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Central pontine myelinolysis

Loss of myelinated fibers at the basilar part of the pons in the brainstem (Luxol-Fast blue stain) Source: Wikipedia: Jensflorian https://en.wikipedia.org/wiki/Central_pontine_myelinolysis#Pathophysiology

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: , Mohsen Basiri M.D.

Synonyms and keywords:

Overview

Overview

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]


Overview

The most common cause of central pontine myelinolysis is a rapid correction(>48-hours duration) of hyponatremia in patients with the history of prolonged hyponatremia. Other causes of central pontine myelinolysis may include: Lengthened orthotopic liver transplantation, hypophosphatemia secondary to refeeding syndrome, deficiencies in neuronal/glial cell energy supply and utilization which produce glial cell apoptosis and thus the clinical syndrome of central pontine myelinolysis and prolonged ischemia. The most potent risk factor in the development of central pontine myelinolysis is hyponatremia. Other risk factors include: Liver dysfunction and liver diseases, hypocholesterolemia, alcoholism, malnutrition, systemic medical disease and hemodialysis. Brain MRI is the gold standard test for the diagnosis of central pontine myelinolysis. The following findings on performing Brain MRI are confirmatory for central pontine myelinolysis: T2 hyperintensity in the central pontine region in the axial plane and hyperintense lesion in the midpons in the midsagittal T2-weighted MRI. Treatment of patients with central pontine myelinolysis is mainly supportive because once the osmotic demyelination has begun, there is no cure or specific treatment. Alcoholic patients should receive vitamin supplementation including vitamin B6, B9 and B12 and evaluation of their nutritional status.

Historical Perspective

Central pontine myelinolysis was first discovered by Raymond Delacy Adams, an an American neurologist, in 1959. Raymond Delacy Adams and colleagues observed a rapidly evolving quadriplegia and pseudobulbar palsy in a young alcoholic man whose postmortem examination showed a large, symmetrical, essentially demyelinative lesion occupying the greater part of the base of the pons In 1950.

Pathophysiology

It is understood that central pontine myelinolysis is caused by the rapid correction of hyponatremia. The CNS is particularly susceptible to reductions in plasma osmolarity, specially during hyponatremia which is the most commonly encountered electrolyte disturbance. When a decrease in the plasma osmolarity happens, neural cells first swell but then they are able to regain their original volume through the release of inorganic and organic osmolytes and exit of osmotically obligated water. Subsequent exposure to hypertonic stress(e.g., correction of hyponatremia with hypertonic I.V. solutions)resulting from a rapid correction of hyponatremia causes the ions to quickly re-enter the intracellular space and compels the water to follow. If the serum sodium levels rise too rapidly, the increased extracellular tonicity will continue to drive water out of the brain’s cells because the brain cells do not have enough time to bring extracellular sodium into the cell, so the water goes out very fast. This can lead to cellular dysfunction and central pontine myelinolysis and finally death.

Causes

The most common cause of central pontine myelinolysis is rapid correction(>48-hours duration) of hyponatremia in patients with the history of prolonged hyponatremia. Other causes of central pontine myelinolysis may include: Lengthened orthotopic liver transplantation, hypophosphatemia secondary to refeeding syndrome, deficiencies in neuronal/glial cell energy supply and utilization which produce glial cell apoptosis and thus the clinical syndrome of central pontine myelinolysis and prolonged ischemia.

Differentiating central pontine myelinolysis from Other Diseases

On the basis central pontine myelinolysis must be differentiated diseases that cause acute confusion, lethargy, speech difficulties and bilateral weakness or quadriplegia such as: Posterior leukoencephalopathy syndrome, infective encephalitis, ischemic Brain stem infarction, thalamus infarction due thrombosis of the basilar artery, diffuse hypoxic encephalopathy, metastasis to the brain and brain tumors such as glioma.

Epidemiology and Demographics

The prevalence of central pontine myelinolysis is approximately 250–500 per 100,000 in the general population. Among hospitalized patients in the ICU the incidence of central pontine myelinolysis is approximately 2500 per 100,000 patients. Among patients undergoing liver transplantation the incidence of central pontine myelinolysis is approximately 10,000 per 100,000 patients. The case-mortality rate of central pontine myelinolysis is approximately 12%. Patients of all age groups may develop central pontine myelinolysis but the incidence of central pontine myelinolysis increases with age. There is no racial predilection to central pontine myelinolysis. Central pontine myelinolysis affects men and women equally. There is no regional predilection to central pontine myelinolysis.

Risk Factors

The most potent risk factor in the development of central pontine myelinolysis is hyponatremia. Other risk factors include: Liver dysfunction and liver diseases, hypocholesterolemia, alcoholism, malnutrition, systemic medical disease and hemodialysis.

Natural History, Complications, and Prognosis

The symptoms of central pontine myelinolysis typically develop immediately after injury to the neurons of the brain stem. Patients, with Central Pontine Myelinolysis, may develop permanent neurological damages. Common complications of central pontine myelinolysis include: Locked-in syndrome, quadriparesis, ataxia, acute Psychosis, pseudobulbar palsy, parkinson’s disease symptoms, dystonia, pneumonia, coma and death. The mortality of patients with central pontine myelinolysis is approximately 8% in the acute setting. Approximately 65% of patients with central pontine myelinolysis may achieve a good or moderate outcome (no functional deficit or independence despite minor deficits). Depending on the time of the diagnosis, the prognosis may vary and the disease may be potentially reversible when therapeutic interventions are initiated rapidly.

Diagnosis

Diagnostic Study of Choice

Brain MRI is the gold standard test for the diagnosis of central pontine myelinolysis. The following findings on performing Brain MRI are confirmatory for central pontine myelinolysis: T2 hyperintensity in the central pontine region in the axial plane and hyperintense lesion in the midpons in the midsagittal T2-weighted MRI.

History and Symptoms

Patients with central pontine myelinolysis may have a positive history of: Malnutrition, alcohol use disorder, chronic liver disease, hyperemesis gravidarum, hypophosphatemia secondary to refeeding syndrome and prolonged ischemia. These patients with central pontine myelinolysis most commonly have a history of rapid sodium correction, greater than 0.5-1.0 mEq/L per hour. The most susceptible patients are those with: Chronic hyponatremia (>48 hours), severe hyponatremia (Na <120 mEq/L) and both chronic hyponatremia and severe hyponatremia. Common symptoms of central pontine myelinolysis include: Spastic quadriparesis, dysarthria, pseudobulbar palsy and altered mental status. In some patients, parkinsonian features, behavioral manifestations, and neuropsychological symptoms can also be present: Personality changes, labile affect, disinhibition, poor judgment, paranoid delusions, emotional lability, delirium, hallucinations and catatonia.

Laboratory Findings

Laboratory finding consistent with the diagnosis of central pontine myelinolysis is hypoosmotic hyponatremia and the rapid correction of hyponatremia is the cause of central pontine myelinolysis.

CT scan

Brain CT scan may be helpful in the diagnosis of central pontine myelinolysis. Findings on CT scan suggestive of central pontine myelinolysis include: A symmetric, centrally located region of low attenuation within the pons and symmetric low-attenuation foci within the lateral thalami.

MRI

Brain and spinal cord MRIs may be helpful in the diagnosis of Central pontine myelinolysis. Findings on MRI diagnostic of Central pontine myelinolysis include: Symmetric signal intensity abnormality in the central pons at T2-weighted and FLAIR imaging which may progress to classic hyperintense “trident-shaped” central pontine abnormality, with sparing of the ventrolateral pons and corticospinal tracts, decreased T1 signal intensity, fluid attenuated inversion recovery (FLAIR) hyperintense lesion in the pons and intramedullary central T2 hyperintensity at axial T2W of spinal cord and sagittal T2W of thoracic spinal cord.

Treatment

Medical Therapy

Treatment of patients with central pontine myelinolysis is mainly supportive because once the osmotic demyelination has begun, there is no cure or specific treatment. Alcoholic patients should receive vitamin supplementation including vitamin B6, B9 and B12 and evaluation of their nutritional status.

Primary Prevention

To minimize the risk of central pontine myelinolysis developing from its most common cause, overly rapid reversal of hyponatremia, the hyponatremia should be corrected slowly. The primary goals of treating hypernatremia are estimating the magnitude of water deficit, determining the proper rate of correction, addressing the concurrent electrolyte or volume deficits and calculating the fluid deficit regimen using the estimated water deficit and desired rate of correction. Correcting sodium level is vital in order to prevent any permanent brain damage.

References


Template:WikiDoc Sources

Historical Perspective

Historical Perspective

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: , Mohamadmostafa Jahansouz M.D.[2]

Overview

Central pontine myelinolysis was first discovered by Raymond Delacy Adams, an an American neurologist, in 1959. Raymond Delacy Adams and colleagues observed a rapidly evolving quadriplegia and pseudobulbar palsy in a young alcoholic man whose postmortem examination showed a large, symmetrical, essentially demyelinative lesion occupying the greater part of the base of the pons In 1950.

Historical Perspective

Discovery

  • Central pontine myelinolysis was first discovered by Raymond Delacy Adams, an an American neurologist, in 1959.[1][2][3][4]
  • Raymond Delacy Adams and colleagues observed a rapidly evolving quadriplegia and pseudobulbar palsy in a young alcoholic man whose postmortem examination showed a large, symmetrical, essentially demyelinative lesion occupying the greater part of the base of the pons In 1950.[2][3][4]

References

  1. ↑ Fisher CM (2010). “Dr. Raymond Delacy Adams (1911-2008): an appreciation”. Neurologist. 16 (3): 141–2. doi:10.1097/NRL.0b013e3181c9280a. PMID 20445423.
  2. ↑ 2.0 2.1 ADAMS RD, VICTOR M, MANCALL EL (1959). “Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients”. AMA Arch Neurol Psychiatry. 81 (2): 154–72. PMID 13616772.
  3. ↑ 3.0 3.1 Mohammed AS, Boddu P, Yazdani DF (2016). “Clinical Evolution of Central Pontine Myelinolysis in a Patient with Alcohol Withdrawal: A Blurred Clinical Horizon”. Case Rep Med. 2016: 6065259. doi:10.1155/2016/6065259. PMC 5004014. PMID 27610136.
  4. ↑ 4.0 4.1 Kumar S, Fowler M, Gonzalez-Toledo E, Jaffe SL (2006). “Central pontine myelinolysis, an update”. Neurol Res. 28 (3): 360–6. doi:10.1179/016164106X110346. PMID 16687066.

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Classification

Classification

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Overview

There is no established system for the classification of central pontine myelinolysis.

Classification

There is no established system for the classification of central pontine myelinolysis.

References

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Pathophysiology

Pathophysiology

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

Overview

It is understood that central pontine myelinolysis is caused by the rapid correction of hyponatremia. The CNS is particularly susceptible to reductions in plasma osmolarity, specially during hyponatremia which is the most commonly encountered electrolyte disturbance. When a decrease in the plasma osmolarity happens, neural cells first swell but then they are able to regain their original volume through the release of inorganic and organic osmolytes and exit of osmotically obligated water. Subsequent exposure to hypertonic stress(e.g., correction of hyponatremia with hypertonic I.V. solutions)resulting from a rapid correction of hyponatremia causes the ions to quickly re-enter the intracellular space and compels the water to follow. If the serum sodium levels rise too rapidly, the increased extracellular tonicity will continue to drive water out of the brain’s cells because the brain cells do not have enough time to bring extracellular sodium into the cell, so the water goes out very fast. This can lead to cellular dysfunction and central pontine myelinolysis and finally death.

Pathophysiology

Pathogenesis

  • It is understood that central pontine myelinolysis is caused by rapid correction of hyponatremia.[1][2]
  • The CNS is particularly susceptible to reductions in plasma osmolarity, specially during hyponatremia which is the most commonly encountered electrolyte disturbance.
  • When a decrease in the plasma osmolarity happens, neural cells first swell but then they are able to regain their original volume through the:[2][3]
    1. Release of inorganic and organic osmolytes
    2. Exit of osmotically obligated water
  • A subsequent exposure to hypertonic stress(e.g., correction of hyponatremia with hypertonic I.V. solution’s)resulting from a rapid correction of hyponatremia causes the ions to quickly re-enter the intracellular space and compels the water to follow.[4][2][1]
  • If the serum sodium levels rise too rapidly, the increased extracellular tonicity will continue to drive water out of the brain neurons because the brain cells do not have enough time to bring extracellular sudiom into the cell, so the water goes out very fast instead.[2][1]
  • This can lead to cellular dysfunction and central pontine myelinolysis and finally death.[2][1]

Genetics

There is no association between central pontine myelinolysis and genetic factors.

Gross Pathology

  • On gross pathology of patients with central pontine myelinolysis a red, central, triangular region of softening may be seen with preservation of the surrounding parenchyma on opened sagittally pons and medulla from the ventral aspect.[5]
  • Central and symetrical demyelination in the base of the pons in cross section samples[6]


Microscopic Pathology

On microscopic histopathological analysis, the following features are characteristic findings of central pontine myelinolysis:[7][6]

    • Loss of myelinated fibers at the base of the pons that is usually central and symmetrical but also can be neither
    • The lesions are sharply demarcated and contain sheets of lipid‐laden macrophages and large numbers of reactive astrocytes.
    • Infiltration by lymphocytes is sparse or absent.
    • Axons and neurons are mostly well preserved.
    • Symmetrically spared “islands” of myelinated white matter


    References

    1. ↑ 1.0 1.1 1.2 1.3 Mascarenhas JV, Jude EB (2014). “Central pontine myelinolysis: electrolytes and beyond”. BMJ Case Rep. 2014. doi:10.1136/bcr-2013-203516. PMC 3975522. PMID 24682140.
    2. ↑ 2.0 2.1 2.2 2.3 2.4 Khosya S, Meena H (2013). “Central pontine myelinolysis”. Indian J Med Res. 137 (5): 993–4. PMC 3734697. PMID 23760391.
    3. ↑ Burg MB, Ferraris JD (2008) Intracellular organic osmolytes: function and regulation. J Biol Chem 283 (12):7309-13. DOI:10.1074/jbc.R700042200 PMID: 18256030
    4. ↑ Sheikh AB, Afzal RM, Sagheer S, Bukhari MM, Javed A, Nasrullah A et al. z (2018). “The Dilemma of Inadvertent Pontine Demyelinosis: A Review of Literature”. Cureus. 10 (8): e3174. doi:10.7759/cureus.3174. PMC 6197531. PMID 30357070.
    5. ↑ Haynes HR, Gallagher PJ, Cordaro A, Likeman M, Love S (2018). “A case of chronic asymptomatic central pontine myelinolysis with histological evidence of remyelination”. Forensic Sci Med Pathol. 14 (1): 106–108. doi:10.1007/s12024-017-9933-y. PMC 5830465. PMID 29177819.
    6. ↑ 6.0 6.1 Robinson CA, Adiele RC, Tham M, Lucchinetti CF, Popescu BF (2014). “Early and widespread injury of astrocytes in the absence of demyelination in acute haemorrhagic leukoencephalitis”. Acta Neuropathol Commun. 2: 52. doi:10.1186/2051-5960-2-52. PMC 4035095. PMID 24887055.
    7. ↑ Love S (2006). “Demyelinating diseases”. J Clin Pathol. 59 (11): 1151–9. doi:10.1136/jcp.2005.031195. PMC 1860500. PMID 17071802.

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    Causes

    Causes

    Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: , Mohamadmostafa Jahansouz M.D.[2]

    Overview

    The most common cause of central pontine myelinolysis is rapid correction(>48-hours duration) of hyponatremia in patients with the history of prolonged hyponatremia. Other causes of central pontine myelinolysis may include: Lengthened orthotopic liver transplantation, hypophosphatemia secondary to refeeding syndrome, deficiencies in neuronal/glial cell energy supply and utilization which produce glial cell apoptosis and thus the clinical syndrome of central pontine myelinolysis and prolonged ischemia.

    Causes

    Common Causes

    The most common cause of central pontine myelinolysis is rapid correction(>48-hours duration) of hyponatremia in patients with the history of prolonged hyponatremia.[1][2]

    Other causes of central pontine myelinolysis may include:[3][4][5][6]

    References

    1. ↑ Seliger S, Kestenbaum B (2018). “Commentary on Treatment of Severe Hyponatremia”. Clin J Am Soc Nephrol. 13 (4): 650–651. doi:10.2215/CJN.13381217. PMC 5969468. PMID 29295827.
    2. ↑ Yu J, Zheng SS, Liang TB, Shen Y, Wang WL, Ke QH (2004). “Possible causes of central pontine myelinolysis after liver transplantation”. World J Gastroenterol. 10 (17): 2540–3. doi:10.3748/wjg.v10.i17.2540. PMC 4572157. PMID 15300900.
    3. ↑ Norenberg MD (2010). “Central pontine myelinolysis: historical and mechanistic considerations”. Metab Brain Dis. 25 (1): 97–106. doi:10.1007/s11011-010-9175-0. PMID 20182780.
    4. ↑ Yamashita C, Shigeto H, Maeda N, Torii T, Ohyagi Y, Kira J (2015). “A Case of Central Pontine Myelinolysis Caused by Hypophosphatemia Secondary to Refeeding Syndrome”. Case Rep Neurol. 7 (3): 196–203. doi:10.1159/000440711. PMC 4637517. PMID 26557081.
    5. ↑ Ashrafian H, Davey P (2001). “A review of the causes of central pontine myelinosis: yet another apoptotic illness?”. Eur J Neurol. 8 (2): 103–9. PMID 11430268.
    6. ↑ Giannetti AV, Pittella JE (1993). “Ischemic and hemorrhagic necrosis of the pons with anatomical location similar to that of central pontine myelinolysis in a chronic alcoholic patient”. Clin Neuropathol. 12 (3): 156–9. PMID 8324964.

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    Differentiating Central pontine myelinolysis from other Diseases

    Differentiating Central pontine myelinolysis from other Diseases

    Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

    Overview

    On the basis central pontine myelinolysis must be differentiated diseases that cause acute confusion, lethargy, speech difficulties and bilateral weakness or quadriplegia such as: Posterior leukoencephalopathy syndrome, infective encephalitis, ischemic Brain stem infarction, thalamus infarction due thrombosis of the basilar artery, diffuse hypoxic encephalopathy, metastasis to the brain and brain tumors such as glioma.

    Differentiating central pontine myelinolysis from other Diseases

    On the basis central pontine myelinolysis must be differentiated diseases that cause acute confusion, lethargy, speech difficulties and bilateral weakness or quadriplegia such as:[1][2][3][4][5][6][7]

    Diseases Clinical manifestations Para-clinical findings Gold standard Additional findings
    Lab Findings Imaging
    Confusion Weakness and quadriplegia Speech difficulties Hypoosmotic hyponatremia MRI
    Central pontine myelinolysis ++ ++ ++ ++
    • Symmetric signal intensity abnormality in the central pons at T2-weighted and FLAIR imaging
    • Decreased T1 signal intensity
    • Fluid attenuated inversion recovery (FLAIR) hyperintense lesion in the pons
    • Intramedullary central T2 hyperintensity at axial T2W of spinal cord and sagittal T2W of thoracic spinal cord
    		 MRI The most common cause of central pontine myelinolysis is rapid correction(>48-hours duration) of hyponatremia in patients with the history of prolonged hyponatremia
    Posterior leukoencephalopathy syndrome ++ -/+ -/+ – Multiple cortico-subcortical areas of T2-weighted hyperintense signal involving the occipital and parietal lobes bilaterally and pons. MRI Other symptoms include: Seizure, headache, visual disturbances, focal neurologic signs, and status epilepticus.

    Many cases resolve within 1–2 weeks of controlling blood pressure and eliminating the inciting factor.

    Ischemic Brain stem infarction -/+ + -/+ – Abnormal high signal within the brainstem white matter and gray matter MRI


    The brain stem infarct can affect essential body functions, such as:

    • Breathing
    • Swallowing
    • Eye movement
    • Facial movement and sensation
    • Hearing
    • Heart rate
    • Blood pressure
    • Body temperature
    Hypertensive encephalopathy -/+ -/+ + –
    • Diffuse extensive periventricular deep white matter T2 hyperintensity changes
    • Ex vacuo ventriculomegaly
    • Global, symmetrical, supra and infratentorial involutional changes
    		 MRI Symptoms typically start to occur 12–48 hours after a sudden and sustained increase in blood pressure.

    The first symptom is a severe headache.

    Other symptoms include:

    Impaired judgement and memory

    Infective encephalitis -/+ -/+ -/+ –
    • High signal in both white and grey matter
    • Region of hypodensity
    		 MRI


    References

    1. ↑ Kawabori M, Murata J, Abe S, Saito H (2009). “[A case of brainstem variant of reversible posterior leukoencephalopathy syndrome]”. No Shinkei Geka. 37 (11): 1105–9. PMID 19938667.
    2. ↑ Osman Y, Imam YZ, Salem K, Al-Hail H, Uthman B, Deleu D (2013). “Isolated brainstem involvement in a patient with hypertensive encephalopathy”. Case Rep Neurol Med. 2013: 540947. doi:10.1155/2013/540947. PMC 3600275. PMID 23533856.
    3. ↑ Uchino A, Sawada A, Takase Y, Kudo S (2004). “Symmetrical lesions of the middle cerebellar peduncle: MR imaging and differential diagnosis”. Magn Reson Med Sci. 3 (3): 133–40. doi:10.2463/mrms.3.133. PMID 16093630.
    4. ↑ Uzkeser M, Akoz A, Ozdemir G, Emet M, Bayramoglu A (2012). “Wide central pontine, bulbar and thalamic myelinolysis with sequela”. Eurasian J Med. 44 (3): 179–81. doi:10.5152/eajm.2012.42. PMC 4261386. PMID 25610237.
    5. ↑ Choi JM, Kim YH, Roh SY (2013). “Acute hepatic encephalopathy presenting as cortical laminar necrosis: case report”. Korean J Radiol. 14 (2): 324–8. doi:10.3348/kjr.2013.14.2.324. PMC 3590348. PMID 23482893.
    6. ↑ Quattrocchi CC, Errante Y, Rossi Espagnet MC, Galassi S, Della Sala SW, Bernardi B; et al. (2016). “Magnetic resonance imaging differential diagnosis of brainstem lesions in children”. World J Radiol. 8 (1): 1–20. doi:10.4329/wjr.v8.i1.1. PMC 4731345. PMID 26834941.
    7. ↑ Shalchi Z, Bennett A, Hargroves D, Nash J (2009). “Diagnostic delay in a case of herpes simplex encephalitis”. BMJ Case Rep. 2009. doi:10.1136/bcr.12.2008.1350. PMC 3028237. PMID 21686359.

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    Epidemiology and Demographics

    Epidemiology and Demographics

    Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

    Overview

    The prevalence of central pontine myelinolysis is approximately 250–500 per 100,000 in the general population. Among hospitalized patients in the ICU the incidence of central pontine myelinolysis is approximately 2500 per 100,000 patients. Among patients undergoing liver transplantation the incidence of central pontine myelinolysis is approximately 10,000 per 100,000 patients. The case-mortality rate of central pontine myelinolysis is approximately 12%. Patients of all age groups may develop central pontine myelinolysis but the incidence of central pontine myelinolysis increases with age. There is no racial predilection to central pontine myelinolysis. Central pontine myelinolysis affects men and women equally. There is no regional predilection to central pontine myelinolysis.

    Epidemiology and Demographics

    Incidence and prevalence

    • The prevalence of central pontine myelinolysis is approximately 250–500 per 100,000 in the general population.[1]
    • Among hospitalized patients in the ICU the incidence of central pontine myelinolysis is approximately 2500 per 100,000 patients.[2][1]
    • Among patients undergoing liver transplantation the incidence of central pontine myelinolysis is approximately 10,000 per 100,000 patients.[2][3][1]

    Mortality rate

    Age

    • Patients of all age groups may develop central pontine myelinolysis but the incidence of central pontine myelinolysis increases with age.

    Race

    • There is no racial predilection to central pontine myelinolysis.

    Gender

    • Central pontine myelinolysis affects men and women equally.[4]

    Region

    • There is no regional predilection to central pontine myelinolysis.

    References

    1. ↑ 1.0 1.1 1.2 1.3 Rao PB, Azim A, Singh N, Baronia AK, Kumar A, Poddar B (2015). “Osmotic demyelination syndrome in Intensive Care Unit”. Indian J Crit Care Med. 19 (3): 166–9. doi:10.4103/0972-5229.152760. PMC 4366916. PMID 25810613.
    2. ↑ 2.0 2.1 Menger H, Jörg J (1999). “Outcome of central pontine and extrapontine myelinolysis (n = 44)”. J Neurol. 246 (8): 700–5. PMID 10460448.
    3. ↑ Lampl C, Yazdi K (2002). “Central pontine myelinolysis”. Eur Neurol. 47 (1): 3–10. doi:10.1159/000047939. PMID 11803185.
    4. ↑ Bansal LR, Zinkus T (2019). “Osmotic Demyelination Syndrome in Children”. Pediatr Neurol. 97: 12–17. doi:10.1016/j.pediatrneurol.2019.03.018. PMID 31128892.

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    Risk Factors

    Risk Factors

    Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

    Overview

    The most potent risk factor in the development of central pontine myelinolysis is hyponatremia. Other risk factors include: Liver dysfunction and liver diseases, hypocholesterolemia, alcoholism, malnutrition, systemic medical disease and hemodialysis.

    Risk Factors

    Common Risk Factors

    References

    1. ↑ Lee EM, Kang JK, Yun SC, Kim KH, Kim SJ, Hwang KS; et al. (2009). “Risk factors for central pontine and extrapontine myelinolysis following orthotopic liver transplantation”. Eur Neurol. 62 (6): 362–8. doi:10.1159/000242426. PMID 19797900.
    2. ↑ Jacob S, Gupta H, Nikolic D, Gundogdu B, Ong S (2014). “Central pontine and extrapontine myelinolysis: the great masquerader-an autopsy case report”. Case Rep Neurol Med. 2014: 745347. doi:10.1155/2014/745347. PMC 3970353. PMID 24716023.
    3. ↑ Martin RJ (2004). “Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes”. J Neurol Neurosurg Psychiatry. 75 Suppl 3: iii22–8. doi:10.1136/jnnp.2004.045906. PMC 1765665. PMID 15316041.
    4. ↑ Oo TN, Smith CL, Swan SK (2003). “Does uremia protect against the demyelination associated with correction of hyponatremia during hemodialysis? A case report and literature review”. Semin Dial. 16 (1): 68–71. PMID 12535304.

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    Screening

    Screening

    Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

    Overview

    There is insufficient evidence to recommend routine screening for central pontine myelinolysis.

    Screening

    There is insufficient evidence to recommend routine screening for central pontine myelinolysis.

    References

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    Natural History, Complications and Prognosis

    Natural History, Complications and Prognosis

    Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohamadmostafa Jahansouz M.D.[2]

    Overview

    The symptoms of central pontine myelinolysis typically develop immediately after injury to the neurons of the brain stem. Patients, with Central Pontine Myelinolysis, may develop permanent neurological damages. Common complications of central pontine myelinolysis include: Locked-in syndrome, quadriparesis, ataxia, acute Psychosis, pseudobulbar palsy, parkinson’s disease symptoms, dystonia, pneumonia, coma and death. The mortality of patients with central pontine myelinolysis is approximately 8% in the acute setting. Approximately 65% of patients with central pontine myelinolysis may achieve a good or moderate outcome (no functional deficit or independence despite minor deficits). Depending on the time of the diagnosis, the prognosis may vary and the disease may be potentially reversible when therapeutic interventions are initiated rapidly.

    Natural History, Complications, and Prognosis

    Natural History

    • The symptoms of central pontine myelinolysis typically develop immediately after injury to the neurons of the brain stem.

    Complications

    • Patients, with Central Pontine Myelinolysis, may develop permanent neurological damages.

    Prognosis

    • The mortality of patients with central pontine myelinolysis is approximately 8% in the acute setting.[7]
    • Approximately 65% of patients with central pontine myelinolysis may achieve a good or moderate outcome (no functional deficit or independence despite minor deficits).[8][7]
    • Depending on the time of the diagnosis, the prognosis may vary and the disease may be potentially reversible when therapeutic interventions are initiated rapidly.[9]

    References

    1. ↑ Sohn MK, Nam JH (2014). “Locked-in Syndrome due to Central Pontine Myelinolysis: Case Report”. Ann Rehabil Med. 38 (5): 702–6. doi:10.5535/arm.2014.38.5.702. PMC 4221401. PMID 25379502.
    2. ↑ Gopal M, Parasram M, Patel H, Ilorah C, Nersesyan H (2017). “Acute Psychosis as Main Manifestation of Central Pontine Myelinolysis”. Case Rep Neurol Med. 2017: 1471096. doi:10.1155/2017/1471096. PMC 5368399. PMID 28392953.
    3. ↑ Pfister HW, Einhäupl KM, Brandt T (1985). “Mild central pontine myelinolysis: a frequently undetected syndrome”. Eur Arch Psychiatry Neurol Sci. 235 (3): 134–9. PMID 4092709.
    4. ↑ Dolciotti C, Nuti A, Cipriani G, Borelli P, Baldacci F, Logi C; et al. (2010). “Cerebellar ataxia with complete clinical recovery and resolution of MRI lesions related to central pontine myelinolysis: case report and literature review”. Case Rep Neurol. 2 (3): 157–62. doi:10.1159/000323429. PMC 3098816. PMID 21607027.
    5. ↑ Seiser A, Schwarz S, Aichinger-Steiner MM, Funk G, Schnider P, Brainin M (1998). “Parkinsonism and dystonia in central pontine and extrapontine myelinolysis”. J Neurol Neurosurg Psychiatry. 65 (1): 119–21. doi:10.1136/jnnp.65.1.119. PMC 2170170. PMID 9667573.
    6. ↑ Grech R, Galvin L, Brennan P, Thornton J (2013). “Central pontine myelinolysis”. BMJ Case Rep. 2013. doi:10.1136/bcr-2013-008920. PMC 3645781. PMID 23608854.
    7. ↑ 7.0 7.1 Graff-Radford J, Fugate JE, Kaufmann TJ, Mandrekar JN, Rabinstein AA (2011). “Clinical and radiologic correlations of central pontine myelinolysis syndrome”. Mayo Clin Proc. 86 (11): 1063–7. doi:10.4065/mcp.2011.0239. PMC 3202996. PMID 21997578.
    8. ↑ Menger H, Jörg J (1999). “Outcome of central pontine and extrapontine myelinolysis (n = 44)”. J Neurol. 246 (8): 700–5. doi:10.1007/s004150050435. PMID 10460448.
    9. ↑ Rebedew DL (2016). “Is Central Pontine Myelinolysis Reversible?”. WMJ. 115 (6): 326–8. PMID 29095576.

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