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Autonomic Dysreflexia in Acute Disseminated Encephalomyelitis
Pediatric Neurology 47 (2012) 309e311
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Case Report
Autonomic Dysreflexia in Acute Disseminated Encephalomyelitis Machinary P. Jayakrishnan MD a, *, Padinharath Krishnakumar MD, DNB b, Rajendran Gauthamen MBBS a, Sasidharanpillai Sabitha MD a, Ellezhuthil Devarajan MD c a
Department of Pediatrics, Institute of Maternal and Child Health, Medical College, Calicut, Kerala, India Institute of Mental Health and Neurosciences, Medical College, Calicut, Kerala, India c Department of Radiodiagnosis, Medical College, Calicut, Kerala, India b
article information
abstract
Article history: Received 8 April 2012 Accepted 7 June 2012
Autonomic dysreflexia occurs in patients with spinal cord injury, and is characterized by unbalanced sympathetic discharge, precipitated by noxious stimuli from a site below the spinal cord lesion. An 11-year-old boy with acute disseminated encephalomyelitis and spinal cord involvement manifested episodes of intense flushing and sweating, confined to the head and neck region, and associated with hypertension and tachycardia. His signs improved after changing a partly blocked bladder catheter. The clinical features suggested autonomic dysreflexia. Early recognition of autonomic dysreflexia is important because removal of the trigger precipitating the event may be life-saving. Ó 2012 Elsevier Inc. All rights reserved.
Introduction
Autonomic dysreflexia is a syndrome characterized by unbalanced sympathetic discharge in patients with spinal cord injury, and is precipitated by a noxious stimulus below the spinal cord lesion [1,2]. The clinical features of autonomic dysreflexia include hypertension and alterations in heart rate, along with flushing and sweating confined to the head and neck region. Visual changes, cardiac arrhythmias, and nasal congestion may also be involved. The recognition of autonomic dysreflexia and the prompt removal of the precipitating stimulus are important, because this syndrome can sometimes result in serious complications or even death. Although autonomic dysreflexia is known to occur in patients with traumatic spinal cord injury, a few reports have associated it with nervous system demyelination. To the best of our knowledge, no reports have described children with acute disseminated encephalomyelitis. Case Report An 11-year-old boy, the third child of nonconsanguineous parents, was admitted with a history of fever for 2 days and altered behavior of * Communications should be addressed to: Dr. Jayakrishnan; Department of Pediatrics; Institute of Maternal and Child Health; Calicut Medical College; Calicut 8; PIN 673 008, Kerala, India. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2012.06.004
1-day duration. Behavioral problems included crying spells, irrelevant talk, and the frequent clenching of teeth. No history of headache, vomiting, weakness of limbs, seizures, loss of consciousness, visual disturbances, or features related to bowel or bladder dysfunction was reported. Moreover, no history of skin rash or bleeding was reported. He had manifested earache and nasal discharge 1 week before the onset of the present illness, which subsided within 2 days. No history of recent immunization was reported. His developmental milestones were normal, and his academic performance had been good. At the time of admission, the child was drowsy. His respiratory rate was 24/minute, with 95% oxygen saturation in room air and normal breathing. His heart rate was 96/minute, and his blood pressure was 100/60 mm Hg. No focal neurologic deficits or signs of meningeal irritation were evident. His plantar reflex was bilaterally extensor. Initial investigations revealed normal blood glucose, blood counts, and serum electrolytes. Tests of renal and hepatic function produced normal results. Analysis of his cerebrospinal fluid indicated a mild increase in proteins (52 mg/dL) and no pleocytosis. An electroencephalogram revealed normal results. A provisional diagnosis of meningoencephalitis was rendered, and 10 mg/kg intravenous acyclovir every 8 hours and 50 mg/kg ceftriaxone every 12 hours were initiated. The next day his condition worsened with generalized seizures, which were controlled by lorazepam and phenytoin sodium. During the next few days he also developed left-sided hemiparesis and abnormal movements of his right upper limb. On day 5 of admission, he developed paradoxic breathing, indicating diaphragmatic weakness. Magnetic resonance imaging (Figs 1 and 2) on day 12 of admission revealed multiple, altered signal intensity lesions (hyperintense in T2 fluid attenuated inversion recovery, and hypointense in T1) in the centrum semiovale and subcortical white matter (U fibers) of both cerebral hemispheres. Lesions were also evident in the deep grey matter, midbrain, and pons. In the cervical cord, T2 hyperintense lesions were
310
M.P. Jayakrishnan et al. / Pediatric Neurology 47 (2012) 309e311 encephalomyelitis. Intravenous methylprednisolone at 30 mg per kg was administered for 3 days, and was continued with oral prednisolone at a dose of 2 mg per kg daily. On day 18 of admission, he manifested frequent episodes of increased sweating and intense flushing involving the face and neck, each episode lasting for about 5 minutes. During these episodes, he also demonstrated tachycardia, hypertension, and dilated pupils. His systolic blood pressure rose to 150 mm mercury at this time. His blood pressure and heart rate were normal between episodes. His bladder catheter was revealed to be partly blocked because of sludge. A diagnosis of autonomic dysreflexia was considered, and the bladder catheter was removed, which relieved his sweating episodes and agitation. His neurologic deficits persisted despite continued treatment with steroids. Hence he received 2 g/kg intravenous immunoglobulin for 5 days. He recovered gradually, with no neurologic deficits at time of discharge from the hospital on day 49 of his illness. At follow-up after 1 year, no neurologic deficits were evident.
Discussion
Figure 1. Multiple T2 hyperintense lesions of varying size (arrows) involve the bilateral centrum semiovale and extend to the subcortical white matter (U fibers). present at the C4, C5, and C6 levels. Multiple T2 hyperintense lesions were also observed in the thoracic cord. The lesions presented mild contrast enhancement, but no significant mass effect. The clinical and radiologic findings led to a diagnosis of acute disseminated
Figure 2. The cervical cord contains a T2 hyperintense lesion at the C4, C5, and C6 levels (upper arrow). Multiple T2 hyperintense lesions are also evident in the thoracic cord (lower arrows).
Autonomic dysreflexia is classically described as a complication in patients with spinal cord injury at or above the level of T6. The clinical manifestations can be explained on the basis of the anatomic location of the sympathetic and parasympathetic tracts. The sympathetic outflow leaves the spinal cord via the first thoracic to the third or fourth lumbar spinal nerves. The parasympathetic outflow occurs via the third, seventh, ninth, and tenth cranial nerves and the second to fourth sacral spinal nerves. The spinal reflexes remain intact below the lesion in spinal cord injuries. A noxious stimulus from a site below the spinal cord lesion (such as bladder distension, bladder irrigation, urinary tract infection, fecal impaction, or pressure sores) causes a generalized sympathetic response, resulting in vasoconstriction and hypertension. Through intact baroreceptors, the brain detects this hypertension and attempts to lower the blood pressure by stimulating the parasympathetic system. However, the spinal cord lesion prevents such signals from descending through the spinal cord. The excessive parasympathetic output above the level of the lesion results in peripheral vasodilation, headache, flushing in the head and neck region, and nasal congestion [1]. The present patient manifested lesions of acute disseminated encephalomyelitis involving his spinal cord. The intense flushing and sweating confined to the head and neck region, along with his hypertension and tachycardia, suggested the possibility of autonomic dysreflexia. When a trigger was sought, his bladder catheter was revealed to be partly blocked with sludge, and dramatic improvement occurred after the removal of the catheter. The signs of autonomic dysreflexia were not persistent, probably because the catheter was not fully blocked and permitted intermittent urine flow. Because the episodes of increased sweating and intense flushing were confined to the face and neck only, and because the episodes were fluctuating, we excluded the possibility that the patient’s signs being caused by anxiety because of a distended bladder. Although bradycardia is classically described in patients during autonomic dysreflexia, tachycardia may also occur [3,4]. The occurrence of tachycardia or bradycardia depends on whether the pulse rate is recorded during the rising or falling phase of blood pressure. This difference is observed because during the rising phase of blood pressure, the transmission from the baroreceptors triggering the vagal response will be intense, leading to bradycardia, and the
M.P. Jayakrishnan et al. / Pediatric Neurology 47 (2012) 309e311
transmission may cease when the pressure is at a steady high or dropping, resulting in tachycardia [4]. To the best of our knowledge, this case report is the first of a child with acute disseminated encephalomyelitis presenting with features of autonomic dysreflexia. Two previous reports involved autonomic dysreflexia in patients with multiple sclerosis and lesions in the spinal cord [3,5]. Autonomic dysreflexia can be life-threatening because it can result in complications such as myocardial ischemia and intracranial hemorrhage [6-8]. Although this condition is likely to be encountered by surgeons and physiatrists treating patients with spinal cord trauma, awareness of autonomic dysreflexia is low among physicians, especially pediatric physicians [5,9]. Because autonomic dysreflexia can lead to death, early recognition is important in removing the underlying trigger. The present case highlights the importance of considering this condition in children with spinal cord involvement in acute disseminated encephalomyelitis. The authors acknowledge the assistance of Geeta M. Govindaraj, MD, Medical College, Calicut, Kerala, India, in the preparation of the manuscript.
311
References [1] Blackmer J. Rehabilitation medicine: Autonomic dysreflexia. Can Med Assoc J 2003;169:931e5. [2] Cragg J, Krassioukov A. Autonomic dysreflexia. Can Med Assoc J 2012;184:66. [3] Kulcu DG, Akbas B, Citci B, Cihangiroglu M. Autonomic dysreflexia in a man with multiple sclerosis. J Spinal Cord Med 2009;32: 198e203. [4] Lindan R, Joiner E, Freehafer A, Bazel C. Incidence and clinical features of autonomic dysreflexia in patients with spinal cord injury. Paraplegia 1980;18:285e92. [5] Bateman AM, Goldish GD. Autonomic dysreflexia in multiple sclerosis. J Spinal Cord Med 2002;25:40e2. [6] Vaidyanathan S, Soni BM, Singh G, Hughes PL, Pulya K, Oo T. Infarct of the right basal ganglia in a male spinal cord injury patient: Adverse effect of autonomic dysreflexia. Sci World J 2011;11: 666e72. [7] Yoo KY, Jeong CW, Kim WM, et al. Fatal cerebral hemorrhage associated with autonomic hyperreflexia during surgery in the prone position in a quadriplegic patient: A case report. Minerva Anestesiol 2010;76:554e8. [8] Ho CP, Krassioukov AV. Autonomic dysreflexia and myocardial ischemia. Spinal Cord 2010;48:714e5. [9] Jackson CR, Acland R. Knowledge of autonomic dysreflexia in the emergency department. Emerg Med J 2011;28:866e9.
Contents lists available at ScienceDirect
Pediatric Neurology journal homepage: www.elsevier.com/locate/pnu
Case Report
Autonomic Dysreflexia in Acute Disseminated Encephalomyelitis Machinary P. Jayakrishnan MD a, *, Padinharath Krishnakumar MD, DNB b, Rajendran Gauthamen MBBS a, Sasidharanpillai Sabitha MD a, Ellezhuthil Devarajan MD c a
Department of Pediatrics, Institute of Maternal and Child Health, Medical College, Calicut, Kerala, India Institute of Mental Health and Neurosciences, Medical College, Calicut, Kerala, India c Department of Radiodiagnosis, Medical College, Calicut, Kerala, India b
article information
abstract
Article history: Received 8 April 2012 Accepted 7 June 2012
Autonomic dysreflexia occurs in patients with spinal cord injury, and is characterized by unbalanced sympathetic discharge, precipitated by noxious stimuli from a site below the spinal cord lesion. An 11-year-old boy with acute disseminated encephalomyelitis and spinal cord involvement manifested episodes of intense flushing and sweating, confined to the head and neck region, and associated with hypertension and tachycardia. His signs improved after changing a partly blocked bladder catheter. The clinical features suggested autonomic dysreflexia. Early recognition of autonomic dysreflexia is important because removal of the trigger precipitating the event may be life-saving. Ó 2012 Elsevier Inc. All rights reserved.
Introduction
Autonomic dysreflexia is a syndrome characterized by unbalanced sympathetic discharge in patients with spinal cord injury, and is precipitated by a noxious stimulus below the spinal cord lesion [1,2]. The clinical features of autonomic dysreflexia include hypertension and alterations in heart rate, along with flushing and sweating confined to the head and neck region. Visual changes, cardiac arrhythmias, and nasal congestion may also be involved. The recognition of autonomic dysreflexia and the prompt removal of the precipitating stimulus are important, because this syndrome can sometimes result in serious complications or even death. Although autonomic dysreflexia is known to occur in patients with traumatic spinal cord injury, a few reports have associated it with nervous system demyelination. To the best of our knowledge, no reports have described children with acute disseminated encephalomyelitis. Case Report An 11-year-old boy, the third child of nonconsanguineous parents, was admitted with a history of fever for 2 days and altered behavior of * Communications should be addressed to: Dr. Jayakrishnan; Department of Pediatrics; Institute of Maternal and Child Health; Calicut Medical College; Calicut 8; PIN 673 008, Kerala, India. E-mail address: [email protected] 0887-8994/$ - see front matter Ó 2012 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.pediatrneurol.2012.06.004
1-day duration. Behavioral problems included crying spells, irrelevant talk, and the frequent clenching of teeth. No history of headache, vomiting, weakness of limbs, seizures, loss of consciousness, visual disturbances, or features related to bowel or bladder dysfunction was reported. Moreover, no history of skin rash or bleeding was reported. He had manifested earache and nasal discharge 1 week before the onset of the present illness, which subsided within 2 days. No history of recent immunization was reported. His developmental milestones were normal, and his academic performance had been good. At the time of admission, the child was drowsy. His respiratory rate was 24/minute, with 95% oxygen saturation in room air and normal breathing. His heart rate was 96/minute, and his blood pressure was 100/60 mm Hg. No focal neurologic deficits or signs of meningeal irritation were evident. His plantar reflex was bilaterally extensor. Initial investigations revealed normal blood glucose, blood counts, and serum electrolytes. Tests of renal and hepatic function produced normal results. Analysis of his cerebrospinal fluid indicated a mild increase in proteins (52 mg/dL) and no pleocytosis. An electroencephalogram revealed normal results. A provisional diagnosis of meningoencephalitis was rendered, and 10 mg/kg intravenous acyclovir every 8 hours and 50 mg/kg ceftriaxone every 12 hours were initiated. The next day his condition worsened with generalized seizures, which were controlled by lorazepam and phenytoin sodium. During the next few days he also developed left-sided hemiparesis and abnormal movements of his right upper limb. On day 5 of admission, he developed paradoxic breathing, indicating diaphragmatic weakness. Magnetic resonance imaging (Figs 1 and 2) on day 12 of admission revealed multiple, altered signal intensity lesions (hyperintense in T2 fluid attenuated inversion recovery, and hypointense in T1) in the centrum semiovale and subcortical white matter (U fibers) of both cerebral hemispheres. Lesions were also evident in the deep grey matter, midbrain, and pons. In the cervical cord, T2 hyperintense lesions were
310
M.P. Jayakrishnan et al. / Pediatric Neurology 47 (2012) 309e311 encephalomyelitis. Intravenous methylprednisolone at 30 mg per kg was administered for 3 days, and was continued with oral prednisolone at a dose of 2 mg per kg daily. On day 18 of admission, he manifested frequent episodes of increased sweating and intense flushing involving the face and neck, each episode lasting for about 5 minutes. During these episodes, he also demonstrated tachycardia, hypertension, and dilated pupils. His systolic blood pressure rose to 150 mm mercury at this time. His blood pressure and heart rate were normal between episodes. His bladder catheter was revealed to be partly blocked because of sludge. A diagnosis of autonomic dysreflexia was considered, and the bladder catheter was removed, which relieved his sweating episodes and agitation. His neurologic deficits persisted despite continued treatment with steroids. Hence he received 2 g/kg intravenous immunoglobulin for 5 days. He recovered gradually, with no neurologic deficits at time of discharge from the hospital on day 49 of his illness. At follow-up after 1 year, no neurologic deficits were evident.
Discussion
Figure 1. Multiple T2 hyperintense lesions of varying size (arrows) involve the bilateral centrum semiovale and extend to the subcortical white matter (U fibers). present at the C4, C5, and C6 levels. Multiple T2 hyperintense lesions were also observed in the thoracic cord. The lesions presented mild contrast enhancement, but no significant mass effect. The clinical and radiologic findings led to a diagnosis of acute disseminated
Figure 2. The cervical cord contains a T2 hyperintense lesion at the C4, C5, and C6 levels (upper arrow). Multiple T2 hyperintense lesions are also evident in the thoracic cord (lower arrows).
Autonomic dysreflexia is classically described as a complication in patients with spinal cord injury at or above the level of T6. The clinical manifestations can be explained on the basis of the anatomic location of the sympathetic and parasympathetic tracts. The sympathetic outflow leaves the spinal cord via the first thoracic to the third or fourth lumbar spinal nerves. The parasympathetic outflow occurs via the third, seventh, ninth, and tenth cranial nerves and the second to fourth sacral spinal nerves. The spinal reflexes remain intact below the lesion in spinal cord injuries. A noxious stimulus from a site below the spinal cord lesion (such as bladder distension, bladder irrigation, urinary tract infection, fecal impaction, or pressure sores) causes a generalized sympathetic response, resulting in vasoconstriction and hypertension. Through intact baroreceptors, the brain detects this hypertension and attempts to lower the blood pressure by stimulating the parasympathetic system. However, the spinal cord lesion prevents such signals from descending through the spinal cord. The excessive parasympathetic output above the level of the lesion results in peripheral vasodilation, headache, flushing in the head and neck region, and nasal congestion [1]. The present patient manifested lesions of acute disseminated encephalomyelitis involving his spinal cord. The intense flushing and sweating confined to the head and neck region, along with his hypertension and tachycardia, suggested the possibility of autonomic dysreflexia. When a trigger was sought, his bladder catheter was revealed to be partly blocked with sludge, and dramatic improvement occurred after the removal of the catheter. The signs of autonomic dysreflexia were not persistent, probably because the catheter was not fully blocked and permitted intermittent urine flow. Because the episodes of increased sweating and intense flushing were confined to the face and neck only, and because the episodes were fluctuating, we excluded the possibility that the patient’s signs being caused by anxiety because of a distended bladder. Although bradycardia is classically described in patients during autonomic dysreflexia, tachycardia may also occur [3,4]. The occurrence of tachycardia or bradycardia depends on whether the pulse rate is recorded during the rising or falling phase of blood pressure. This difference is observed because during the rising phase of blood pressure, the transmission from the baroreceptors triggering the vagal response will be intense, leading to bradycardia, and the
M.P. Jayakrishnan et al. / Pediatric Neurology 47 (2012) 309e311
transmission may cease when the pressure is at a steady high or dropping, resulting in tachycardia [4]. To the best of our knowledge, this case report is the first of a child with acute disseminated encephalomyelitis presenting with features of autonomic dysreflexia. Two previous reports involved autonomic dysreflexia in patients with multiple sclerosis and lesions in the spinal cord [3,5]. Autonomic dysreflexia can be life-threatening because it can result in complications such as myocardial ischemia and intracranial hemorrhage [6-8]. Although this condition is likely to be encountered by surgeons and physiatrists treating patients with spinal cord trauma, awareness of autonomic dysreflexia is low among physicians, especially pediatric physicians [5,9]. Because autonomic dysreflexia can lead to death, early recognition is important in removing the underlying trigger. The present case highlights the importance of considering this condition in children with spinal cord involvement in acute disseminated encephalomyelitis. The authors acknowledge the assistance of Geeta M. Govindaraj, MD, Medical College, Calicut, Kerala, India, in the preparation of the manuscript.
311
References [1] Blackmer J. Rehabilitation medicine: Autonomic dysreflexia. Can Med Assoc J 2003;169:931e5. [2] Cragg J, Krassioukov A. Autonomic dysreflexia. Can Med Assoc J 2012;184:66. [3] Kulcu DG, Akbas B, Citci B, Cihangiroglu M. Autonomic dysreflexia in a man with multiple sclerosis. J Spinal Cord Med 2009;32: 198e203. [4] Lindan R, Joiner E, Freehafer A, Bazel C. Incidence and clinical features of autonomic dysreflexia in patients with spinal cord injury. Paraplegia 1980;18:285e92. [5] Bateman AM, Goldish GD. Autonomic dysreflexia in multiple sclerosis. J Spinal Cord Med 2002;25:40e2. [6] Vaidyanathan S, Soni BM, Singh G, Hughes PL, Pulya K, Oo T. Infarct of the right basal ganglia in a male spinal cord injury patient: Adverse effect of autonomic dysreflexia. Sci World J 2011;11: 666e72. [7] Yoo KY, Jeong CW, Kim WM, et al. Fatal cerebral hemorrhage associated with autonomic hyperreflexia during surgery in the prone position in a quadriplegic patient: A case report. Minerva Anestesiol 2010;76:554e8. [8] Ho CP, Krassioukov AV. Autonomic dysreflexia and myocardial ischemia. Spinal Cord 2010;48:714e5. [9] Jackson CR, Acland R. Knowledge of autonomic dysreflexia in the emergency department. Emerg Med J 2011;28:866e9.