Abstract
Osmotic demyelination syndrome (ODS), previously known as central pontine myelinolysis, is a rare neurological condition characterized by demyelination of the pons or extrapontine areas including the midbrain, thalamus, basal nuclei, and cerebellum, resulting in upper motor neuron dysfunction and pseudobulbar palsy. We report a case of a 45-year-old woman with a history of alcohol dependence and end stage liver disease complicated by hepatic encephalopathy who developed symptoms suspicious of recurrent hepatic encephalopathy and experienced a generalized seizure during an inpatient stay. After 10 days of treatment with no improvement, it was noted that the patient had locked-in syndrome and that her sodium levels had rapidly risen 2 days prior. This led to a clinical suspicion of ODS, which was confirmed on T2-weighted MRI and subsequently on autopsy. In this clinical vignette, we review the clinical presentation, prognosis, and diagnostic considerations of ODS.
Author contributions: All the authors have accepted responsibility for the entire content of this submitted manuscript and approved submission.
Research funding: None declared.
Employment or leadership: None declared.
Honorarium: None declared.
Competing interests: The funding organization(s) played no role in the study design; in the collection, analysis, and interpretation of data; in the writing of the report; or in the decision to submit the report for publication.
References
1. Adams RA, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholics and malnourished patients. Arch Neurol Psychiatry 1959;81: 154–72.10.1001/archneurpsyc.1959.02340140020004Search in Google Scholar
2. Martin RJ. Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry 2004;75(Suppl 3):iii22–8.10.1136/jnnp.2004.045906Search in Google Scholar PubMed PubMed Central
3. Singh TD, Fugate JE, Rabinstein AA. Central pontine and extrapontine myelinolysis: a systematic review. Eur J Neurol 2014;21:1443–50.10.1111/ene.12571Search in Google Scholar PubMed
4. Berner ES, Graber ML. Overconfidence as a cause of diagnostic error in medicine. Am J Med 2008;121(Suppl 5):S2–23.10.1016/j.amjmed.2008.01.001Search in Google Scholar PubMed
5. Munasque A. ‘Thinking about thinking:’ Heuristics and the emergency physician. Emerg Med News 2009;31.10.1097/01.EEM.0000361895.47298.fbSearch in Google Scholar
6. Smith E, Delargy M. Locked-in syndrome. Br Med J 2005;330:406–9.10.1136/bmj.330.7488.406Search in Google Scholar PubMed PubMed Central
7. Wolf DC. Hepatic encephalopathy. Medscape [internet]. 2015 [cited 2015 Jun 22].Search in Google Scholar
8. Kallakatta RN, Radhakrishnan A, Fayaz RK, Unnikrishnan JP, Kesavadas C, Sarma SP. Clinical and functional outcome and factors predicting prognosis in osmotic demyelination syndrome (central pontine and/or extrapontine myelinolysis) in 25 patients. J Neurol Neurosurg Psychiatry 2011;82:326–31.10.1136/jnnp.2009.201764Search in Google Scholar PubMed
9. Laureno R, Karp BI. Myelinolysis after correction of hyponatremia. Ann Intern Med 1997;126:57–62.10.7326/0003-4819-126-1-199701010-00008Search in Google Scholar PubMed
10. Verbalis JG, Goldsmith SR, Greenberg A, Korzelius C, Schrier RW, Sterns RH, et al. Diagnosis, evaluation, and treatment of hyponatremia: expert panel recommendations. Am J Med 2013;126(10 Suppl 1):S1–42.10.1016/j.amjmed.2013.07.006Search in Google Scholar PubMed
11. Sterns RH. Overview of the treatment of hyponatremia. Uptodate [internet]. 2015 [cited 22 June 2015].Search in Google Scholar
12. Graff-Radford J, Fugate JE, Kaufmann TJ, Mandrekar JN, Rabinstein AA. Clinical and radiologic correlations of central pontine myelinolysis syndrome. Mayo Clin Proc 2011;86:1063–7.10.4065/mcp.2011.0239Search in Google Scholar PubMed PubMed Central
13. Menger H, Jörg J. Outcome of central pontine and extrapontine myelinolysis (n = 44). J Neurol 1999;246:700–5.10.1007/s004150050435Search in Google Scholar PubMed
14. Musana AK, Yale SH. Central pontine myelinolysis: case series and review. WMJ 2005;104:56–60.Search in Google Scholar
15. Brunner JE, Redmond JM, Haggar AM, Kruger DF, Elias SB. Central pontine myelinolysis and pontine lesions after rapid correction of hyponatremia: a prospective magnetic resonance imaging study. Ann Neurol 1990;27:61–6.10.1002/ana.410270110Search in Google Scholar PubMed
©2016 by De Gruyter
Articles in the same Issue
- Frontmatter
- Opinion Paper
- Healthcare is a giant with feet of clay
- Original Articles
- Diagnosis is a team sport – partnering with allied health professionals to reduce diagnostic errors
- An investigation into method of diagnosis in clinicopathologic conferences (CPCs)
- Primary care pediatricians’ interest in diagnostic error reduction
- Spurious elevation of serum potassium concentration measured in samples with thrombocytosis
- Mobile phone exposure influences some erythrocytes parameters in vitro. A novel source of preanalytical variability?
- Case Reports
- Those eyes don’t lie: a case of osmotic demyelination syndrome in a patient with hepatic encephalopathy
- CA-125: What does that lab really mean?
- Letter to the Editor
- Assessment of reticulated platelets with automated hemocytometers: are we measuring the same thing?
- Congress Abstracts
- Diagnostic Error in Medicine 1st European Conference June 30–July 1, 2016
Articles in the same Issue
- Frontmatter
- Opinion Paper
- Healthcare is a giant with feet of clay
- Original Articles
- Diagnosis is a team sport – partnering with allied health professionals to reduce diagnostic errors
- An investigation into method of diagnosis in clinicopathologic conferences (CPCs)
- Primary care pediatricians’ interest in diagnostic error reduction
- Spurious elevation of serum potassium concentration measured in samples with thrombocytosis
- Mobile phone exposure influences some erythrocytes parameters in vitro. A novel source of preanalytical variability?
- Case Reports
- Those eyes don’t lie: a case of osmotic demyelination syndrome in a patient with hepatic encephalopathy
- CA-125: What does that lab really mean?
- Letter to the Editor
- Assessment of reticulated platelets with automated hemocytometers: are we measuring the same thing?
- Congress Abstracts
- Diagnostic Error in Medicine 1st European Conference June 30–July 1, 2016