Central pontine myelinolysis is a serious neurological condition caused by damage to the nerves in the area of the brainstem known as the pons. Antemortem computed tomography ct had been performed in nine, and ante or postmortem magnetic resonance mr imaging in 11. Chronic alcoholism associated cpm tends to be benign with a favorable prognosis compared to cpm secondary to rapid correction of hyponatremia. Central pontine myelinolysis cpm is a demyelinating disease of the brainstem but rarely found in other cerebral regions. Central pontine myelinolysis journal of neuropsychiatry and. Charness, in office practice of neurology second edition, 2003. Download pdf brain edema cerebello pontine angle tumors.
Ataxic form of central pontine myelinolysis somarajan a. Chronic alcoholism or rapid correction of hyponatremia was present in over 75% of cases. Rhythmic fingerwork bagpipe pdf download ciematame. Central pontine myelinolysis information page national. Central pontine myelinolysis cpm is a neurological disorder that affects the brain. Central pontine myelinolysis occurs frequently in alcoholism and chronic liver disease. 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. This is a demyelinating disease which was first described in the year 1959.
Despite its relatively ambiguous pathogenesis, it is believed that rapid correction of hyponatremia plays a pivotal role in pathogenesis of ods. However, it rarely occurs with the correction of hypernatremia and hyperosmolarity and involves extrapontine areas. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Our case differs in that the underlying cause of the myelinolysis was a complication of hemodialysis as a treatment for endstage renal failure. Early diagnosis of central pontine myelinolysis with diffusionweighted imaging. 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. Osmotic demyelination syndrome ubaidur rahaman senior resident, critical care medicine sanjay gandhi post graduate institute of medical sciences, lucknow, india 2.
Magnetic resonance imaging in central pontine myelinolysis. Cerebellar peduncular myelinolysis in a patient receiving. Central pontine myelinolysis cpm know it all duration. 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. Central pontine myelinolysis in a previously healthy 4yearold. Magnetic resonance imaging mri was performed in two patients in whom a clinical diagnosis of central pontine myelinolysis cpm had been made. This condition can be fatal or cause long term disability. Also known as osmotic demyelination syndrome ods, pm is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis. It is caused by the destruction of the layer myelin sheath covering nerve cells in the middle of the brainstem pons. Cerebellopontine angle tumors otolaryngologists perspective.
Pdf central pontine and extrapontine myelinolysis researchgate. 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. A manual of 141 cerebello pontine myelinolysis pdf download rhythmic fingerwork bagpipe pdf download energy medicine pdf free download literatura brasileira ii pdf download. The movement disorders of epm represent a treatable manifestation of the osmotic demyelination syndrome in that a rewarding symptomatic. 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.
When the myelin sheath that covers nerve cells is destroyed, signals from one nerve to another arent properly transmitted. Central pontine myelinolysis cpm is a syndrome that is characterised by rapid destruction of. Conventional ct and mr imaging findings lag the clinical manifestations of cpm. 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. Extrapontine myelinolysis presenting with extra pyramidal features suggestive of parkinsonism may be a challenging clinical syndrome. A 56yearold chronic alcoholic had been admitted with a history of confusion. Primary solitary intracranial melanoma is a rare tumour and its occurrence in the cerebellopontine angle is extremely uncommon. Central pontine myelinolysis fulltext european neurology 2009.
The clinical features vary depending on the extent of involvement. 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. Resolving mr features in osmotic myelinolysis central pontine and extra pontine myelinolysis vincent b. Of the 34 patients for whom followup data were available, 32 survived. The cerebellopontine angle cistern is a subarachnoid cistern formed by the cerebellopontine. The movement disorders of epm represent a treatable manifestation of the osmotic demyelination syndrome in that a rewarding symptomatic improvement can. Diffuse leptomeningeal melanomas preferentially affect children and may be part of neuro. A 41yearold woman presented with acute parkinsonism like features while on a holiday. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Possible causes of central pontine myelinolysis after liver transplantation. Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. Central pontine myelinolysis cpm, a neurologic disorder caused most frequently by rapid correction of hyponatre mia, is characterized by demyelination that. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Read more about symptoms, diagnosis, treatment, complications, causes and prognosis.
Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs when sodium deficiency is treated too rapidly. Cerebello pontine angle tumors otolaryngologists perspective dr t balasubramanian drtbalus otolaryngology online otolaryngology online. This condition, however, has also been found in the malnourished, transplant recipients, or chronically debilitated patients,in particular, those in whom electrolyte. In 1976 a link between these disorders and the rapid correction of sodium in hyponatraemic patients was. Diffusionweighted mr findings of central pontine and extrapontine myelinolysis. Early diagnosis of central pontine myelinolysis with. Central pontine myelinolysis cpm, a potentially fatal and debilitating neurological condition, was first described in 1959 in a study on alcoholic and malnourished patients.
Myelinolysis definition of myelinolysis by the free. Central and extrapontine myelinolysis 02 after alcohol. Electroencephalography hyponatremia and central pontine. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. Osmotic demyelination syndrome linkedin slideshare.
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 rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. It is a condition most frequently related to rapid correction of hyponatremia. Cerebellar peduncular myelinolysis in a patient receiving hemodialysis article in journal of the neurological sciences 25312.
My wife, 50, suffered central pontine myelinolysis in august 2000. Osmotic demyelination syndrome ods is brain cell dysfunction. Yuhming chang department of neurology, hsinchu mackay memorial hospital, taiwan. Central pontine myelinolysis cpm was first termed in 1959 by adams to describe a symmetric, demyelinating focus prominent in the central pons. Parkinsonism and recovery in central and extrapontine. Central pontine myelinolysis associated with diabetic. 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. Plasma exchange successfully treats central pontine. Parkinsonism and recovery in central and extrapontine myelinolysis a.
The original patient population included only chronic alcoholics. Central pontine myelinolysis an overview sciencedirect. Behavioral manifestations of central pontine myelinolysis. 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. Central pontine myelinolysis cpm was described by adams et al in 1959 as a disease affecting alcoholics and the malnourished. Through a mechanism that is only partly understood, the shift. Central pontine myelinolysis genetic and rare diseases. Cerebellar ataxia with recovery related to central pontine. 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 was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished.
Pdf central pontine myelinolysis cpm commonly presents as a complication of treatment in patients with profound life threatening hyponatraemia. Resolving mr features in osmotic myelinolysis central. Central pontine myelinolysis is a concentrated, frequently symmetric, noninflammatory demyelination within the central basis pontis. Extrapontine myelinolysis presenting as acute parkinsonism. Instances of related myelinolysis in the cerebral white matter have led to the term extrapontine myelinolysis 3. Mri showed lesions in the pons in both cases about 2 years after the illness, at a time when the spastic quadriparesis and pseudobulbar palsy had recovered. Imaging of the cerebellopontine angle practical neurology. Recommendations for treatment of symptomatic hyponatremia.
The findings in 44 patients 42 of whom were chronic alcoholics with central pontine myelinolysis show that the outcome does not depend on the severity of neurological deficits during the acute phase of the condition or on concomitant internal diseases, including the degree of hyponatremia. In summary, poisoned children present with a wide variety of symptoms and inaccurate histories, which makes diagnosis difficult. Clinicians should maintain their vigilance while correcting electrolyte imbalances, especially with associated comorbidity. This diagnosis was confirmed by magnetic resonance imaging mri in an alcoholic man. Primary intracranial melanomas are either solitary or of diffuse variety. 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. The said disease affects specifically the central portion of. Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia. Central pontine myelinolysis, extrapontine myelinolysis, osmotic demyelination, hyponatremia, magnetic resonance imaging, adolescence. Damned if we do, damned if we dont oh ms, kim hi, carrol hj. Symptoms include lessened awareness and difficulty speaking and swallowing. Click download or read online button to brain edema cerebello pontine angle tumors book pdf for free now.
It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis. The cerebellopontine angle syndrome is a distinct neurological syndrome of deficits that can arise due to the closeness of the cerebellopontine angle to specific cranial nerves. Indications include unilateral hearing loss 85%, speech impediments, disequilibrium, tremors or other loss of motor control. Pontine refers to the stem of the brain, called the pons. Albeit mild, the righthanded clumsiness, the dysarthria, and the rate of clinical resolution are consistent with this diagnosis.
This group of tumors account for nearly 10% of all intracranial tumors. 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. Central pontine myelinolysis cpm was described in 1959 by adams et al. Osmotic demyelination syndrome ods primarily occurs after rapid correction of severe hyponatremia. This concept was extended in 1962 with the recognition that lesions can occur outside the pons, socalled extrapontine myelinolysis epm. 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. Outcome of central pontine and extrapontine myelinolysis.664 1080 301 849 537 201 660 1210 1609 1105 1522 396 635 1406 1076 815 1310 37 581 1020 1116 44 1279 9 128 984 758 93 483 732 782 1306 349 593 44 1003 1480