Clinical evolution of central pontine myelinolysis in a. With the introduction of ct and mri there was an increasing number of reports on nonfatal cases of cpm. Central pontine myelinolysis in a case of alcohol dependence. It is a condition most frequently related to rapid correction of hyponatremia. Hence, the slow correction of low levels of sodium would prevent its occurrence. Diabetic ketoacidosis, alcoholic ketoacidosis, starvation ketosis and hypoglycaemic ketoacidosis. Central pontine myelinolysis is manifested by rapidly evolving paraparesis or quadriparesis, pseudobulbar palsy, and impaired consciousness and is further discussed in chapter 17.
In patients with this condition, the myelin sheath which protects the nerves is damaged, interfering with their ability to signal to each other. In the background of such a history with normal mra, a diagnosis of central pontine. Here you can read posts from all over the web from people who wrote about central pontine myelinolysis and cerebral edema, and check the relations between central pontine myelinolysis and cerebral edema. Central pontine myelinolysis cpm is a rare, acute, and uniformly fatal demyelinative process that. Central pontine myelinolysis cpm was described in 1959 by adams et al. In this manner, the incidence rate of cpm can also be greatly reduced. Although the mechanism is not fully understood and the.
On rare occasions, demyelination occurs outside the pons and is termed extrapontine myelinolysis epm. Central pontine myelinolysis pathophysiology, causes. Charness, in office practice of neurology second edition, 2003. 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. 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 a rare, acute, and uniformly fatal demyelinative process that involves the pons almost exclusively. My wife, 50, suffered central pontine myelinolysis in august 2000. Parkinsonism and recovery in central and extrapontine myelinolysis a.
Early diagnosis of central pontine myelinolysis with diffusionweighted imaging. Early diagnosis of central pontine myelinolysis with. Ozgurilhan i, demirbas h, yalcin ga, yucesan c, dogan yb. Garzon t, mellibovsky l, roquer j, perich x, diezperez a. Chronic alcoholism associated cpm tends to be benign with a favorable prognosis compared to cpm secondary to rapid correction of hyponatremia. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Conventional ct and mr imaging findings lag the clinical manifestations of cpm. Central pontine and extrapontine myelinolysis after alcohol. 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. 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.
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 hyponatremia and central. The clinical dilemma of proper treatment of severe hyponatremia is. Pdf a case of central pontine myelinolysis caused by. In the course of the past 18 months, he has seen a decline in his abilities. Central pontine myelinolysis, or osmotic demyelination syndrome, is a neurological disease caused by severe damage of the myelin sheath of nerve cells in the brainstem, more precisely in the pons, predominately of iatrogenic etiology. 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.
Oct 01, 2015 a case of central pontine myelinolysis caused by hypophosphatemia secondary to refeeding syndrome. 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. With corticobulbar pathway involvement, patients are affected by dysarthria and dysphagia 3. Introduction pontine myelinolysis pm was first described in 1959 by adams et al.
Central pontine myelinolysis cpm is a neurologic disorder once thought to be uniformly fatal. The neurologists attributed it to a high blood pressure blood drug called moduretic and 48 hours of vomiting. Mutism after brain injury and central pontine myelinolysis. Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the central basis pontis. Symptoms include lessened awareness and difficulty speaking and swallowing. Central pontine myelinolysis symptoms, treatment and prognosis.
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. Hyponatremia at time of admission was 104 meq, corrected to 121 meq within 24hrs. 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. Decreased diffusion in central pontine myelinolysis.
Central pontine myelinolysis cpm, a frequently fatal disorder, is often associated with hyponatremia. A manual of 141 cerebello pontine myelinolysis pdf download rhythmic fingerwork bagpipe pdf download energy medicine pdf free download literatura brasileira ii pdf download. Central pontine myelinosis and osmotic demyelination syndrome. Rhythmic fingerwork bagpipe pdf download ciematame. The main cause of central pontine myelinolysis is a rapid correction of low sodium levels in the body. Intravenous immunoglobulin ivig in treatment of central. Central pontine myelinolysis occurs frequently in alcoholism and chronic liver disease. Also known as osmotic demyelination syndrome ods, pm is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis epm 2. It has been decades since the disease has been identified, still development of treatment is undergoing. 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 associated with diabetic. Central pontine myelinolysis information page national. A case of osmotic demyelination syndrome occurred after. The clinical dilemma of proper treatment of severe hyponatremia is exemplified by this. Intravenous immunoglobulin ivig in treatment of central pontine and extrapontine myelinolysis shahram izadyar, shumaila sultan, joseph s. Cerebellum hyponatremia and central pontine myelinolysis. Diffusionweighted mr findings of central pontine and extrapontine myelinolysis. It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis. Messing, in aminoffs neurology and general medicine fifth edition, 2014.
Central pontine myelinolysis cpm, a neurologic disorder caused most frequently by rapid correction of hyponatremia, is characterized by demyelination that affects the central portion of the base of the pons. Parkinsonism and recovery in central and extrapontine. Common clinical manifestations of cpm include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees. Osmotic demyelination syndrome ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical sciences, lucknow, india 2.
A holistic approach in the treatment course should be provided in order to attain recovery. The patient died before repeat images could be obtained. Thanks to the ninjadoc, i have been made aware of another way that a person can develop hyponatremia, which increases their risk for cpm. Nov 15, 2019 how can central pontine myelinolysis be prevented. Myelinolysis definition of myelinolysis by the free. A unique extrapontine location of a similar process is noted in the medulla of a 6yearold girl. A case of osmotic demyelination syndrome occurred after the. A case of central pontine myelinolysis caused by hypophosphatemia secondary to refeeding syndrome. Chronic alcoholism is frequently associated with this condition which may have. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients.
Goldsmith department of neurology, baylor college of medicine, houston, texas central pontine myelinolysis cpm extrapontine myelinolysis epm is usually associated with rapid. Alcoholic case of central pontine myelinolysis with mainly cerebellar signs. Central pontine myelinolysis is considered to require emergent attention once suspected or diagnosed. Central pontine myelinolysis cpm is a noninflammatory, demyelinating lesion usually localised in the basis pontis. Pathology outlines pontine and extrapontine myelinolysis.
A 41yearold woman presented with acute parkinsonism like features while on a holiday. Damned if we do, damned if we dont oh ms, kim hi, carrol hj. Because of the locality of the damage to the pons, i am going to suggest that the white matter of the cerebellum can also be impacted. 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.
Nearly all reports on nonfatal cases describe severe clinical syndromes with tetraparesis, bulbar palsy, and coma. On admission mri t2w images show t2 hyperintensity confined to centre of pons. Central pontine myelinolysis cpm is known to be almost universally fatal. The extrapontine myelinolysis of osmotic demyelination syndrome ods is a wellknown but uncommon disorder of the central nervous system. The said disease affects specifically the central portion of. For more information on subscription options, click below on the option that best describes you. Recovery after central pontine myelinolysis springerlink. Acute psychosis as main manifestation of central pontine. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. 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. 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. At admission the patient appeared restless and confused.
This concept was extended in 1962 with the recognition that lesions can occur outside the pons, socalled extrapontine myelinolysis epm. The most common cause of central pontine myelinolysis is a quick change in the bodys sodium levels. This most often occurs when someone is being treated for low blood levels of sodium hyponatremia and the sodium is replaced too fast. This condition can be fatal or cause long term disability. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Osmotic demyelination syndrome radiology reference article. Most published cases are based on autopsy reports, whereas antemortem diagnosis is rare. My dad was diagnosed with central pontine myelinolysis last month. Recommendations for treatment of symptomatic hyponatremia. Central pontine myelinolysis cpm 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. Osmotic demyelination syndrome linkedin slideshare. Now, this was interesting because central pontine myelinolysis is an injury that generally impacts the pons.
Central pontine myelinolysis an overview sciencedirect topics. Learn what causes it and which symptoms to watch out for. Recovery from probable central pontine myelinolysis. On viewing the imaging findings, history of recent treatment was looked into.
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. Extrapontine myelinolysis presenting as acute parkinsonism. Ataxic form of central pontine myelinolysis somarajan a, paul. Ataxic form of central pontine myelinolysis somarajan a. Central pontine myelinolysis an overview sciencedirect. Central pontine myelinolysis, also known as osmotic demyelination syndrome, is a neurological disease caused by severe damage of the myelin sheath of. Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Pdf extrapontine myelinolysis of osmotic demyelination. At admission the patient appeared restless and confused and severe. We describe the case of a 21yearold woman who was hospitalized at week 10 of gestation because of severe hyperemesis. It also can occasionally occur when high levels of sodium in the body hypernatremia are corrected too.
This is a neurological disease, with a specific location area known as the pons and iatrogenic etiology. Central pontine myelinolysis and medullary myelinolysis. Central pontine myelinolysis is a serious neurological condition caused by damage to the nerves in the area of the brainstem known as the pons. Three cases diagnosed at autopsy illustrate the characteristic clinical course and pathologic features of cpm. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished adams et al. Central pontine myelinolysis medical condition youtube. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. Yuhming chang department of neurology, hsinchu mackay memorial hospital, taiwan. 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.
It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Mar 27, 2019 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. If the pons and the corticospinal and bulbar tracts are affected in cpm, patients present with frequent encephalopathies and signs of damage to the brainstem. Central pontine myelinolysis cpm, a potentially fatal and debilitating neurological condition, was first described in 1959 in a study on alcoholic and malnourished patients. Central pontine myelinolysis genetic and rare diseases. Myelinolysis definition of myelinolysis by the free dictionary. We present a case in which the diagnosis of cpm was confirmed by computed tomography ct. Michael developed central pontine myelinolysis a few years ago. A history of alcoholism is common and found in 39 percent of affected patients.