Acute psychosis as main manifestation of central pontine. Also known as osmotic demyelination syndrome ods, pm. Central pontine myelinolysis cpm is a disease that may present with coma, quadriplegia. 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. Extrapontine myelinolysis in a child with nephrotic. Pdf central pontine and extrapontine myelinolysis researchgate.
We evaluated a 58yearold woman with pancreatic encephalopathy associated to pontine and extrapontine myelinolysis involving the brain and spinal cord. Central pontine myelinolysis an overview sciencedirect. Hyponatremia at time of admission was 104 meq, corrected to 121 meq within 24hrs. Results compulsive water drinking or psychogenic polydipsia is a common cause of hyponatremia among individuals with chronic mental illness. Lesions can also occur outside the pons as extrapontine myelinolysis epm. Central pontine myelinolysis an overview sciencedirect topics. Extrapontine myelinolysis presenting as acute parkinsonism. On viewing the imaging findings, history of recent treatment was looked into. Extrapontine myelinolysis has not been described in children previously.
Rapid correction of hyponatremia can cause a brain disease known as myelinolysis. Extrapontine myelinolysis resulting in transient cortical. We describe the resolving mr features in an adolescent who has experienced complete neurologic recovery. However, the pontine and extrapontine lesions of myelinolysis have been shown to occur very rarely secondary to dds in end stage renal failure patients. Osmotic myelinolysis is a rare, acute, demyelinating process that involves the pons central pontine myelinolysis and other locations of the central nervous system extrapontine myelinolysis. The concept was extended from 1962 with the recognition that lesions can occur outside the pons, socalled extrapontine myelinolysis epm. Although early descriptions of this phenomenon emphasized its grave prognosis, recent reports have documented recovery in mild cases 17, 21 23. Extrapontine lesions can occur in the basal ganglia, internal capsule, lateral geniculate body, and cortex. Jul 21, 2011 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Clinical evidence of the presence of such lesions, however, have rarely been described. Central pontine and extrapontine myelinolysis after alcohol. Central pontine and extrapontine myelinolysis can occur despite appropriate sodium correction, likely related to severity, duration of initial hyponatraemia and the underlying cause. It has a wide range of consequences, ranging from mild neurological symptoms to death. Age, sex, decade of diagnosis, neurologic presentation, outcome, and attributed causes were extracted.
Myelinolysis has been reported after the rapid sodium correction that can result from dialysis 4. In the background of such a history with normal mra, a diagnosis of central pontine. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are well recognized syndromes related to the rapid correction of hyponatremia, which. Extrapontine myelinolysis with involvement of the hippocampus. A 65 year old female known case of hypertension, first time detected type 2 diabetes mellitus was admitted in smhs hospital with history of altered sensorium for 1 day. Intravenous immunoglobulin ivig in treatment of central.
On admission, the infant had impaired consciousness, mild dehydration, and severe hypernatremia 190 mmoll. Idiopathic central pontine and extrapontine myelinolysis in a child. Also known as osmotic demyelination syndrome ods, pm is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis epm 2. A 58yearold woman presented with subacute onset of tetraparesis, anarthria, and dysphagia after rapid correction of hyponatremia caused by repeated vomiting. Central pontine myelinolysis cpm is a noninflammatory demyelinating disease of the pons characterized by loss of myelin, and was described by adams in 1959. An unusual presentation of pancreatic encephalopathy. Central pontine myelinosis and osmotic demyelination. Central pontine and extrapontine myelinolysis despite. We propose that in a significant number of cases, central pontine myelinolysis may be predicted by doing an early mri of the brain with diffusionweighted imaging. The mechanism is not fully understood and the treatment is controversial. Central pontine myelinolysis and extrapontine myelinolysis. Central pontine myelinolysis is a demyelinating disorder characterized by the loss of myelin in the center of the basis pontis usually caused by. Here we report a case 7 months old chid presented as severe hypernatremic dehydration, resulting.
Central and extrapontine myelinolysis 02 after alcohol. In two patients the diagnosis of central pontine myelinolysis cpm, suspected on clinical grounds, was supported by computerized tomographic ct sections made perpendicular to the pons, and confirmed on postmortem examination. 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. The clinical features vary depending on the extent of involvement. We describe a 6yearold girl with nephrotic syndrome who presented with symptomatic hyponatremia, and who developed acute quadriparesis with pseudobulbar palsy during rapid correction of the hyponatremia. However, it rarely occurs with the correction of hypernatremia and hyperosmolarity and involves extrapontine areas. Described here is a novel case of transient cortical blindness in association with imaging features of extrapontine myelinolysis, which occurred in a child with carbamoyl phosphate synthetase deficiency after rapid correction of hyperammonemia. Brain mri showed pontine and extrapontine lesions typical for the osmotic demyelination syndrome figure, a. On rare occasions, demyelination occurs outside the pons and is termed extrapontine myelinolysis epm.
Pdf extrapontine osmotic myelinolysis researchgate. The extrapontine myelinolysis of osmotic demyelination syndrome ods is a wellknown but uncommon disorder of the central nervous system. Hemiparesis, encephalopathy, and extrapontine osmotic myelinolysis in the setting of hyperosmolar hyperglycemia. There may be a lag between neurological symptoms and radiographic findings in this condition. Extrapontine myelinolysis and behavioral change after. Pdf extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical. The authors discuss the relevance of the results for theories of pathogenesis and for diagnosis of patients. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Clinicians should maintain their vigilance while correcting electrolyte imbalances, especially with associated comorbidity.
Neuropsychological findings of extrapontine myelinolysis without. The extrapontine myelinolysis epm of osmotic demyelination syndrome ods is a rare and dangerous disorder of the central nervous system. Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Central pontine and extrapontine myelinolysis after. Background central pontine and extrapontine myelinolysis cpmepm are severe neurologic complications after liver transplantation methods the present work retrospectively evaluated singlecenter prevalence of cpmepm and associated risk factors. Extrapontine myelinolysis epm is a common metabolic disorder and it is associated with neurological complications. Extrapontine myelinolysis presenting as acute parkinsonism bmc. Extrapontine myelinolysis following extreme hypernatremia. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished adams et al. This worldwide, iatrogenic problem has been documented in medical, surgical, pediatric, psychiatric, and obstetric settings. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. We report the first case of an infant with epm without central pontine myelinolysis cpm presenting with severe hypernatremia. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Sequential mri in pontine and extrapontine myelinolysis.
Sep 10, 2006 extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Extrapontine myelinolysis of osmotic demyelination syndrome in a. Pancreatic encephalopathy refers to a gamut of neuropsychiatric symptoms complicating acute pancreatitis. Osmotic demyelination syndrome ods, which embraces central pontine myelinolysis cpm and extrapontine myelinosis epm, is often underdiagnosed in clinical practice, but can be. Computerized tomography in the diagnosis of central and.
Osmotic myelinolysis is a known complication of pancreatic encephalopathy. Extrapontine myelinolysis epm is an uncommon disorder in children, with few pediatric cases reported to date. Mri changes associated with pontine and extrapontine myelinolysis first occur on diffusionweighted imaging. Central and extrapontine myelinolysis affecting the brain. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We report two unusual cases of such a rare condition. Objective description of a case of osmotic myelinolysis associated with hyponatremia produced as a consequence of compulsive water drinking.
We would like to present a case of isolated extrapontine myelinolysis of the osmotic demyelination syndrome which has been reported only a few times in the literature. Extrapontine myelinolysis of osmotic demyelination syndrome. Frontiers central and extrapontine myelinolysis affecting. Myelinolysis definition of myelinolysis by the free dictionary. Osmotic demyelination syndrome ods is a devastating neurologic disorder often associated with a precipitous rise in serum sodium. Hyperintense lesions are seen on t2, and hypointense lesions on t1 weighted images.
Pontine and extrapontine myelinolysis secondary to glycemic. Osmotic demyelination syndrome ods refers to central pontine myelinolysis and extrapontine myelinolysis. Introduction pontine myelinolysis pm was first described in 1959 by adams et al. View the article pdf and any associated supplements and figures for a period of 48 hours. Striatal syndrome following hyponatremia and its rapid. From the national institute of neurologic disorders and stroke, national institutes of health. In epm, demyelinating lesions occur in brain structures other than the pons, including the basal ganglia e. Extrapontine myelinolysis epm is a pathophysiologically similar condition affecting 10% of patients with cpm. Central and extrapontine myelinolysis affecting the brain and spinal cord. During hyponatraemia correction, rapid osmotic shifts. 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.
A rather rare complication can be extrapontine myelinolysis 24, which is often associated with an overcorrection of hyponatremia. Central pontine myelinolysis and extrapontine myelinolysis are characterized by symmetric demyelination subsequent to rapid shifts in serum osmolality. Extrapontine myelinolysis caused by rapid correction of. Wright divisions of neuropathology and neurology, cleveland metropolitan general hospital, and case western reserve university, school of medicine, cleveland. Pediatric osmotic demyelination syndrome ods is a rare condition with a very few cases reported in the world literature. There are few reports of epm occurred after endonasal endoscopic resection of pediatric craniopharyngioma. Pdf download for central pontine and extrapontine myelinolysis in. Extrapontine myelinolysis was suggested on ct scans in both cases. Although the disease was unknown 50 years ago, a clinical diagnosis of myelinolysis can now be made with confidence. Mar 27, 2019 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. Pontine and extrapontine myelinolysis a neurologic disorder following rapid correction of hyponatremia. Extrapontine myelinolysis could make a complete recovery with symptomatic support alone. Psychogenic polydipsia, hyponatremia and osmotic myelinolysis. Central pontine myelinolysis information page national.
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. Rodriguez porcel fj, agha c, flaster m, morales vidal s, schneck mj, lee j and biller j 2012 central and extrapontine myelinolysis affecting the brain and spinal cord. Intravenous immunoglobulin ivig in treatment of central pontine and extrapontine myelinolysis shahram izadyar, shumaila sultan, joseph s. Pathology outlines pontine and extrapontine myelinolysis. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. The results showed that the diagnosis, course, and outcomes of central pontine and extrapontine myelinolysis clearly have changed over the past few decades. Central myelinolysis was first described in 1959 by adams as demyelinating lesions occurring in the central pons in alcoholic and undernourished patients. 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. Several pathologic reports have indicated the occurrence of extrapontine sites of myelinosis associated with electrolyte disturbance and its rapid correction.
Central pontine myelinolysis genetic and rare diseases. The concept was extended from 1962 with the recognition that lesions can occur at extrapontine sites, socalled extrapontine myelinolysis epm. Central pontine and extrapontine myelinolysis in children. Parkinsonism as well as dystonic signs are rarely seen in central pontine myelinolysis and extrapontine myelinolysis. 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. Cranial magnetic resonance imaging demonstrated bilateral, symmetric basal ganglia lesions extrapontine myelinolysis. Central pontine myelinolysis cpm and extrapontine myelinolysis. These disorders are characterized by insults to regions of the brain with anatomical features predisposing white matter tracts to myelin injury in the setting of osmotic disturbances and their attempted correction. A 41yearold woman presented with acute parkinsonism like features while on a holiday. 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 is an osmotic demyelination syndrome which, in the majority of cases, occurs after rapid correction of hyponatraemia. Keywords central pontine myelinolysis, extrapontine myelinolysis. Cns nontumor pontine and extrapontine myelinolysis. A 56yearold chronic alcoholic had been admitted with a history of confusion. Although the mechanism is not fully understood and the treatment is controversial, hyponatremia is probably considered to be the main pathophysiological basis.
Regions of involvement manifested increased t2 signal intensity. 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 is a rare neurologic disorder of unclear etiology characterized by symmetric midline demyelination of the central pons. Myelinolysis after correction of hyponatremia annals of. 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. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. Myelinolysis definition of myelinolysis by the free. Hemiparesis, encephalopathy, and extrapontine osmotic. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was suggested, and.
Resolving mr features in osmotic myelinolysis central. Extrapontine myelinolysis is an acute demyelination involving other than central pontine regions due to rapidly fluctuating serum osmolality. A 51 year old woman developed central pontine myelinolysis and extrapontine myelinolysis with parkinsonism after severe vomiting which followed alcohol and drug intake, even though marked hyponatraemia had been corrected gradually over six days. Laboratory examinations showed severe hypernatremia and hyperosmolarity. 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. Extrapontine myelinolysis hyponatremia and central. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. There are few reports of ods caused by a sellar lesion. Jun 27, 2018 the extrapontine myelinolysis epm of osmotic demyelination syndrome ods is a rare and dangerous disorder of the central nervous system.
To our knowledge, this is the first clinic pathological case. Here we describe a rare case of extrapontine myelinolysis that developed after a rapid correction of symptomatic hyponatremia induced by pituitrin to treat a patient with massive hemoptysis. Osmotic myelinolysis is a distinctive clinical syndrome with characteristic mr features in the central pons central pontine myelinolysis and in other locations extrapontine myelinolysis. Extrapontine myelinolysis in a child with nephrotic syndrome. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are considered iatrogenic diseases caused by a rapid rise in serum sodium during the correction of hyponatremia. Extrapontine myelinolysis following extreme hypernatremia and. Seventysix pediatric cases were identified in the literature. Extrapontine myelinolysis radiology reference article. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. We report three children with severe hypernatremia and extrapontine myelinolysis involving various combinations of thalamus, basal ganglia, external and extreme capsules, and cerebellar vermis. Central pontine and extrapontine myelinolysis request pdf. Pdf extrapontine myelinolysis presenting as acute parkinsonism. Caudal and lentiform nuclei myelinolysis following.
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