In vivo diffusion tensor imaging and 1hmagnetic resonance. Central pontine myelinolysis and mri 71 volume, issue 1, january 2014 initial ct scan did not show any low attenuated areas. Revert 22 years female, case of dm type 1 with ckd history of frequent rapid change in glucose admitted with depression of. We report a patient who developed cpm secondary to profound hyponatraemia and who recovered. We describe demographics, clinical presentation, and early magnetic resonance imaging mri findings. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly.
Diffusionweighted mr findings of central pontine and extrapontine myelinolysis. A diffusionweighted image c and adc image d show increased signal intensity in the bilateral basal ganglia, thalami, and cerebral cortices. Value of diffusionweighted imaging in central pontine and. As such the condition is described in the osmotic demyelination syndrome article. Dtistudio software cmrm, johns hopkins medical institute, md, usa was. Whether diffusionweighted imaging dwi might facilitate an earlier diagnosis has not yet been studied systematically. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Central pontine myelinolysis cpm, also referred to as osmotic demyelination syndrome, is a demyelinating disease that is caused by significant damage to the myelin sheath of nerve cells in the middle of the brainstem, specifically the pons region. When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted.
Flair mri hyponatremia and central pontine myelinolysis. Clinical and functional outcome and factors predicting prognosis in osmotic demyelination syndrome central pontine andor extrapontine myelinolysis in. Central pontine myelinolysis definition of central. Learn what causes it and which symptoms to watch out for. The latter correlated with preservation of centrally located groups of longitudinal myelinated nerve fibres shown at autopsy. Magnetic resonance imaging mri of the brain at supratentorial. The neurologists attributed it to a high blood pressure blood drug called moduretic and 48 hours of vomiting. Antemortem computed tomography ct had been performed in nine, and ante or postmortem magnetic resonance mr imaging in 11. The prognosis is very variable from complete regression of clinical symptomatology to signs of significant quadruparesis, a vegetative state and death.
Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We present a case in which restricted diffusion was identified within the central pons by using mr diffusion. Central pontine myelinolysis cpm is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Of 19 cases in which diffusionweighted sequences were obtained, 10 53%. Through a mechanism that is only partly understood, the shift. Kallakatta rn, radhakrishnan a, fayaz rk, unnikrishnan jp, kesavadas c, sarma sp. Central pontine myelinolysis cpm is a neurological condition involving severe damage to the myelin sheath of nerve cells in the pons an area of the brainstem. A case of osmotic demyelination syndrome occurred after. Osmotic demyelination syndrome linkedin slideshare. Osmotic demyelination syndrome radiology reference article. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia.
Studies show that lesions can occur with or without cpm. Diffusionweighted imaging is particularly important in the early. In a fatal case of central pontine myelinolysis cpm a low field strength 0. May involve basal ganglia and cerebral white matter without pontine involvement is called extra pontine myelinolysis. An mri scan is much recommended and considered as the main diagnostic tool for central pontine myelinolysis. Therefore, we concur with the other authors that a repeat mri after 12 weeks in all clinically suspected cases of ods is very helpful. By continuing to browse this site you are agreeing to our use of cookies. T2 hyperintensity confined to center of pons seen in 50% of cases. Mri changes associated with pontine and extrapontine myelinolysis first occur on diffusionweighted imaging. Does extrapontine myelinolysis precede central pontine myelinolysis. Immediate mri of the brain was advised and requested to be. Posted in central pontine myelinolysis extrapontine myelinolysis and tagged alzheimers disease, brain injury, cognition, cpmepm, diffusion tensor imaging, electroencephalography, extrapontine myelinolysis, fmri, high definition fiber tracking, magnetic resonance imaging, mri neurography, nfl, pet scan, positron emission tomography, post. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are disorders frequently associated with serum osmotic imbalance.
Central pontine myelinolysis an overview sciencedirect. So the other name for central pontine myelinolysis is osmotic demyelination syndrome. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are rare neurological disorders characterized by demyelination in andor outside the pons. Decreased diffusion in central pontine myelinolysis.
Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. We describe the case of a 21yearold woman who was hospitalized at week 10 of gestation because of severe hyperemesis. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. Keywords central pontine myelinolysis, extrapontine myelinolysis, methylprednisolone. Early diagnosis of central pontine myelinolysis with. Osmotic demyelination syndrome ods may be observed as a result of a rapid change in serum osmolarity, such as that induced by an overly rapid correction of serum sodium levels in hyponatraemic patients. Central pontine myelinolysis cpm is a rare condition characterised by spastic tetraparesis, pseudobulbar palsy and the lockedin syndrome. It is the more recent term replacing central pontine myelinolysis, recognizing that. N2 central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Previous mri studies of the brains of rats with chronic alcohol. There are no proven effective therapies for ods, but we describe the first case showing the successful treatment of central pontine myelinolysis cpm by plasma exchange, which occurred after rapid development of hypernatremia from intravenous sodium bicarbonate therapy. Central pontine myelinolysis information page national. The clinical features vary depending on the extent of involvement.
Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by rapid correction of chronic hyponatremia. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Central and extrapontine myelinolysis 02 after alcohol. Isolated extra pontine myelinolysis rajitha et al case. Central pontine myelinolysis cpm, steroidresponsive encephalopathy associated with autoimmune thyroiditis sreat, bickerstaffs brainstem encephalitis bbe are some of the disorders that might present with a localized lesion that specifically involves the pontine area 3. A physical examination can help in attaining the final diagnosis as long as the major symptoms are present. Mri of the brain demonstrating central pontine arrow hyperintensity in a diffusionweighted image a and t2weighted image b. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. If you have the appropriate software installed, you can download article. So, central pontine myelinolysis is the destruction of the myelin sheath around nerve cells that are in the pons. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Central pontine myelinolysis associated with diabetic. Diffusion weighted imaging and diffusion tensor imaging on followup.
The earliest change is seen on dwi with restriction in the lower pons. Autopsy validation of mri in central pontine myelinolysis. Central pontine myelinolysis genetic and rare diseases. Role of mri in diagnosis of osmotic myelinolysis is also well established. Common clinical manifestations of cpm include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees. Central pontine myelinolysis cpm is a disease characterized by the destruction of the myelin in the brainstem, generally associated with alcoholism, rapid correction of hyponatremia and other. Early detection of central pontine myelinolysis with mri.
The effect of white matter integrity on functional outcome in central. T2weighted t2w and diffusionweighted dw magnetic resonance imaging mri of brain showed central pontine hyperintensity with peripheral sparing figure 1 consistent with acute central pontine myelinolysis cpm. Magnetic resonance imaging mri with diffusion weighted imaging dwi is the modality of choice for early diagnosis of this entity. Sequential mri in pontine and extrapontine myelinolysis following. Early diagnosis of central pontine myelinolysis with diffusionweighted imaging. Clinical and radiologic correlations of central pontine myelinolysis. Central pontine myelinolysis symptoms, treatment and.
Central pontine myelinolysis synonyms, central pontine myelinolysis pronunciation, central pontine myelinolysis translation, english dictionary definition of central pontine myelinolysis. My wife, 50, suffered central pontine myelinolysis in august 2000. In mri scan results, damage to the pons is more visible compared with ct scan results. Osmotic demyelination syndrome central pontine and. Plasma exchange successfully treats central pontine. Central pontine demyelination, diffusion tensor imaging, prognosis. Central pontine myelinolysis appears an area of hyperintensity on t2weighted mri sequences, and may show contrast enhancement early in the course of disease miller et al. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. T1 early diagnosis of central pontine myelinolysis with diffusionweighted imaging. At admission the patient appeared restless and confused. Volume of signal abnormality on brain magnetic resonance imaging mri was quantified by.
It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Electroencephalography hyponatremia and central pontine. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. Diffusion may be normal or may show faint high signal. Charness, in office practice of neurology second edition, 2003. Osmotic demyelination syndrome ods is brain cell dysfunction. Therapeutic effect of steroids in osmotic demyelination of infancy. Value of diffusionweighted imaging in central pontine and extrapontine myelinolysis. Diffusion weighted imaging and diffusion tensor imaging on followup shalini r nair 1, norlisah m ramli 1, kartini rahmat 1, sharon t meiling 2 1 department of biomedical imaging, university malaya research imaging centre, kuala lumpur, malaysia 2 department of medicine, neurology unit, faculty of medicine, university malaya, kuala lumpur, malaysia. Central pontine myelinolysis cpm is a rare neurologic disorder defined by. Cramer sc, stegbauer kc, schneider a, mukai j, maravilla kr. Central pontine myelinolysis cpm is now more commonly referred to as osmotic demyelination syndrome, which recognizes that the same phenomenon is also seen in other areas of the brain previously known as extrapontine myelinolysis. Central pontine myelinolysis is a disorder of cerebral white matter that usually affects alcoholics but also occurs in nonalcoholics with liver disease, including wilsons disease, malnutrition, anorexia, burns, cancer, addisons disease, and severe electrolyte disorders, such as thiazide.