You can also develop cpmepm if you are an alcoholic without developing hyponatremia. On admission mri t2w images show t2 hyperintensity confined to centre of pons. 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. Charness, in office practice of neurology second edition, 2003.
Introduction central pontine myelinolysis cpm is a syndrome that is characterised by rapid destruction of myelin sheaths of mainly. It has been observed to be more prevalent in liver transplant recipients, with worse prognosis. Of the 34 patients for whom followup data were available, 32 survived. 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. Also known as osmotic demyelination syndrome ods, pm is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis. The most common cause of central pontine myelinolysis is a quick change in the bodys sodium levels. By continuing to browse this site you are agreeing to our use of cookies. Cpm mostly affects the pons area of the brain and is characterized by acute paralysis, difficulty swallowing dysphagia, difficulty speaking. Central pontine myelinolysis cpm, a frequently fatal disorder, is often associated with hyponatremia. Five cases of central pontine myelinolysis cpm were detected by neuropathological examination in a series of 50 patients coming to necropsy after liver transplantation. Plasma exchange successfully treats central pontine. Central pontine and extrapontine myelinolysis after alcohol. Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the central basis pontis. Central pontine myelinolysis pathophysiology, causes.
This case report underlines the importance of recognising risk factors predisposing the patient to the development of central pontine myelinolysis. Central pontine myelinolysis a 60 y o female with altered consciousness accompanying with a file of previous hospital admission and lab reports mentioning abnormal levels of na and k. Damned if we do, damned if we dont oh ms, kim hi, carrol hj. 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. A holistic approach in the treatment course should be provided in order to attain recovery. Recommendations for treatment of symptomatic hyponatremia. Mar 27, 2019 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Intravenous immunoglobulin ivig in treatment of central. The condition commonly results from a rapid increase in the sodium levels of the human body. Jul 21, 2011 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Classical clinical manifestations range from a depressed level of consciousness to dysarthria and flaccid quadriplegia. 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.
A 9 year old boy presented with fever, drowsiness, quadriparesis and facial myokymia. Central pontine myelinolysis cpm is a noninflammatory, demyelinating condition that has been described mostly in the context of rapid correction of hyponatraemia. All the mechanisms of hyponatremia and hypernatremia were summarized here in our case report. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. Electroencephalography hyponatremia and central pontine. It is not clearly known how the myelin sheath damage occurs. Cns nontumor pontine and extrapontine myelinolysis. This website is intended for pathologists and laboratory personnel, who understand that medical information is imperfect and must be interpreted using reasonable medical judgment.
However, it may develop in the context of normal serum. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. In other words, drinking chronically can lead to demyelination of your pontine area, basal ganglia, or other areas of your brain. N2 central pontine myelinolysis is a disease process described by adams, victor, and mancall in 1959 in which there is destruction of the myelin sheaths in the basis pontis in a central well demarcated symmetric area.
Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Apr 11, 2014 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 was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. Alcoholism hyponatremia and central pontine myelinolysis. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Osmotic demyelination syndrome linkedin slideshare. 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. Benign type of central pontine myelinolysis in alcoholism. A history of alcoholism is common and found in 39 percent of affected patients.
It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis. Learn what causes it and which symptoms to watch out for. Central pontine and extrapontine myelinolysis cpepm is a rare demyelinating syndrome. Messing, in aminoffs neurology and general medicine fifth edition, 2014. Keywords autocorrection, central pontine myelinolysis, hyponatraemia, osmotic demyelinisation syndrome abstract central pontine myelinolysis is a rare and potentially lifethreatening complication of a sudden rise in serum osmolality. Acute psychosis as main manifestation of central pontine. This condition, however, has also been found in the malnourished, transplant recipients, or chronically debilitated patients,in particular, those in whom electrolyte. Through a mechanism that is only partly understood, the shift. Central pontine myelinolysis information page national. Osmotic demyelination syndrome radiology reference article. The majority of them had taken part in binge drinking and had a subsequent consciousness disturbance for 18. Conventional ct and mr imaging findings lag the clinical manifestations of cpm.
Intravenous immunoglobulin ivig in treatment of central pontine and extrapontine myelinolysis shahram izadyar, shumaila sultan, joseph s. It was eventually recognized that demyelinating lesions can also be seen outside the pons, termed extratrapontine myelinolysis 2,3. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Jun 26, 20 magnetic resonance image mri in central pontine myelinolysis. Central pontine and extrapontine myelinolysis in children. Recovery after central pontine myelinolysis springerlink. Central pontine myelinolysis associated with diabetic. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells.
When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Central pontine myelinolysis northwestern scholars. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was suggested, and. Pdf central pontine myelinolysis induced by alcohol. Central pontine myelinolysis occurs most often as a complication of treatment of patients with profound, lifethreatening 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. The main cause of central pontine myelinolysis is a rapid correction of low sodium levels in the body. The cause is traditionally associated with overzealous correction of hyponatraemia in patients who are malnourished, alcoholic or chronically ill. Central pontine myelinolysis cpm, first described in 1959 by adams et al, 1 is characterized by demyelination that involves the central portion of the base of the pons. We describe the case of a 21yearold woman who was hospitalized at week 10 of gestation because of severe hyperemesis. Osmotic demyelination syndrome ods is brain cell dysfunction. Current concepts in pontine myelinolysis translational biomedicine. In patients with this condition, the myelin sheath which protects the nerves is damaged, interfering with their ability to signal to each other.
Antemortem computed tomography ct had been performed in nine, and ante or postmortem magnetic resonance mr imaging in 11. Truncal ataxia and gait instability were present in most of the patients after recovery. Central pontine myelinolysis market research report. Its clinical presentation varies, but may include acute paralysis, dysarthria and dysphagia. Keywords central pontine myelinolysis, refeeding syndrome, coma, rehabilitation. The said disease affects specifically the central portion of. Nine alcoholic patients with central pontine myelinolysis cpm,who showed a favorable prognosis, are reported. Nov 15, 2019 how can central pontine myelinolysis be prevented.
Central pontine myelinolysis is considered to require emergent attention once suspected or diagnosed. Central pontine myelinolysis or cpm is a rare neurological disorder that primarily affects the myelin sheath of our nerve cells neurons found in the brainstem. None of the patients had had acute correction of hyponatremia. Central pontine myelinolysis symptoms, treatment and prognosis. Extrapontine myelinolysis radiology reference article. Central pontine myelinolysis symptoms, treatment and. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. The clinical features vary depending on the extent of involvement. Recovery from probable central pontine myelinolysis. Chronic alcoholism or rapid correction of hyponatremia was present in over 75% of cases. Rosenbaum1 central pontine myelinolysis cpm was first described by adams et al. Osmotic myelinolysis central pontine myelinolysis and extrapontine myelinolysis. It has been decades since the disease has been identified, still development of treatment is undergoing.
Central pontine myelinolysis is manifested by rapidly evolving paraparesis or quadriparesis, pseudobulbar palsy, and impaired consciousness and is further discussed in chapter 17. Central pontine myelinolysis genetic and rare diseases. At admission the patient appeared restless and confused and severe. Central pontine myelinolysis is a rare entity of osmotic demyelinating disease that mostly affects the basis pontis in brainstem. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. Parkinsonism and recovery in central and extrapontine myelinolysis.
Early diagnosis of central pontine myelinolysis with. Common clinical manifestations of cpm include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees. Central pontine and extrapontine myelinolysis cpem are recognised complications of hyponatraemia and its overly rapid correction. 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 cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished adams et al. This is a demyelinating disease which was first described in the year 1959. She developed acute hypernatremia due to the stage of pregnancy and other comorbidities. 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, extrapontine myelinolysis, osmotic demyelination, hyponatremia, magnetic resonance imaging, adolescence.
Central pontine myelinolysis in liver transplantation. 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 an overview sciencedirect topics. Central pontine myelinolysis cpm is a neurological condition involving severe damage to the myelin sheath of nerve cells in the pons it is predominately. Central pontine myelinolysis cpm, also known as osmotic demyelination syndrome or central pontine demyelination, is a neurological disorder caused by severe damage of the myelin sheath of neurons. The clinical dilemma of proper treatment of severe hyponatremia is exemplified by this. The first cases were described in patients with a history of alcoholism and malnutrition. Also known as osmotic demyelination syndrome or central. Central pontine myelinolysis cpm is a demyelinating disorder characterized by the loss of myelin in the center of the basis pons, and is mainly caused by the rapid correction of hyponatremia. Most published cases are based on autopsy reports, whereas antemortem diagnosis is rare.
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. Central pontine myelinolysis cpm is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are well recognized syndromes related to the rapid correction of hyponatremia, which. Central pontine myelinolysis, osmotic demyelination syndrome. This case has picture graph representation of parameters that led to.
A 42yearold high risk pregnant female presented with hyponatremia from multiple causes and was treated with total parenteral nutrition. Goldsmith department of neurology, baylor college of medicine, houston, texas central pontine myelinolysis cpm extrapontine myelinolysis epm is usually associated with rapid. Central pontine myelinolysis cpm is known to be almost universally fatal. Neuropsychological findings of extrapontine myelinolysis without. Outcome of central pontine and extrapontine myelinolysis n. Recovery of lockedin syndrome in central pontine myelinolysis. A case of osmotic demyelination syndrome occurred after. 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. Hence, the slow correction of low levels of sodium would prevent its occurrence. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Osmotic myelinolysis central pontine myelinolysis and. The original patient population included only chronic alcoholics.
B axial and c sagittal t2weighted images show the lesion in the corresponding region as d, the coronar. At admission the patient appeared restless and confused. Extrapontine myelinolysis epm is one of the complications occurring secondary to rapid correction of hyponatremia, and is, along with central pontine myelinolysis encompassed by the more recent term osmotic demyelination syndrome in the vast majority of cases it is associated with central pontine myelinolysis but it can also rarely occur as an isolated entity. The clinical dilemma of proper treatment of severe hyponatremia is.
If you are an alcoholic, you are at great risk for developing it. Pdf catatonia due to central pontine and extrapontine. A case of osmotic demyelination syndrome occurred after the. Initially described in chronic alcohol abuse and malnourished patients,1 cpm has also been associated with the rapid correction of hyponatremia and alterations in blood glucose homeostasis. Central pontine myelinolysis cpm is a rare neurologic entity of varied etiology characterized by the destruction of the myelin sheath in pontine structures. We present a case in which the diagnosis of cpm was confirmed by computed tomography ct. Koci t m, chiang f, chow p et al, thalamic extrapontine lesion in central pontine myelinolysis, am. Central pontine myelinolysis is a serious neurological condition caused by damage to the nerves in the area of the brainstem known as the pons. This is a neurological disease, with a specific location area known as the pons and iatrogenic etiology. Five 20% had central pontine myelinolysis, seven 28% had extrapontine myelinolysis, and 52% had both. Central pontine myelinolysis cpm was first termed in 1959 by adams to describe a symmetric, demyelinating focus prominent in the central pons. However, changes in sodium levels can lead to rapid movement of fluids in and out of the myelin sheath. A diagnosis of central pontine myelinolysis was made. Central pontine and extrapontine myelinolysis with a report of 58 cases, cln neuropathol, 66 26270, 1987,pmid 3322623 5.
Hyponatraemia and hypokalaemia were noted in 20 80% and 10 40% patients respectively. In this manner, the incidence rate of cpm can also be greatly reduced. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. The more you drink, the more likely you are to develop hyponatremia. Central pontine myelinolysis is characterised by focal osmotic demyelination within the pons. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Yuhming chang department of neurology, hsinchu mackay memorial hospital, taiwan. Central pontine myelinolysis is also called osmotic demyelination syndrome.
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. This condition can be fatal or cause long term disability. 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. Pontine myelinolysis following excessive consumption of. Central pontine myelinolysis an overview sciencedirect. Pathology outlines pontine and extrapontine myelinolysis. A the axial diffusionweighted image shows marked signal intensity in the pons. Evolution of central pontine myelinolysis on ct shelley rosenbloom,1 david buchholz,2 ashok j. Central pontine myelinolysis secondary to hyperglycaemia. Osmotic demyelination syndrome ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical sciences, lucknow, india 2.
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