- Email: [email protected]
Extrapontine Myelinolysis in a Child With Nephrotic Syndrome
Extrapontine Myelinolysis in a Child With Nephrotic Syndrome
myelinolysis is rarer, and the occurrence of extrapontine myelinolysis in children is extremely unusual [1,2,4,5]. The usual clinical scenario involves an association of the myelinolysis with a rapid correction of severe and prolonged hyponatremia [6]. We report on an unusual case of extrapontine myelinolysis in a patient with nephrotic syndrome who underwent rapid correction for chronic hyponatremia. Case Report
Milind S. Tullu, MD, Isha Deshmukh, MBBS, Mamta N. Muranjan, MD, Archana S. Kher, MD, and Keya R. Lahiri, MD Extrapontine myelinolysis is rare in children. We describe a 6-year-old girl with nephrotic syndrome who presented with symptomatic hyponatremia, and who developed acute quadriparesis with pseudobulbar palsy during rapid correction of the hyponatremia. Cranial magnetic resonance imaging demonstrated bilateral, symmetric basal ganglia lesions (extrapontine myelinolysis). The extrapontine myelinolysis was caused by rapid correction of severe and prolonged hyponatremia with intravenous 3% sodium chloride. The child demonstrated complete neurologic recovery. Prevention of this rare condition involves recognizing patients at risk for the disorder, and avoiding rapid correction of severe and prolonged hyponatremia. To the best of our knowledge, this is the first case report of extrapontine myelinolysis in a child with nephrotic syndrome. Ó 2010 by Elsevier Inc. All rights reserved. Tullu MS, Deshmukh I, Muranjan MN, Kher AS, Lahiri KR. Extrapontine myelinolysis in A child with nephrotic syndrome. Pediatr Neurol 2010;43:139-141.
Introduction Central pontine myelinolysis was first described in 1959, and most reports concern the adult population [1-4]. Compared with pontine myelinolysis, extrapontine
From the Department of Pediatrics, Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Maharashtra, Mumbai, India.
Ó 2010 by Elsevier Inc. All rights reserved. doi:10.1016/j.pediatrneurol.2010.04.006 0887-8994/$—see front matter
A 6-year-old girl was admitted to our institute with an episode of giddiness, followed by frequent bouts of nonbilious and nonprojectile vomiting for 4 days. There was no history of abdominal pain, visual complaints, auditory complaints, convulsions, altered sensorium, or bowel/ bladder incontinence. At age 2 years, the patient was diagnosed with focal segmental glomerulosclerosis, on the basis of a kidney biopsy performed for frequently relapsing nephrotic syndrome with persistent proteinuria and hypertension. She had been treated intermittently with prednisolone, furosemide, and salt restriction since age 1 year. Enalapril was also administered for 3 years (discontinued 1 year previously). She had been treated with cyclophosphamide for 12 weeks in the recent past, and had been receiving low dose oral prednisolone (0.5 mg/kg/day) for the past 6 months. Despite all these treatments, her proteinuria persisted. On admission, she was conscious and irritable, with normal vital parameters, bilateral pitting pedal edema, and cushingoid facies. Her blood pressure was normal. A systemic examination produced normal results. Investigations revealed a serum sodium level of 109 mEq/L, and a serum potassium level of 1.4 mEq/L (Table 1). Her leukocyte count was normal (9800 cells/mm3), her level of blood urea nitrogen was 9 mg/dL, and her serum creatinine level was 0.86 mg/dL. She was treated with antiemetics and intravenous fluids, with concentrated Ringer lactate solution and potassium chloride for her dyselectrolytemia. On admission, she received 20 mL of concentrated Ringer lactate solution (containing 3 mEq/mL of sodium) and 30 mL of potassium chloride (containing 1 mEq/mL of potassium) over a period of 24 hours. On day 2, she received 3% hypertonic saline infusion at 6 mL/kg over 6 hours (rapid sodium correction). On day 3 after admission, she developed drowsiness and an inability to move all her limbs, along with weak voice and a poor gag reflex. A neurologic examination revealed generalized hypotonia, with grade 2/5 muscle power in both lower extremities, and grade 3/5 power in both upper extremities. Her deep tendon reflexes were exaggerated, with a bilateral Babinski response. The examination of her sensory system produced normal results. No tremors or orobuccal movements were evident. A clinical diagnosis of quadriparesis with pseudobulbar palsy (possibly because of spinal cord compression by atlanto-axial subluxation, or a fracture dislocation of the cervical vertebrae) was considered. A radiograph of the cervical spine produced normal results, and a computed tomographic scan of the brain and spinal cord did not reveal a fracture or dislocation. Cranial magnetic resonance imaging (T2-weighted images) 2 days later revealed symmetric, bilateral, hyperintense lesions in the basal ganglia,
Communications should be addressed to: Dr. Tullu; Sankalp Siddhi; Block No. 1, Ground Floor; Kher Nagar, Service Road; Bandra (East); Maharashtra, Mumbai 400051, India. E-mail: [email protected] Received November 12, 2009; accepted April 5, 2010.
Tullu et al: Extrapontine Myelinolysis 139
Table 1. Daily details of electrolyte levels
Serum sodium (meq/L) Serum potassium (meq/L)
Day 1
Day 2
Day 3
Day 4
109 1.4
107 1.2
128 4.9
125 3.9
suggestive of extrapontine myelinolysis (Fig 1). The patient did not manifest any lesions in the pons, cortex, cerebellum, or thalamus. This finding was attributed to the rapid correction of hyponatremia (the patient had received 3% hypertonic saline infusion at 6 mL/kg during 6 hours on day 2 of admission, i.e., 121 meq/L/day) (Table 1). Ten days later, she was able to stand with support, her voice improved, and her gag reflex was normal. She was discharged from the hospital with the advice to undergo limb physiotherapy. At 10 months of follow-up, the patient demonstrated complete neurologic recovery, and repeated cranial magnetic resonance imaging produced normal results.
Discussion Osmotic demyelination syndrome is a neurologic complication associated with the rapid correction of severe and prolonged hyponatremia [5,7]. The myelinolysis frequently involves symmetric, noninflammatory demyelination within the central basis pontis or extrapontine sites (in 10% of cases), e.g., the midbrain, thalamus, basal nuclei, cerebellum, and subcortical white matter [2,5,7,8]. The pathogenesis of the condition remains controversial. The cellular edema, attributable to fluctuations in electrolyte forces, may result in the compression and subsequent demyelination of fiber tracts [2,7]. Predisposing conditions include alcoholism, liver disease, malnutrition, hyponatremia,
Figure 1. Cranial magnetic resonance imaging (T2-weighted diffusion image) indicates symmetric, bilateral hyperintense lesions in the basal ganglia, suggesting extrapontine myelinolysis.
140 PEDIATRIC NEUROLOGY Vol. 43 No. 2
hypernatremia, hyperglycemia, burn injuries, hemodialysis, peritoneal dialysis, chronic ventilation, immunosuppressant drugs (our patient had received cyclophosphamide), diabetic ketoacidosis, and neoplasia [2,5,7,9-11]. Some investigators emphasized the importance of hypoglycemia, hypokalemia, and azotemia, rather than sodium levels, as causes of myelinolysis [2,5,9-11]. Our patient did not manifest hypoglycemia or azotemia. The associated risk factors for myelinolysis include prolonged and severe hyponatremia of less than 120 meq/L for 24-48 hours, the development of normonatremia or hypernatremia during treatment for hyponatremia, and intravenous hypertonic saline infusions, with serum sodium values increasing by more than 10 meq/L/day during 24 hours [6,7,11,12]. The hyponatremia in our patient may have been attributable to the prolonged use of furosemide, coupled with a salt-restricted diet. The clinical features of osmotic demyelination syndrome include confusion, delirium or coma, pseudobulbar palsy, quadriplegia, hypertonia or hypotonia, hyperactive reflexes, a positive Babinski sign, horizontal gaze palsy, vertical ophthalmoparesis, dysphagia, dysarthria, and ataxia [1,2,58,12]. The pontine features such as hyperreflexia, pseudobulbar palsy, and tetraparesis in our patient may have been attributable to a pontine involvement that was not detected with magnetic resonance imaging; magnetic resonance imaging features can be delayed [10]. We had considered differential diagnoses such as spinal cord compression, mitochondrial cytopathy, deep venous occlusion, vascular problems, and acute disseminated encephalomyelitis, but excluded them on the basis of the clinical features and the findings of magnetic resonance imaging (suggesting extrapontine myelinolysis). The rapid occurrence of clinical findings was an unusual feature in our patient, especially insofar as clinical and radiographic findings may occur 7-14 days after an acute osmotic shift. The discordance between clinical and radiologic findings is well known in osmotic demyelination syndrome, especially during clinical recovery [1,4,11]. Cranial magnetic resonance imaging is the modality of choice when T2-weighted fluid-attenuated inversion recovery images demonstrate hyperintense lesions of demyelination caused by relatively increased water content in those areas [2,5,7,9,11]. In the acute phase of the demyelination, magnetic resonance imaging reveals pontine and extrapontine hyperintensities on T2-weighted and hypointensities on T1-weighted images [2,4,5,9]. Similar changes may also observed in disorders such as ischemia, multiple sclerosis, neoplasms, and metabolic diseases, and when symmetric, they are diagnostic of osmotic demyelination syndrome [4]. True demyelination can be demonstrated only after the regression of lesions, which reach their peak size 1-2 weeks after the initial symptoms and signs [4,11]. At this stage, extrapontine hyperintensities persist. Hence, symmetric basal ganglia hyperintensities mandate the consideration of osmotic demyelination syndrome in the differential diagnosis [4]. The findings of magnetic resonance imaging may correlate
with the clinical status. However, clinical improvement is independent of imaging, and the clinical improvement precedes the findings of imaging [1,4,7]. Prognostically, osmotic demyelination syndrome is highly heterogenous. Variable recovery and a reversal of magnetic resonance imaging findings are reported in some cases, but progression and death occur in other cases, and no specific treatment has been convincingly demonstrated to reverse osmotic demyelination syndrome or halt its progress [1,2,6,11]. Hence, prevention remains the mainstay of management. Some treatment modalities that were reported to be variably effective include plasmapheresis, corticosteroids, myoinositol, vasopressin receptor antagonist (vaptans), continuous veno-venous hemofiltration, and immunoglobulin therapy [2,3,6,10,13]. In conclusion, we report on a patient with osmotic demyelination syndrome/extrapontine myelinolysis secondary to a rapid correction of severe and prolonged hyponatremia, using hypertonic saline infusion to manage the hyponatremia in a patient with nephrotic syndrome. In our patient, the hyponatremia was probably attributable to a saltrestricted diet and long term diuretic therapy. Patients with nephrotic syndrome, and especially those with frequent relapses, are at high risk of developing myelinolysis if their hyponatremia is corrected rapidly. To the best of our knowledge, this is the first case report in the medical literature of extrapontine myelinolysis in a pediatric patient with nephrotic syndrome. The authors thank Sanjay Oak, MS, MCH, Director (Medical Education and Major Hospitals, Municipal Corporation of Greater Mumbai) and Dean of Seth Gorhandas Sunderdas Medical College and King Edward Memorial Hospital, for granting permission to publish the manuscript.
References [1] Niehaus L, Kulozik A, Lehmann R. Reversible central pontine and extrapontine myelinolysis in a 16-year-old girl. Childs Nerv Syst 2001;17: 294-6. [2] Bonkowsky JL, Filloux FM. Extrapontine myelinolysis in a pediatric case of diabetic ketoacidosis and cerebral edema. J Child Neurol 2003; 18:144-7. [3] Bibl D, Lampl C, Gabriel C, Jungling G, Brock H, Kostler G. Treatment of central pontine myelinolysis with therapeutic plasmapharesis. Lancet 1999;353:1155. [4] Karakas HM, Erdem G, Yakinci C. Osmotic demyelination syndrome in a 40-day-old infant. Diagn Interv Radiol 2007;13:121-4. [5] Brown WD, Caruso JM. Extrapontine myelinolysis with involvement of the hippocampus in three children with severe hypernatremia. J Child Neurol 1999;14:428-33. [6] Snell DM, Bartley C. Osmotic demyelination syndrome following rapid correction of hyponatraemia. Anaesthesia 2008;63:92-5. [7] Simon EE, Hamrahian SM. Hyponatremia. Available at: http:// emedicine.medscape.com/article/242166-overview. Accessed July 6, 2009. [8] Tomita I, Satoh H, Satoh A, Seto M, Tsujihata M, Yoshimura T. Extrapontine myelinolysis presenting with parkinsonism as a sequel of rapid correction of hyponatremia. J Neurol Neurosurg Psychiatry 1997; 62:422-3. [9] Bekiesinska-Figatowska M, Bulski T, Rozyczka I, Furmanek M, Walecki J. MR imaging of seven presumed cases of central pontine and extrapontine myelinolysis. Acta Neurobiol Exp (Warsz) 2001;61: 141-4. [10] Aydin OF, dem Uner C, Senbil N, Bek K, Erdogan O, Gurer YKY. Central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. J Child Neurol 2003;18:292-6. [11] Brito AR, Vasconcelos MM, Cruz Junior LC, et al. Central pontine and extrapontine myelinolysis: Report of a case with a tragic outcome. J Pediatr (Rio J) 2006;82:157-60. [12] Wang AH. Osmotic demyelination syndrome associated with 3% hypertonic saline administration. Intern Med J 2008;38:219-20. [13] Finsterer J, Engelmayer E, Trnka E, Stiskal M. Immunoglobulins are effective in pontine myelinolysis. Clin Neuropharmacol 2000;23: 110-3.
Tullu et al: Extrapontine Myelinolysis 141
myelinolysis is rarer, and the occurrence of extrapontine myelinolysis in children is extremely unusual [1,2,4,5]. The usual clinical scenario involves an association of the myelinolysis with a rapid correction of severe and prolonged hyponatremia [6]. We report on an unusual case of extrapontine myelinolysis in a patient with nephrotic syndrome who underwent rapid correction for chronic hyponatremia. Case Report
Milind S. Tullu, MD, Isha Deshmukh, MBBS, Mamta N. Muranjan, MD, Archana S. Kher, MD, and Keya R. Lahiri, MD Extrapontine myelinolysis is rare in children. We describe a 6-year-old girl with nephrotic syndrome who presented with symptomatic hyponatremia, and who developed acute quadriparesis with pseudobulbar palsy during rapid correction of the hyponatremia. Cranial magnetic resonance imaging demonstrated bilateral, symmetric basal ganglia lesions (extrapontine myelinolysis). The extrapontine myelinolysis was caused by rapid correction of severe and prolonged hyponatremia with intravenous 3% sodium chloride. The child demonstrated complete neurologic recovery. Prevention of this rare condition involves recognizing patients at risk for the disorder, and avoiding rapid correction of severe and prolonged hyponatremia. To the best of our knowledge, this is the first case report of extrapontine myelinolysis in a child with nephrotic syndrome. Ó 2010 by Elsevier Inc. All rights reserved. Tullu MS, Deshmukh I, Muranjan MN, Kher AS, Lahiri KR. Extrapontine myelinolysis in A child with nephrotic syndrome. Pediatr Neurol 2010;43:139-141.
Introduction Central pontine myelinolysis was first described in 1959, and most reports concern the adult population [1-4]. Compared with pontine myelinolysis, extrapontine
From the Department of Pediatrics, Seth Gordhandas Sunderdas Medical College and King Edward Memorial Hospital, Maharashtra, Mumbai, India.
Ó 2010 by Elsevier Inc. All rights reserved. doi:10.1016/j.pediatrneurol.2010.04.006 0887-8994/$—see front matter
A 6-year-old girl was admitted to our institute with an episode of giddiness, followed by frequent bouts of nonbilious and nonprojectile vomiting for 4 days. There was no history of abdominal pain, visual complaints, auditory complaints, convulsions, altered sensorium, or bowel/ bladder incontinence. At age 2 years, the patient was diagnosed with focal segmental glomerulosclerosis, on the basis of a kidney biopsy performed for frequently relapsing nephrotic syndrome with persistent proteinuria and hypertension. She had been treated intermittently with prednisolone, furosemide, and salt restriction since age 1 year. Enalapril was also administered for 3 years (discontinued 1 year previously). She had been treated with cyclophosphamide for 12 weeks in the recent past, and had been receiving low dose oral prednisolone (0.5 mg/kg/day) for the past 6 months. Despite all these treatments, her proteinuria persisted. On admission, she was conscious and irritable, with normal vital parameters, bilateral pitting pedal edema, and cushingoid facies. Her blood pressure was normal. A systemic examination produced normal results. Investigations revealed a serum sodium level of 109 mEq/L, and a serum potassium level of 1.4 mEq/L (Table 1). Her leukocyte count was normal (9800 cells/mm3), her level of blood urea nitrogen was 9 mg/dL, and her serum creatinine level was 0.86 mg/dL. She was treated with antiemetics and intravenous fluids, with concentrated Ringer lactate solution and potassium chloride for her dyselectrolytemia. On admission, she received 20 mL of concentrated Ringer lactate solution (containing 3 mEq/mL of sodium) and 30 mL of potassium chloride (containing 1 mEq/mL of potassium) over a period of 24 hours. On day 2, she received 3% hypertonic saline infusion at 6 mL/kg over 6 hours (rapid sodium correction). On day 3 after admission, she developed drowsiness and an inability to move all her limbs, along with weak voice and a poor gag reflex. A neurologic examination revealed generalized hypotonia, with grade 2/5 muscle power in both lower extremities, and grade 3/5 power in both upper extremities. Her deep tendon reflexes were exaggerated, with a bilateral Babinski response. The examination of her sensory system produced normal results. No tremors or orobuccal movements were evident. A clinical diagnosis of quadriparesis with pseudobulbar palsy (possibly because of spinal cord compression by atlanto-axial subluxation, or a fracture dislocation of the cervical vertebrae) was considered. A radiograph of the cervical spine produced normal results, and a computed tomographic scan of the brain and spinal cord did not reveal a fracture or dislocation. Cranial magnetic resonance imaging (T2-weighted images) 2 days later revealed symmetric, bilateral, hyperintense lesions in the basal ganglia,
Communications should be addressed to: Dr. Tullu; Sankalp Siddhi; Block No. 1, Ground Floor; Kher Nagar, Service Road; Bandra (East); Maharashtra, Mumbai 400051, India. E-mail: [email protected] Received November 12, 2009; accepted April 5, 2010.
Tullu et al: Extrapontine Myelinolysis 139
Table 1. Daily details of electrolyte levels
Serum sodium (meq/L) Serum potassium (meq/L)
Day 1
Day 2
Day 3
Day 4
109 1.4
107 1.2
128 4.9
125 3.9
suggestive of extrapontine myelinolysis (Fig 1). The patient did not manifest any lesions in the pons, cortex, cerebellum, or thalamus. This finding was attributed to the rapid correction of hyponatremia (the patient had received 3% hypertonic saline infusion at 6 mL/kg during 6 hours on day 2 of admission, i.e., 121 meq/L/day) (Table 1). Ten days later, she was able to stand with support, her voice improved, and her gag reflex was normal. She was discharged from the hospital with the advice to undergo limb physiotherapy. At 10 months of follow-up, the patient demonstrated complete neurologic recovery, and repeated cranial magnetic resonance imaging produced normal results.
Discussion Osmotic demyelination syndrome is a neurologic complication associated with the rapid correction of severe and prolonged hyponatremia [5,7]. The myelinolysis frequently involves symmetric, noninflammatory demyelination within the central basis pontis or extrapontine sites (in 10% of cases), e.g., the midbrain, thalamus, basal nuclei, cerebellum, and subcortical white matter [2,5,7,8]. The pathogenesis of the condition remains controversial. The cellular edema, attributable to fluctuations in electrolyte forces, may result in the compression and subsequent demyelination of fiber tracts [2,7]. Predisposing conditions include alcoholism, liver disease, malnutrition, hyponatremia,
Figure 1. Cranial magnetic resonance imaging (T2-weighted diffusion image) indicates symmetric, bilateral hyperintense lesions in the basal ganglia, suggesting extrapontine myelinolysis.
140 PEDIATRIC NEUROLOGY Vol. 43 No. 2
hypernatremia, hyperglycemia, burn injuries, hemodialysis, peritoneal dialysis, chronic ventilation, immunosuppressant drugs (our patient had received cyclophosphamide), diabetic ketoacidosis, and neoplasia [2,5,7,9-11]. Some investigators emphasized the importance of hypoglycemia, hypokalemia, and azotemia, rather than sodium levels, as causes of myelinolysis [2,5,9-11]. Our patient did not manifest hypoglycemia or azotemia. The associated risk factors for myelinolysis include prolonged and severe hyponatremia of less than 120 meq/L for 24-48 hours, the development of normonatremia or hypernatremia during treatment for hyponatremia, and intravenous hypertonic saline infusions, with serum sodium values increasing by more than 10 meq/L/day during 24 hours [6,7,11,12]. The hyponatremia in our patient may have been attributable to the prolonged use of furosemide, coupled with a salt-restricted diet. The clinical features of osmotic demyelination syndrome include confusion, delirium or coma, pseudobulbar palsy, quadriplegia, hypertonia or hypotonia, hyperactive reflexes, a positive Babinski sign, horizontal gaze palsy, vertical ophthalmoparesis, dysphagia, dysarthria, and ataxia [1,2,58,12]. The pontine features such as hyperreflexia, pseudobulbar palsy, and tetraparesis in our patient may have been attributable to a pontine involvement that was not detected with magnetic resonance imaging; magnetic resonance imaging features can be delayed [10]. We had considered differential diagnoses such as spinal cord compression, mitochondrial cytopathy, deep venous occlusion, vascular problems, and acute disseminated encephalomyelitis, but excluded them on the basis of the clinical features and the findings of magnetic resonance imaging (suggesting extrapontine myelinolysis). The rapid occurrence of clinical findings was an unusual feature in our patient, especially insofar as clinical and radiographic findings may occur 7-14 days after an acute osmotic shift. The discordance between clinical and radiologic findings is well known in osmotic demyelination syndrome, especially during clinical recovery [1,4,11]. Cranial magnetic resonance imaging is the modality of choice when T2-weighted fluid-attenuated inversion recovery images demonstrate hyperintense lesions of demyelination caused by relatively increased water content in those areas [2,5,7,9,11]. In the acute phase of the demyelination, magnetic resonance imaging reveals pontine and extrapontine hyperintensities on T2-weighted and hypointensities on T1-weighted images [2,4,5,9]. Similar changes may also observed in disorders such as ischemia, multiple sclerosis, neoplasms, and metabolic diseases, and when symmetric, they are diagnostic of osmotic demyelination syndrome [4]. True demyelination can be demonstrated only after the regression of lesions, which reach their peak size 1-2 weeks after the initial symptoms and signs [4,11]. At this stage, extrapontine hyperintensities persist. Hence, symmetric basal ganglia hyperintensities mandate the consideration of osmotic demyelination syndrome in the differential diagnosis [4]. The findings of magnetic resonance imaging may correlate
with the clinical status. However, clinical improvement is independent of imaging, and the clinical improvement precedes the findings of imaging [1,4,7]. Prognostically, osmotic demyelination syndrome is highly heterogenous. Variable recovery and a reversal of magnetic resonance imaging findings are reported in some cases, but progression and death occur in other cases, and no specific treatment has been convincingly demonstrated to reverse osmotic demyelination syndrome or halt its progress [1,2,6,11]. Hence, prevention remains the mainstay of management. Some treatment modalities that were reported to be variably effective include plasmapheresis, corticosteroids, myoinositol, vasopressin receptor antagonist (vaptans), continuous veno-venous hemofiltration, and immunoglobulin therapy [2,3,6,10,13]. In conclusion, we report on a patient with osmotic demyelination syndrome/extrapontine myelinolysis secondary to a rapid correction of severe and prolonged hyponatremia, using hypertonic saline infusion to manage the hyponatremia in a patient with nephrotic syndrome. In our patient, the hyponatremia was probably attributable to a saltrestricted diet and long term diuretic therapy. Patients with nephrotic syndrome, and especially those with frequent relapses, are at high risk of developing myelinolysis if their hyponatremia is corrected rapidly. To the best of our knowledge, this is the first case report in the medical literature of extrapontine myelinolysis in a pediatric patient with nephrotic syndrome. The authors thank Sanjay Oak, MS, MCH, Director (Medical Education and Major Hospitals, Municipal Corporation of Greater Mumbai) and Dean of Seth Gorhandas Sunderdas Medical College and King Edward Memorial Hospital, for granting permission to publish the manuscript.
References [1] Niehaus L, Kulozik A, Lehmann R. Reversible central pontine and extrapontine myelinolysis in a 16-year-old girl. Childs Nerv Syst 2001;17: 294-6. [2] Bonkowsky JL, Filloux FM. Extrapontine myelinolysis in a pediatric case of diabetic ketoacidosis and cerebral edema. J Child Neurol 2003; 18:144-7. [3] Bibl D, Lampl C, Gabriel C, Jungling G, Brock H, Kostler G. Treatment of central pontine myelinolysis with therapeutic plasmapharesis. Lancet 1999;353:1155. [4] Karakas HM, Erdem G, Yakinci C. Osmotic demyelination syndrome in a 40-day-old infant. Diagn Interv Radiol 2007;13:121-4. [5] Brown WD, Caruso JM. Extrapontine myelinolysis with involvement of the hippocampus in three children with severe hypernatremia. J Child Neurol 1999;14:428-33. [6] Snell DM, Bartley C. Osmotic demyelination syndrome following rapid correction of hyponatraemia. Anaesthesia 2008;63:92-5. [7] Simon EE, Hamrahian SM. Hyponatremia. Available at: http:// emedicine.medscape.com/article/242166-overview. Accessed July 6, 2009. [8] Tomita I, Satoh H, Satoh A, Seto M, Tsujihata M, Yoshimura T. Extrapontine myelinolysis presenting with parkinsonism as a sequel of rapid correction of hyponatremia. J Neurol Neurosurg Psychiatry 1997; 62:422-3. [9] Bekiesinska-Figatowska M, Bulski T, Rozyczka I, Furmanek M, Walecki J. MR imaging of seven presumed cases of central pontine and extrapontine myelinolysis. Acta Neurobiol Exp (Warsz) 2001;61: 141-4. [10] Aydin OF, dem Uner C, Senbil N, Bek K, Erdogan O, Gurer YKY. Central pontine and extrapontine myelinolysis owing to disequilibrium syndrome. J Child Neurol 2003;18:292-6. [11] Brito AR, Vasconcelos MM, Cruz Junior LC, et al. Central pontine and extrapontine myelinolysis: Report of a case with a tragic outcome. J Pediatr (Rio J) 2006;82:157-60. [12] Wang AH. Osmotic demyelination syndrome associated with 3% hypertonic saline administration. Intern Med J 2008;38:219-20. [13] Finsterer J, Engelmayer E, Trnka E, Stiskal M. Immunoglobulins are effective in pontine myelinolysis. Clin Neuropharmacol 2000;23: 110-3.
Tullu et al: Extrapontine Myelinolysis 141