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Case ReportA 26-year-old female with Turner Syndrome (TS), treated with oestrogen replacement therapy, presented with vertigo, ataxia and paraesthesia of the left arm immediately following chiropractic neck manipulation. Magnetic resonance angiography revealed dissection and partial occlusion of the left vertebral artery at the C2/3 level with an associated acute left cerebellar infarct (Figure 1). A non-occlusive dissection involving the right subclavian artery extending 4cm superiorly into the proximal right vertebral artery was also identified (Figure 2). The patient was commenced on anticoagulant therapy and her symptoms resolved completely over the following 3 days. Figure 1: 3D Time of Flight MRA: Absent flow related enhancement V3/V4 segment of the left vertebral artery secondary to dissection (short arrows). Intramural haematoma right subclavian artery (long arrow). Figure 2: Axial proton density with fat suppression: Right vertebral crescentic intramural haematoma (short arrow) and flow void (long arrow) Case DiscussionTurner Syndrome, affecting 1:2,500 live female births, results from the absence or partial deletion of one X chromosome. Cardiovascular anomalies are the major cause of increased mortality in TS, with aortic dissection a well-recognised complication.1 However, arterial wall abnormalities in TS extend beyond the aorta, with demonstrable dilatation and intimal thickening of the carotid and brachial arteries.2 This vasculopathy may predispose TS patients to cervical arterial dissection from minimal trauma. There have been two previous case reports of spontaneous cervical artery dissection in TS, describing a bilateral vertebral artery dissection and unilateral carotid artery dissection respectively.3,4 The literature, and this case report, suggest TS patients should be advised against cervical manipulation and also support the recommendation that clinicians have a low threshold for requesting imaging studies of the cervical and intracerebral vasculature in TS patients presenting with focal neurological deficits.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Jonathan Stevenson, Wellington Hospital, Department of Medicine; Dr Steven Soule, Christchurch Hospital, Department of Endocrinology; Dr Philip Parkin, Christchurch Hospital, Department of Neurology; Dr David ONeill-Kerr, Christchurch Hospital, Department of Radiology

Acknowledgements

Correspondence

Dr Jonathan Stevenson, Department of Medicine, Wellington Hospital, Private Bag 7902, Wellington, New Zealand

Correspondence Email

jsastevenson@gmail.com

Competing Interests

- Elsheikh M, Dunger DB, Conway GS, Wass JA. 2002. Turners syndrome in adulthood. Endocr Rev 23:120-140 Ostberg JE, Donald AE, Halcox JP, Storry C, McCarthy C, Conway GS. 2005. Vasculopathy in Turner Syndrome: Arterial Dilatation and Intimal Thickening without Endothelial Dysfunction. J Clin Endocrinol Metab 90(9):5161-5166 Muscat P, Lidov M, Nahar T, Tuhrim S, Weinberger J. 2001. Vertebral Artery Dissection in Turners Syndrome: Diagnosis by Magnetic Resonance Imaging. J Neuroimaging 11:50-54 Fuentes K, Silveira DC, Papamitsakis NI. 2007. Spontaneous Carotid Artery Dissection in a Patient with Turner Syndrome. Cerebrovasc Dis 24(6):543-544-

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Case ReportA 26-year-old female with Turner Syndrome (TS), treated with oestrogen replacement therapy, presented with vertigo, ataxia and paraesthesia of the left arm immediately following chiropractic neck manipulation. Magnetic resonance angiography revealed dissection and partial occlusion of the left vertebral artery at the C2/3 level with an associated acute left cerebellar infarct (Figure 1). A non-occlusive dissection involving the right subclavian artery extending 4cm superiorly into the proximal right vertebral artery was also identified (Figure 2). The patient was commenced on anticoagulant therapy and her symptoms resolved completely over the following 3 days. Figure 1: 3D Time of Flight MRA: Absent flow related enhancement V3/V4 segment of the left vertebral artery secondary to dissection (short arrows). Intramural haematoma right subclavian artery (long arrow). Figure 2: Axial proton density with fat suppression: Right vertebral crescentic intramural haematoma (short arrow) and flow void (long arrow) Case DiscussionTurner Syndrome, affecting 1:2,500 live female births, results from the absence or partial deletion of one X chromosome. Cardiovascular anomalies are the major cause of increased mortality in TS, with aortic dissection a well-recognised complication.1 However, arterial wall abnormalities in TS extend beyond the aorta, with demonstrable dilatation and intimal thickening of the carotid and brachial arteries.2 This vasculopathy may predispose TS patients to cervical arterial dissection from minimal trauma. There have been two previous case reports of spontaneous cervical artery dissection in TS, describing a bilateral vertebral artery dissection and unilateral carotid artery dissection respectively.3,4 The literature, and this case report, suggest TS patients should be advised against cervical manipulation and also support the recommendation that clinicians have a low threshold for requesting imaging studies of the cervical and intracerebral vasculature in TS patients presenting with focal neurological deficits.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Jonathan Stevenson, Wellington Hospital, Department of Medicine; Dr Steven Soule, Christchurch Hospital, Department of Endocrinology; Dr Philip Parkin, Christchurch Hospital, Department of Neurology; Dr David ONeill-Kerr, Christchurch Hospital, Department of Radiology

Acknowledgements

Correspondence

Dr Jonathan Stevenson, Department of Medicine, Wellington Hospital, Private Bag 7902, Wellington, New Zealand

Correspondence Email

jsastevenson@gmail.com

Competing Interests

- Elsheikh M, Dunger DB, Conway GS, Wass JA. 2002. Turners syndrome in adulthood. Endocr Rev 23:120-140 Ostberg JE, Donald AE, Halcox JP, Storry C, McCarthy C, Conway GS. 2005. Vasculopathy in Turner Syndrome: Arterial Dilatation and Intimal Thickening without Endothelial Dysfunction. J Clin Endocrinol Metab 90(9):5161-5166 Muscat P, Lidov M, Nahar T, Tuhrim S, Weinberger J. 2001. Vertebral Artery Dissection in Turners Syndrome: Diagnosis by Magnetic Resonance Imaging. J Neuroimaging 11:50-54 Fuentes K, Silveira DC, Papamitsakis NI. 2007. Spontaneous Carotid Artery Dissection in a Patient with Turner Syndrome. Cerebrovasc Dis 24(6):543-544-

For the PDF of this article,
contact nzmj@nzma.org.nz

View Article PDF

Case ReportA 26-year-old female with Turner Syndrome (TS), treated with oestrogen replacement therapy, presented with vertigo, ataxia and paraesthesia of the left arm immediately following chiropractic neck manipulation. Magnetic resonance angiography revealed dissection and partial occlusion of the left vertebral artery at the C2/3 level with an associated acute left cerebellar infarct (Figure 1). A non-occlusive dissection involving the right subclavian artery extending 4cm superiorly into the proximal right vertebral artery was also identified (Figure 2). The patient was commenced on anticoagulant therapy and her symptoms resolved completely over the following 3 days. Figure 1: 3D Time of Flight MRA: Absent flow related enhancement V3/V4 segment of the left vertebral artery secondary to dissection (short arrows). Intramural haematoma right subclavian artery (long arrow). Figure 2: Axial proton density with fat suppression: Right vertebral crescentic intramural haematoma (short arrow) and flow void (long arrow) Case DiscussionTurner Syndrome, affecting 1:2,500 live female births, results from the absence or partial deletion of one X chromosome. Cardiovascular anomalies are the major cause of increased mortality in TS, with aortic dissection a well-recognised complication.1 However, arterial wall abnormalities in TS extend beyond the aorta, with demonstrable dilatation and intimal thickening of the carotid and brachial arteries.2 This vasculopathy may predispose TS patients to cervical arterial dissection from minimal trauma. There have been two previous case reports of spontaneous cervical artery dissection in TS, describing a bilateral vertebral artery dissection and unilateral carotid artery dissection respectively.3,4 The literature, and this case report, suggest TS patients should be advised against cervical manipulation and also support the recommendation that clinicians have a low threshold for requesting imaging studies of the cervical and intracerebral vasculature in TS patients presenting with focal neurological deficits.

Summary

Abstract

Aim

Method

Results

Conclusion

Author Information

Jonathan Stevenson, Wellington Hospital, Department of Medicine; Dr Steven Soule, Christchurch Hospital, Department of Endocrinology; Dr Philip Parkin, Christchurch Hospital, Department of Neurology; Dr David ONeill-Kerr, Christchurch Hospital, Department of Radiology

Acknowledgements

Correspondence

Dr Jonathan Stevenson, Department of Medicine, Wellington Hospital, Private Bag 7902, Wellington, New Zealand

Correspondence Email

jsastevenson@gmail.com

Competing Interests

- Elsheikh M, Dunger DB, Conway GS, Wass JA. 2002. Turners syndrome in adulthood. Endocr Rev 23:120-140 Ostberg JE, Donald AE, Halcox JP, Storry C, McCarthy C, Conway GS. 2005. Vasculopathy in Turner Syndrome: Arterial Dilatation and Intimal Thickening without Endothelial Dysfunction. J Clin Endocrinol Metab 90(9):5161-5166 Muscat P, Lidov M, Nahar T, Tuhrim S, Weinberger J. 2001. Vertebral Artery Dissection in Turners Syndrome: Diagnosis by Magnetic Resonance Imaging. J Neuroimaging 11:50-54 Fuentes K, Silveira DC, Papamitsakis NI. 2007. Spontaneous Carotid Artery Dissection in a Patient with Turner Syndrome. Cerebrovasc Dis 24(6):543-544-

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