As such the condition is described in the osmotic demyelination syndrome article. The situation is usually seen in the setting of rapid or prolonged overcorrection of the hyponatraemia. The widespread use of ct scan and mri has made diagnosis easier and. 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. Osmotic demyelination syndrome in children pediatric. The part of the brain called the pons is especially sensitive and too much sodium can damage nerve fibers. Central pontine myelinolysis cpm was first described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. It is characterized by spastic quadriplegia, pseudobulbar palsy and encephalopathy. Brain magnetic resonance imaging mri was prescribed one week after the onset of symptoms. The t2weighted images showed highintensity abnormalities in the pons, bilateral lenticular nuclei, and head of caudate nuclei see figure 1. 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. 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. Central pontine myelinolysis or osmotic demyelination syndrome is a disorder consisting of noninflammatory demyelination, involving the pons.
In both patients, affected white matter showed hyperintensity on diffusionweighted images associated with a decrease in apparent diffusion coefficient adc values. 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 and extrapontine myelinolysis 02 after alcohol. Pontine myelinolysis lesions are usually symmetrical and bilateral 2.
The contribution of measured organic osmolytes and electrolytes to the total brain osmolality change was 23 and 72%, respectively. It is the more recent term replacing central pontine myelinolysis, recognizing that extrapontine structures can also be affected, previously known as extrapontine myelinolysis. Traumatic brain injury hyponatremia and central pontine. However, it is believed that there could be a significantly higher number of people. Diagnostic examination for central pontine myelinolysis includes imaging tests. Outcome of central pontine and extrapontine myelinolysis. This is the first case report of combined central pontine and extrapontine demyelination after alcohol withdrawal and correction of hypernatremia in a chronic alcoholic. An mri scan is much recommended and considered as the main diagnostic tool for central pontine myelinolysis.
Osmotic myelinolysis is an acute, rare, demyelinating process. Extrapontine myelinolysis hyponatremia and central. In this disease, myelin and oligodendrocytes are damaged, while the neurons and axons are relatively spared. The osmotic demyelination syndrome j neurol neurosurg psychiatry 2004. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after too rapid medical correction of sodium deficiency hyponatremia. 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.
Also known as osmotic demyelination syndrome ods, pm is subdivided into central pontine myelinolysis cpm and extrapontine myelinolysis epm 2. Central pontine myelinolysis cpm was first described in 1959 by adams et al. Behavioral manifestations of central pontine myelinolysis. Central pontine and extrapontine myelinolysis in children. The lesions occur symmetrically in the thalamus, pons, striatum, cerebral cortex, cerebellum and other areas. This study aimed to identify the causes and contributing factors, neurologic presentation, and outcomes of central pontine and extrapontine myelinolysis and to examine any trends in the presentation and course of these disorders over the past 50 years. Central pontine myelinolysis, osmotic demyelination. Central pontine and extrapontine myelinolysis in a patient. Postpartum hypernatremic cerebral encephalopathy with osmotic myelinolysis. Central pontine myelinolysis in a undernourished boy with acute gastroenteritis. Among all 106 cases, 49 presented with isolated central pontine myelinolysis. Central pontine myelinolysis cpm is a neurological disorder that most frequently occurs after overrapid medical correction of sodium deficiency hyponatremia. Osmotic demyelination syndrome refers to acute demyelination seen in the setting of osmotic changes, typically with the rapid correction of hyponatremia.
Sequential mri in pontine and extrapontine myelinolysis. Central pontine myelinolysis information page national. Prognosis is not uniformly bad mri changes may be delayed mri severity is not prognostic r j martin central pontine and extrapontine myelinolysis. However, in 1962, it was found that similar lesions can occur outside the pons, the socalled extrapontine myelinolysis epm. Pontine stroke is a type of stroke that happens when the blood flow in the brain stem is disrupted. With corticobulbar pathway involvement, patients are affected by dysarthria and dysphagia 3. Central pontine myelinolysis pathogenesis and pathophysiology cpm was initially described by adams and colleagues8 in 1959 as a disease affecting alcoholic andor malnourished persons. What is pontine strokecausessymptomstreatmentrecovery. Central pontine myelinolysis as a complication of the disease due to burns. Pdf central pontine and extrapontine myelinolysis owing.
Initial signs and symptoms appear within two to three days and include a depressed level of awareness, difficulty speaking, and difficulty swallowing. Pontine and extrapontine myelinolysis1, brain, volume 102, issue 2, 1 june 1979, pages 3685, we use cookies to enhance your experience on our website. Osmotic demyelination syndrome radiology reference. Central pontine myelinosis and osmotic demyelination syndrome. Central pontine myelinolysis genetic and rare diseases. Hyponatremia can be treated using hypertonic saline solution. 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 and extrapontine myelinolysis owing to disequilibrium syndrome. Postpartum hypernatremic cerebral encephalopathy with. Central pontine and extrapontine myelinolysis were impressed. It is characterized by demyelinization of the white matter. Through a mechanism that is only partly understood, the shift in water and brain molecules leads to the. Pontine and extrapontine myelinolysis can occur after the rapid correction of hyponatremia. Mielinolisis central pontina en trasplante hepatico medicina intensiva.
Several options surround the prevention and treatment of pm. Pdf central pontine myelinolysis is a demyelinating lesion in the basis pontis which in most cases is caused by a rapid correction of hyponatremia. Behavioral manifestations of central pontine myelinolysis bruce h. It is predominately iatrogenic treatmentinduced, and is characterized by acute paralysis, dysphagia difficulty swallowing, dysarthria difficulty speaking, and other neurological symptoms. Central pontine myelinolysis is a demyelinating condition primarily seen in chronic alcohol abusers who become hyponatraemic. A central nervous system disorder caused by demyelination within the central basis pontis of the brain. Demyelination can also affect other parts of the brain, in extrapontine myelinolysis epm. The rapid rise in sodium concentration is accompanied by the movement of small molecules and pulls water from brain cells. Pdf central pontine myelinolysis cpm was described by adams and colleagues in 1959 as a disease affecting alcoholics and the malnourished. Myelinolysis definition of myelinolysis by medical. Marsel mesulam, md \sb\a young woman with a clinical history and magnetic resonance imaging scan consistent with central pontine myelinolysis came to medical attention because of prominent behavioral symptoms. A case of osmotic demyelination syndrome occurred after. Hippocampus, putamen, cerebral cortex, thalamus, cerebellum, lateral geniculate body, ex ternal capsule lesions can also be included 6. Ods was formerly called central pontine myelinolysis, but the name was changed for several reasons.
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. Jci study of brain electrolytes and organic osmolytes. 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. The ct scan and mri scan are two conventional procedures for cpm. There may be no safe limit for the rate of rise of na. Introduction pontine myelinolysis pm was first described in 1959 by adams et al. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved.
Osmotic demyelination syndrome is an acute demyelination process that usually occurs several days following an osmotic stress. With rapid correction by 5% nacl infusion, significant brain dehydration and elevation of brain na and cl levels above the normal range occurred at 24 h. Two patients with central pontine myelinolysis cpm were studied with diffusionweighted mr imaging 1 week after onset of tetraplegia. Decreased diffusion in central pontine myelinolysis. The brain stem is responsible for breathing, heart function, digestion and alertness.
Mielinolisis extrapontina secundaria a hiponatremia por crisis. In mri scan results, damage to the pons is more visible compared with ct scan results. Central pontine myelinolysis an overview sciencedirect. Central pontine myelinolysis in a patient admitted for. Of the 34 patients for whom followup data were available, 32 survived. Conventional ct and mr imaging findings lag the clinical manifestations of cpm. Central pontine myelinolysis or osmolar demielinization syn drome is a very. The clinical features vary depending on the extent of involvement. Know the causes, symptoms, treatment, recovery period, survival rate and prognosis of pontine. To differentiate, a person with a traumatic brain injury usually needs to be hospitalized for their injury, and in regards to a mild traumatic brain injury, a person sustains ongoing issues after receiving a hit to the head, but did not require hospitalization. Mri of different brain regions for pontine myelinolysis.
Pontine and extrapontine myelinolysis1 brain oxford. In one patient studied serially, adc values normalized by 3 weeks after. We describe the case of a 21yearold woman who was hospitalized at week 10 of gestation because of severe hyperemesis. Learn what causes it and which symptoms to watch out for. It is observed in patients with severe hyponatremia, particularly when the hyponatremia is corrected too rapidly. Central pontine myelinolysis cpm occurs in the setting of rapidly corrected hyponatremia, especially in chronically debilitated patients. Central pontine myelinolysis has been well known for more than 40 years and mainly occurs in alcoholics. Central pontine myelinolysis cpm is now more commonly referred to as osmotic demyelination syndrome, which recognizes that the same phenomenon is also seen in other areas of the brain previously known as extrapontine myelinolysis.
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