Acute psychosis as main manifestation of central pontine. Magnetic resonance imaging scan of the brain revealed characteristic findings of central pontine myelinolysis cpm. Central pontine myelinolysis, pathological laughing and crying, cognition, emotion 1. Central pontine myelinolysis cpm is a disease that may present with coma, quadriplegia, or no symptoms at all. Thirtynine case studies of patients with central pontine myelinolysis have appeared in the literature 115 since the lesion was first recognized as an unusual demyelinative process in the basis pontis by adams et al in 1959. Treatment with highdose intravenous thiamine only led to a partial clinical improvement. Myelin is a covering that protects the pontine nerve cells. Central pontine myelinolysis cpm is a rare, acute, and uniformly.
Pdf extra and central pontine myelinolysis in a child with. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. 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 an overview sciencedirect. Central pontine myelinolysis cpm is a noninflammatory demyelinating lesion of the pons. Orolingual dyskinesia in central pontine myelinolysis. The etiology was not known then but the authors suspected the cause to be either a toxin or a nutritional deficiency. Neuroimaging revealed a striking triangular central pontine defect in keeping with central pontine myelinolysis cpm. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Cpm and extrapontine demyelination epm are together termed osmotic demyelination syndrome ods, a known and serious complication of acute correction of hyponatremia. A case of chronic asymptomatic central pontine myelinolysis with.
Occult central pontine myelinolysis post liver transplant. Nov 16, 2020 background central pontine myelinolysis cpm is a noninflammatory demyelinating lesion of the pons. Seventysix pediatric cases were identified in the literature. Central pontine myelinolysis cpm is a demyelinating disease of the brainstem but rarely found in other cerebral regions. Central pontine and extrapontine myelinolysis sciencedirect. Indeed, a rapid correction of hyponatremia has been regarded as the main cause of osmotic demyelination syndrome 2, which explains why the disease was first described in. Dec 01, 2016 central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. The cause is traditionally associated with overzealous correction of hyponatraemia in patients who are malnourished, alcoholic or chronically ill. Apr 25, 2018 central pontine myelinolysis secondary to rapid sodium correction can still be seen in patients who have their serum sodium levels normalized at the suggested appropriate rate.
Cpm has also been linked to the kept above 9 gdl, and these children had a tolerable quality of life. 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. Chronic alcoholism associated cpm tends to be benign with a favorable prognosis compared to cpm secondary to rapid correction of hyponatremia. Osmotic demyelination syndromealso known as central pontine myelinolysis or extrapontine myelinolysisusually occurs after rapid correction.
Its exact incidence is not well defined, but studies show a prevalence of 0. Results mri changes associated with pontine and extrapontine myelinolysis first occur on diffusionweighted imaging. Most children be kept below 05 mmol1 per hour to avoid central pontine were transfused every 46 weeks, the haemoglobin usually being myelinolysis cpm. Central pontine myelinolysis during treatment of hyperglycemic. Central pontine myelinolysis with complete recovery keilson. Central pontine myelinolysis in a previously healthy 4yearold. However, it may develop in the context of normal serum sodium.
Goldhammer, extrapyramidal syndrome responsive to dopaminergic treatment following recovery from central pontine myelinolysis, eur neurol 33 1993, 4850. Pdf central pontine myelinolysis cpm commonly presents as a complication of treatment in patients with profound life threatening. Pontine myelinolysis caused by hypovolemic hypernatremia. Central pontine myelinolysis in a child with leukemia. Central pontine myelinolysis fulltext european neurology 2009. Central pontine myelinolysis cpm was first described as a disease affecting the alcoholics and the malnourished by adams and colleagues in 19591. Osmotic demyelination syndrome ods, also known as central pontine myelinolysis, is a neurological disorder characterised by myelin loss in the central pons. Central pontine myelinolysis is a serious condition that may result in neuropathological sequelae and mortality, and clinicians should be aware of its potential presence in patients with malignancies. Central pontine and extrapontine myelinolysis was experimentally produced in dogs by the rapid correction of severe, sustained, vasopressin.
In the supratentorial area, she presented a significant hyperintensity in the periventricular white substance. Korogi y, takahashi m, shinzato j, sakamoto y, mitsuzaki k, hirai t, yoshizumi k. This study aimed to identify the causes and contributing factors, neurologic presentation, and outcomes of central pontine and extrapontine myelinolysis and to examine any trends in the presentation and course of these disorders over the past 50 years. It is a condition most frequently related to rapid correction of hyponatremia. The authors discuss the relevance of the results for theories of pathogenesis and for diagnosis of patients.
The temporal resolution of the patients symptoms suggests that prompt recognition of central pontine and epm and conversion from tacrolimus to rapamycin during the early post. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Osmotic demyelination syndrome is a rare, severe neurological disease that includes extrapontine and central pontine myelinolysis cpm, with the latter being. Central pontine myelinolysis not associated with rapid correction of hyponatraemia.
Central pontine myelinolysis is characterised by focal osmotic demyelination within the pons. In the course of our studies of the neuropathology of alcoholism, which were begun at the neurological unit, boston city hospital, and have continued in the lab. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Cognitive and emotional dysfunction after central pontine. It is a rare condition that predominantly affects the pons but may involve extrapontine structures including the midbrain, thalamus and cerebellum. Pontine and extra pontine myelinolysis ism due to pontine and extrapontine myelinolysis in japanese. Central pontine myelinolysis cpm is also referred to as osmotic demyelination syndrome. Conventional ct and mr imaging findings lag the clinical manifestations of cpm. Common clinical manifestations of cpm include spastic quadriparesis, dysarthria, pseudobulbar palsy, and encephalopathy of various degrees. C and d, axial t2weighted mr images 200080 on day 24. Central pontine myelinolysisextrapontine myelinolysisosmotic endothelial. Although usually associated with rapid correction of.
Pdf idiopathic central pontine myelinolysis in childhood. Thalamic extrapontine lesions in central pontine myelinolysis. Oct 01, 2011 central pontine myelinolysis cpm is a clinically heterogeneous, demyelinating condition originally thought to occur only in the central pons. This paper follows the historical evolution of central pontine myelinolysis and the changing concepts of its metabolic aetiol. Cognitive and emotional dysfunction after central pontine myelinolysis central pontine myelinolysis cpm, though rare, has increasingly been recognizedas a neurologicaldisorder associated with a lesion of the central portion of the basis pontis 1. Central pontine myelinolysis in a patient with j neurol neurosurg psychiatry 1993.
Central pontine myelinolysis cpm is a wellknown neurologic event that is classically associated with. Central pontine myelinolysis following rapid correction of. Pdf central pontine myelinolysis and changes in serum. Central pontine myelinolysis in a child with leukemia jama. Central pontine myelinolysis is an acute noninflammatory demyelinating disorder first described by adams et al in 1958. 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 the american journal of medicine. Its clinical presentation varies, but may include acute paralysis, dysarthria and dysphagia. Subacute central pontine myelinolysis secondary to hyperglycaemia. Introduction pontine myelinolysis pm was first described in 1959 by adams et al.
Charness, in office practice of neurology second edition, 2003 central pontine myelinolysis. The initial cases were seen in patients with alcohol use disorder and malnutrition. Central pontine myelinolysis secondary to hyperglycaemia. Osmotic demyelination syndrome in a normonatremic patient with. The authors describe two patients with presumed central pontine myelinolysis whose clinical symptoms were mild despite extensive. It is one of the most serious neurological complications occurring in liver.
Desmopressininduced severe hyponatremia with central pontine. Parkinsonism after correction of hyponatremia with radiological central pontine myelinolysis and changes in the basal ganglia. 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 is an acute demyelinating neurological disorder affecting primarily the central pons and is frequently associated with rapid correction of hyponatremia. Magnetic resonance mr imaging findings were consistent with central pontine and epm. Pdf central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. Sep 01, 2008 both central pontine and extra pontine myelinolysis are very uncommon disorders.
Dystonia in central pontine myelinolysis without evidence of extrapontine myelinolysis. Signs of pseudobulbar palsy with dysphagia and dysarthria, paraparesis or quadriparesis are first symptoms, which usually appear within 26 days after correction of hyponatraemia. Central pontine and extrapontine myelinolysis in children. Myelinolysis or lateonset imbalance in dialysis nefrologia. It was evaluated as pontine and extrapontine myelinolysis. Central pontine myelinolysis cpm was first described in 1959 by adams and his colleagues in a report of four patients with pseudobulbar palsy and quadriplegia. Cpm is a neurological disorder defined as a demyelination of the nerve cells, in the pontine area and. Desmopressininduced severe hyponatremia with central. Klintmalm, in transplantation of the liver third edition, 2015 central pontine myelinolysis. Central pontine myelinolysis genetic and rare diseases.
Central pontine myelinolysis an overview sciencedirect topics. Central pontine myelinolysis information page national. Central pontine myelinolysis is a recently recognized clinical and pathologic entity. Their pathophysiology is classically related to changes in serum sodium concentration 1. 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. Treatment of central pontine myelinolysis with therapeutic. Study of brain electrolytes and organic osmolytes during correction of chronic hyponatremia. We report the case of a patient with clinical cpm whose diagnosis was confirmed by the now characteristic. It was first identified approximately 50 years ago. Pdf central pontine and extrapontine myelinolysis owing.
Central pontine myelinolysis cpm is a neurological demyelinating disease of the pons. Initial immunosuppression included tacrolimus, mycophenolate mofetil, and corticosteroids. Oct 05, 2018 objective this study describes the mri changes associated with pontine and extrapontine myelinolysis secondary to rapid correction of hyponatremia in dogs. Thirtyseven cases of central pontine myelinolysis cpm with miscellaneous underlying disorders were found in 1,000 consecutive autopsies, of which 636 brains were examined. A hitherto undescribed disease occurring in alcoholic and malnourished patients. Signs of pseudobulbar palsy with dysphagia and dysarthria, paraparesis or quadriparesis are first symptoms, which usually appear within 26 days after correction of. Conversely, hyperglycemic hyperosmolarity syndrome hhs develops in patients with type 2 diabetes who still have some. Sequential mri in pontine and 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.
Clinical evolution of central pontine myelinolysis in a. Pdf extra and central pontine myelinolysis in a child. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are wellrecognized syndromes that are. Central pontine and extrapontine myelinolysis after alcohol. Studies show that lesions can occur with or without cpm. Central pontine myelinolysis cpm is a demyelinating disease of the pons and is associated with a rapid correction of hyponatremia 172 or large fluid shifts occurring during transplant. A rare case of pontine and extrapontine myelinolysis in a pediatric. 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. Early diagnosis of central pontine myelinolysis with. Central pontine myelinolysis is characterized by the occurrence of acute demyelinating lesions of cells in the pons secondary to abrupt oscillations of serum osmolarity.
The patient had normal sodium and glucose levels throughout his admission and for the previous 12month period. Central pontine myelinolysis cpm, which is a component of the osmotic demyelination syndrome. Pdf central pontine myelinolysis in association with. Recently, reports of the roentgenographic features in six cases of central pontine myelinolysis cpm have appeared. Also known as osmotic demyelination syndrome ods, pm pontine myelinosis is subdivided into central pontine myelinolysis cpm and extrapontine. Resolving mr features in osmotic myelinolysis central pontine and extrapontine myelinolysis.
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