Case Report
Painless Hypoglossal Palsy as an Isolated Symptom of Spontaneous Carotid Dissection J. M. Hennings, MD,* D. Hoehn, MD,† E. Schumann-Spaeth, MD,† and F. Weber, MD*
Spontaneous internal carotid artery dissection (sICAD) occurs annually in 2.5 to 3 per 100,000 presenting with signs of ischemic events in the majority of cases. In contrast, lower cranial nerve palsy due to peripheral nerve affection is seldom the presenting clinical sign. In symptomatic cases (>90%), sICAD is most commonly accompanied by local pain. We report a case of a 49-year old woman with a left sICAD presenting with isolated ipsilateral hypoglossal palsy as the sole clinical sign. Compared to other cases, local pain was absent and other cranial nerves were not affected. Further, sICAD could not be detected in repeated Doppler-/ Duplex-sonography, but magnetic resonance imaging and MR-angiography only. Key Words: Hypoglossal palsy—carotid dissection—ICAD—magnetic resonance imaging—cranial nerve—beauty parlor stroke syndrome. Ó 2014 by National Stroke Association
Spontaneous internal carotid artery dissection (sICAD) occurs annually in 2.5-3 per 100,0001 presenting with signs of ischemic events in most cases. In contrast, lower cranial nerve palsy because of peripheral nerve affection is seldom the presenting clinical sign. In symptomatic cases (.90%), spontaneous ICAD is most commonly accompanied by local pain. The 49-year-old woman, a habitual cigarette smoker, presented to the emergency room with a 10-day history of progressive weakness of tongue motility. On protrusion of the tongue, a pseudohypertrophia of the left side was observed, and movement to the left side as well as swallowing and articulation was impaired (Fig 1, A-C). No further clinical symptoms were present including cerFrom the *Section of Neurology, Max Planck Institute of Psychiatry, Munich; and †Neuroimaging Research Group, Max Planck Institute of Psychiatry, Munich, Germany. Received December 2, 2013; revision received January 30, 2014; accepted February 1, 2014. The authors have no conflict of interest. Address correspondence to J.M. Hennings, MD, Max Planck Institute of Psychiatry, Kraepelinstr. 2-10, 80804 Munich, Germany. E-mail: [email protected]. 1052-3057/$ - see front matter Ó 2014 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2014.02.001
vical, cranial, or facial. Beside a visit to a hairdresser, where her head had been lying reclined in a washing basin for about 30 minutes a few days before symptom onset, the patient denied any further manipulation or trauma of the head or neck. Repeated ultrasound examinations did not reveal any signs of cervical artery dissection. Only magnetic resonance imaging lead to the identification of a dissection of the left ICA closely related to the skull base (Fig 1, DF). A blood screen for vasculitis was normal (including human immunodeficiency virus testing, erythrocyte sedimentation rate, rheumatoid factor, C-reactive protein, antinuclear antibodies, perinuclear and cytoplasmic antineutrophil cytoplasmic antibodies, antibodies against double-stranded DNA, myeloperoxidase, and proteinase 3). A dissection of the renal arteries as a sign of fibromuscular dysplasia was excluded by sonography and there were no clinical signs for connective tissue disease (such as Ehlers–Danlos or Marfan’s syndrome). Although a major trauma was not reported in the present case, arterial dissections and stroke have been reported even after minor neck extensions like after shampooing at a hairdresser, known as beauty parlor stroke syndrome,2 which might have played a role in our patient too. Blood pressure was normal throughout the clinical observation
Journal of Stroke and Cerebrovascular Diseases, Vol. -, No. - (---), 2014: pp 1-3
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J.M. HENNINGS ET AL.
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Figure 1. Left hypoglossal palsy caused pseudohypertrophia of the left tongue (A) and impaired movement to the left side (B), whereas movement to the unaffected side was normal (C). sICAD (arrow) visualized by coronal T1-weighted fast spin-echo (D) and axial T2-weighted fast spin-echo proton density (E) magnetic resonance imaging scans as well as gadolinium-enhanced MRA (F) performed in a 1.5-T Signa LX (GE Healthcare, Milwaukee, WI). Note the double lumen caused by the pseudoaneurysm and the local effect of mass close to the skull base (D, E). In follow-up MRA scans after 3 weeks (G), 3 months (H), and 5 months (I) a new dissection in the V3 segment of the left vertebral artery (asterisk) was detectable. Although of reduced size, the sICAD still showed a prefunded second lumen in time-of-flight MRA after 5 months (J). Abbreviations: MRA, magnetic resonance angiography; sICAD, spontaneous cervical internal carotid artery dissection.
period. Nevertheless, in a control magnetic resonance angiography after 3 weeks, a new spontaneous but clinically asymptomatic dissection of the left vertebral artery close to the arch of the atlas was observed (Fig 1, G-J). Under oral anticoagulation with phenprocoumon (international standardized ratio 2:3), hypoglossal palsy remitted within 2 months and pseudo-aneurysms were reduced in size. Particular to the present case is that no typical clinical signs of cerebral artery dissection such as pain, carotid territory ischemia, or Horner syndrome were present, and dissection was restricted to an anatomic region that was not accessible to ultrasound diagnostics. Other cases3-7 of isolated hypoglossal palsy and sICAD have at least reported local pain suggestive of cervical artery dissection. Furthermore, a second, clinically asymptomatic dissection of the left vertebral artery occurred within 3 weeks in the absence of any predisposing condition. Of note, with better availability and more frequent use of noninvasive neurovascular imaging, even high located dissections of the carotids and vertebral arteries or dissections of the intracranial vessels are increasingly recognized.1,8
Spontaneous multiple cervical artery dissections or dissections in a short time frame occur in 15%-25% of patients, but numbers might be underestimated owing to frequent spontaneous recanalization and possible oligosymptomatic presentation.7,8 There is no consensus for an optimal treatment approach of sICAD, and a recent meta-analysis did not find superiority of anticoagulation or antiplatelet therapy in prevention of stroke after carotid and vertebral artery dissection.9 In summary, in patients with unilateral lower cranial nerve palsy, sICAD should be actively excluded even if further clinical signs suggestive of cervical artery dissection are absent. Acknowledgment: The authors thank Gudrun Kochinke and Reinhold Borschke for excellent technical assistance.
References 1. Redekop G. Extracranial carotid and vertebral artery dissection: a review. Can J Neurol Sci 2008;35:146-152. 2. Weintraub MI. Beauty parlor stroke syndrome: report of five cases. JAMA 1993;269:2085-2086.
HYPOGLOSSAL PALSY AND SPONTANEOUS CAROTID DISSECTION 3. Spitzer C, Mull M, T€ opper R. Isolated hypoglossal nerve palsy caused by carotid artery dissection the necessity of MRI for diagnosis. J Neurol 2001;248:909-910. 4. Marin LF, Bichuetti DB, Felıcio AC, et al. Hypoglossal nerve palsy as the sole manifestation of spontaneous internal carotid artery dissection. Arq Neuropsiquiatr 2009;67:107-108. 5. Ursekar MA, Singhal BS, Konin BL. Hypoglossal nerve palsy due to spontaneous dissection of the internal carotid artery. Clin Radiol 2000;55:978-979. 6. Lieschke GJ, Davis S, Tress BM, et al. Spontaneous internal carotid artery dissection presenting as hypoglossal nerve palsy. Stroke 1988;19:1151-1155.
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7. Ferlazzo E, Gasparini S, Arcudi L, et al. Isolated hypoglossal nerve palsy due to spontaneous carotid artery dissection: a neuroimaging study. Neurol Sci 2013. Epub ahead of print. 8. Ono H, Nakatomi H, Tsutsumi K, et al. Symptomatic recurrence of intracranial arterial dissections: follow-up study of 143 consecutive cases and pathological investigation. Stroke 2013;44:126-131. 9. Kennedy F, Lanfranconi S, Hicks C, et al. Antiplatelets vs anticoagulation for dissection: CADISS nonrandomized arm and meta-analysis. Neurology 2012;79: 686-689.
Painless Hypoglossal Palsy as an Isolated Symptom of Spontaneous Carotid Dissection J. M. Hennings, MD,* D. Hoehn, MD,† E. Schumann-Spaeth, MD,† and F. Weber, MD*
Spontaneous internal carotid artery dissection (sICAD) occurs annually in 2.5 to 3 per 100,000 presenting with signs of ischemic events in the majority of cases. In contrast, lower cranial nerve palsy due to peripheral nerve affection is seldom the presenting clinical sign. In symptomatic cases (>90%), sICAD is most commonly accompanied by local pain. We report a case of a 49-year old woman with a left sICAD presenting with isolated ipsilateral hypoglossal palsy as the sole clinical sign. Compared to other cases, local pain was absent and other cranial nerves were not affected. Further, sICAD could not be detected in repeated Doppler-/ Duplex-sonography, but magnetic resonance imaging and MR-angiography only. Key Words: Hypoglossal palsy—carotid dissection—ICAD—magnetic resonance imaging—cranial nerve—beauty parlor stroke syndrome. Ó 2014 by National Stroke Association
Spontaneous internal carotid artery dissection (sICAD) occurs annually in 2.5-3 per 100,0001 presenting with signs of ischemic events in most cases. In contrast, lower cranial nerve palsy because of peripheral nerve affection is seldom the presenting clinical sign. In symptomatic cases (.90%), spontaneous ICAD is most commonly accompanied by local pain. The 49-year-old woman, a habitual cigarette smoker, presented to the emergency room with a 10-day history of progressive weakness of tongue motility. On protrusion of the tongue, a pseudohypertrophia of the left side was observed, and movement to the left side as well as swallowing and articulation was impaired (Fig 1, A-C). No further clinical symptoms were present including cerFrom the *Section of Neurology, Max Planck Institute of Psychiatry, Munich; and †Neuroimaging Research Group, Max Planck Institute of Psychiatry, Munich, Germany. Received December 2, 2013; revision received January 30, 2014; accepted February 1, 2014. The authors have no conflict of interest. Address correspondence to J.M. Hennings, MD, Max Planck Institute of Psychiatry, Kraepelinstr. 2-10, 80804 Munich, Germany. E-mail: [email protected]. 1052-3057/$ - see front matter Ó 2014 by National Stroke Association http://dx.doi.org/10.1016/j.jstrokecerebrovasdis.2014.02.001
vical, cranial, or facial. Beside a visit to a hairdresser, where her head had been lying reclined in a washing basin for about 30 minutes a few days before symptom onset, the patient denied any further manipulation or trauma of the head or neck. Repeated ultrasound examinations did not reveal any signs of cervical artery dissection. Only magnetic resonance imaging lead to the identification of a dissection of the left ICA closely related to the skull base (Fig 1, DF). A blood screen for vasculitis was normal (including human immunodeficiency virus testing, erythrocyte sedimentation rate, rheumatoid factor, C-reactive protein, antinuclear antibodies, perinuclear and cytoplasmic antineutrophil cytoplasmic antibodies, antibodies against double-stranded DNA, myeloperoxidase, and proteinase 3). A dissection of the renal arteries as a sign of fibromuscular dysplasia was excluded by sonography and there were no clinical signs for connective tissue disease (such as Ehlers–Danlos or Marfan’s syndrome). Although a major trauma was not reported in the present case, arterial dissections and stroke have been reported even after minor neck extensions like after shampooing at a hairdresser, known as beauty parlor stroke syndrome,2 which might have played a role in our patient too. Blood pressure was normal throughout the clinical observation
Journal of Stroke and Cerebrovascular Diseases, Vol. -, No. - (---), 2014: pp 1-3
1
J.M. HENNINGS ET AL.
2
Figure 1. Left hypoglossal palsy caused pseudohypertrophia of the left tongue (A) and impaired movement to the left side (B), whereas movement to the unaffected side was normal (C). sICAD (arrow) visualized by coronal T1-weighted fast spin-echo (D) and axial T2-weighted fast spin-echo proton density (E) magnetic resonance imaging scans as well as gadolinium-enhanced MRA (F) performed in a 1.5-T Signa LX (GE Healthcare, Milwaukee, WI). Note the double lumen caused by the pseudoaneurysm and the local effect of mass close to the skull base (D, E). In follow-up MRA scans after 3 weeks (G), 3 months (H), and 5 months (I) a new dissection in the V3 segment of the left vertebral artery (asterisk) was detectable. Although of reduced size, the sICAD still showed a prefunded second lumen in time-of-flight MRA after 5 months (J). Abbreviations: MRA, magnetic resonance angiography; sICAD, spontaneous cervical internal carotid artery dissection.
period. Nevertheless, in a control magnetic resonance angiography after 3 weeks, a new spontaneous but clinically asymptomatic dissection of the left vertebral artery close to the arch of the atlas was observed (Fig 1, G-J). Under oral anticoagulation with phenprocoumon (international standardized ratio 2:3), hypoglossal palsy remitted within 2 months and pseudo-aneurysms were reduced in size. Particular to the present case is that no typical clinical signs of cerebral artery dissection such as pain, carotid territory ischemia, or Horner syndrome were present, and dissection was restricted to an anatomic region that was not accessible to ultrasound diagnostics. Other cases3-7 of isolated hypoglossal palsy and sICAD have at least reported local pain suggestive of cervical artery dissection. Furthermore, a second, clinically asymptomatic dissection of the left vertebral artery occurred within 3 weeks in the absence of any predisposing condition. Of note, with better availability and more frequent use of noninvasive neurovascular imaging, even high located dissections of the carotids and vertebral arteries or dissections of the intracranial vessels are increasingly recognized.1,8
Spontaneous multiple cervical artery dissections or dissections in a short time frame occur in 15%-25% of patients, but numbers might be underestimated owing to frequent spontaneous recanalization and possible oligosymptomatic presentation.7,8 There is no consensus for an optimal treatment approach of sICAD, and a recent meta-analysis did not find superiority of anticoagulation or antiplatelet therapy in prevention of stroke after carotid and vertebral artery dissection.9 In summary, in patients with unilateral lower cranial nerve palsy, sICAD should be actively excluded even if further clinical signs suggestive of cervical artery dissection are absent. Acknowledgment: The authors thank Gudrun Kochinke and Reinhold Borschke for excellent technical assistance.
References 1. Redekop G. Extracranial carotid and vertebral artery dissection: a review. Can J Neurol Sci 2008;35:146-152. 2. Weintraub MI. Beauty parlor stroke syndrome: report of five cases. JAMA 1993;269:2085-2086.
HYPOGLOSSAL PALSY AND SPONTANEOUS CAROTID DISSECTION 3. Spitzer C, Mull M, T€ opper R. Isolated hypoglossal nerve palsy caused by carotid artery dissection the necessity of MRI for diagnosis. J Neurol 2001;248:909-910. 4. Marin LF, Bichuetti DB, Felıcio AC, et al. Hypoglossal nerve palsy as the sole manifestation of spontaneous internal carotid artery dissection. Arq Neuropsiquiatr 2009;67:107-108. 5. Ursekar MA, Singhal BS, Konin BL. Hypoglossal nerve palsy due to spontaneous dissection of the internal carotid artery. Clin Radiol 2000;55:978-979. 6. Lieschke GJ, Davis S, Tress BM, et al. Spontaneous internal carotid artery dissection presenting as hypoglossal nerve palsy. Stroke 1988;19:1151-1155.
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7. Ferlazzo E, Gasparini S, Arcudi L, et al. Isolated hypoglossal nerve palsy due to spontaneous carotid artery dissection: a neuroimaging study. Neurol Sci 2013. Epub ahead of print. 8. Ono H, Nakatomi H, Tsutsumi K, et al. Symptomatic recurrence of intracranial arterial dissections: follow-up study of 143 consecutive cases and pathological investigation. Stroke 2013;44:126-131. 9. Kennedy F, Lanfranconi S, Hicks C, et al. Antiplatelets vs anticoagulation for dissection: CADISS nonrandomized arm and meta-analysis. Neurology 2012;79: 686-689.
