The cerebello pontine angle may be involved by secondary extension of tumors that originate in neighbouring structures. On rare occasions, demyelination occurs outside the pons and is termed extrapontine myelinolysis epm. Pdf file of the complete article 603k, or click on a page image below to browse page by page. This condition can be fatal or cause long term disability. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. All the mechanisms of hyponatremia and hypernatremia were summarized here in our case report. Pontocerebellar hypoplasia genetic and rare diseases. People who have cpm cant be cured, but their symptoms can be treated. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. 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. Most published cases are based on autopsy reports, whereas antemortem diagnosis is rare. Ods was formerly called central pontine myelinolysis, but the name was changed for several reasons. This appears to be the first report of a case of central pontine myelinolysis associated with chronic alcoholism and liver cirrhosis in the united kingdom. Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome.
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 cpm is a demyelinative disorder of unknown origin. Get a printable copy pdf file of the complete article 316k, or click on a page image below to browse page by page. The authors present an important clinical observation on a favorable recovery of lockedin syndrome due to central pontine myelinolysis cpm in association with liver transplantation, hyponatremia. A rare case of central pontine myelinolysis in overcorrection. Noninvasive cerebellar stimulation a consensus paper. A case of osmotic demyelination syndrome occurred after. Early detection of central pontine myelinolysis with mri.
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. Central pontine myelinolysis is a rare and potentially life threatening complication of a sudden rise in serum osmolality. Behavioral manifestations of central pontine myelinolysis. Prognosis is not uniformly bad mri changes may be delayed mri severity is not prognostic r j martin central pontine and extrapontine myelinolysis. In 1976 a link between these disorders and the rapid correction of. Central pontine myelinolysis cpm now termed osmotic demyelination syndrome ods, characterized by damage to regions of the brain most commonly pontine white matter tracts after rapid correction of metabolic disturbances such as hyponatremia low amounts of sodium in the blood. The cpm, now known as osmotic demyelination syndrome, is an acquired demyelinating lesion of the pons that typically occurs after rapid correction of hyponatremia 3. Central and extrapontine myelinolysis 02 after alcohol. Jul 21, 2011 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. There is some evidence that rapid changes in osmolality during treatment may be the precipitant of cpm. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms.
In mri scan results, damage to the pons is more visible compared with ct scan results. Diffuse large b cell lymphoma with central pontine. Click download or read online button to brain edema cerebello pontine angle tumors book pdf for free now. Our case differs in that the underlying cause of the myelinolysis was a complication of hemodialysis as a treatment for endstage renal failure. 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 cpm is a neurological condition involving severe damage to the myelin sheath of nerve cells in the pons an area of the brainstem. Parkinsonism and recovery in central and extrapontine. 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. Whilst dka presents within hours of onset, hhs comes on over many days, and consequently the dehydration and metabolic disturbances are more extreme. Some of the tumors that extend into the cerebello pontine angle have been discussed in earlier chapters.
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. Development of severe ataxia and mild pyramidal signs without mental deterioration, tetraparesis or pseudobulbar palsy during recovery from withdrawal delirium and initial hyponatraemia are unusual clinical features consistent with central pontine myelinolysis. Cpm usually happens as the result of another illness or medical condition. As will be described below, almost all patients who develop ods present with. Recovery from probable central pontine myelinolysis. Get a printable copy pdf file of the complete article 10k, or click on a page. Central pontine myelinolysis often is associated with a rapid correction of hyponatremia. 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. Atypical presentation of central pontine myelinolysis in. Diagnostic examination for central pontine myelinolysis includes imaging tests. Extrapontine myelinolysis radiology reference article. Pdf central pontine myelinolysis in liver transplantation. References to any names, marks, products, or services of third parties or hypertext links to third. Despite the pronounced fluctuations in serum osmolality, cpm is rarely seen in diabetics.
Pdf central pontine and extrapontine myelinolysis researchgate. Cpm was initially reported in alcoholic patients and was thought to be a complication of alcoholism. The ct scan and mri scan are two conventional procedures for cpm. Central pontine myelinolysis occurs frequently in alcoholism and chronic liver disease. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was suggested, and. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. Myelinolysis means that the myelin a covering that protects the pontine nerve cells is being destroyed. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers.
A 41yearold woman presented with acute parkinsonism like features while on a holiday. Pdf central pontine and extra pontine myelinosis is commonly associated rapid correction of sodium. Central pontine myelinolysis cpm, a frequently fatal disorder, is often associated with hyponatremia. Central pontine myelinolysis an overview sciencedirect. 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 thiazideinduced hyponatremia. Pdf central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the. Recovery after central pontine myelinolysis springerlink. This is a case report of cpm associated with hyperglycemia. We know of 66 different cases that have been recorded to date.
Central pontine myelinolysis cpm is a neurological disorder that affects the brain. Early diagnosis of central pontine myelinolysis with. Central pontine myelinolysis genetic and rare diseases. Only one case of cerebellar peduncular myelinolysis without the involvement of central pontine area has been reported, and here, also, the patient was an alcoholic. 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. Extrapontine myelinolysis presenting as acute parkinsonism. Central pontine myelinolysis cpm, which was originally considered to. Primary solitary intracranial melanoma is a rare tumour and its occurrence in the cerebello pontine angle is extremely uncommon.
She developed acute hypernatremia due to the stage of pregnancy and other comorbidities. The said disease affects specifically the central portion of. The original patient population included only chronic alcoholics. Central pontine myelinolysis cpm, steroidresponsive encephalopathy associated with autoimmune thyroiditis sreat, bickerstaffs brainstem encephalitis bbe are some of the disorders that might present with a localized lesion that specifically involves the pontine area 3. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. Pontocerebellar hypoplasia pch is a group of conditions affecting the brain characterized by underdevelopment of the cerebellum and pons. This case report demonstrates that if serum sodium is re lowered early in the course of osmotic demyelination syndrome ods, many of its devastating consequences may be avoided. 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. The neurologic manifestations associated with overly rapid correction have been called the osmotic demyelination syndrome ods. Central pontine myelinolysis symptoms, treatment and prognosis. This is a demyelinating disease which was first described in the year 1959. This case has picture graph representation of parameters that led to changes in serum sodium and radiological findings of central pontine myelinolysis on mri. Central pontine myelinolysis cpm usually occurs with rapid correction of severe chronic hyponatremia. Central pontine myelinolysis cpm was first termed in 1959 by adams to describe a symmetric, demyelinating focus prominent in the central pons.
The rapid correction of electrolytes and especially serum sodium in these patients is thoughtto lead first to improvement, and then, in three to ten days, to the pontine myelinolysis with associated cerebel. At admission the patient appeared restless and confused. Central pontine myelinolysis is also found in patients with chronic alcoholism, malnutrition, hyponatremia, liver disease and infections 4. Pontine refers to the stem of the brain, called the pons. Central pontine myelinolysis jama internal medicine. Conventional ct and mr imaging findings lag the clinical manifestations of cpm. Central pontine myelinolysis symptoms, treatment and. Osmotic demyelination syndrome radiology reference article.
Clinicians should maintain their vigilance while correcting electrolyte imbalances, especially with associated comorbidity. 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. Key points r j martin central pontine and extrapontine myelinolysis. Central pontine myelinolysis cpm is a clinically heterogeneous, demyelinating condition originally thought to occur only in the central pons. Mar 20, 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. Decompression of the trigeminal root and the posterior. Eight primary solitary cerebello pontine angle melanomas have been reported in the literature till 1992.
Learn what causes it and which symptoms to watch out for. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. In at least 10% of patients with central pontine myelinolysis, demyelination also occurs in extrapontine regions, including. Osmotic myelinolysis om is an acute, noninflammatory demyelinating disease that can develop following rapid correction of hyponatremia from any cause. However, it rarely occurs with the correction of hypernatremia and hyperosmolarity and involves extrapontine areas. Hameed raina 1, irfan yusuf wani 2 abstractpurpose. The clinical dilemma of proper treatment of severe hyponatremia is. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. 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. As additional cases were identified, it became apparent that lesions were not confined to the pons, but also occurred in the basal ganglia, thalamus, graywhite junction of cerebral and cerebellar cortices, lateral geniculate and at. The clinical features vary depending on the extent of involvement. Osmotic demyelination syndrome due to hyperosmolar. There may be no safe limit for the rate of rise of na. This case report underlines the importance of recognising risk factors predisposing the patient to the development of central pontine myelinolysis.
Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Diffuse leptomeningeal melanomas preferentially affect children and may be part of neuro. Cerebellar ataxia with recovery related to central pontine. Chronic alcoholism or rapid correction of hyponatremia was present in over 75% of cases. This condition, however, has also been found in the malnourished, transplant recipients, or chronically debilitated patients,in. This loop consists of the cerebellar afferent pathways and the cerebellar efferent pathways including cerebellar output fibres fig. 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. Recovery of lockedin syndrome in central pontine myelinolysis. Sep 10, 2006 extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Brain edema cerebello pontine angle tumors download brain edema cerebello pontine angle tumors ebook pdf or read online books in pdf, epub, and mobi format. Central pontine myelinolysis an overview sciencedirect topics.
Central pontine myelinolysisextrapontine myelinolysisosmotic endothelial injuryosmotic dysmyelinationhyponatraemia. Central pontine myelinolysis cpm is known to be almost universally fatal. Since the first description of the central myelinolytic lesion of the pons by adams et al in 1959, 1 and aptly named central pontine myelinolysis cpm, an increasing number of cases have been reported in the literature. Full text full text is available as a scanned copy of the original print version. Plasma exchange successfully treats central pontine. Central pontine myelinolysis definition of central pontine. Mar 23, 2020 there is no known cure for central pontine myelinolysis.
Central pontine myelinolysis, extrapontine myelinolysis, osmotic demyelination, hyponatremia, magnetic resonance imaging, adolescence. Download pdf brain edema cerebello pontine angle tumors. Physical therapy may help maintain muscle strength, mobility, and function in weakened arms and legs. Subsequently, it was found also in patients with burns, sepsis, cancer, liver disease, liver transplantation, malnutrition, fluid and electrolyte imbalance, and in other settings, including pediatric patients. Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Early diagnosis of central pontine myelinolysis with diffusionweighted imaging kimberly a. It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis. Mar 27, 2019 central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. The pathological features and theories of aetiology are briefly discussed. Xray, cat scan, or mri may reveal mass lesion in meckels cave or in pontine cistern. In patients with this condition, the myelin sheath which protects the nerves is damaged, interfering with their ability to signal to each other. This diagnosis was confirmed by magnetic resonance imaging mri in an alcoholic man. A ct brain performed on day 12 revealed mild diffuse. Central pontine myelinolysis fulltext european neurology 2009.
An mri scan is much recommended and considered as the main diagnostic tool for central pontine myelinolysis. Primary intracranial melanomas are either solitary or of diffuse variety. Osmotic myelinolysis central pontine myelinolysis and. Decompression of the trigeminal root and the posterior part of the ganglion as treatment in trigeminal neuralgia preliminary communication. References to any names, marks, products, or services of third parties or hypertext links to thirdparty sites or information are provided solely as a convenience to you and do. Central pontine myelinolysis cpm is a rare condition characterised by. The management of the hyperosmolar hyperglycaemic state. Ataxic form of central pontine myelinolysis somarajan a, paul. Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the central basis pontis. The patient presented with one month history of headache, diplopia, facial asymmetry and ataxia. Central pontine myelinolysis information page national. Apr 18, 20 central pontine myelinolysis cpm know it all duration. We describe the case of a 21yearold woman who was hospitalized at week 10 of gestation because of severe hyperemesis.
Central pontine and extrapontine myelinolysis after alcohol. Central pontine myelinolysis and mri 71 volume, issue 1, january 2014 initial ct scan did not show any low attenuated areas. Antemortem computed tomography ct had been performed in nine, and ante or postmortem magnetic resonance mr imaging in 11. 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. Location this is an irregularly shaped potential space located at the posterior cranial fossa.
The osmotic demyelination syndrome j neurol neurosurg psychiatry 2004. Introduction central pontine myelinolysis cpm is a syndrome that is characterised by rapid destruction of myelin sheaths of mainly. Immediate mri of the brain was advised and requested to be. Pdf central pontine myelinolysisa rare case report researchgate. Relief surgical intervention directed to the underlying cause. Central pontine myelinolysis in liver transplantation article pdf available in journal of clinical pathology 4411. A 56yearold chronic alcoholic had been admitted with a history of confusion.
Cerebellopontine angle tumors otolaryngologists perspective. Central pontine myelinolysis lockedin syndrome youtube. We present a case in which the diagnosis of cpm was confirmed by computed tomography ct. In rare cases, cpm can lead to lockedin syndrome, coma, or even death. 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 myelinolysis cpm know it all duration. Cerebellar peduncular myelinolysis in a patient receiving. Feb 09, 2014 there may be no safe limit for the rate of rise of na. 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. The cerebellum normally coordinates movement and the pons located in the brainstem transmits signals from.
657 688 437 885 224 738 1129 1464 474 654 1014 193 648 583 847 1111 1460 311 219 1580 703 363 1152 140 613 613 1407 824