Eur J Pediatr DOI 10.1007/s00431-014-2281-y
CASE REPORT
Hypertensive encephalopathy as a late complication of autonomic dysreflexia 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 not well recognised, and paediatricians seem to be generally unaware of the complexity and diversity of their clinical manifestations. We report a 12-year-old boy presenting with hypertensive encephalopathy caused by autonomic dysreflexia. Conclusion: This observation emphasises the importance of the recognition of this rare autonomic disorder, which can have potentially life-threatening neurological complications. Keywords Hypertensive encephalopathy . Autonomic dysreflexia
Introduction Autonomic dysreflexia (AD) is a syndrome affecting patients with lesions of the spinal cord above the mid-thoracic level, leading to paroxysmal hypertension, bradycardia, excessive sweating, facial flushing, nasal congestion and headache. It is due to an exaggerated autonomous response to stimuli such as distention of the bladder or rectum [1, 2]. It mostly occurs in children aged 6 to 13 years, with an estimated lifetime prevalence among patients with spinal cord injury (SCI) ranging from 19 to 70 % [3]. To our current knowledge, there are still Communicated by Patrick Van Reempts B. Bjelakovic (*) : E. Golubovic Clinic of Pediatrics, Clinical Center, Medical Faculty, University of Nis, Zorana Djindjica 48 Boulevard, 18000 Nis, Serbia e-mail: [email protected] L. Dimitrijevic Clinic of Physical Medicine and Rehabilitation Clinical Center, Medical Faculty, University of Nis, Nis, Serbia S. Lukic Clinic of Neurology, Clinical Center, Medical Faculty, University of Nis, Nis, Serbia
no published reports describing hypertensive encephalopathy due to AD in the paediatric population [4–6].
Case report A 12-year-old boy was brought to our emergency clinic for loss of consciousness and general seizures. According to the patient’s mother, who witnessed the scene 1 h earlier, the boy suddenly complained of intense headache; shortly after this, he became unconscious and started shaking uncontrollably. Notably, his medical history included a serious neck injury 3 months earlier, further complicated with haematoma, compression and subsequent spinal cord atrophy at the C6/C5 and Th1/Th2 levels (Fig. 1). Prior to being hospitalised, he was on regular physiotherapy for the management of spastic paraplegia and had no other health difficulties. His only medication was baclofen, with the last dose taken the day before his admission. Upon admission to our institution, the boy was afebrile and unconscious, with a slightly rapid respiration rate of 42 breaths/ min, marked uncontrolled shaking of the arms and oxygen saturation of 98 to 100 % on room air. The patient’s pulse rate was 60/min, while his blood pressure was 180/105 mmHg. Other positive clinical findings were bladder distension and a neurological deficit in the form of spastic paraplegia. Initially, we performed standard haematological, biochemical and blood gas analyses, fundus examination and a multislice computed tomography (MSCT) scan of the head. Except for 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 examination and MSCT of the head, were normal. After the initial diagnostic workup, the patient was given the following medicines intravenously: 0.1 mg/kg midazolam, 1 mg/kg furosemide and 1 g/kg mannitol 20 %. A paediatric
Eur J Pediatr
Fig. 1 Magnetic resonance image of the cervical spine in the neutral position reveals segmental spinal cord atrophy at the C5/C6 and Th1/Th2 spinal segments
surgeon placed a urinary catheter for drainage. The patient regained consciousness within the next 30 min, and 1 h later, his blood pressure dropped to 115/70 mmHg. During further hospitalisation, the patient was closely observed. Additional laboratory analyses (urine vanillyl mandelic acid level and abdominal ultrasound) were normal. On the fourth and fifth day of hospitalisation, during 24-h ambulatory blood pressure monitoring, the patient experienced a second and a third hypertensive crisis, characterised by intense headache but no loss of consciousness and seizurelike neurological signs. The patient’s maximal blood pressure was similar on both occasions, reaching 170/80 mmHg. While the first hypertensive episode was associated with difficulties in bowel emptying, the second one was associated with urinary retention and bladder distention. Taking into account all the relevant findings, the diagnosis of AD 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 for 6 months, the hypertensive crises did not recur and the patient continued with his regular physical rehabilitation.
Discussion A number of cardiovascular abnormalities associated with autonomic and central nervous system (CNS) disorders have been described [5]. However, the scope of paediatric autonomic disorders is not well recognised, and paediatricians
seem to be generally unaware of the complexity and diversity of their clinical manifestations [1]. One of the frequently overlooked autonomic disorders in patients with SCI at the T6 level or above is AD. This is characterised by uncontrolled sympathetic response secondary to a precipitant, various vegetative disturbances and potentially fatal complication in the form of malignant hypertension and subsequent hypertensive encephalopathy and/or cerebral haemorrhage. The first-line therapy for such disorders is positioning the patient upright and eliminating any precipitating stimulus like bladder or rectal distension [2]. Since paediatricians, and especially paediatric cardiologists, have little knowledge about the causes, clinical features and appropriate treatment options in AD patients, the early recognition of this rare clinical entity remains a diagnostic challenge to them. According to data in the literature, the lifetime prevalence of AD in SCI patients ranges from 19 to 70 % [3]. Hence, it is quite possible for paediatricians, and particularly paediatric cardiologists, to come across this potentially fatal medical disorder. It is worth noting that our literature search of the most reputable paediatric cardiology journals (Cardiology in the Young and Pediatric Cardiology) using the keyword ‘autonomic dysreflexia’ revealed no published reports on this subject. To our knowledge, there are also still no published articles describing hypertensive encephalopathy due to AD in paediatric patients, although we did find a case of acute disseminated encephalomyelitis with spinal cord involvement and AD in the paediatric literature [4]. To summarise, since cardiovascular disturbances are the leading causes of morbidity and mortality in both the acute and chronic stages of SCI, this observation emphasises the importance of recognising this rare autonomic complication of which paediatricians are often unaware. Acknowledgments This work was supported by a grant no. 175092 from the Ministry of Science and Technology of the Republic of Serbia.
References 1. Bycroft J, Shergill IS, Chung EA, Arya N, Shah PJ (2005) Autonomic dysreflexia: a medical emergency. Postgrad Med J 81:232–235 2. Grigorean VT, Sandu AM, Popescu M, Iacobini MA, Stoian R, Neascu C, Strambu V, Popa F (2009) Cardiac dysfunctions following spinal cord injury. J Med Life 2:133–145 3. Hickey KJ, Vogel LC, Willis KM, Anderson CJ (2004) Prevalence and etiology of autonomic dysreflexia in children with spinal cord injuries. J Spinal Cord Med 27(Suppl 1):S54–S60 4. Jayakrishnan MP, Krishnakumar P, Gauthamen R, Sabitha S, Devarajan E (2012) Autonomic dysreflexia in acute disseminated encephalomyelitis. Pediatr Neurol 47:309–311 5. Krassioukov A (2009) Autonomic function following cervical spinal cord injury. Respir Physiol Neurobiol 169:157–164 6. Matias AC, Rocha J, Cerqueira ME, Pereira JM (2013) Autonomic dysreflexia and posterior reversible encephalopathy syndrome. Am J Phys Med Rehabil 92:453–458
CASE REPORT
Hypertensive encephalopathy as a late complication of autonomic dysreflexia 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 not well recognised, and paediatricians seem to be generally unaware of the complexity and diversity of their clinical manifestations. We report a 12-year-old boy presenting with hypertensive encephalopathy caused by autonomic dysreflexia. Conclusion: This observation emphasises the importance of the recognition of this rare autonomic disorder, which can have potentially life-threatening neurological complications. Keywords Hypertensive encephalopathy . Autonomic dysreflexia
Introduction Autonomic dysreflexia (AD) is a syndrome affecting patients with lesions of the spinal cord above the mid-thoracic level, leading to paroxysmal hypertension, bradycardia, excessive sweating, facial flushing, nasal congestion and headache. It is due to an exaggerated autonomous response to stimuli such as distention of the bladder or rectum [1, 2]. It mostly occurs in children aged 6 to 13 years, with an estimated lifetime prevalence among patients with spinal cord injury (SCI) ranging from 19 to 70 % [3]. To our current knowledge, there are still Communicated by Patrick Van Reempts B. Bjelakovic (*) : E. Golubovic Clinic of Pediatrics, Clinical Center, Medical Faculty, University of Nis, Zorana Djindjica 48 Boulevard, 18000 Nis, Serbia e-mail: [email protected] L. Dimitrijevic Clinic of Physical Medicine and Rehabilitation Clinical Center, Medical Faculty, University of Nis, Nis, Serbia S. Lukic Clinic of Neurology, Clinical Center, Medical Faculty, University of Nis, Nis, Serbia
no published reports describing hypertensive encephalopathy due to AD in the paediatric population [4–6].
Case report A 12-year-old boy was brought to our emergency clinic for loss of consciousness and general seizures. According to the patient’s mother, who witnessed the scene 1 h earlier, the boy suddenly complained of intense headache; shortly after this, he became unconscious and started shaking uncontrollably. Notably, his medical history included a serious neck injury 3 months earlier, further complicated with haematoma, compression and subsequent spinal cord atrophy at the C6/C5 and Th1/Th2 levels (Fig. 1). Prior to being hospitalised, he was on regular physiotherapy for the management of spastic paraplegia and had no other health difficulties. His only medication was baclofen, with the last dose taken the day before his admission. Upon admission to our institution, the boy was afebrile and unconscious, with a slightly rapid respiration rate of 42 breaths/ min, marked uncontrolled shaking of the arms and oxygen saturation of 98 to 100 % on room air. The patient’s pulse rate was 60/min, while his blood pressure was 180/105 mmHg. Other positive clinical findings were bladder distension and a neurological deficit in the form of spastic paraplegia. Initially, we performed standard haematological, biochemical and blood gas analyses, fundus examination and a multislice computed tomography (MSCT) scan of the head. Except for 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 examination and MSCT of the head, were normal. After the initial diagnostic workup, the patient was given the following medicines intravenously: 0.1 mg/kg midazolam, 1 mg/kg furosemide and 1 g/kg mannitol 20 %. A paediatric
Eur J Pediatr
Fig. 1 Magnetic resonance image of the cervical spine in the neutral position reveals segmental spinal cord atrophy at the C5/C6 and Th1/Th2 spinal segments
surgeon placed a urinary catheter for drainage. The patient regained consciousness within the next 30 min, and 1 h later, his blood pressure dropped to 115/70 mmHg. During further hospitalisation, the patient was closely observed. Additional laboratory analyses (urine vanillyl mandelic acid level and abdominal ultrasound) were normal. On the fourth and fifth day of hospitalisation, during 24-h ambulatory blood pressure monitoring, the patient experienced a second and a third hypertensive crisis, characterised by intense headache but no loss of consciousness and seizurelike neurological signs. The patient’s maximal blood pressure was similar on both occasions, reaching 170/80 mmHg. While the first hypertensive episode was associated with difficulties in bowel emptying, the second one was associated with urinary retention and bladder distention. Taking into account all the relevant findings, the diagnosis of AD 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 for 6 months, the hypertensive crises did not recur and the patient continued with his regular physical rehabilitation.
Discussion A number of cardiovascular abnormalities associated with autonomic and central nervous system (CNS) disorders have been described [5]. However, the scope of paediatric autonomic disorders is not well recognised, and paediatricians
seem to be generally unaware of the complexity and diversity of their clinical manifestations [1]. One of the frequently overlooked autonomic disorders in patients with SCI at the T6 level or above is AD. This is characterised by uncontrolled sympathetic response secondary to a precipitant, various vegetative disturbances and potentially fatal complication in the form of malignant hypertension and subsequent hypertensive encephalopathy and/or cerebral haemorrhage. The first-line therapy for such disorders is positioning the patient upright and eliminating any precipitating stimulus like bladder or rectal distension [2]. Since paediatricians, and especially paediatric cardiologists, have little knowledge about the causes, clinical features and appropriate treatment options in AD patients, the early recognition of this rare clinical entity remains a diagnostic challenge to them. According to data in the literature, the lifetime prevalence of AD in SCI patients ranges from 19 to 70 % [3]. Hence, it is quite possible for paediatricians, and particularly paediatric cardiologists, to come across this potentially fatal medical disorder. It is worth noting that our literature search of the most reputable paediatric cardiology journals (Cardiology in the Young and Pediatric Cardiology) using the keyword ‘autonomic dysreflexia’ revealed no published reports on this subject. To our knowledge, there are also still no published articles describing hypertensive encephalopathy due to AD in paediatric patients, although we did find a case of acute disseminated encephalomyelitis with spinal cord involvement and AD in the paediatric literature [4]. To summarise, since cardiovascular disturbances are the leading causes of morbidity and mortality in both the acute and chronic stages of SCI, this observation emphasises the importance of recognising this rare autonomic complication of which paediatricians are often unaware. Acknowledgments This work was supported by a grant no. 175092 from the Ministry of Science and Technology of the Republic of Serbia.
References 1. Bycroft J, Shergill IS, Chung EA, Arya N, Shah PJ (2005) Autonomic dysreflexia: a medical emergency. Postgrad Med J 81:232–235 2. Grigorean VT, Sandu AM, Popescu M, Iacobini MA, Stoian R, Neascu C, Strambu V, Popa F (2009) Cardiac dysfunctions following spinal cord injury. J Med Life 2:133–145 3. Hickey KJ, Vogel LC, Willis KM, Anderson CJ (2004) Prevalence and etiology of autonomic dysreflexia in children with spinal cord injuries. J Spinal Cord Med 27(Suppl 1):S54–S60 4. Jayakrishnan MP, Krishnakumar P, Gauthamen R, Sabitha S, Devarajan E (2012) Autonomic dysreflexia in acute disseminated encephalomyelitis. Pediatr Neurol 47:309–311 5. Krassioukov A (2009) Autonomic function following cervical spinal cord injury. Respir Physiol Neurobiol 169:157–164 6. Matias AC, Rocha J, Cerqueira ME, Pereira JM (2013) Autonomic dysreflexia and posterior reversible encephalopathy syndrome. Am J Phys Med Rehabil 92:453–458
