In 1976 a link between these disorders and the rapid correction of. 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. Eight primary solitary cerebellopontine angle melanomas have been reported in the literature till 1992. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Outlook prognosis the nerve damage caused by central pontine myelinolysis is often longlasting. The syndrome entails central pontine myelinolysis cpm and. 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 was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. 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.
Diffuse large bcell lymphoma presenting with central. The patient presented with one month history of headache, diplopia, facial asymmetry and ataxia. Central pontine myelinolysis associated with diabetic. Central pontine myelinolysis cpm, a neurologic disorder caused most frequently by. Appearances were suggestive of central pontine myelinolysis. This case has picture graph representation of parameters that led to changes in serum sodium and radiological findings of central pontine myelinolysis on mri. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. Neurons of the nuclei pontis are relatively spared. It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis. There may be no safe limit for the rate of rise of na. Disease involving the cerebellopontine angle cpa may arise from structures located within the cistern or from extension of lesions located primarily outside the cisternfrom the brainstem, fourth ventricle, choroid plexus and bony skull base. The original patient population included only chronic alcoholics. We report a case of diffuse large bcell lymphoma with central pontine myelinolysis developing at the onset of disease.
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. Central pontine myelinolysisextrapontine myelinolysisosmotic endothelial injuryosmotic dysmyelinationhyponatraemia. Sep 10, 2006 extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Cerebellum hyponatremia and central pontine myelinolysis. First, let me apologize to anyone who has tried to find my site in the past few weeks. 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 genetic and rare diseases. 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. Download pdf brain edema cerebello pontine angle tumors. This condition, however, has also been found in the malnourished, transplant recipients, or chronically debilitated patients,in. There is loss of myelin and less severe loss of axons. 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. Three cases diagnosed at autopsy illustrate the characteristic clinical course and pathologic features of cpm. 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.
Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia. Cerebellar peduncular myelinolysis in a patient receiving. The clinical features vary depending on the extent of involvement. All the mechanisms of hyponatremia and hypernatremia were summarized here in our case report. However, it rarely occurs with the correction of hypernatremia and hyperosmolarity and involves extrapontine areas. Hyperintense lesions are seen on t2, and hypointense lesions on t1 weighted images. Osmotic demyelination syndrome ods refers to central pontine myelinolysis and extrapontine myelinolysis. Rosenbaum1 central pontine myelinolysis cpm was first described by adams et al. To report a rare association of central pontine myelinolysis cpm with hyperosmolar hyperglycaemic state hhs. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis. The ct scan and mri scan are two conventional procedures for cpm. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Psychiatric symptoms and limb tremors associated with central.
Ataxic form of central pontine myelinolysis somarajan a, paul. The symptoms and signs of cpa disease are nonspecific, relating either to compression of the neural structures found within it or from compression. The cerebellopontine angle is surrounded by meninges of cerebellum pontine. Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the central basis pontis. Conventional ct and mr imaging findings lag the clinical manifestations of cpm. Central and extrapontine myelinolysis 02 after alcohol. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. Central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished adams et al. Disorders associated with cpm and extrapontine myelinolysis. 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.
Central pontine myelinolysis cpm and 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. 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 in the hyperosmolar hyperglycaemic. This condition can be fatal or cause long term disability. There is no known cure for central pontine myelinolysis. Central pontine myelinolysis and medullary myelinolysis. A read is counted each time someone views a publication summary such as the title, abstract, and list of authors, clicks on a figure, or views or downloads the fulltext. Central pontine myelinolysis fulltext european neurology 2009. Recovery of lockedin syndrome in central pontine myelinolysis. A case is presented of solitary fibrous tumor occurring in the cerebello pontine angle. There is no selective involvement of fiber systems. 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. The most common cause of central pontine myelinolysis is an overly rapid correction of hyponatremia, although it can also occur in patients with any condition leading to nutritional or electrolyte stress.
Knowledge of the normal anatomy of the cerebellopontine angle and of the frequent artifacts occurring in this area is essential for correct. Cerebellopontine anglesyndrome with uncommon aetiology a. Central pontine myelinolysis occurs frequently in alcoholism and chronic liver disease. Central pontine myelinolysis an overview sciencedirect. Diffuse leptomeningeal melanomas preferentially affect children and may be part of neuro. A case of cerebello pontine angle syndrome is reported in which the cause was an atherosclerotic and tortuous basilar artery, with functional loss of the acoustic nerve and irritation of the trigeminal nerve. On rare occasions, demyelination occurs outside the pons and is termed extrapontine myelinolysis epm. A 56yearold chronic alcoholic had been admitted with a history of confusion. A rare case of primary malignant melanoma in the cerebello pontine angle, in a 17 year old girl is presented. 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.
Now, this was interesting because central pontine myelinolysis is an injury that generally impacts the pons. Central pontine myelinolysis, or cpm, is a neurological disorder that affects the brain. 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 a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Lesions can also occur outside the pons as extrapontine myelinolysis epm. Central pontine myelinolysis cpm is a disease that may present with coma, quadriplegia. Diagnostic examination for central pontine myelinolysis includes imaging tests. Cerebellar peduncular myelinolysis in a patient receiving hemodialysis article in journal of the neurological sciences 25312. To the best of our knowledge, hematological malignancies presenting. 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. Imaging of the cerebellopontine angle practical neurology.
Ataxic form of central pontine myelinolysis somarajan a. Central pontine myelinolysis cpm was described by adams et al in 1959 as a disease affecting alcoholics and the malnourished. The cerebellopontine angle is the site of the cerebellopontine angle cistern one of the subarachnoid cisterns that contains cerebrospinal fluid, arachnoid tissue, cranial nerves, and associated vessels. A case of cerebello pontine angle syndrome is reported in which the cause was an atherosclerotic and tortuous basilar artery, with functional loss. The osmotic demyelination syndrome j neurol neurosurg psychiatry 2004. A 41yearold woman presented with acute parkinsonism like features while on a holiday. In patients with this condition, the myelin sheath which protects the nerves is damaged, interfering with their ability to signal to each other. 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. One of the first articles that i found was in regards to children who have developed mutism after having cerebellar surgery. Extrapontine myelinolysis presenting as acute parkinsonism. An mri scan is much recommended and considered as the main diagnostic tool for central pontine myelinolysis. 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.
Ct anatomy of the cerebellopontine angle springerlink. Pdf extraaxial cerebellopontine angle medulloblastoma. Primary solitary intracranial melanoma is a rare tumour and its occurrence in the cerebellopontine angle is extremely uncommon. A 56yearold chronic alcoholic had been admitted with a. Central pontine myelinolysis cpm, also known as osmotic demyelination syndrome, is a rare demyelinating disorder characterized by the loss. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was suggested, and. Primary intracranial melanomas are either solitary or of diffuse variety. 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. 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. In this report, a 23yearold male patient with a two month history of. Osmotic demyelination syndrome radiology reference article.
The authors present an important clinical observation on a favorable recovery of lockedin syndrome due to central pontine myelinolysis cpm in. Feb 09, 2014 there may be no safe limit for the rate of rise of na. She developed acute hypernatremia due to the stage of pregnancy and other comorbidities. Osmotic myelinolysis central pontine myelinolysis and. The said disease affects specifically the central portion of. Central pontine and extrapontine myelinolysis after alcohol. Pdf solitary fibrous tumor of the cerebellopontine angle. Central pontine myelinolysis lockedin syndrome youtube. Early diagnosis of central pontine myelinolysis with. For more information on subscription options, click below on the option that best describes you.
Knowledge of the normal anatomy of the cerebellopontine angle and of the frequent artifacts occurring in this area is essential for correct interpretation of pathology in the cerebellopontine angle. Cpm is a degeneration of a symmetrical midline patch of the basis pontis. 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 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. Central pontine myelinolysis cpm was first termed in 1959 by adams to describe a symmetric, demyelinating focus prominent in the central pons. 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. Chronic alcoholism or rapid correction of hyponatremia was present in over 75% of cases. Cerebellopontine anglesyndrome with uncommon aetiology. Central pontine myelinolysis is a serious neurological condition caused by damage to the nerves in the area of the brainstem known as the pons. The cerebellopontine angle is the site of the cerebellopontine angle cistern one of the subarachnoid cisterns that contains cerebrospinal fluid, arachnoid tissue. Click download or read online button to brain edema cerebello pontine angle tumors book pdf for free now.
Evolution of central pontine myelinolysis on ct shelley rosenbloom,1 david buchholz,2 ashok j. Plasma exchange successfully treats central pontine. A rare case of central pontine myelinolysis in overcorrection. Central pontine myelinolysis is a rare and potentially life threatening complication of a sudden rise in serum osmolality. Central pontine myelinolysis cpm is a rare, acute, and uniformly fatal demyelinative process that involves the pons almost exclusively.
In mri scan results, damage to the pons is more visible compared with ct scan results. Central pontine myelinolysis cpm know it all duration. Central pontine myelinolysis is caused by a rapid change in the bodys sodium levels, either from treating low sodium hyponatremia or high sodium hypernatremia in a health care setting. Medulloblastoma is quite uncommon in the adult population and even rarer in extraaxial site in cerebello pontine cp angle. Clinicians should maintain their vigilance while correcting electrolyte imbalances, especially with associated comorbidity. Central pontine myelinolysis information page national. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. I faced a challenge, and i have to say it is an ongoing challenge of. Central pontine myelinolysis mayo clinic proceedings. Yuhming chang department of neurology, hsinchu mackay memorial hospital, taiwan. This is a demyelinating disease which was first described in the year 1959. Central pontine myelinolysis symptoms, treatment and prognosis. Diffuse large bcell lymphoma presenting with central pontine.
There have been only two other reported cases of a solitary fibrous tumors in this region. Physical therapy may help maintain muscle strength, mobility, and function in weakened arms and legs. Learn what causes it and which symptoms to watch out for. Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Pdf central pontine and extrapontine myelinolysis researchgate.
Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. Central pontine myelinolysis an overview sciencedirect topics. Central pontine myelinolysis symptoms, treatment and.