Intern Emerg Med (2014) 9:201–205 DOI 10.1007/s11739-013-1038-3

IM - CASE RECORD

A challenging multifactorial migraine with aura Luca Naldi • Margherita De Stefano • Andrea Berni • Marco Torri • Loredana Poggesi

Received: 15 November 2013 / Accepted: 17 December 2013 / Published online: 14 January 2014 Ó SIMI 2014

Case presentation Dr. Naldi and Dr. De Stefano We present the case of a 48-year-old man with sudden onset at rest of migraine with a visual aura characterized by scintillating scotoma. Minutes later, fluency aphasia, blurred vision of the left eye, and paresthesia of the left hand developed, resolving after 10 min. Thirty minutes later, another event of transient fluency aphasia, blurred vision and paresthesia, similar to the previous, occurred. He took a non-steroidal antiinflammatory drug (NSAID) with a complete resolution of both migraine and neurological symptoms after 1 h. The past medical history of the patient included hyperlipidemia and unilateral left fronto-temporal migraine with visual aura (scintillating scotoma) usually responsive to NSAID. He was a non-smoker. He had been well until 2 weeks earlier, when several episodes of subcontinuous occipital headache of moderate intensity occurred, without fever, nausea or vomiting, partially responsive to NSAID. The episodes differed from the previous ones for the lower intensity of pain, the localization at the occipital region and the absence of aura. During the previous days, he found high values of blood pressure by self-measurement (170/110 mmHg). The patient had never experienced aphasia, blurred vision and paresthesia, and for this reason he decided to go to the emergency department (ED.)

L. Naldi  M. De Stefano  A. Berni (&)  M. Torri  L. Poggesi Dipartimento di Medicina Sperimentale e Clinica, Universita` degli Studi di Firenze, Azienda Ospedaliero-Universitaria Careggi, Viale Morgagni, 85-50134 Florence, Italy e-mail: [email protected]

He arrived at the ED 90 min after the onset of symptoms. He appeared alert and oriented. The migraine had resolved. At that time, he did not have visual changes, aphasia, sensory loss, weakness, or constitutional symptoms. He reported no recent head or neck trauma. He was right-handed. On examination, the blood pressure was 160/105 mmHg symmetrically, the pulse 90 beats per minute, the temperature 36.5 °C, and the oxygen saturation 98 % on room air. A full neurological examination was unremarkable, and no pupillary asymmetry or ptosis was seen. A slight left carotid bruit was detectable. The rest of the general physical examination was normal. Laboratory testing on admission showed a mild increase of fasting glucose (121 mg/dL). A complete blood count, the results of renal and liver tests and plasma levels of electrolytes were normal. An electrocardiogram showed a normal sinus rhythm at a rate of 68 beats per minute.

Differential diagnosis Dr. Berni The patient was relatively young, but already had cardiovascular risk factors (hyperlipidemia, recently reported high values of blood pressure, and a first report of an impaired fasting glucose). He presented an association of acute and transient neurological manifestations (fluent aphasia, blurred vision of the left eye, paresthesia of the left hand), triggered by a typical episode of migraine with scintillating scotoma, the latter already experienced in the past. Neurological symptoms resolved after 10 min, but the migraine remained. The same neurological manifestation relapsed within a few minutes and disappeared together with migraine after NSAID. In his recent clinical history, several episodes of a different type of headache occurred, localized bilaterally in the occipital region.

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In a migraineur patient, recurrent attacks of reversible neurological symptoms associated with the migraine may simply represent a new type of aura during the headache. Nevertheless, transient neurological manifestations combined with headache should lead to a focus on a cerebrovascular event due to hemorrhagic (e.g., subarachnoid hemorrhage or parenchymal brain hemorrhage) or a thrombotic cause (e.g., carotid- or vertebral-artery dissection, venous sinus thrombosis, or ischemic brain lesion with hemorrhagic transformation, especially on cardioembolic etiology). Unusual cerebrovascular diseases with concomitant neurological deficit and headache may be due to a reversible cerebral vasoconstriction syndrome and primary angiitis of the central nervous system. Migraine followed by visual, sensory or motor neurological manifestations may be associated with a seizure and represent a pre-ictal migraine. Finally, hypertensive crises, brain mass, and meningitis/ encephalitis may also present with headache and neurological deficits.

Preliminary diagnosis Prof. Poggesi The computed tomography (CT) of the head was normal. The color duplex sonography of the neck showed a demodulation of the blood flow among the left extracranial internal carotid. The transthoracic echocardiography revealed an atrial septal aneurysm with a normal left ventricular systolic function. The patient was transferred to our department for further investigations. On account of the left carotid bruit and the related demodulation of the blood flow, a diagnostic procedure was performed. A CT angiography of the head and neck (Fig. 1) showed no alterations of the brain vessels and parenchyma, but revealed ectasia in the proximal portion of the internal carotid artery near its origin, followed by an irregular narrowing extending up to the skull base. Irregular focal expansion of the distal cervical left internal carotid artery with a kinking and a saccular ‘‘pseudoaneurysm’’ (15 9 9 mm) were present near the skull base. An intimal flap at the pre-petrosal tract was also present, without extension into the petrous carotid canal. The vertebral arteries were normal. These features were consistent with dissection of the extracranial left internal carotid artery with an associated ‘‘pseudoaneurysm’’.

Further investigations Dr. Naldi and Dr. De Stefano The electroencephalography showed no abnormalities. Transesophageal cardiac ultrasonography confirmed the presence of an atrial septal aneurysm and revealed a patent foramen ovale (PFO) with

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spontaneous intracardial right-to-left shunt. Color duplex sonography of the lower limbs was then performed, and showed deep vein thrombosis of the right segmental peroneal vein. There was no recent trauma of the legs or immobilization that had occurred, and lupus anticoagulant, antiphospholipid antibodies, and other coagulation genetic disorders were negative. During hospitalization, the diagnoses of arterial hypertension and diabetes mellitus were made. The patient reported no neck pain, headache or prodromic symptoms, but reported a continuous left ear pulsatile tinnitus. The neurological examination remained completely normal.

Diagnosis and therapy Dr. Torri and Dr. Berni CT angiography showed dissection of the extracranial left internal carotid artery with pseudoaneurysm at the pre-petrosal tract, symptomatic for recurrent neurological symptoms, occipital headache and continuous left ear pulsatile tinnitus. The vascular surgeon did not recommend surgical or endovascular procedures. Subcutaneous low-molecular-weight heparin at anticoagulant dose was started, followed by warfarin with a range of international normalized ratio (INR) 2.0-3.0. The patient was advised to avoid forceful neck movements, and we recommended the continuation of warfarin at least until the repeat of the CT angiography of the head and neck at three months could be performed.

Discussion Dr. Torri Spontaneous (non-traumatic) cervical artery dissection (SCAD) is a relevant cause of transient ischemic attack (TIA) and stroke in young and middle-aged patients. Indeed, although SCAD accounts for only about 2 % of all ischemic strokes, its prevalence rises up to 10–24 % in patients between 15 and 45 years of age [1–3]. SCAD is characterized by mural hematoma located in the arterial wall, which usually arises from an intimal tear. Less commonly, a medial or subadventitial dissection causing an intramural hematoma may occur. The most frequent involved vessel is the extracranial segment of internal carotid artery— typically 2 cm above the bifurcation—which is affected about twice as often as vertebral artery [4–6]. The subintimal dissection may cause a stenosis of the arterial lumen, whereas a subadventitial dissection may bring about an aneurysmal dilatation, the inappropriately so called ‘‘pseudoaneurysm’’. In fact, the walls of hematoma are composed of blood vessel elements (i.e., media and adventitia). Minor traumas and mechanical stress are important predisposing factors for SCAD, especially when they are

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Fig. 1 CT angiogram of the head and neck scan in axial plane (a), reformatted in coronal (b) and sagittal plane (c), demonstrates a saccular pseudoaneurysm (white arrowheads; 15 9 9 mm) of the distal cervical left internal carotid artery with an intimal flap (white arrow). Volume rendering image of cervical left internal carotid artery (d) shows: ectasia in the proximal portion of the internal carotid artery near its origin followed by (white arrowheads) an irregular narrowing extending up to the skull base; pseudoaneurysm and kinking near the skull base (white arrow)

characterized by sudden or high-speed hyperextension or rotation of the neck. Some underlying structural defects of the arterial wall make a vessel predisposed to dissection, such as vascular-type Ehlers-Danlos syndrome, Marfan’s syndrome, and type I osteogenesis imperfecta. Nevertheless, SCAD may also develop in regions of arterial loop and redundancies, as we observed in our patient, which had a kinking of the pre-petrosal tract of cervical left internal carotid artery. Carotid artery dissection should be suspected from the presence of local signs and symptoms, separately or in various combinations, which often precede cerebral ischemic manifestations. The typical presentation is ipsilateral pain in the head, face and neck, changeably associated to cranial nerve palsy (mainly hypoglossal nerve), pulsatile tinnitus and ocular sympathetic palsy, the latter referred to as a partial Horner’s syndrome (miosis and ptosis ipsilateral to the dissection without facial anhidrosis) [5].

Cerebral ischemic events occur in about 70 % of patients [5, 7] and are related to artery-to-artery embolism or to an impaired blood supply from a stenotic or occluded artery. The delay between local and ischemic manifestations can vary from a few minutes to several weeks, and is usually less than 1 month. In our case, transient episodes of aphasia, blurred vision of the left eye and paresthesia of the left hand presented 2 weeks after the beginning of occipital headache. Headache is the most common symptom of carotid artery dissection, since it is reported in about 70 % of patients and is frequently the earliest [5, 7]. In most cases, headache occurs ipsilateral to the dissection, mainly in the frontal or fronto-temporal area. Occipital headache is an uncommon localization, which was reported by our patient. The onset is usually gradual, and the quality is frequently continuous, although occasionally throbbing. Indeed, he reported several episodes of moderate subcontinuous

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bilateral occipital headache, which differed from the usual migraine for the localization, the intensity and the absence of aura. Nevertheless, about 36 % of patients with SCAD have a history of migraine [5, 7, 8]. Accordingly, Rist et al. [9] report that patients with migraine have a twofold increased risk of SCAD, independently of the presence of aura. Moreover, during the acute phase of ischemic stroke and TIA due to SCAD, headache is reported more frequently in migraneurs than in non-migraneurs patients [8], but only about a fourth of migraineur patients consider the headache to be similar to the previous migraine [10]. Diagnosis of SCAD requires that one think of the diagnosis and search for it aggressively. Imaging plays a major role because the cornerstone of diagnosis is based on direct visualization of the intramural hematoma and narrowing or occlusion of the arterial lumen. In our patient, because of the recurrent ischemic manifestations and the occipital headache, an extracranial color duplex sonography was performed initially, and revealed a markedly reduced blood flow velocity of the left internal carotid artery. The reported sensitivity of neurovascular (extracranial and intracranial) color duplex ultrasound varies from 80 to 96 % in the ability to detect symptomatic dissection of internal carotid artery [11, 12]. The accuracy may not be optimal when SCAD affects anatomic sites difficult to assess by ultrasound or when intramural hematoma is too small to cause hemodynamically significant stenosis. Nevertheless, when SCAD is clinically suggested, in particular when hemodynamic information of ultrasonography is not clear, confirmatory testing with CT angiography or magnetic resonance angiography is mandatory. Multisection CT angiography provides useful information about the arterial lumen and vessel wall using contrast and maximum intensity projection or multiplanar performation technique. Although intimal flap or a double lumen is a pathognomonic feature of SCAD, a long tapered stenosis or a dissection aneurysm (the improperly so called ‘‘pseudoaneurysm’’) is a typical finding [6]. As in our case, stenosis usually starts about 2 or 3 cm distal to the carotid bulb and shows an aneurysmal dilatation at the distal subcranial segment. Cervical magnetic resonance imaging is able to directly show intramural hematoma using the T1 fat suppression technique, in which it appears as hyperintense area surrounding the vessel lumen. To date, the choice of appropriate therapy for SCAD with ischemic symptoms remains controversial, as carotid dissections can heal on their own. Dissection and ischemia recurrence are very low (1–5 %) and recanalization occurs in a large proportion of patients (82 %) within the first year. However, based on the putative embolic mechanism,

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the treatment of choice is anticoagulation with heparin followed by vitamin K antagonists (INR therapeutic range between 2.0 and 3.0). The duration of anticoagulation is at least 3 months. At that time, a follow-up imaging is recommended to determine the continuation of therapy. Anticoagulation is continued for another 3 months in case of residual luminal irregularity. Then, in case of persistent arterial abnormalities a shift to long-term antiaggregant is suggested. Initiation of anticoagulant treatment is not indicated in patients with severe stroke—e.g., National Institutes of Health Stroke Scale (NHISS) C15—or in the presence of intracranial aneurysm or dissection. In these cases, platelet aggregation inhibitors have to be considered. On the other hand, antiplatelet treatment is otherwise indicated for SCAD without ischemic symptoms. When anticoagulation is contraindicated or medical management of SCAD is insufficient (e.g., recurrent ischemic symptoms despite optimum medical treatment), invasive interventions such as surgery or endovascular therapy can be considered in highly selected patients. Prof. Poggesi Nevertheless, our migraneur patient presented a potential concomitant indication to anticoagulation treatment. In the absence of SCAD, ischemic symptoms may be attributed to paradoxical embolism because of the presence of PFO with deep venous thrombosis. The transesophageal echocardiography showed PFO with spontaneous intracardial right-to-left shunt and atrial septal aneurysm. PFO is an often asymptomatic slit-like interatrial defect that is quite common in the general population, with a prevalence of approximately 25 %. Atrial septal aneurysm is a congenital abnormality of the interatrial septum characterized by a redundant central part of the septum primum. An association between migraine and PFO has been widely described. The main hypothesis is that intracardiac right-to-left shunt of vasoactive substances or paradoxical microembolism may trigger migraine attack. Unfortunately, conflicting data regarding causal relationship between the two conditions are also reported. Garg et al. [13] report the same prevalence of PFO (about 26 %) between migraneurs with or without aura and healthy subjects. PFO has also been recognized as a potential mediator of cerebral ischemic events. Atrial septal aneurysm may itself facilitate paradoxical embolism by leading to a more frequent and wider opening of the PFO channel. Nowadays, the association between any stroke and PFO or atrial septal aneurysm is not causative, but still probabilistic, because of the difficulties of the differential diagnosis among paradoxical embolism, in situ thrombosis, and transient atrial dysrhythmia [14]. Nevertheless, in case of cryptogenetic TIA/stroke, PFO with or without atrial septal aneurysm, or

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atrial septal aneurysm alone, should be considered as potential sources of cardioembolism, especially in adults \50 years. Management of patients with cryptogenic TIA/stroke and PFO is provided by antiplatelet therapy. Oral anticoagulant therapy with vitamin K antagonist should be considered in the presence of specific conditions (e.g., recurrent ischemic events, coexisting atrial septal aneurysm, venous thromboembolism) [14]. Moreover, the coexistence of at least one anatomical risk factor, such as atrial septal aneurysm, basal right-left shunt and PFO [4 mm, or at least one clinical risk factor, such as recurrent ischemic events, deep vein thrombosis and multiple ischemic lesions, makes percutaneous closure of PFO an alternative to medical therapy. Percutaneous closure of PFO should be considered in case of ischemic events occurring during antithrombotic therapy [14]. In conclusion, in our case recurrent transient ischemic manifestations were undoubtedly due to internal carotid artery dissection. Nevertheless, the same case history could be explained by paradoxical embolism. Oral anticoagulant therapy is currently recommended for TIA and ischemic stroke due to both causes. On the other hand, the duration of oral anticoagulant therapy remains controversial, since discontinuing it after 6 months is recommended only for internal carotid artery dissection. Conflict of interest

None.

References 1. Putaala J, Metso AJ, Metso TM, Konkola N, Kraemer Y, Haapaniemi E, Kaste M, Tatlisumak T (2009) Analysis of 1,008 consecutive patients aged 15 to 49 with first-ever ischemic stroke: the Helsinki young stroke registry. Stroke 40:1195–1203 2. Ji R, Schwamm LH, Pervez MA, Singhal AB (2013) Ischemic stroke and transient ischemic attack in young adults. Risk factors, diagnostic yield, neuroimaging, and thrombolysis. JAMA Neurol 70:51–57 3. Nedeltchev K, Der Maur TA, Georgiadis D, Arnold M, Caso V, Mattle HP, Schroth G, Remonda L, Sturzenegger M, Fischer U, Baumgartner RW (2005) Ischaemic stroke in young adults: predictors of outcome and recurrence. J Neurol Neurosurg Psychiatry 76:191–195 4. Debette S, Leys D (2009) Cervical-artery dissections: predisposing factors, diagnosis, and outcome. Lancet Neurol 8:668–678

205 5. Von Babo M, De Marchis GM, Sarikaya H, Stapf C, Buffon F, Fischer U, Heldner MR, Gralla J, Jung S, Goeggel Simonetti B, Mattle HP, Baumgartner RW, Bousser MG, Arnold M (2013) Dissections of the internal carotid artery and the vertebral artery. Stroke 44:1537–1542 6. Schievink WI (2001) Spontaneous dissection of the carotid and vertebral arteries. N Engl J Med 344:898–906 7. Debette S, Grond-Ginsbach C, Bodenant M, Kloss M, Engelter S, Metso T, Pezzini A, Brandt T, Caso V, Touze´ E, Metso A, Canaple S, Abboud S, Giacalone G, Lyrer P, Del Zotto E, Giroud M, Samson Y, Dallongeville J, Tatlisumak T, Leys D, Martin JJ, For the Cervical Artery Dissection Ischemic Stroke Patients (CADISP) Group (2011) Differential features of carotid and vertebral artery dissections. The CADISP study. Neurology 77:1174–1181 8. Metso TM, Tatlisumak T, Debette S, Dallongeville J, Engelter ST, Lyrer PA, Thijs V, Bersano A, Abboud S, Leys D, GrondGinsbach C, Kloss M, Touze´ E, Pezzini A, Metso AJ, CADISP group (2012) Migraine in cervical artery dissection and ischemic stroke patients. Neurology 78:1221–1228 9. Rist PM, Diener HC, Kurth T, Schu¨rks M (2011) Migraine, migraine aura, and cervical artery dissection: a systematic review and meta-analysis. Cephalalgia 31:886–896 10. Silbert PL, Mokri B, Schievink WI (1995) Headache and neck pain in spontaneous internal carotid and vertebral artery dissections. Neurology 45:1517–1522 11. Benninger DH, Georgiadis D, Gandjour J, Baumgartner RW (2006) Accuracy of color duplex ultrasound diagnosis of spontaneous carotid dissection causing ischemia. Stroke 37:377–381 12. Dittrich R, Dziewas R, Ritter MA, Kloska SP, Bachmann R, Nassenstein I, Kuhlenbaumer G, Heindel W, Ringelstein EB, Nabavi DG (2006) Negative ultrasound findings in patients with cervical artery dissection. Negative ultrasound in CAD. J Neurol 253:424–433 13. Garg P, Servoss SJ, Wu JC, Bajwa ZH, Selim MH, Dineen A, Kuntz RE, Cook EF, Mauri L (2010) Lack of association between migraine headache and patent foramen ovale. Results of a casecontrol study. Circulation 121:1406–1412 14. Pristipino C, Anzola GP, Ballerini L, Bartorelli A, Cecconi M, Chessa M, Donti A, Gaspardone A, Neri G, Onorato E, Palareti G, Rakar S, Rigatelli G, Santoro G, Toni D, Ussia GP, Violini R, on behalf of: Italian Society of Invasive Cardiology (SICI-GISE); Italian Stroke Association (ISA-AIS); Italian Association of Hospital Neurologists, Neuroradiologists, Neurosurgeons (SNO); Congenital Heart Disease Study Group of Italian Society Of Cardiology; Italian Association Of Hospital Cardiologists (ANMCO); Italian Society Of Pediatric Cardiology (SICP); Italian Society of Cardiovascular Echography (SIEC); Italian Society of Hemostasis and Thrombosis (SISET) (2013) Management of patients with patent foramen ovale and cryptogenic stroke: a collaborative, multidisciplinary, position paper: executive summary. Catheter Cardiovasc Interv 82:122–129

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A challenging multifactorial migraine with aura.

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