We describe a case of cpm occurring secondary to nephrogenic diabetes insipidus di, which developed as a consequence of severe hypokalaemia. There may be no safe limit for the rate of rise of na. Central pontine myelinolysis in patient with normal serum sodium. 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. Extrapontine myelinolysis presenting as acute parkinsonism. Pdf central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. The osmotic demyelination syndrome j neurol neurosurg psychiatry 2004. References to any names, marks, products, or services of third parties or hypertext links to third. Pdf the case is described of a 46 year old female alcoholic. We report the first case of an infant with epm without central pontine myelinolysis cpm presenting with severe hypernatremia.
Central pontine myelinolysis cpm is known to be almost universally fatal. 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. We report a case of a 39yearold woman with a history of chronic alcoholism. 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. Central pontine myelinolysis is an acute noninflammatory demyelinating disorder first described by adams et al in 1958. Pdf central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a. Central pontine myelinolysis information page national. Neurons of the nuclei pontis are relatively spared. Among them 4 cases were male, 2 cases were female, with age 24 to 55 years old and average age 36 years old. Central pontine myelinolysis and the rate of correction of. Mri changes associated with pontine and extrapontine myelinolysis first occur on diffusionweighted imaging.
Learn what causes it and which symptoms to watch out for. In about 50% of cases isolated central pontine myelinolysis cpm. 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. Any information contained in this pdf file is automatically generated from digital material submitted to epos by third parties in the form of scientific presentations. Neuropsychological findings of extrapontine myelinolysis without. Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Central pontine and extrapontine myelinolysis despite. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain.
View enhanced pdf access article on wiley online library html view download pdf for offline viewing. 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 correction of 1. Central pontine myelinolysis an overview sciencedirect topics. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. 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 bicarbonate therapy. Evidence of aquaporin involvement in human central pontine. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia. A case of osmotic demyelination syndrome occurred after. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished.
Central pontine myelinolysis cpm, which was originally considered to be the result of rapid correction of chronic. However, there are other predisposing risk factors chronic alcoholism, hypokalaemia that perpetuate the development of ods. Central pontine myelinolysis cpm is heterogeneous group of demyelinating. Central pontine myelinolysis is characterised by focal osmotic demyelination within the pons. Central pontine myelinolysis with minimal hyponatremia in. However, there are case reports of this disease in the setting of normal serum sodium or minimal hyponatremia. A 41yearold woman presented with acute parkinsonism like features while on a holiday. Central pontine myelinolysis cpm usually occurs with rapid correction of severe chronic hyponatremia. Cpm is a degeneration of a symmetrical midline patch of the basis pontis. Central pontine myelinolysis cpm, a frequently fatal disorder, is often associated with hyponatremia.
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. 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. Most published cases are based on autopsy reports, whereas antemortem diagnosis is rare. There is no selective involvement of fiber systems.
A case of osmotic demyelination syndrome occurred after the correction of severe hyponatraemia in hyperemesis gravidarum. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Central pontine myelinolysis cpm and extrapontine myelinolysis epm are well recognized syndromes related to the rapid correction of hyponatremia, which. 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. This study describes the mri changes associated with pontine and extrapontine myelinolysis secondary to rapid correction of hyponatremia in dogs. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. Clinicians should maintain their vigilance while correcting electrolyte imbalances, especially with associated comorbidity. There may be a lag between neurological symptoms and radiographic findings in this condition. Despite the pronounced fluctuations in serum osmolality, cpm is rarely seen in diabetics. Atypical presentation of central pontine myelinolysis in. 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. Central pontine myelinolysis cpm is a demyelinating disorder of the. 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.
Central pontine and extrapontine myelinolysis can occur despite appropriate sodium correction, likely related to severity, duration of initial hyponatraemia and the underlying cause. Atypical presentation of central pontine myelinolysis in hyperglycemia. Rehabilitation of the pontine ataxiadysmetria syndrome. Pdf central pontine and extrapontine myelinolysis researchgate. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Sequential mri in pontine and extrapontine myelinolysis. Central pontine myelinolysis secondary to hyperglycaemia.
We present a case in which the diagnosis of cpm was confirmed by computed tomography ct. Line chart showing the trend of serum sodium increment. We report a case of a 39yearold woman with a history of chronic alcoholism who. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. For language access assistance, contact the ncats public information officer. Central pontine myelinolysis symptoms, treatment and. This is a demyelinating disease which was first described in the year 1959.
However, it may develop in the context of normal serum. These cases have been hypothesized to be secondary to other metabolic disturbances such as hyperglycemia or hypophosphatemia. Prognosis is not uniformly bad mri changes may be delayed mri severity is not prognostic r j martin central pontine and extrapontine myelinolysis. Central pontine myelinolysis secondary to hypokalaemic. Central pontine myelinolysis is a syndrome of symmetric osmotic demyelination involving the pons. Central pontine myelinolysis fulltext european neurology 2009. Albeit mild, the righthanded clumsiness, the dysarthria, and the rate of clinical resolution are consistent with this diagnosis. Central pontine and extrapontine myelinolysis after. If you have problems viewing pdf files, download the latest version of adobe reader. A patient with a history of chronic alcohol abuse presenting with a twoday history of lethargy, confusion and a progressive deterioration in the level of consciousness following a fall downstairs. The clinical dilemma of proper treatment of severe hyponatremia is. Central pontine myelinolysis also known as osmotic demyelination, as it not only affects the pons refers to acute demyelination of the white matter tracts traversing the pons.
Central pontine and extrapontine myelinolysis request pdf. Central pontine myelinolysis an overview sciencedirect. Plasma exchange successfully treats central pontine. The authors discuss the relevance of the results for theories of pathogenesis and for diagnosis of patients. Severe hypernatremia, defined as a rise in the serum sodium concentration to a value exceeding 160meql, leads to the failure of the brain defense mechanism to adapt itself to change, and therefore, it is associated with high mortality due to encephalopathy1,2,3. Behavioral manifestations of central pontine myelinolysis. Plasmapheresis to treat osmotic demyelination syndrome. It is a rare condition that predominantly affects the pons but may involve extrapontine structures including the midbrain, thalamus and cerebellum. Central pontine myelinolysis and extrapontine myelinolysis extrapontine myelinolysis. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly.
This is a pdf file of an unedited manuscript that has been accepted for publication. 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. Its clinical presentation varies, but may include acute paralysis, dysarthria and dysphagia. Occasionally gadolinium enhancement is also demonstrated, as in this case, just as in.
Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. Genetic and rare diseases information center gard po box 8126, gaithersburg, md 208988126 tollfree. The said disease affects specifically the central portion of the. This is a case report of cpm associated with hyperglycemia. Download as pdf print show related cases notify admin. The clinical features vary depending on the extent of involvement.
Pathology outlines pontine and extrapontine myelinolysis. Cns nontumor pontine and extrapontine myelinolysis. For more information on subscription options, click below on the option that best describes you. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was suggested, and.
Figure 2 relative proportions of central pontine myelinolysis cpm, extrapontine myelinolysis epm, and cpm with. Recovery after central pontine myelinolysis springerlink. Extrapontine myelinolysis epm is an uncommon disorder in children, with few pediatric cases reported to date. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. As a service to our customers we are providing this early version of the manuscript. Central pontine myelinolysis cpm is a demyelinating disease of the pons and is associated with a rapid correction of. The cause is traditionally associated with overzealous correction of hyponatraemia in patients who are malnourished, alcoholic or chronically ill. Plasma exchange successfully treats central pontine myelinolysis. Central pontine myelinolysis cpm is classically attributed to overly rapid correction of profound hyponatremia. Central pontine myelinolysis genetic and rare diseases. This article is from acta neuropathologica communications, volume 1.
This paper follows the historical evolution of central pontine myelinolysis and the changing concepts of its metabolic aetiology. Osmotic demyelination syndrome ods, which includes both central pontine myelinolysis cpm and. Central pontine myelinolysis cpm has been described in alcoholic patients and in the aftermath of rapid correction of chronic hyponatraemia. Central pontine myelinolysis is a demyelinating condition primarily. On admission, the infant had impaired consciousness, mild dehydration, and severe hypernatremia 190 mmoll.