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Table of Contents
CASE REPORT
Year : 2020  |  Volume : 16  |  Issue : 3  |  Page : 124-126

Posterior reversible encephalopathy syndrome due to autonomic dysreflexia in medullary infarction


1 Division of Stroke Centre of Neurosciences, Amrita Institute Medical Sciences, Kochi, Kerala, India
2 Department of Neurology, Amrita Institute Medical Sciences, Kochi, Kerala, India
3 Department of Health Sciences Research, Amrita Institute Medical Sciences, Kochi, Kerala, India

Date of Submission03-Mar-2020
Date of Acceptance14-Mar-2020
Date of Web Publication09-Oct-2020

Correspondence Address:
Dr. Remya Sudevan
Department of Health Sciences Research, Amrita institute of Medical Sciences, Ponekkara, Kochi - 682 041, Kerala
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/AMJM.AMJM_20_20

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  Abstract 


The simultaneous occurrence of cervicocephalic arterial dissection and posterior reversible encephalopathy syndrome (PRES) is a rare entity. A healthy 33-year-old male with no known comorbidities had sudden onset pain in the neck, while he was doing strenuous physical labor. Over the next 2 days, he developed symptoms such as swaying to either side while walking, numbness on the left side of his body, painless progressive bilateral blurring of vision, and drooping of bilateral eyelids. On arrival at the emergency room, he was drowsy with a Glasgow Coma Score of 14 and complained of headache in addition to the preexisting symptoms. He also reported that his entire visual field was clouded and dark. On examination, he was detected to have a blood pressure of 220/120 mmHg with bilateral mild ptosis and nonreactive pupils. He had cortical blindness at the time of admission. He had significant gait and truncal ataxia but no other cerebellar signs. Motor system examination was unremarkable and plantars were flexor. No objective sensory deficit was demonstrable. Magnetic resonance imaging showed acute infarct in the vermis with vertebral artery dissection at V4 extending to the basilar artery. In addition, there were diffuse T2-hyperintensities in the bilateral occipital areas without restricted diffusion suggestive of posterior reversible encephalopathy syndrome [Figure 1]. He was admitted to the intensive care unit; the high blood pressure was treated aggressively with labetalol and hydralazine. Gradually, his vision improved over 48 h and back to normal acuity by 5 days. His magnetic resonance angiography with the neck vessels showed a dissection of the vertebral artery. His symptoms of medullary infarct persisted for a few weeks and then resolved completely. Young brainstem stroke with neck pain points to vertebral artery dissection. Concurrent worsening with autonomic fluctuation can be due to associated PRES and is to be considered as this situation is eminently treatable with good control of blood pressure at the earliest.

Keywords: Autonomic dysreflexia, medullary infarction, posterior reversible encephalopathy syndrome


How to cite this article:
Nambiar K V, Nair JN, Ajai V A, Sudevan R. Posterior reversible encephalopathy syndrome due to autonomic dysreflexia in medullary infarction. Amrita J Med 2020;16:124-6

How to cite this URL:
Nambiar K V, Nair JN, Ajai V A, Sudevan R. Posterior reversible encephalopathy syndrome due to autonomic dysreflexia in medullary infarction. Amrita J Med [serial online] 2020 [cited 2020 Oct 30];16:124-6. Available from: https://www.ajmonline.org.in/text.asp?2020/16/3/124/297552




  Introduction Top


The simultaneous occurrence of cervicocephalic arterial dissection and posterior reversible encephalopathy syndrome (PRES) is a rare entity and has mostly been described in the literature as a postpartum complication. We describe a case of vertebral artery dissection in a young male with concurrent PRES.


  Case Report Top


A previously healthy 33-year-old male with no known comorbidities was brought to the emergency room 3 days after sudden onset pain in the neck which started, while he was helping the construction of a well by carrying the mud from the partly dug well with repeated neck movements. He ignored the pain and attributed it to the unaccustomed work. Over the next 2 days his symptoms worsened in the form of swaying to either side while walking, numbness on the left side of his body, painless progressive bilateral blurring of vision, and drooping of both eyelids. These worsening symptoms prompted him to seek help at a nearby hospital from where he was transferred to our care. His past medical and surgical history were unremarkable except for a hernia repair he underwent a few years ago. He had no significant family history either. On arrival at the emergency room, he was drowsy with a Glasgow Coma Score of 14 and complained of a headache in addition to the preexisting symptoms. He also reported that his entire visual field was clouded and dark. On examination, he was detected to have a blood pressure of 220/120 mmHg with bilateral mild ptosis and nonreactive pupils. He had cortical blindness at the time of admission. He had significant gait and truncal ataxia but no other cerebellar signs. Motor system exam was unremarkable and plantar were flexor. No objective sensory deficit was demonstrable. Magnetic resonance imaging showed acute infarct in the vermis with vertebral artery dissection at V4 extending to the basilar artery. In addition, there were diffuse T2-hyperintensities in the bilateral occipital areas without restricted diffusion suggestive of posterior reversible encephalopathy syndrome [Figure 1]. He was admitted to the intensive care unit; the high blood pressure was treated aggressively with labetalol and hydralazine. Gradually, his vision improved over 48 h and back to normal acuity by 5 days. His magnetic resonance angiography with the neck vessels showed a dissection of the vertebral artery. His symptoms of medullary infarct persisted for a few weeks and then resolved completely.
Figure 1: Magnetic resonance imaging image showing posterior reversible encephalopathy syndrome. Acute infarct in the vermis, diffuse T2-hyper intensities in the bilateral occipital areas without restricted diffusion suggestive of posterior reversible encephalopathy syndrome

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  Discussion Top


Arterial dissection is not an uncommon cause of stroke in the young. These can be post-traumatic after a minor trauma or may be spontaneous as in many inherited connective tissue disorders such as Ehlers-Danlos syndrome or Marfan syndrome.[1] Many arterial dissections may be asymptomatic but a significant proportion will cause arterial thrombosis and embolism to the distal arteries.[2] Vertebral artery dissection in the current patient resulted in medullary infarcts. Brain stem infarcts can cause dysfunction in the baroreflex mechanisms and can lead to uncontrollable hypertension.[3] The present patient developed vertebral dissection due to the unaccustomed exertion involving neck which eventually led to cerebellar and medullary infarct subsequently leading to autonomic fluctuations and high blood pressures. These high blood pressures resulted in poor autoregulation and endothelial dysfunction with PRES in both occipital regions. Occipital areas are the most common locations for the syndrome due to the poor sympathetic innervation of the occipital lobes.[4] The present patient had a headache and visual disturbances with radiological features showing vasogenic subcortical edema, the features suggestive of PRES.[5] PRES has been described in autonomic dysreflexia in patients of traumatic quadriplegia.[6] There are existing case reports of association of PRES and cervicocephalic arterial dissection as a postpartum complication and is attributed to the common risk factors.[7] One previous report raises the possibility that dissection may be the cause of the PRES due to impaired baroreflex mechanisms. Paroxysmal hypertension can lead to disruption in cerebral autoregulation in the posterior region and can cause vasogenic edema and reversible ischemia.[8] Even though PRES is reversible in many cases it causes infarction in several patients due to prolonged vasoconstriction and endothelial damage. Here, the prompt control of the blood pressure had brought the complete clinical and radiological resolution.


  Conclusion Top


Young brainstem stroke with neck pain points to vertebral artery dissection. Concurrent worsening with autonomic fluctuation can be due to associated PRES and is to be considered as this situation is eminently treatable with good control of blood pressure at the earliest.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Mellion ML, Rizvi S. Spontaneous bilateral carotid artery dissection and posterior reversible encephalopathy syndrome. Neurology 2005;65:1990.  Back to cited text no. 1
    
2.
Biffl WL, Moore EE, Offner PJ, Brega KE, Franciose RJ, Elliott JP, et al. Optimizing screening for blunt cerebrovascular injuries. Am J Surg 1999;178:517-22.  Back to cited text no. 2
    
3.
Phillips AM, Jardine DL, Parkin PJ, Hughes T, Ikram H. Brain stem stroke causing baroreflex failure and paroxysmal hypertension. Stroke 2000;31:1997-2001.  Back to cited text no. 3
    
4.
Caplan LR, Biousse V. Cervicocranial arterial dissections. J Neuroophthalmol 2004;24:299-305.  Back to cited text no. 4
    
5.
Iso T, Hamamori Y, Kedes L. Notch signaling in vascular development. Arterioscler Thromb Vasc Biol 2003;23:543-53.  Back to cited text no. 5
    
6.
Matias AC, Rocha J, Cerqueira ME, Pereira JM. Autonomic dysreflexia and posterior reversible encephalopathy syndrome. Am J Phys Med Rehabil 2013;92:453-8.  Back to cited text no. 6
    
7.
Arnold M, Camus-Jacqmin M, Stapf C, Ducros A, Viswanathan A, Berthet K, et al. Postpartum cervicocephalic artery dissection. Stroke 2008;39:2377-9.  Back to cited text no. 7
    
8.
Fugate JE, Claassen DO, Cloft HJ, Kallmes DF, Kozak OS, Rabinstein AA. Posterior reversible encephalopathy syndrome: Associated clinical and radiologic findings. Mayo Clin Proc 2010;85:427-32.  Back to cited text no. 8
    


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