important cause of ischemic disorders mainly in young patients [ 6 , 7 , 8 , 9 , 10 , 11 , 12 , 13 , 14 ] .
while previous cases have been associated with migraine [ 5 , 10 ] , we describe a patient with recurrent non - migrainous vasospasms of the extracranial ica .
only a few such cases have been reported in the literature to date [ 6 , 8 , 11 , 12 , 13 , 14 ] .
it is noteworthy that the recurrent migrainous and non - migrainous vasospasms of the extracranial ica vasospasms appear to occur exclusively in the intermediate portion of the ica [ 8 , 11 , 13 , 14 ] .
the reason for this finding is currently unclear , but could be associated with a distinct pattern of sympathetic vasomotor innervation in the intermediate ica segment , enhancing the susceptibility of this vessel segment to sympathetic stimuli among others .
it should also be emphasized that recurrent vasospasms of the extracranial ica can only be observed during a short time period , and , therefore , they can only be detected using serial ultrasound or other vessel imaging modalities .
it is thus likely that the incidence of this entity is underestimated . in young patients with an acute ischemic stroke due to an ica occlusion
, the most important differential diagnosis is a dissection of the carotid artery . in light of a missing wall hematoma on repeated t1-weighted fat - suppressed images , as well as the recurrence of the episodes with spontaneous resolution of the left ica occlusion within a few days ,
it is very unlikely that repeated dissections were responsible for the clinical course in our patient .
the rapid spontaneous resolution of the ica occlusion is also an argument against the presence of an inflammatory vessel disease .
although the beaded appearance of the left ica could principally be suggestive of vasculitis , extensive laboratory evaluations were performed to rule out the presence of such a disorder . in particular
, there was no evidence of wegener 's disease due to missing oropharyngeal inflammation , chest x - rays were inconspicuous and c - and p - anca were negative .
reversible vessel occlusions can also be observed in the context of rcvs . in the past few years , this disorder has emerged as a distinct entity and is characterized by thunderclap headaches accompanied by multifocal cerebral artery vasoconstriction . for the diagnosis of rcvs , a complete reversibility of the latter feature within 12 weeks of onset is required .
the missing headaches , as well as the time course argue against the presence of rcvs as the cause of the reversible ica occlusions in our patient .
currently , the optimal treatment of patients with recurrent vasospasms of the extracranial ica remains unclear .
in contrast to other case reports [ 6 , 7 , 12 ] which reported positive effects of calcium channel inhibitors and corticoids , this treatment was not effective in our patient .
in addition , neither oral anticoagulation nor platelet inhibitors prevented new stroke episodes in our case .
recently , moeller et al .   demonstrated a sympathetic hypersensitivity in a patient with an isolated vasospasm of the extracranial ica , which partially resolved using the -1-blocker prazosin .
spontaneous vasospasms of the extracranial ica should be thought of in recurrent ischemic symptoms in young stroke patients .
recurrent non - migrainous vasospasms of the extracranial ica likely reflect a distinct disease entity which can cause ischemic strokes .
 vasospasms of the intracranial arteries are a well - known complication of subarachnoid hemorrhage and are also frequently encountered in other disorders such as migraine , cerebral vasculitis or reversible cerebral vasoconstriction syndrome . 
 in contrast , recurrent spontaneous vasospasms of the extracranial circulation appear to be extremely rare and have most often been associated with migraine . 
 we present a patient with recurrent strokes due to spontaneous transient vasospastic occlusions of the internal carotid artery ( ica ) without migraine over a time period of at least 13 years . 
 initially , the patient had presented with a bilateral ica occlusion and a cerebral infarct on the right side . 
 while the right ica remained occluded , a reopening of the left ica could be detected 3 days after this initial event . in subsequent years 
 , both duplex sonography and magnetic resonance angiography revealed recurrent occlusions of the left ica , which resolved spontaneously within days . 
 this case and other rare previous reports indicate that recurrent non - migrainous vasospasms of the extracranial carotid artery likely reflect a distinct entity which can cause ischemic strokes . 
Introduction
Case Report
Discussion
Conclusion