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1/8. Central and extrapontine myelinolysis in a patient in spite of a careful correction of hyponatremia.

    We report the case of a 54-year-old alcoholic female patient who was hospitalized for neurologic alterations along with a severe hyponatremia (plasma Na : 97 mEq/l). She suffered from potomania and was given, a few days before admission, a thiazide diuretic for hypertension. A careful correction of plasma Na levels was initiated over a 48-hour period (rate of correction < 10 mEq/l/24h) in order to avoid brain demyelination. After a 2-day period of clinical improvement, her neurologic condition started to deteriorate. By the 5th day of admission, she became tetraplegic, presented pseudobulbar palsy, ataxia, strabism, extrapyramidal stiffness and clouding of consciousness. Scintigraphic and MRI investigations demonstrated pontine and extrapontine lesions associated with Gayet-wernicke encephalopathy. After correction of ionic disorders (hyponatremia, hypokaliemia) and vitamin B (thiamine) deficiency, the patient almost completely recovered without notable disabilities. This case illustrates that profound hyponatremia, in a paradigm of slow onset, can be compatible with life. It also demonstrates that demyelinating lesions, usually considered as a consequence of a too fast correction of hyponatremia, may occur despite the strict observance of recent guidelines. There is increasing evidence to suggest that pontine swelling and dysfunction may sometimes occur in alcoholic patients even in absence of disturbance in plasma Na levels. It is therefore of importance, while managing a hyponatremic alcoholic patient, to identify additional risk factors (hypokaliemia, hypophosphoremia, seizure-induced hypoxemia, malnutrition with vitamin b deficiency) for brain demyelination and to correct them appropriately.
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2/8. A 47-year-old alcoholic man with progressive abnormal gait.

    Central pontine myelinolysis should be considered in the differential diagnosis of a patient with a history of alcoholism and malnutrition presenting with ataxia, regardless of serum sodium values. T2-weighted images are the most sensitive imaging technique, but changes may not be evident for weeks after the insult, and in addition, the insult may not be known. Supportive care is important to prevent complications, but no treatment has been found to be effective in treating the illness. Patient outcomes vary considerably and are difficult to predict.
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3/8. Osmotic myelinolysis following chronic hyponatremia corrected at an overall rate consistent with current recommendations.

    Rapid correction of chronic hyponatremia (CH) may lead to the development of osmotic myelinolysis (OM), a condition with high mortality and high incidence of devastating neurological sequelae. Treatment guidelines suggest "safe" overall rates of correction of serum sodium concentration ([Na](s)) over the first 24 and 48 hours of treatment of CH. We report a patient with CH who developed fatal OM despite overall rates of correction of [Na](s) that were within the recommended rates. The potential risk factors for the development of OM in this patient included short (within a few hours) rises in [Na](s) exceeding 0.5 mmol/l per hour and the presence of severe protein malnutrition. We suggest that the rate of correction of [Na](s) in CH should be uniformly slow and that the overall rate of correction should be slower than the currently recommended rate in severely malnourished patients.
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4/8. Central pontine myelinolysis after liver transplantation: a case report.

    A patient development deteriorating mental status, quadriparesis, and severe pseudobulbar palsy with the inability to speak or swallow following orthotopic liver transplantation (OLT). Subsequently, abnormalities were found in the pons on MRI that were consistent with central pontine myelinolysis (CPM). Marked recovery occurred following transfer to the rehabilitation medicine service. Seven months following development of CPM, a mild dysarthria has persisted, but full ambulation has returned. Although no significant fluctuations in serum sodium were seen perioperatively, multiple risk factors associated with the development of CPM were present, including end-stage liver disease, a history of alcohol abuse, malnutrition, hypoxia, and use of cyclosporin medication postoperatively. This case demonstrates that the development of CPM may occur following OLT despite meticulous attention to serum sodium concentrations. We conclude that CPM is multifactorial in nature. There can be a great variation in its clinical course.
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5/8. Central pontine myelinolysis in chronic hyponatremic patient: a case report.

    A 59-year-old woman had chronic hyponatremia from inappropriate secretion of antidiuretic hormone (SIADH) and malnutrition after recurrent cholecystitis for 2 months. She developed dysarthria, dysphagia, bilateral ptosis, clonic convulsions and delayed onset Parkinsonian features. magnetic resonance imaging showed increased signal density in the central pons on T2-weighted images. She was also later diagnosed as having systemic lupus erythematosus (SLE). This case is reported because central pontine myelinolysis (CPM) developed in chronic hyponatremia without correction, and manifested with atypical, delayed-onset Parkinsonian features. The patient recovered well from her neurological illness, unlike the poor outcome in previously reported cases of CPM. In addition, the coincidence of CPM and SLE has not, to knowledge, been reported before.
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6/8. Central pontine myelinolysis following 'slow' correction of hyponatremia.

    Two patients with central pontine myelinolysis are described for the peculiar mode of development. Both patients were in chronic renal failure and admitted in a stuporous state due to hyponatremia. Both developed central pontine myelinolysis during the hospital stay following slow and judicious correction of hyponatremia. The role of chronicity of hyponatremia prior to its correction, in the genesis of central pontine myelinolysis, particularly in the patients who have chronic debilitating illness, septicemia or malnutrition, is highlighted.
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7/8. Central pontine myelinolysis associated with acquired folate depletion.

    After long-standing malnutrition a 15-month-old boy with signs of kwashiorkor was admitted in a moribund state with serious hyponatraemic dehydration, hypothermia, somnolence, and signs of a pontine disconnection syndrome. folic acid levels were below the detection level in the presence of normal cobalamin levels. MRI of the brain showed global volume loss and signal abnormalities on the T2-weighted images suggestive for central pontine myelinolysis (CPM). Brainstem acoustic evoked responses have remained normal. The serious metabolic and nutritional derangements required substitution of folic acid, vitamins and trace elements as well as slow correction of hyponatraemic dehydration with return of the sodium level over a period of four days. This therapeutic regimen resulted in complete neurological recovery. Follow-up MRI documented normalisation of the initial pathologic findings. The hypothesis was put forward linking the pathogenesis of CPM with the combination of folate depletion and superimposed hyponatraemic dehydration. The previously acquired folate depletion could affect normal appositional function of myelin basic protein molecules due to insufficient methylation of arginine in position 107. The subsequent development of intramyelinic edema and CPM will then be triggered by the superimposed hyponatraemic dehydration. The verification of this hypothesis requires further investigations.
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8/8. Central pontine myelinolysis in AIDS.

    Central pontine myelinolysis (CPM) is an uncommon complication in sick patients with severe underlying disorders such as chronic alcoholism, malignancy, malnutrition and hyponatraemia. We report two patients with advanced hiv infection who developed CPM. In one case the diagnosis was not suspected in life, in the other the diagnosis was made just before death, on the basis of magnetic resonance (MR) imaging appearances. At post mortem there was a close correlation between the MR abnormalities and the anatomic changes in the pons.
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