Central Pontine Myelinolysis

(Redirected from Central Pontine Myelinosis)

Original Editor - Wendy Walker

Top Contributors -

Wendy Walker, Laura Ritchie and Naomi O'Reilly

Contents

Clinically Relevant Anatomy

The Pons is situated in the brainstem. It lies above the medulla, below the midbrain and anterior to the cerebellum.


 

Mechanism of Injury / Pathological Process

Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the pons. The area involved is usually confined to the basis pontis but occasionally involves the tegmentum too.

It is one of the demyelinating conditions and was first described by Adams et al in 1959.[1] In at least 10% of patients with central pontine myelinolysis, demyelination also occurs in extrapontine regions, including the mid brain, thalamus, basal nuclei and cerebellum. The exact mechanism that strips the myelin sheath is unknown.

Central pontine myelinolysis occurs most often as a complication of treatment of patients with profound, life-threatenin-g hyponatremia (low sodium) and is a consequence of a rapid rise in serum tonicity following treatment in individuals with chronic, severe hyponatraemia who have made intracellular adaptations to the prevailing hypotonicity. 

In some cases, demyelination occurs outside the pons too; cases which include extrapontine demyelination are labelled "osmotic demyelination syndrome", "extra pontine myelinosis" or "osmotic myelinosis".[2]

Microscopically the lesion shows degeneration and loss of oligodendrocytes with preservation of axons unless the lesion is very advanced.

Clinical Presentation

[3]

The classic clinical presentation of central pontine myelinolysis is progressive spastic quadriparesis with pseudobulbar palsy.[4]

Clinically CPM presents in a biphasic pattern:

1st phase = acute encephalopathy, caused by the electrolyte abnormalities.

Once treatment is given causing rapid reversal of this abnormality, the patient improves for 2 to 3 days before progressing onto the classic CPM features. These consist of:

  • spastic quadriparesis
  • pseudobulbar palsy
  • changes in levels of consciousness
  • coma or death

Causes

Conditions predisposing patients to central pontine myelinolysis include alcoholism, liver disease, malnutrition and hyponatraemia (an electrolyte disturbance in which the sodium ion concentration in the plasma is lower than normal).

CPM may occur following liver transplantation surgery.

Burn patients may develop CPM and it can also occur with Wilson disease and neoplasia.

Diagnostic Procedures

[5]

MRI is the most useful imaging technique in these cases.

T2-weighted MRI images demonstrate hyperintense or bright areas where demyelination has occurred and has been caused by relatively increased water content in those regions[6].

Management / Interventions

Patients who survive central pontine myelinolysis likely require extensive and prolonged neurorehabilitation. Incorporate occupational, physical, speech, and language therapists early in the care of such patients. Swallowing studies are necessary to evaluate for dysphagia and determine the risk for aspiration pneumonia.

Differential Diagnosis

  • Demyelination, including multiple sclerosis (MS)
  • Infarction from basilar perforators can be central, although more often brainstem infarcts stop at the midline
  • Pontine neoplasms including astrocytomas

Key Evidence

Graff-Radford et al[7] examined the clinical records of patients diagnosed as having CPM identified by a search of Mayo Clinic medical records from January 1, 1999, to December 31, 2010. Diagnosis was made by clinical and radiologic features. Their objective was to characterize clinical and radiologic features of patients with central pontine myelinolysis (CPM) and identify variables that predict outcome. They concluded: "Clinical outcome in patients with CPM is not predicted by the volume of radiologic T2 signal abnormality on MRI or the severity of hyponatremia. Serial brain imaging is of value because a substantial proportion of patients have normal findings on initial MRI".

Recent Related Research (from Pubmed)

References


  1. Adams RD, Victor M, Mancall EL. Central pontine myelinolysis: a hitherto undescribed disease occurring in alcoholic and malnourished patients. AMA Arch Neurol Psychiatry. Feb 1959;81(2):154-72
  2. Martin RJ. Central pontine and extrapontine myelinolysis: the osmotic demyelination syndromes. J Neurol Neurosurg Psychiatry. Sep 2004;75 Suppl 3:iii22-8
  3. Medical Condition Information. Central pontine myelinolysis (Medical Condition). Available from: http://www.youtube.com/watch?v=347y-o2IKAs [last accessed 13/02/16]
  4. Laureno R, Karp BI. Myelinolysis after correction of hyponatremia. Ann Intern Med. Jan 1 1997;126(1):57-62
  5. T. Bee Teh. Lock In Syndrome, low sodium, Central Pontine Myelinolysis, Extra Pontine Myelinolysis. Available from: http://www.youtube.com/watch?v=v-_xNbZ5ef8 [last accessed 13/02/16]
  6. DeWitt LD, Buonanno FS, Kistler JP, et al. Central pontine myelinolysis: demonstration by nuclear magnetic resonance. Neurology. May 1984;34(5):570-6. [Medline].
  7. Jonathan Graff-Radford, Jennifer E. Fugate, Timothy J. Kaufmann, Jay N. Mandrekar, Alejandro A. RabinsteinfckLRMayo Clin Proc. 2011 November; 86(11): 1063–1067