CASE REPORT

Hypertensive encephalopathy as a late complication of autonomicdysreflexia in a 12-year-old boy with a previous spinal cord injury

Bojko Bjelakovic & Lidija Dimitrijevic & Stevo Lukic &

Emilija Golubovic

Received: 12 December 2013 /Revised: 25 January 2014 /Accepted: 30 January 2014# Springer-Verlag Berlin Heidelberg 2014

Abstract The scope of paediatric autonomic disorders is notwell recognised, and paediatricians seem to be generally un-aware of the complexity and diversity of their clinical mani-festations. We report a 12-year-old boy presenting with hy-pertensive encephalopathy caused by autonomic dysreflexia.Conclusion: This observation emphasises the importance ofthe recognition of this rare autonomic disorder, which canhave potentially life-threatening neurological complications.

Keywords Hypertensive encephalopathy . Autonomicdysreflexia

Introduction

Autonomic dysreflexia (AD) is a syndrome affecting patientswith lesions of the spinal cord above the mid-thoracic level,leading to paroxysmal hypertension, bradycardia, excessivesweating, facial flushing, nasal congestion and headache. It isdue to an exaggerated autonomous response to stimuli such asdistention of the bladder or rectum [1, 2]. It mostly occurs inchildren aged 6 to 13 years, with an estimated lifetime prev-alence among patients with spinal cord injury (SCI) rangingfrom 19 to 70 % [3]. To our current knowledge, there are still

no published reports describing hypertensive encephalopathydue to AD in the paediatric population [4–6].

Case report

A 12-year-old boy was brought to our emergency clinic forloss of consciousness and general seizures. According to thepatient’s mother, who witnessed the scene 1 h earlier, the boysuddenly complained of intense headache; shortly after this,he became unconscious and started shaking uncontrollably.

Notably, his medical history included a serious neck injury3 months earlier, further complicated with haematoma, com-pression and subsequent spinal cord atrophy at the C6/C5 andTh1/Th2 levels (Fig. 1).

Prior to being hospitalised, he was on regular physiothera-py for the management of spastic paraplegia and had no otherhealth difficulties. His only medication was baclofen, with thelast dose taken the day before his admission.

Upon admission to our institution, the boy was afebrile andunconscious, with a slightly rapid respiration rate of 42 breaths/min, marked uncontrolled shaking of the arms and oxygensaturation of 98 to 100 % on room air. The patient’s pulse ratewas 60/min, while his blood pressure was 180/105 mmHg.Other positive clinical findings were bladder distension and aneurological deficit in the form of spastic paraplegia.

Initially, we performed standard haematological, biochem-ical and blood gas analyses, fundus examination and a multi-slice computed tomography (MSCT) scan of the head. Exceptfor respiratory alkalosis (pH=7.66, pCO2=18 mmHg, pO2=75 mmHg, HCO3=27.2 mmol/l, BE=2.9 mmol/l, O2 sat=98 %), all other laboratory findings, including fundus exam-ination and MSCT of the head, were normal.

After the initial diagnostic workup, the patient was giventhe following medicines intravenously: 0.1 mg/kg midazolam,1 mg/kg furosemide and 1 g/kg mannitol 20 %. A paediatric

Communicated by Patrick Van Reempts

B. Bjelakovic (*) : E. GolubovicClinic of Pediatrics, Clinical Center, Medical Faculty, University ofNis, Zorana Djindjica 48 Boulevard, 18000 Nis, Serbiae-mail: bojko968@gmail.com

L. DimitrijevicClinic of Physical Medicine and Rehabilitation Clinical Center,Medical Faculty, University of Nis, Nis, Serbia

S. LukicClinic of Neurology, Clinical Center, Medical Faculty, University ofNis, Nis, Serbia

Eur J PediatrDOI 10.1007/s00431-014-2281-y

surgeon placed a urinary catheter for drainage. The patientregained consciousness within the next 30 min, and 1 h later,his blood pressure dropped to 115/70 mmHg. During furtherhospitalisation, the patient was closely observed. Additionallaboratory analyses (urine vanillyl mandelic acid level andabdominal ultrasound) were normal.

On the fourth and fifth day of hospitalisation, during 24-hambulatory blood pressure monitoring, the patient experi-enced a second and a third hypertensive crisis, characterisedby intense headache but no loss of consciousness and seizure-like neurological signs. The patient’s maximal blood pressurewas similar on both occasions, reaching 170/80mmHg.Whilethe first hypertensive episode was associated with difficultiesin bowel emptying, the second one was associated with uri-nary retention and bladder distention.

Taking into account all the relevant findings, the diagnosis ofAD was established and the patient was given an alpha-blocker(2.5 mg Xatral), an angiotensin-converting-enzyme inhibitor(enalapril) and a beta-blocker (Presolol). During follow-up for6 months, the hypertensive crises did not recur and the patientcontinued with his regular physical rehabilitation.

Discussion

A number of cardiovascular abnormalities associated withautonomic and central nervous system (CNS) disorders havebeen described [5]. However, the scope of paediatric auto-nomic disorders is not well recognised, and paediatricians

seem to be generally unaware of the complexity and diversityof their clinical manifestations [1].

One of the frequently overlooked autonomic disorders inpatients with SCI at the T6 level or above is AD. This ischaracterised by uncontrolled sympathetic response second-ary to a precipitant, various vegetative disturbances and po-tentially fatal complication in the form of malignant hyperten-sion and subsequent hypertensive encephalopathy and/or ce-rebral haemorrhage. The first-line therapy for such disorders ispositioning the patient upright and eliminating any precipitat-ing stimulus like bladder or rectal distension [2].

Since paediatricians, and especially paediatric cardiolo-gists, have little knowledge about the causes, clinical featuresand appropriate treatment options in AD patients, the earlyrecognition of this rare clinical entity remains a diagnosticchallenge to them. According to data in the literature, thelifetime prevalence of AD in SCI patients ranges from 19 to70 % [3]. Hence, it is quite possible for paediatricians, andparticularly paediatric cardiologists, to come across this po-tentially fatal medical disorder. It is worth noting that ourliterature search of the most reputable paediatric cardiologyjournals (Cardiology in the Young and Pediatric Cardiology)using the keyword ‘autonomic dysreflexia’ revealed no pub-lished reports on this subject. To our knowledge, there are alsostill no published articles describing hypertensive encephalop-athy due to AD in paediatric patients, although we did find acase of acute disseminated encephalomyelitis with spinal cordinvolvement and AD in the paediatric literature [4].

To summarise, since cardiovascular disturbances are theleading causes of morbidity and mortality in both the acuteand chronic stages of SCI, this observation emphasises theimportance of recognising this rare autonomic complication ofwhich paediatricians are often unaware.

Acknowledgments This work was supported by a grant no. 175092from the Ministry of Science and Technology of the Republic of Serbia.

References

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3. Hickey KJ, Vogel LC,Willis KM, Anderson CJ (2004) Prevalence andetiology of autonomic dysreflexia in children with spinal cord injuries.J Spinal Cord Med 27(Suppl 1):S54–S60

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6. Matias AC, Rocha J, Cerqueira ME, Pereira JM (2013) Autonomicdysreflexia and posterior reversible encephalopathy syndrome. Am JPhys Med Rehabil 92:453–458

Fig. 1 Magnetic resonance image of the cervical spine in the neutralposition reveals segmental spinal cord atrophy at the C5/C6 and Th1/Th2spinal segments

Eur J Pediatr