MR demonstration of mesencephalic lesions in osmotic demyelination syndrome (central pontine myelinolysis)

1987 ◽  
Vol 29 (6) ◽  
pp. 582-584 ◽  
Author(s):  
Elizabeth Gerard ◽  
M. E. Healy ◽  
J. R. Hesselink
Keyword(s):  
Central Pontine Myelinolysis ◽  
Osmotic Demyelination Syndrome ◽  
Osmotic Demyelination ◽  
Pontine Myelinolysis ◽  
Central Pontine

scholarly journals Osmotic Demyelination Syndrome after Primary Hypoadrenocorticism Crisis Management

2021 ◽  
Vol 49 ◽  
Author(s):  
Álan Gomes Pöppl ◽  
Érico Haas Pires ◽  
Claudia Ruga Barbieri ◽  
Lucas Marques Colomé
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Central Pontine Myelinolysis ◽  
Osmotic Demyelination Syndrome ◽  
Resonance Imaging ◽  
Present Case Report ◽  
Osmotic Demyelination ◽  
Pontine Myelinolysis ◽  
Central Pontine ◽  
Rapid Correction

Background: Primary hypoadrenocorticism is a rare condition resulting from immune-mediated destruction of the adrenal cortices. It can also occur due to necrosis, neoplasms, infarctions and granulomas. The clinical and laboratory changes are due to deficient secretion of glucocorticoids and mineralocorticoids, which leads to electrolyte disorders associated with hyponatremia and hyperkalemia. These disorders can cause hypotension, hypovolemia and shock, putting a patient's life at risk if inadequate hydroelectrolytic supplementation and hormone replacement is provided. Nevertheless, rapid sodium chloride supplementation is contraindicated due to the risk of central pontine myelinolysis induction. The present study aims to describe a thalamic osmotic demyelination syndrome after management of a primary hypoadrenocorticism crisis in a 2-year-old, female West White Highland Terrier. Case: The patient had a presumptive diagnosis of hypoadrenocorticism already receiving oral prednisolone and gastrointestinal protectants in the last 2 days. After prednisolone dose reduction the dog presented a severe primary hypoadrenocorticism crisis treated with intravenous sodium chloride 0.9% solution along with supportive therapy. Four days after being discharged from the hospital, the patient showed severe neurological impairment and went back to the clinic where a neurological examination revealed mental depression, drowsiness, ambulatory tetraparesis and proprioceptive deficit of the 4 limbs, postural deficits, and cranial nerves with decreased response. Due to these clinical signs, a magnetic resonance imaging was performed. It showed 2 intra-axial circular lesions, symmetrically distributed in both thalamus sides, with approximately 0.8 cm in diameter each without any other anatomical changes on magnetic resonance imaging. The images were compatible with metabolic lesions, suggesting demyelination. Furthermore, liquor analysis did not show relevant abnormalities, except for a slight increase in density and pH at the upper limit of the reference range. After treatment, the patient had a good neurological evolution secondary to standard primary hypoadrenocorticism treatment, without sequelae. Discussion: In the present case report, primary hypoadrenocorticism gastrointestinal signs seemed to be triggered by a food indiscretion episode, not responsive to the symptomatic therapies employed. The patient´s breed and age (young West White Highland Terrier bitch) is in accordance with the demographic profile of patients affected by the disease, where young females are frequently more affected. Regarding the probable thalamic osmotic demyelination syndrome documented in this case, is important to notice that myelinolysis or demyelination is an exceedingly rare noninflammatory neurological disorder, initially called central pontine myelinolysis, which can occur after rapid correction of hyponatremia. It has already been observed in dogs after correction of hyponatremia of different origins, including hypoadrenocorticism and parasitic gastrointestinal disorders. Currently, the terms "osmotic myelinolysis" or “osmotic demyelination syndrome" are considered more suitable when compared to the term "central pontine myelinolysis" since it has been demonstrated in dogs and humans the occurrence of demyelination secondary to the rapid correction of hyponatremia in distinct regions of the central nervous system including pons, basal nuclei, striatum, thalamus, cortex, hippocampus and cerebellum. The present case report emphasizes the difficulties for hormonal confirmation of primary hypoadrenocorticism in a patient already on corticosteroid treatment, as well as proposes that the current term osmotic demyelination syndrome replace the term “central pontine myelinolysis” in veterinary literature related to the management of hypoadrenocorticism crisis.Keywords: Addison Syndrome, hyponatremia, osmotic myelinolysis, magnetic resonance imaging.

scholarly journals Hyponatremia, osmotic demyelination syndrome, and the importance of the patient’s history

2021 ◽  
Vol 9 (37) ◽  
pp. 45-53
Author(s):  
Dominique Gagnon
Keyword(s):  
Demyelinating Disease ◽  
Central Pontine Myelinolysis ◽  
Cell Volume Regulation ◽  
Brain Cell ◽  
Osmotic Demyelination Syndrome ◽  
Severe Hyponatremia ◽  
Hypertonic Saline Solution ◽  
Osmotic Demyelination ◽  
Pontine Myelinolysis ◽  
Electrolyte Abnormalities

Central pontine myelinolysis (CPM), first described in 1959, is a symmetrical non-inflammatory demyelinating disease with loss of oligodendrocytes that occurs most often following a rapid correction of severe hyponatremia (i.e., <120 mmol/L). It presents as a biphasic disease with initial seizure or encephalopathy, followed by clinical improvement and subsequent rapid deterioration with bulbar dysfunction, oculomotor dysfunction, various degree of paresis, and even locked in syndrome. Its occurrence is rare (≈0.6% of severe hyponatremia), it is diagnosed clinically and confirmed with brain imaging, ideally with magnetic resonance image, and it is reversible in approximately half the patients. Lesions are classically identified in the pons but extra pontine lesions (in basal ganglia, cerebellar white matter, thalamus, and hippocampus) have also been identified. The most commonly accepted molecular mechanism involves brain cell volume regulation with a rapid shift of osmole following brain edema which establishes during the chronic hyponatremic phase. For these reasons, osmotic demyelination syndrome (ODS) is a better term. The most identified risk factor is severe hyponatremia, but other electrolyte abnormalities can contribute, in particular, if the patient is an alcoholic or malnourished. This diagnosis should also be suspected in post-op patients with nausea and headache non-responsive to antiemetic and analgesic drugs. An essential step is an appropriate medical history, a list of medications, physical examination, and basic initial lab tests with the goal of identifying possible easily reversible causes of hyponatremia. Correction of severe hyponatremia with neurological symptoms should be done using rapid boluses of hypertonic saline solution in rapid succession with goals of increasing serum sodium by 5-6 mEq/L in the first two hours, which should be stopped if the level has risen by 10 mEq/L in the first five hours, and with the overall correction goal not to exceed 15-20 mEq/L in 48 hrs. This method has been shown safe in all hospital settings studied. Serial measurements of electrolyte levels and neurological examinations are recommended, as are correction of all electrolyte abnormalities, in particular magnesium and potassium. Thiamine should be given to all patients with chronic alcohol use who present with hyponatremia and encephalopathy.

The changing face of osmotic demyelination syndrome

2020 ◽  
pp. 10.1212/CPJ.0000000000000932
Author(s):  
Whitney Fitts ◽  
Andre C. Vogel ◽  
Farrah J. Mateen
Keyword(s):  
Central Pontine Myelinolysis ◽  
International Classification Of Diseases ◽  
Underlying Disease ◽  
Osmotic Demyelination Syndrome ◽  
Post Discharge ◽  
Diverse Range ◽  
Osmotic Demyelination ◽  
Classification Of Diseases ◽  
Pontine Myelinolysis ◽  
Electrolyte Abnormalities

ObjectiveTo describe long-term outcomes of osmotic demyelination syndrome (ODS) in an updated cohort.MethodsWe performed a retrospective medical records review of cases of ODS at the Massachusetts General and Brigham and Women's Hospitals using International Classification of Diseases-9th edition codes and a text-based search for central pontine myelinolysis, extrapontine myelinolysis, and osmotic demyelination syndrome (1999–2018). Cases were individually selected based upon patients having neuroimaging and symptoms consistent with ODS, and no other potentially explanatory etiology. Modified Rankin scale (mRS) scores were extracted at pre-hospitalization, hospital discharge, 6-months post-discharge, and at the most recently available clinical visit.ResultsWe identified 45 cases of ODS (mean age 48.4 years, range 0.07–75 years; 58% female). Common co-morbidities included liver disease (26%, n = 12), alcoholism (43%, n = 20), and kidney failure (20%, n = 9). Twenty-nine percent of patients had a rapid correction of hyponatremia. Twenty-nine percent had other electrolyte abnormalities. Only 59% (24/41) of patients with complete electrolyte data had abnormalities that could explain their ODS. At 6-month follow-up, 16% of patients were deceased and 60% of patients had minimal to no disability (mRS 0–2).ConclusionsODS has a diverse range of clinical presentations. Not all patients have electrolyte abnormalities. The prognosis is generally favorable, although 1 in 6 patients had died at 6 months, likely due to underlying disease states.

scholarly journals Diabetes Induced Osmotic Demyelination of the Pons in a Patient on Peritoneal Dialysis

2021 ◽  
Vol 5 (Supplement_1) ◽  
pp. A375-A375
Author(s):  
Sri Mandava ◽  
Lucas Pfeifer ◽  
Gretel D’Souza ◽  
Mark Wilson
Keyword(s):  
Diabetes Mellitus ◽  
Peritoneal Dialysis ◽  
Serum Sodium ◽  
Central Pontine Myelinolysis ◽  
Rare Event ◽  
Lower Extremities ◽  
Muscle Rigidity ◽  
Osmotic Demyelination ◽  
Pontine Myelinolysis ◽  
Central Pontine

Abstract Introduction: Osmotic demyelination of the pons as a result of uncontrolled diabetes is a rare event. Here we present a unique case of central pontine myelinolysis in a patient with poorly controlled type 2 diabetes mellitus in the setting of peritoneal dialysis. Clinical Case: A 48-year-old male with a history of insulin-dependent diabetes mellitus, hypertension, non-ischemic cardiomyopathy, and end-stage renal disease, treated with peritoneal dialysis, presented to the hospital for slurred speech, inability to walk, and persistent hyperglycemia for 3 days. Approximately 2 years prior to admission, peritoneal dialysis was initiated for ESRD and poor cardiac function. The patient’s outpatient pharmacologic regimen was 30 units of glargine daily and glimepiride. On physical exam, there was evidence of aphasia, dysarthria, and muscle weakness (2/5 in bilateral upper and lower extremities) without muscle rigidity. Deep tendon reflexes were absent in the lower extremities, but sensation to light touch was intact throughout. On admission, the patient was found to be in a hyperosmolar hyperglycemic state without ketosis. Initial lab tests were significant for serum sodium: 138mmol/L (n=134–145 mmol/L), serum sodium corrected for hyperglycemia: 143 mmol/L, serum glucose: 419mg/dL (n=75–99 mg/dL), beta-hydroxybutyrate 2.1 mg/dL (n&lt;4.4 mg/dL), and serum osmolality: 330mosmol/kg (n=275–305 mosmol/kg). Urinalysis showed glucosuria without ketonuria. The cerebrospinal fluid analysis showed protein 57, glucose 109, and WBC 3. Lab studies for meningitis/encephalitis panel were negative. During his 2 years of dialysis, his HbA1C increased from 7.6% to 14.3% (n&lt;5.6%). CT brain without contrast showed midline hypoattenuation of the inferior pons without edema. Magnetic resonance imaging without contrast of the brain demonstrated a lobulated lesion in the pons measuring 1–2 cm that shows T2 hyperintensity without surrounding edema. He was then diagnosed with central pontine myelinolysis in the setting of chronic glycemic changes. Conclusion: Osmotic demyelination of the pons is typically associated with rapid correction of hyponatremia. We describe osmotic demyelination of the pons as a result of poorly controlled diabetes with normal sodium. To our knowledge, this is the first report of this event in association with worsening diabetes after the initiation of peritoneal dialysis.

scholarly journals Central Pontine and Extrapontine Myelinolysis: The Great Masquerader—An Autopsy Case Report

2014 ◽  
Vol 2014 ◽  
pp. 1-5 ◽  
Author(s):  
Sajish Jacob ◽  
Harsh Gupta ◽  
Dejan Nikolic ◽  
Betul Gundogdu ◽  
Shirley Ong
Keyword(s):  
Spinal Cord ◽  
Central Pontine Myelinolysis ◽  
Autopsy Case ◽  
Osmotic Demyelination Syndrome ◽  
Thoracic Region ◽  
Demyelinating Disorder ◽  
First Case ◽  
Chronic Hyponatremia ◽  
Pontine Myelinolysis ◽  
Central Pontine

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. The clinical features vary depending on the extent of involvement. Demyelination can occur outside the pons as well and diagnosis can be challenging if both pontine and extrapontine areas are involved. We herein report a case of myelinolysis involving pons, lateral geniculate bodies, subependymal region, and spinal cord. To the best of our knowledge, this case represents the second case of spinal cord involvement in osmotic demyelination syndrome and the first case of involvement of thoracic region of spinal cord.

scholarly journals Osmotic Demyelination Syndrome: Clinical, Neuroimaging Characteristics, and Outcomes in a Series of 18 Cases

2021 ◽  
Vol 2021 ◽  
pp. 1-9
Author(s):  
Xinhuang Lv ◽  
Qian Hong ◽  
Xiuxiu Lin ◽  
Weian Chen ◽  
Yuan Tian
Keyword(s):  
Complex Disease ◽  
Central Pontine Myelinolysis ◽  
Clinical Information ◽  
The Other ◽  
Extrapyramidal Symptoms ◽  
Osmotic Demyelination Syndrome ◽  
Osmotic Demyelination ◽  
Imaging Results ◽  
Pontine Myelinolysis

Objective. To investigate the etiology, clinical as well as neuroimaging characteristics, and outcomes after proper treatment in a series of 18 patients with osmotic demyelination syndrome. Methods. Medical records, including video records, of 18 patients with osmotic demyelination syndrome were retrospectively examined. Demographic and clinical information, imaging results, plans of management, and outcomes during the follow-up period were collected and analyzed. Results. Eighteen patients, including 10 males and 8 females, were included in the present study. The mean age at diagnosis of CNS insult was 47.4 ± 13.3 years (ranged from 30 to 78 years). Etiologies included rapidly corrected hyponatremia (50%), alcoholism (27.8%), and others. Neurological manifestations included encephalopathy (61.1%), dysphonia (50%), extrapyramidal symptoms (38.9%), and seizures (22.2%). Neuroimaging results showed that 6 patients (33.3%) had central pontine myelinolysis, 5 (27.8%) had extrapontine myelinolysis, and 7 (38.9%) had both. After treatment, 12 patients showed improvement and the other 6 did not. Among these patients, those who showed symptoms of encephalopathy had a favorable outcome. The majority of those who presented with mental retardation, seizures, and no other symptoms recovered better than their counterparts who had other symptoms. Nine out of 11 patients with pseudobulbar paralysis and/or extrapyramidal symptoms showed improvement, but the other 2 did not show improvement. Five patients who did not improve after treatment during admission were followed up for 1-3 months with rehabilitation training recommended, and it was found that 3 showed significant improvement after training, and the other 2 did not respond to this training. Conclusions. Osmotic demyelination syndrome is a complex disease entity due to a variety of etiologies, manifesting with symptoms involving diverse systems of the brain. Early identification and removal/correction of conditions leading to osmotic demyelination syndrome are the key to prevent and/or manage this disease.

Those eyes don’t lie: a case of osmotic demyelination syndrome in a patient with hepatic encephalopathy

Diagnosis ◽  
2016 ◽  
Vol 3 (2) ◽  
pp. 81-85
Author(s):  
Caleb J. Murphy ◽  
Peter L. Cathcart ◽  
Andrew P.J. Olson
Keyword(s):  
Hepatic Encephalopathy ◽  
Central Pontine Myelinolysis ◽  
Pseudobulbar Palsy ◽  
Osmotic Demyelination Syndrome ◽  
Basal Nuclei ◽  
Osmotic Demyelination ◽  
Pontine Myelinolysis ◽  
History Of ◽  
Upper Motor Neuron Dysfunction ◽  
End Stage

AbstractOsmotic demyelination syndrome (ODS), previously known as central pontine myelinolysis, is a rare neurological condition characterized by demyelination of the pons or extrapontine areas including the midbrain, thalamus, basal nuclei, and cerebellum, resulting in upper motor neuron dysfunction and pseudobulbar palsy. We report a case of a 45-year-old woman with a history of alcohol dependence and end stage liver disease complicated by hepatic encephalopathy who developed symptoms suspicious of recurrent hepatic encephalopathy and experienced a generalized seizure during an inpatient stay. After 10 days of treatment with no improvement, it was noted that the patient had locked-in syndrome and that her sodium levels had rapidly risen 2 days prior. This led to a clinical suspicion of ODS, which was confirmed on T2-weighted MRI and subsequently on autopsy. In this clinical vignette, we review the clinical presentation, prognosis, and diagnostic considerations of ODS.

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