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Zontinue Myelnisis
Zontinue Myelnisis
Pathophysiology
Central pontine myelinolysis is a concentrated, frequently symmetric,
noninflammatory demyelination within the central basis pontis. 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.
One theory proposes that in regions of compact interdigitation of white and
gray matter, cellular edema, which is caused by fluctuating osmotic forces,
results in compression of fiber tracts and induces demyelination. Prolonged
hyponatremia followed by rapid sodium correction results in edema. During
the period of hyponatremia, the concentration of intracellular charged protein
moieties is altered; reversal cannot parallel a rapid correction of electrolyte
status. The term osmotic myelinolysis is more appropriate than central pontine
myelinolysis for demyelination occurring in extrapontine regions after the
correction of hyponatremia. [4, 5, 6, 7]
Causes
Conditions predisposing patients to central pontine myelinolysis
include alcoholism, liver disease, malnutrition, and hyponatremia.
Risk factors for central pontine myelinolysis in the hyponatremic patient
include the following:
Serum sodium of less than 120 mEq/L for more than 48 hours
Aggressive IV fluid therapy with hypertonic saline solutions
Development of hypernatremia during treatment
Many patients who have hyponatremia that is corrected rapidly do not develop
central pontine myelinolysis. Thus, other less obvious risk factors probably
exist. Central pontine myelinolysis reportedly occurs occasionally in patients
who are treated for hypernatremia.
Central pontine myelinolysis may complicate liver
transplantation surgery. [8]Consider central pontine myelinolysis when
confusion and/or weakness complicate the liver transplant patient's
postoperative recovery. The author provided consultation for a liver transplant
patient who developed central pontine myelinolysis and critical illness
neuromyopathy. The typical exam findings for central pontine myelinolysis
were masked by peripheral nerve and muscle disease. MRI studies provided
conclusive evidence for brain stem demyelination.
Burn patients with a prolonged period of serum hyperosmolality are prone to
developing central pontine myelinolysis. Central pontine myelinolysis also has
occurred concurrently with Wilson disease and neoplasia.
Epidemiology
The exact incidence of central pontine myelinolysis is unknown. A study by
Singh et al demonstrated that central pontine myelinolysis was present in 29%
of postmortem examinations of liver transplant patients. Two thirds of these
patients had serum sodium fluctuations of only ± 15-20 mEq/L. [9] Central
pontine myelinolysis occurs more frequently in females than in males.
Prognosis
Death is common. Maximum recovery from central pontine myelinolysis may
require several months. Chronic neurologic deficits range from locked-in
syndrome to spastic quadriparesis. Patients with extrapontine lesions may
exhibit tremor and ataxia.
Possible complications include those associated with severe central nervous
system injury and reduced activity, such as the following:
Ventilator dependency
Aspiration pneumonia
Venous thrombosis
Pulmonary embolism
Contractures
Muscle wasting
Decubitus ulcers
Urinary tract infections
Depression
Diagnostic Tests
Cerebral spinal fluid (CSF) probably is not necessary when the etiology and
regions. MRI or CT imaging of the brain stem may not reveal an obvious
anatomic disturbance. A thorough neurologic exam therefore is
indispensable. [14]
Overview
Adams et al described central pontine myelinolysis (CPM) as a unique clinical
entity. They published their findings in 1958, observing that patients who
suffered from alcoholism or malnutrition developed spastic quadriplegia,
pseudobulbar palsy, and varying degrees of encephalopathy or coma from
acute, noninflammatory demyelination that centered within the basis pontis. [1]
Physicians currently recognize that central pontine myelinolysis occurs
inconsistently as a complication of severe and prolonged hyponatremia,
particularly when corrected too rapidly. [2] Standard of care requires judicious
treatment of electrolyte disturbances to reduce the incidence of osmotic
myelinolysis. [3]
Pathophysiology
Central pontine myelinolysis is a concentrated, frequently symmetric,
noninflammatory demyelination within the central basis pontis. 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.
One theory proposes that in regions of compact interdigitation of white and
gray matter, cellular edema, which is caused by fluctuating osmotic forces,
results in compression of fiber tracts and induces demyelination. Prolonged
hyponatremia followed by rapid sodium correction results in edema. During
the period of hyponatremia, the concentration of intracellular charged protein
moieties is altered; reversal cannot parallel a rapid correction of electrolyte
status. The term osmotic myelinolysis is more appropriate than central pontine
myelinolysis for demyelination occurring in extrapontine regions after the
correction of hyponatremia. [4, 5, 6, 7]
Causes
Conditions predisposing patients to central pontine myelinolysis
include alcoholism, liver disease, malnutrition, and hyponatremia.
Risk factors for central pontine myelinolysis in the hyponatremic patient
include the following:
Serum sodium of less than 120 mEq/L for more than 48 hours
Aggressive IV fluid therapy with hypertonic saline solutions
Development of hypernatremia during treatment
Many patients who have hyponatremia that is corrected rapidly do not develop
central pontine myelinolysis. Thus, other less obvious risk factors probably
exist. Central pontine myelinolysis reportedly occurs occasionally in patients
who are treated for hypernatremia.
Central pontine myelinolysis may complicate liver
transplantation surgery. [8]Consider central pontine myelinolysis when
confusion and/or weakness complicate the liver transplant patient's
postoperative recovery. The author provided consultation for a liver transplant
patient who developed central pontine myelinolysis and critical illness
neuromyopathy. The typical exam findings for central pontine myelinolysis
were masked by peripheral nerve and muscle disease. MRI studies provided
conclusive evidence for brain stem demyelination.
Burn patients with a prolonged period of serum hyperosmolality are prone to
developing central pontine myelinolysis. Central pontine myelinolysis also has
occurred concurrently with Wilson disease and neoplasia.
Epidemiology
The exact incidence of central pontine myelinolysis is unknown. A study by
Singh et al demonstrated that central pontine myelinolysis was present in 29%
of postmortem examinations of liver transplant patients. Two thirds of these
patients had serum sodium fluctuations of only ± 15-20 mEq/L. [9] Central
pontine myelinolysis occurs more frequently in females than in males.
Prognosis
Death is common. Maximum recovery from central pontine myelinolysis may
require several months. Chronic neurologic deficits range from locked-in
syndrome to spastic quadriparesis. Patients with extrapontine lesions may
exhibit tremor and ataxia.
Possible complications include those associated with severe central nervous
system injury and reduced activity, such as the following:
Ventilator dependency
Aspiration pneumonia
Venous thrombosis
Pulmonary embolism
Contractures
Muscle wasting
Decubitus ulcers
Urinary tract infections
Depression
Diagnostic Tests
Cerebral spinal fluid (CSF) probably is not necessary when the etiology and
diagnosis are obvious. CSF studies may demonstrate increased opening
pressure, elevated protein, or mononuclear pleocytosis.
MRI is the imaging modality of choice (see the image below). According to
one study, serial brain imaging is of value because a substantial proportion of
patients have normal findings on initial MRI. [13] Typically, 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. MRI or CT imaging of the brain stem may not reveal an obvious
anatomic disturbance. A thorough neurologic exam therefore is
indispensable. [14]