Myelinolysis means that the myelin a covering that protects the pontine nerve cells is being destroyed. Most published cases are based on autopsy reports, whereas antemortem diagnosis is rare. Central pontine myelinolysis an overview sciencedirect. We present a case in which the diagnosis of cpm was confirmed by computed tomography ct. However, there are other predisposing risk factors chronic alcoholism, hypokalaemia that perpetuate the development of ods. Cns nontumor pontine and extrapontine myelinolysis. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Osmotic demyelination syndrome radiology reference article.
Central pontine and extrapontine myelinolysis cpepm is a rare demyelinating syndrome. Pdf only abstract magnetic resonance mr features of a patient with profound hyponatremia and clinical findings of central pontine myelinolysis are presented. Udainiya department of neurology, sms medical college and hospital, jaipur, india central pontine myelinolysis is a demyelinating affection of central pons diagnosed on the basis of characteristic mri finding in an appropriate clinical setting. Central pontine myelinolysis cpm usually occurs with rapid correction of severe chronic hyponatremia. Of the 34 patients for whom followup data were available, 32 survived. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty. Commentary central pontine myelinolysis is a syndrome of symmetric osmotic demyelination involving the pons.
Central pontine myelinolysis cpm is a rare neurologic complication after orthotopic liver transplantation olt. Pdf central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the. Central pontine myelinolysis cpm was described in 1959 by adams et al. At autopsy, an area of extensive demyelination in the center of the pons was found and a pathological diagnosis of central pontine myelinolysis was made. Pathology outlines pontine and extrapontine myelinolysis. However, sometimes ct shows lowattenuation changes in the pons, as seen in our patient. The diagnosis in this patient was central pontine myelinolysis. Pdf extra and central pontine myelinolysis in a child.
Extrapontine myelinolysis presenting as acute parkinsonism. Department of neurology, mayo clinic, rochester, mn, usa. The findings in 44 patients 42 of whom were chronic alcoholics with central pontine myelinolysis show that the outcome does not depend on the severity of neurological deficits during the acute phase of the condition or on concomitant internal diseases, including the degree of hyponatremia. Central pontine myelinolysis is brain cell dysfunction. Kallakatta rn, radhakrishnan a, fayaz rk, unnikrishnan jp, kesavadas c, sarma sp. Marsel mesulam, md \sb\a young woman with a clinical history and magnetic resonance imaging scan consistent with central pontine myelinolysis came to medical attention because of prominent behavioral symptoms. The management of the hyperosmolar hyperglycaemic state hhs. Central pontine myelinolysis the journal of neuropsychiatry and. Through a mechanism that is only partly understood, the shift.
It has been decades since the disease has been identified, still development of treatment is undergoing. Apr 11, 2014 in about 50% of cases isolated central pontine myelinolysis cpm occurs, whereas the remaining cases are more or less equally divided into isolated extra pontine myelinolysis epm or a combination of cpm and epm, with a slight prevalence of the latter condition. This is a neurological disease, with a specific location area known as the pons and iatrogenic etiology. A holistic approach in the treatment course should be provided in order to attain recovery. A welldefined trident shaped focus of marked t2 hyperintensity following nearly csf flow signal symmetrically involves the central pons without mass effect or abnormal enhancement. Osmotic demylenation syndrome neuroradiology teaching files. 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. Central pontine myelinolysis symptoms, treatment and prognosis.
Central pontine myelinolysis in association with hodgkins ncbi central pontine myelinolysis cpm was first described in 1959 by adams, victor and mancall as an area ofnoninflammatory demyelination affecting the central. Osmotic demyelination syndrome ods is also referred to as central pontine myelinolysis cpm. In this report, we present a case of cpm after olt followed. Pontine refers to the stem of the brain, called the pons.
Central pontine myelinolysis is also an earlyoccurring lesion. The mortality rate has significantly decreased, with recent retrospective studies showing 94% survival about 25%40% make a complete recovery without deficits, and 25%30% remain incapacitated. It is characterized by acute paralysis, dysphagia difficulty swallowing, and dysarthria difficulty speaking, and other neurological symptoms. Central pontine myelinolysis associated with acute. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. In about 50% of cases isolated central pontine myelinolysis cpm occurs, whereas the remaining cases are more or less equally divided into isolated extrapontine myelinolysis epm or a combination of cpm and epm, with a slight prevalence of the latter condition. This condition can be fatal or cause long term disability. Outcome of central pontine and extrapontine myelinolysis n. In patients with this condition, the myelin sheath which protects the nerves is damaged, interfering with their ability to signal to each other. Also known as osmotic demyelination syndrome ods, pm is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis epm 2. Central pontine myelinolysis, osmotic demyelination.
A 41yearold woman presented with acute parkinsonism like features while on a holiday. Central pontine myelinolysisextrapontine myelinolysisosmotic endothelial injuryosmotic dysmyelinationhyponatraemia. By continuing to browse this site you are agreeing to our use of cookies. Osmotic demyelination syndrome ods is brain cell dysfunction. The condition was originally described in patients with severe hypona. Introduction pontine myelinolysis pm was first described in 1959 by adams et al. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia. View the article pdf and any associated supplements and figures for a period of 48 hours. This case report underlines the importance of recognising risk factors predisposing the patient to the development of central pontine myelinolysis. 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.
It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. In this case report, we present the pitfalls of severe hypernatremia, its treatment and complications including misinterpretation of radiological imaging, and. Keywords central pontine myelinolysis, refeeding syndrome, coma, rehabilitation. The clinical dilemma of proper treatment of severe hyponatremia is exemplified by this case. Central pontine myelinolysis cpm is neurological disease caused by severe damage of the myelin sheath of nerve cells in the brainstem, more precisely in the area termed the pons, predominately of iatrogenic etiology. As such the condition is described in the osmotic demyelination syndrome article. Extrapontine myelinolysis epm has also been described most often involving the white matter of the cerebellum, lateral geniculate body, putamen.
Jul 21, 2011 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Central pontine myelinolysis cpm, which is a component of the osmotic demyelination syndrome ods, is a frequent neurological complication that follows rapid correction of hyponatraemia. Clinicians should maintain their vigilance while correcting electrolyte imbalances, especially with associated comorbidity. Central pontine myelinolysis can be defined as a medical condition, in which the myelin sheath of the nerve cells in the brainstem become severely damaged. A young pregnant woman showing clinical symptoms of numbness, vertigo, diplopia, and amnesia with associated hypernatremia ran a fulminant course. It is a rare condition that predominantly affects the pons but may involve extrapontine structures including the midbrain, thalamus and cerebellum. Central pontine and extrapontine myelinolysis request pdf. Parkinsonism and recovery in central and extrapontine. 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. In describing a patient with central pontine myelinolysis cpm and behavioral symptoms, price and mesulam 1 considered the development of cpm to be related to the rapid correction of hyponatremia. Central pontine myelinolysis in a case of alcohol dependence. A case of chronic asymptomatic central pontine myelinolysis.
Central pontine myelinolysis definition of central pontine. Osmotic demyelination syndrome in children pediatric neurology. Plasma exchange successfully treats central pontine. Get a printable copy pdf file of the complete article 739k, or click on a page image below to browse page by page.
Whilst dka presents within hours of onset, hhs comes on over many days, and consequently the dehydration and metabolic disturbances are more extreme. Central pontine myelinolysis is a rare entity of disease that is characterized by a noninflammatory demyelination of oligodendrocy tes of the basis pontis 6 mostly, central pontine myelinolysis lesions are usually symmetrical and bilateral 2. Atypical presentation of central pontine myelinolysis in. Introduction central pontine myelinolysis cpm is a syndrome that is characterised by rapid destruction of myelin sheaths of mainly. Apr 28, 2020 central pontine myelinolysis is a serious neurological condition caused by damage to the nerves in the area of the brainstem known as the pons. Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Central pontine myelinolysis central pontine myelinolysis cpm is a demyelinating disease of the pons often associated with demyelination of.
When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted. The management of the hyperosmolar hyperglycaemic state. Central pontine myelinolysis cpm is a neurological disorder that affects the brain. Extrapontine involvement frequently coexisted with or was present without. Central pontine myelinolysis associated with acute haemorrhagic pancreatitis. Recovery after central pontine myelinolysis springerlink. It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis. Pdf central pontine myelinolysis cpm commonly presents as a complication of treatment in patients with profound life threatening hyponatraemia. Central pontine myelinolysis cpm is a clinically heterogeneous, demyelinating condition originally thought to occur only in the central pons.
Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Central pontine myelinolysis with clinical recovery. Central pontine myelinolysis and the rate of correction of. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. Mri of the brain has greater sensitivity to the early increase in the tissue. For language access assistance, contact the ncats public information officer. Central pontine myelinolysis fulltext european neurology 2009. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved.
Central pontine and extrapontine myelinolysis after. Central pontine myelinolysis symptoms, treatment and. Pdf central pontine and extrapontine myelinolysis researchgate. Get a printable copy pdf file of the complete article 10k, or click on a page.
It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. 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 case of osmotic demyelination syndrome occurred after. Osmotic demyelination syndrome linkedin slideshare. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. 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.
The serum sodium concentration in this patient on admission was 114 mmoll and 17 hours later it was 3 mmoll, yielding a calculated rate of. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Despite the pronounced fluctuations in serum osmolality, cpm is rarely seen in diabetics. The treatment of hyponatremia saint lukes health system. Peduncular hallucinosis secondary to central pontine myelinolysis. There is some evidence that rapid changes in osmolality during treatment may be the precipitant of cpm. Extra and central pontine myelinolysis in a child with adrenal insufficiency. Central pontine myelinolysis cpm is characterized by a loss of oligodendrocytes and myelin in the central pons adams et al. A case of osmotic demyelination syndrome occurred after the. Marked clinical recovery occurred despite persistent radiologic abnormalities. It is a neurological disease that is caused when severe damage occurs to the myelin sheath of nerve cells located in the brainstem.
Charness, in office practice of neurology second edition, 2003. Isolated extra pontine myelinolysis rajitha et al case. A number of sites may be involved, besides the pons, and among the most. Central pontine myelinolysis cpm is known to be almost universally fatal. The classic appearance is a trident or triangleshaped central pontine hyperintensity on t2weighted axial images, as seen in our patient. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment. Central pontine myelinolysis was once thought to be fatal, with a mortality of 90% to 100% possibly because it was initially diagnosed at autopsy. Mar 27, 2019 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Central pontine myelinolysis radiology reference article. Central pontine myelinolysis health encyclopedia uf. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. 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. This article is an open access publication abstract.
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. Behavioral manifestations of central pontine myelinolysis. If you have problems viewing pdf files, download the latest version of adobe reader. Pdf peduncular hallucinosis secondary to central pontine. Central pontine myelinolysis without hyponatraemia. Central pontine myelinolysis cpm is a rare condition characterised by. There were some papers on mr diffusion dwi and spectroscopy mrs of cpm15. Central pontine myelinolysis cpm, a frequently fatal disorder, is often associated with hyponatremia.
Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress. Clinical and functional outcome and factors predicting prognosis in osmotic demyelination syndrome central pontine andor extrapontine myelinolysis in. The clinical features vary depending on the extent of involvement. She was known as a chronic alcoholic with nearly three.
Parkinsonism and recovery in central and extrapontine myelinolysis a. This is a case report of cpm associated with hyperglycemia. A case of chronic asymptomatic central pontine myelinolysis with histological evidence of remyelination harry r. Central pontine myelinolysis information page national. Request pdf central pontine myelinolysis case reporta 45yearold woman was found dead on a sofa in her flat by her male partner. It has been observed to be more prevalent in liver transplant recipients, with worse prognosis. Recovery from probable central pontine myelinolysis.
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. Revert 22 years female, case of dm type 1 with ckd history of frequent rapid change in glucose admitted with depression of. Extrapontine myelinolysis epm as well as central pontine myelinolysis cpm can occur due to changes in serum osmolality and are referred to as osmotic demyelination syndrome ods. Central pontine myelinolysis after liver transplantation.
Central pontine myelinolysis is mainly an iatrogenic disorder, and its appearance coincided with the widespread use of intravenous therapy for correction of fluid and electrolyte disorders. Central pontine myelinolysis pathophysiology, causes. 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. Extrapontine myelinolysis following extreme hypernatremia. Among all 106 cases, 49 presented with isolated central pontine myelinolysis. Full text full text is available as a scanned copy of the original print version. While they are very similar conditions, ods is slightly different.
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