Does extrapontine myelinolysis precede central pontine myelinolysis. Diffusion may be normal or may show faint high signal. Diffusionweighted imaging is particularly important in the early. Kallakatta rn, radhakrishnan a, fayaz rk, unnikrishnan jp, kesavadas c, sarma sp. A diffusionweighted image c and adc image d show increased signal intensity in the bilateral basal ganglia, thalami, and cerebral cortices. N2 central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Central pontine myelinolysis information page national.
Central pontine myelinolysis associated with diabetic. Osmotic demyelination syndrome central pontine and. So the other name for central pontine myelinolysis is osmotic demyelination syndrome. Therefore, we concur with the other authors that a repeat mri after 12 weeks in all clinically suspected cases of ods is very helpful. Central pontine myelinolysis and mri 71 volume, issue 1, january 2014 initial ct scan did not show any low attenuated areas. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. In vivo diffusion tensor imaging and 1hmagnetic resonance. In mri scan results, damage to the pons is more visible compared with ct scan results. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Central pontine myelinolysis cpm is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Isolated extra pontine myelinolysis rajitha et al case. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Osmotic demyelination syndrome ods is brain cell dysfunction. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are disorders frequently associated with serum osmotic imbalance.
Central and extrapontine myelinolysis 02 after alcohol. Sequential mri in pontine and extrapontine myelinolysis following. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. 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.
Osmotic demyelination syndrome radiology reference article. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Mri of the brain demonstrating central pontine arrow hyperintensity in a diffusionweighted image a and t2weighted image b. Magnetic resonance imaging mri of the brain at supratentorial. We present a case in which restricted diffusion was identified within the central pons by using mr diffusion. T1 early diagnosis of central pontine myelinolysis with diffusionweighted imaging. 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. Cramer sc, stegbauer kc, schneider a, mukai j, maravilla kr. 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. Clinical and radiologic correlations of central pontine myelinolysis. A case of osmotic demyelination syndrome occurred after. Clinical and functional outcome and factors predicting prognosis in osmotic demyelination syndrome central pontine andor extrapontine myelinolysis in. If you have the appropriate software installed, you can download article.
The clinical features vary depending on the extent of involvement. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Autopsy validation of mri in central pontine myelinolysis. Whether diffusionweighted imaging dwi might facilitate an earlier diagnosis has not yet been studied systematically. Early detection of central pontine myelinolysis with mri. 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. Diffusionweighted mr findings of central pontine and extrapontine myelinolysis. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. Central pontine myelinolysis cpm is a rare neurologic disorder defined by. Central pontine myelinolysis definition of central.
Central pontine demyelination, diffusion tensor imaging, prognosis. Role of mri in diagnosis of osmotic myelinolysis is also well established. Osmotic demyelination syndrome linkedin slideshare. A physical examination can help in attaining the final diagnosis as long as the major symptoms are present. When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted. Plasma exchange successfully treats central pontine. Flair mri hyponatremia and central pontine myelinolysis. At admission the patient appeared restless and confused.
The effect of white matter integrity on functional outcome in central. T2 hyperintensity confined to center of pons seen in 50% of cases. 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. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. The prognosis is very variable from complete regression of clinical symptomatology to signs of significant quadruparesis, a vegetative state and death. Through a mechanism that is only partly understood, the shift. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. 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 genetic and rare diseases. Keywords central pontine myelinolysis, extrapontine myelinolysis, methylprednisolone. My wife, 50, suffered central pontine myelinolysis in august 2000. 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. Dtistudio software cmrm, johns hopkins medical institute, md, usa was. Studies show that lesions can occur with or without cpm. Central pontine myelinolysis medical condition youtube. Central pontine myelinolysis radiology reference article. Immediate mri of the brain was advised and requested to be.
Previous mri studies of the brains of rats with chronic alcohol. Decreased diffusion in central pontine myelinolysis. Electroencephalography hyponatremia and central pontine. 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.
Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. Antemortem computed tomography ct had been performed in nine, and ante or postmortem magnetic resonance mr imaging in 11. Therapeutic effect of steroids in osmotic demyelination of infancy. 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. Mri changes associated with pontine and extrapontine myelinolysis first occur on diffusionweighted imaging. An mri scan is much recommended and considered as the main diagnostic tool for central pontine myelinolysis. Revert 22 years female, case of dm type 1 with ckd history of frequent rapid change in glucose admitted with depression of.
Magnetic resonance imaging mri with diffusion weighted imaging dwi is the modality of choice for early diagnosis of this entity. 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. We present a case in which restricted diffusion was identified within the central pons by using mr diffusionweighted imaging within 24 hours of onset of patient. Central pontine myelinolysis an overview sciencedirect. The neurologists attributed it to a high blood pressure blood drug called moduretic and 48 hours of vomiting. Early diagnosis of central pontine myelinolysis with. Value of diffusionweighted imaging in central pontine and.
Volume of signal abnormality on brain magnetic resonance imaging mri was quantified by. May involve basal ganglia and cerebral white matter without pontine involvement is called extra pontine myelinolysis. Myelinolysis was first discovered in the central pons of autopsied humans 1. The earliest change is seen on dwi with restriction in the lower pons. Diffusion weighted imaging and diffusion tensor imaging on followup. 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. 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. Acute psychosis as main manifestation of central pontine. Value of diffusionweighted imaging in central pontine and extrapontine myelinolysis. So, central pontine myelinolysis is the destruction of the myelin sheath around nerve cells that are in the pons. Learn what causes it and which symptoms to watch out for.
Central pontine myelinolysis cpm and extrapontine myelinolysis epm are rare neurological disorders characterized by demyelination in andor outside the pons. As such the condition is described in the osmotic demyelination syndrome article. Charness, in office practice of neurology second edition, 2003. 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. Conventional ct and mr imaging findings lag the clinical manifestations of cpm.729 211 1109 1127 215 32 11 1398 1534 1431 1149 1501 1070 289 861 1039 701 751 1285 1193 1399 958 696 922 1252 1119 804 178 621 1403 761 783 155 747 239 1167 1329 342 184 1401 881 623 30 294 881