Central Pontine and Extrapontine Myelinolysis in Children

Ranger, Adrianna; Chaudhary, Navjot; Avery, Michael B; Fraser, Douglas D.

doi:10.1177/0883073812445908

Cited by 30 publications

(23 citation statements)

References 103 publications

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“…These disturbances in brain water balance can be viewed as being analogous to those associated with extrapontine myelinolysis, which is typically found in conjunction with fluid/electrolyte disturbances and has been associated with diabetes (21,22). The large U.K. case-control series of DKA-associated cerebral edema did not show an association with any changes in blood glucose after therapy (6).…”

Section: Discussionmentioning

confidence: 99%

Neurological Consequences of Diabetic Ketoacidosis at Initial Presentation of Type 1 Diabetes in a Prospective Cohort Study of Children

et al. 2014

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OBJECTIVETo investigate the impact of new-onset diabetic ketoacidosis (DKA) during childhood on brain morphology and function.RESEARCH DESIGN AND METHODSPatients aged 6–18 years with and without DKA at diagnosis were studied at four time points: <48 h, 5 days, 28 days, and 6 months postdiagnosis. Patients underwent magnetic resonance imaging (MRI) and spectroscopy with cognitive assessment at each time point. Relationships between clinical characteristics at presentation and MRI and neurologic outcomes were examined using multiple linear regression, repeated-measures, and ANCOVA analyses.RESULTSThirty-six DKA and 59 non-DKA patients were recruited between 2004 and 2009. With DKA, cerebral white matter showed the greatest alterations with increased total white matter volume and higher mean diffusivity in the frontal, temporal, and parietal white matter. Total white matter volume decreased over the first 6 months. For gray matter in DKA patients, total volume was lower at baseline and increased over 6 months. Lower levels of N-acetylaspartate were noted at baseline in the frontal gray matter and basal ganglia. Mental state scores were lower at baseline and at 5 days. Of note, although changes in total and regional brain volumes over the first 5 days resolved, they were associated with poorer delayed memory recall and poorer sustained and divided attention at 6 months. Age at time of presentation and pH level were predictors of neuroimaging and functional outcomes.CONCLUSIONSDKA at type 1 diabetes diagnosis results in morphologic and functional brain changes. These changes are associated with adverse neurocognitive outcomes in the medium term.

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Section: Discussionmentioning

confidence: 99%

Neurological Consequences of Diabetic Ketoacidosis at Initial Presentation of Type 1 Diabetes in a Prospective Cohort Study of Children

et al. 2014

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“…As in the mRS, patients were rated from 0‐6 according to the level of their acute disability, but without ADL assessment as that is usually not considered in the acute setting. A similar rating of ODS symptoms was conducted in a review of pediatric ODS cases by Ranger et al…”

Section: Methodsmentioning

confidence: 99%

Incidence of osmotic demyelination syndrome in Sweden: A nationwide study

et al. 2019

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Objective To report the incidence rate of osmotic demyelination syndrome (ODS), associated risk factors, treatment, and long‐term outcomes in a nationwide cohort. Methods We conducted a retrospective study of individuals diagnosed with central pontine myelinolysis (ICD‐10 code G37.2) in the Swedish National Patient Register during 1997‐2011. Results During the study period, we identified 83 individuals with ODS, 47 women and 36 men. Median age at diagnosis was 55 years. The incidence rate of ODS for the entire study period was 0.611 (95% CI: 0.490‐0.754) per million person‐years and increased during the study period from 0.271 (95% CI: 0.147‐0.460) in 1997‐2001 to 0.945 (95% CI: 0.677‐1.234) individuals per million person‐years in 2007‐2011. Most cases (86.7%) were hyponatremic with a median sodium level at admission of 104 mmol/L. All hyponatremic cases were chronic. The cause of hyponatremia was multifactorial, including drugs (56.9%), polydipsia (31.9%), and vomiting or diarrhea (41.7%). A majority of patients (69.9%) were alcoholics. Hyponatremic patients were predominantly treated with isotonic saline (93.1%) and only 4.2% with hypotonic fluids. The median correction rate was 0.72 mmol/L/h. Only six patients were corrected in accordance with national guidelines (≤8 mmol/L/24/h). At three months, 7.2% had died and 60.2% were functionally independent (modified Rankin Scale 0‐2). Interpretation We found an increasing incidence during the study period, which could partly be explained by increased access to magnetic resonance imaging. ODS occurs predominantly in patients with extreme chronic hyponatremia which is corrected too fast with isotonic saline. Most patients survived and became functionally independent.

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“…With the improvement of MRI resolution, the number of case reports on ODS in children has recently been increasing. According to previous reports, EPM without CPM comprised 22% of the cases reviewed (13/76 cases), but EPM without CPM associated with hypernatremia accounted for only three cases in the English‐language literature . EPM without CPM associated with hypernatremia in children was first reported in 1999 by Brown and Caruso, who provided detailed accounts of three cases .…”

Section: Discussionmentioning

confidence: 99%

Extrapontine myelinolysis associated with severe hypernatremia in infancy

Aoki

Morimoto

Takahashi

et al. 2016

Pediatrics International

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Extrapontine myelinolysis (EPM) is an uncommon disorder in children, with few pediatric cases reported to date. We report the first case of an infant with EPM without central pontine myelinolysis (CPM) presenting with severe hypernatremia. On admission, the infant had impaired consciousness, mild dehydration, and severe hypernatremia (190 mmol/L). The following day, the patient developed abnormal involuntary movements. Brain magnetic resonance imaging (MRI) confirmed EPM without CPM. He recovered without sequelae, and clinical examinations were within normal limits approximately 6 months after discharge. Brain MRI at 1 year after onset showed complete disappearance of the previous EPM regions. To the best of our knowledge, this represents the youngest patient with EPM without CPM presenting with severe hypernatremia. Given that treatment for osmotic demyelination syndrome (ODS) is yet to be established, preventing the development of ODS is crucial.

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Central Pontine and Extrapontine Myelinolysis in Children

Cited by 30 publications

References 103 publications

Neurological Consequences of Diabetic Ketoacidosis at Initial Presentation of Type 1 Diabetes in a Prospective Cohort Study of Children

Neurological Consequences of Diabetic Ketoacidosis at Initial Presentation of Type 1 Diabetes in a Prospective Cohort Study of Children

Incidence of osmotic demyelination syndrome in Sweden: A nationwide study

Extrapontine myelinolysis associated with severe hypernatremia in infancy

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