Neuroimaging Spectrum of Severe Hypernatremia in Infants with Neurological Manifestations.

BACKGROUND: Variable neurological manifestations and imaging findings have been described in children with severe hypernatremia. We aimed to describe the spectrum of neuroimaging changes in infants with severe hypernatremia. METHODS: This retrospective study included infants with severe hypernatremia (serum sodium >160 mEq/L), abnormal neurological examination, and an abnormal magnetic resonance imaging (MRI) of the brain over a period of 2 years in a tertiary care hospital. Relevant clinical data, including the feeding practices, clinical features, complications, and biochemical and radiological parameters, were entered in a structured pro forma. MRI findings were classified as vascular (hemorrhages and cerebral sinus venous thrombosis), osmotic demyelination syndrome (pontine and extrapontine myelinolyses), and white matter changes. RESULTS: The common clinical features in the neonates were poor feeding (n = 4) and decreased urine output (n = 4); the older infants presented with gastrointestinal losses (n = 5). All cases had dehydration with encephalopathy. The patterns of radiological injury were vascular (hemorrhages, n = 5 and venous thrombosis, n = 3), osmotic demyelination (n = 8), and white matter changes (n = 7). Coagulopathy was correlated with the vascular complications (r = 0.8, p < 0.0001); the degree of dehydration was correlated with the venous thrombosis (r = 0.7, p < 0.04) and acute kidney injury (r = 0.8, p < 0.001). Neurological sequelae were seen in four cases and correlated with hypernatremia (r = 0.6, p = 0.03) and hyperosmolarity (r = 0.6, p = 0.03). CONCLUSION: Characteristic neuroimaging findings are vascular changes in the form of venous thrombosis and hemorrhages, osmotic demyelination and white matter tract injury, and/or mostly combinations of these findings. Severe hypernatremia and resulting hyperosmolarity frequently cause neurological sequelae in neonates and infants.

An Unusual Cause of Bulbar Palsy in the Emergency Department.

BACKGROUND: Osmotic demyelination syndrome commonly occurs after rapid correction of hyponatremia. But it has also been reported after graded correction of hyponatremia in the presence of other risk factors like chronic alcoholism, malnutrition, liver disease, and hypokalemia. CASE REPORT: We report a case of a 67-year-old man who presented with dysphagia and nasal regurgitation and had features suggestive of bulbar palsy on neurological examination. He had spontaneous rapid correction of hyponatremia from a serum sodium level of 122 mEq/L to 132 mEq/L after discharge from our hospital. Neuroimaging was suggestive of extrapontine myelinolysis involving the basal ganglia. WHY SHOULD AN EMERGENCY PHYSICIAN BE AWARE OF THIS?: An emergency physician should be aware of this because osmotic demyelination syndrome should also be considered in the differential diagnosis of patients presenting with bulbar palsy to the emergency department.

Central pontine myelinolysis during pregnancy: Pathogenesis, diagnosis and management.

Central pontine myelinolysis (CPM) is a rare condition usually caused by rapid sodium correction in hyponatraemia after a severe neurological syndrome. Only few cases have been reported during pregnancy, most of which were reported in patients with hyperemesis. We describe the successful management of the first case of twin pregnancy in a patient who presented with CPM after treatment for premature labour and then review the literature on CPM in pregnancy (aetiology, diagnosis and management). Our patient required emergency delivery to achieve electrolyte and fluid balance. At six months, the twins remained asymptomatic and the mother had minor sequelae. The aetiology is not clear, and there is no evidence regarding the optimal treatment or prognosis of CPM. In our patient, desmopressin-contaminated atosiban showed a certain probability in the Karch-Lasagne algorithm of a causality relationship between hyponatraemia and the drug. To our knowledge, this is the first case of myelinolysis reported in a twin pregnancy possibly related to desmopressin-contaminated atosiban.

Extrapontine myelinolysis associated with severe hypernatremia in infancy.

Extrapontine myelinolysis (EPM) is an uncommon disorder in children, with few pediatric cases reported to date. We report the first case of an infant with EPM without central pontine myelinolysis (CPM) presenting with severe hypernatremia. On admission, the infant had impaired consciousness, mild dehydration, and severe hypernatremia (190 mmol/L). The following day, the patient developed abnormal involuntary movements. Brain magnetic resonance imaging (MRI) confirmed EPM without CPM. He recovered without sequelae, and clinical examinations were within normal limits approximately 6 months after discharge. Brain MRI at 1 year after onset showed complete disappearance of the previous EPM regions. To the best of our knowledge, this represents the youngest patient with EPM without CPM presenting with severe hypernatremia. Given that treatment for osmotic demyelination syndrome (ODS) is yet to be established, preventing the development of ODS is crucial.

Extrapontine myelinolysis manifested selectively by acute severe parkinsonian syndrome. Case Report.

OBJECTIVES: Osmotic demyelination syndrome (ODMS) is a rare and serious neurologic disorder with acute myelin disintegration, usually in the pontine area (central pontine myelinolysis) and to a lesser extent, even in other areas of the central nervous system (extrapontine myelinolysis). The main underlying mechanism is the change of serum osmolality with quick correction of low mineral levels, mainly hyponatraemia. Clinical manifestation is various and depends on the localization. DESIGN: We describe an acute isolated extrapontine myelinolysis causing acute onset of parkinsonism in a 61-year-old man who developed quickly progressing parkinsonian syndrome after the rapid correction of hyponatraemia. RESULTS: Brain MRI revealed lesions only in the striatum, sparing the globus pallidus. Substitution therapy with high doses of levodopa significantly improved his clinical condition. CONCLUSION: Extrapontine myelinolysis with isolated affection of basal ganglia is extremely rare. In such case, clinical manifestation of acute severe parkinsonism could be successfully treated by high dose of levodopa.

Urinary incontinence a first presentation of central pontine myelinolysis: a case report.

An 84-year-old lady was treated for hyperosmolar hyperglycaemia with IV insulin, fluids and catheterisation for fluid balance monitoring. Trial without catheter failed as the patient complained of new-onset urinary incontinence and lack of awareness of bladder filling. In light of her breast cancer history, we excluded cauda equina. Ultrasound KUB showed an enlarged bladder. Whole-body MRI revealed a lesion in the pons which was highly suggestive of central pontine myelinolysis (CPM). Her electrolytes were normal throughout her admission; thus, the rapid fluctuation in osmolality, secondary to her hyperglycaemic state, was the likely cause of CPM. CPM has been reported secondary to hyperglycaemia; however, this is the first reported case of CPM presenting as urinary incontinence and loss of bladder sensation.

Central pontine and extrapontine myelinolysis: a systematic review.

The purpose was to perform a systematic review of studies on central pontine and extrapontine myelinolysis [forms of osmotic demyelination syndrome (ODS)] and define the spectrum of causes, risk factors, clinical and radiological presentations, and functional outcomes of this disorder. A thorough search of the literature was conducted using multiple databases (PubMed, Ovid Medline and Google) and bibliographies of key articles to identify all case series of adult patients with ODS published from 1959 to January 2013. Only series with five or more cases published in English were considered. Of the 2602 articles identified, 38 case series were included comprising a total of 541 patients who fulfilled our inclusion criteria. The most common predisposing factor was hyponatremia (78%) and the most common presentation was encephalopathy (39%). Favorable recovery occurred in 51.9% of patients and death in 24.8%. Liver transplant patients with ODS had a combined rate of death and disability of 77.4%, compared with 44.7% in those without liver transplantation (P < 0.001). ODS is found to have a good recovery in more than half of cases and its mortality has decreased with each passing decade. Favorable prognosis is possible in patients of ODS, even with severe neurological presentation. Further research is required to confirm the differences found in liver transplant recipients.

Central pontine myelinolysis following pediatric living donor liver transplantation: a case report and review of literature.

CPM is one of the most serious neurological complications that can occur after OLT and is characterized by symmetrical demyelinization in the basis pontis. The etiology of CPM remains unclear, although the rapid correction of the serum sodium and CNI concentrations may be associated with the development of CPM. With recent advances in MRI technology, early diagnosis of CPM has become possible. Here, we present the case of a five-yr-old female who developed CNI-associated CPM after undergoing LDLT. A decreased level of consciousness and dysphasia was noted one wk after LDLT, and MRI revealed findings compatible with a diagnosis of CPM. The patient fully recovered from the neurological deficits related to CPM following the switch from the CNI to sirolimus. We propose MRI to be promptly considered for patients with abnormal neurological findings, together with the substitution of CNI with an mTOR inhibitor as a management regimen for CNI-related CPM.

Plasma exchange successfully treats central pontine myelinolysis after acute hypernatremia from intravenous sodium bicarbonate therapy.

BACKGROUND: Osmotic demyelination syndrome (ODS) primarily occurs after rapid correction of severe hyponatremia. 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. CASE PRESENTATION: A 40-year-old woman presented with general weakness, hypokalemia, and metabolic acidosis. The patient was treated with oral and intravenous potassium chloride, along with intravenous sodium bicarbonate. Although her bicarbonate deficit was 365 mEq, we treated her with an overdose of intravenous sodium bicarbonate, 480 mEq for 24 hours, due to the severity of her acidemia and her altered mental status. The next day, she developed hypernatremia with serum sodium levels rising from 142.8 mEq/L to 172.8 mEq/L. Six days after developing hypernatremia, she exhibited tetraparesis, drooling, difficulty swallowing, and dysarthria, and a brain MRI revealed high signal intensity in the central pons with sparing of the peripheral portion, suggesting CPM. We diagnosed her with CPM associated with the rapid development of hypernatremia after intravenous sodium bicarbonate therapy and treated her with plasma exchange. After two consecutive plasma exchange sessions, her neurologic symptoms were markedly improved except for mild diplopia. After the plasma exchange sessions, we examined the patient to determine the reason for her symptoms upon presentation to the hospital. She had normal anion gap metabolic acidosis, low blood bicarbonate levels, a urine pH of 6.5, and a calyceal stone in her left kidney. We performed a sodium bicarbonate loading test and diagnosed distal renal tubular acidosis (RTA). We also found that she had Sjögren's syndrome after a positive screen for anti-Lo, anti-Ra, and after the results of Schirmer's test and a lower lip biopsy. She was discharged and treated as an outpatient with oral sodium bicarbonate and potassium chloride. CONCLUSION: This case indicates that serum sodium concentrations should be carefully monitored in patients with distal RTA receiving intravenous sodium bicarbonate therapy. We should keep in mind that acute hypernatremia and CPM can be associated with intravenous sodium bicarbonate therapy, and that CPM due to acute hypernatremia may be effectively treated with plasma exchange.

Extrapontine Myelinolysis in a patient with Primary Adrenal Insufficiency.

PURPOSE: To report the development of estrapontine myelinolysis (EPM) in a patient with adrenal insufficiency and review similar in the literature. CASE REPORT: A 49-year-old female with insufficiency presented with acute dysarthria, stuttering, and parkinsonism. She received isotonic saline hydration for adrenal crisis and hyponatremia 18 days before the onset of symtoms. The brain MRI and MRS showed demyelination at bilateral basal ganglia and the thalamus, which was compatible with EPM and resolved within 3 months after steroid treatment. CONCLUSION: Development of acute parkinsonism after rapid correction of hyponatremia may indicate the occurrence of EPM and underlying adrenal insufficiency should be excluded in these patients.

Osmotic demyelination syndrome.

Formerly known as central pontine myelinolysis, osmotic demyelination syndrome (ODS) is defined by a symmetrical destruction of myelin sheaths involving mainly the central portion of the basis pontis without evidence of vascular involvement. We report the case of a 60-year-old man who presented to the emergency department with a 2-week history of progressive confusion, memory loss, and lower extremity weakness with limited ambulation. A computed tomography scan of the head revealed areas of low attenuation within the pons, and brain magnetic resonance imaging (MRI) confirmed the changes as compatible with ODS.

[A case of central pontine and extrapontine myelinolysis, without hyponatremia, during alcohol withdrawal with favorable outcome]. / Un cas clinique de myélinolyse centropontine et extrapontine, sans hyponatrémie, au décours d'un sevrage alcoolique et d'évolution favorable.

Central pontine and extra-pontine myelinolysis (CPM/EPM) is a rare neurological disorder, well documented for more than 50 years but whose pathogenesis remains obscure. The existence of predisposing factors occurs in the most cases; chronic alcohol abuse is one of the most commonly encountered, among many others. Alcohol withdrawal represents an additional vulnerability factor, being responsible for electrolyte imbalances which are not always demonstrable but are certainly involved in the development of CPM and/or EPM. CPM/EPM may be responsible for severe morbidity and is potentially life-threatening. The diagnosis of CPM/ EPM remains mostly clinical and is confirmed by magnetic resonance imaging of the brain. It should be considered in the setting of any unexplained neurological symptoms during the course of alcohol withdrawal or for any patient with chronic alcohol abuse, as promptly as possible, given the potentially fatal outcome.

Central pontine myelinolysis in the hyperosmolar hyperglycaemic state.

OBJECTIVE: To report a rare association of central pontine myelinolysis (CPM) with hyperosmolar hyperglycaemic state (HHS). CLINICAL PRESENTATION AND INTERVENTION: A diabetic female presented with HHS and prolonged severe hypernatraemia. The metabolic derangement was adequately treated with proper correction of both hyperglycaemia and hypernatraemia. Lack of improvement in the presenting confusional state and the development of a fresh neurological deterioration led to the suspicion of CPM that was confirmed with magnetic resonance imaging. She fully recovered after 4 weeks with no specific medical treatment. CONCLUSION: This case report showed that osmotic demyelination was linked to hypernatraemia and that CPM could result from severe hypernatraemia of HHS.

A woman with focal neurological deficit following treatment for cholera.

A 41-year old woman was treated for cholera at one of the health centers in Blantyre. Two days after discharge from the treatment unit, she developed weakness of all 4 limbs and difficulties with speech. She was referred to the Queen Elizabeth Central Hospital. A CT scan of the brain showed hypodense lesions in the pons. A diagnosis of central pontine myelinolysis was made. She recovered slowly and was discharged from hospital 17 days after admission.

[Reversible central pontine myelinolysis without hyponatremia in a child with acute lymphoblastic leukemia: a case report]. / Myélinolyse centropontine réversible sans hyponatrémie chez un enfant atteint de leucémie aigüe lymphoblastique: à propos d'un cas.

Central pontine myelinolysis is a demyelinating disorder mainly affecting the central pons. In some cases, it is associated with extrapontine myelinolysis. It is usually caused by rapid correction of hyponatremia and osmotic shock. We here report the case of a 3.5-year-old girl diagnosed with acute lymphoblastic leukemia admitted to our Oncology Unit with neutropenic fever and diarrhea. Laboratory tests showed mild neutropenia, normochromic normocytic anemia. Electrolyte tests were normal without hyponatremia. She received antibiotic therapy with Metronidazole. Five days later, she developed flaccid quadriparesis with mutism. Computerized tomography (CT) scan was normal, cerebrospinal fluid (CSF) examination was normal (there was no evidence of leukemic cells) and ophthalmological examination did not show any abnormalities. Brain MRI found hyperintense signal in the pons. The child improved without specific treatment, and clinical and complete neurological recovery was noted. This case highlights that myelinolysis can occur under some circumstances not related with hyponatremia such as malignancy, chemotherapy.

Long-term outcome of patients hospitalized in intensive care units with central or extrapontine myelinolysis*.

OBJECTIVE: Very few data are available for critically ill patients with central or extrapontine myelinolysis and according to available evidence, the prognosis seems to be poor. We aimed to describe the baseline characteristics, the management, the long-term prognosis, and the prognostic factors in central or extrapontine myelinolysis. DESIGN: Retrospective observational study considering modified Rankin Scale score >3 or death as an unfavorable outcome. SETTING: Forty-six French intensive care units. PATIENTS: Thirty-six patients with central or extrapontine myelinolysis treated in 2000-2010. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: At baseline, 31 (86%) patients were alcoholics and 33 (92%) presented with hyponatremia. Mechanical ventilation was required in 32 (89%) patients. At 1-yr follow-up, 11 (31%) patients have died, whereas 14 (56%) survivors have returned to a Rankin score ≤ 1. Life-supporting therapies were withheld in 11 (31%) patients. Severe cerebral motor disability was the most frequently cited reason. However, five of them were still alive at 1 yr with Rankin score ≤ 1 for four of them. We found no statistical difference between the 18 (50%) patients with a favorable outcome and the 18 (50%) patients with an unfavorable outcome with regard to severity of illness, suggesting that recovery is possible and unpredictable on the basis of clinical presentation. Chronic alcoholism was less frequent in patients with a favorable outcome as compared with patient with an unfavorable outcome (13 [72%] vs. 18 [100%], p = .04). CONCLUSIONS: The prognosis of critically ill patients with central or extrapontine myelinolysis is better than thus far thought despite initial severe clinical manifestations. Regarding the high rate of decisions to withhold life-supporting therapies, the probability of a favorable outcome might be underestimated by intensivists.

Central pontine myelinolysis: a case report and clinical-pathological review.

An 11-yr-old child presented with acute mental status changes and spastic quadriplegia after orthotopic liver transplantation. Magnetic resonance (MR) imaging findings were consistent with central pontine and EPM. Initial immunosuppression included tacrolimus, mycophenolate mofetil, and corticosteroids. Given that neurotoxicity is a well-established side effect of CNI, the patient was converted to rapamycin and subsequently experienced significant neurologic recovery. The temporal resolution of the patient's symptoms suggests that prompt recognition of central pontine and EPM and conversion from tacrolimus to rapamycin during the early post-operative course may have therapeutic benefits for patients undergoing pediatric transplant with CNI-related neurotoxicity.

The magnetic resonance spectroscopy findings of extrapontine myelinolysis in a patient with acute lymphoblastic leukemia.

The magnetic resonance (MR) spectroscopy findings of extrapontine myelinolysis have been rarely reported. Herein, we present MR spectroscopy findings as well as the conventional MR and diffusion MR findings of an acute lymphoblastic leukemia patient with extrapontine myelinolysis. Advanced MR imaging including diffusion-weighted imaging and MR spectroscopy may be helpful to exclude other pathologies in the differential diagnosis and make the diagnosis when there is a diagnostic difficulty on cases clinically suspicious for extrapontine myelinolysis.