Case Series of Osmotic Demyelination Syndrome Treated With Plasmapheresis: Experience From Two Tertiary Hospitals.
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SUBMITTER: Lim KY
PROVIDER: S-EPMC8762491 | biostudies-literature | 2022 Jan
REPOSITORIES: biostudies-literature
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Case Series of Osmotic Demyelination Syndrome Treated With Plasmapheresis: Experience From Two Tertiary Hospitals.
Lim Kuan Yee KY Chia Yuen Kang YK Khoo Ching Soong CS Tan Hui Jan HJ
Journal of clinical neurology (Seoul, Korea) 20220101 1
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Project description:BackgroundTreatment options for chronic osmotic demyelination syndrome are limited to case reports and only a very few show complete recovery. We report a case of complete recovery of chronic osmotic demyelination syndrome with plasmapheresis.Case presentationA 43-year-old Sri Lankan man presented with fever, repeated vomiting, unsteady gait, increased tonicity of his right upper limb and paucity of speech for three days. He was treated in the local hospital with antibiotics and antivirals as per central nervous system infection. He had hyponatraemia, which was rapidly corrected with hypertonic saline from 97 to 119 mmol/L. He was transferred to our hospital because of progressive reduction of consciousness, rapidly worsening rigidity and bradykinesia of all four limbs and worsening dysarthria and bradyphrenia. Magnetic resonance imaging of the brain was compatible with osmotic demyelination syndrome. He was commenced on plasmapheresis twenty-two days after rapid correction of sodium. He regained independent mobility with complete resolution of rigidity, bradykinesia and speech dysfunction after five cycles of alternate day plasmapheresis.ConclusionPlasmapheresis can be considered as an effective treatment modality in chronic osmotic demyelination syndrome.
| S-EPMC7798224 | biostudies-literature
Project description:ObjectiveTo investigate the etiology, clinical as well as neuroimaging characteristics, and outcomes after proper treatment in a series of 18 patients with osmotic demyelination syndrome.MethodsMedical 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.ResultsEighteen 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.ConclusionsOsmotic 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.
| S-EPMC8178002 | biostudies-literature
Project description:Osmotic demyelination syndrome (ODS) is well known to be associated with the rapid correction of hyponatremia. However, there is limited literature on its link to persistent or corrected hyperglycemic states in diabetic patients. We report two young patients with ODS and hyperglycemia. Neuroimaging and blood tests indicated central pontine and extrapontine myelinolysis with a hyperglycemic hyperosmolar state (HHS). These unusual cases provide insight into the neurologic complications of hyperglycemia.
| S-EPMC11575862 | biostudies-literature
Project description:Abrupt osmotic changes during rapid correction of chronic hyponatremia result in demyelinative brain lesions, but the sequence of events linking rapid osmotic changes to myelin loss is not yet understood. Here, in a rat model of osmotic demyelination syndrome, we found that massive astrocyte death occurred after rapid correction of hyponatremia, delineating the regions of future myelin loss. Astrocyte death caused a disruption of the astrocyte-oligodendrocyte network, rapidly upregulated inflammatory cytokines genes, and increased serum S100B, which predicted clinical manifestations and outcome of osmotic demyelination. These results support a model for the pathophysiology of osmotic brain injury in which rapid correction of hyponatremia triggers apoptosis in astrocytes followed by a loss of trophic communication between astrocytes and oligodendrocytes, secondary inflammation, microglial activation, and finally demyelination.
| S-EPMC3365278 | biostudies-literature
Project description:Osmotic demyelination syndrome (ODS) is characterized by loss of myelin in various parts of the central nervous system. It is mainly caused by a rapid correction of hyponatremia, although other factors that may cause rapid rise in serum osmolality can also be associated with its development. Its prognosis is poor and the recovery rate is unknown. The authors report a rare case of a patient with multiple risk factors for ODS, without hyponatremia, who developed ODS and surprisingly recovered. This case report highlights the importance of recognizing risk factors for the development of ODS, even if the main one is not present.
| S-EPMC7125463 | biostudies-literature
Project description:BackgroundOsmotic demyelination syndrome (ODS) can be a central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) based on the regions involved even though they share the same disease process, aetiopathogenesis and time course.ObjectivesPresent study aims to characterize the clinical, radiological features and the outcome of patients with ODS with movement disorders as the forthcoming manifestation.MethodsChart review of patients with ODS with movement disorders. Demographic, clinical and radiological details of the patients were reviewed.ResultsEleven patients (six females; mean age: 48.3 ± 17.6 years) were included in the study. Parkinsonism alone and parkinsonism with dystonia was noted in four patients each (36.4%) while dystonia alone was noted in the other 3 (27.3%). Five patients (45.5%) had postural tremors. While 5 patients had dystonia early in the course of illness (3-7 days), it was delayed (6-9 months) in the other 2. A triphasic course was noted in two patients. The first phase of hyponatremia induced neurological impairment was followed by a second phase of worsening due to the immediate effect of ODS and a third delayed phase of worsening due to delayed effect of ODS. MRI showed both EPM and CPM in eight patients, EPM alone in two patients and CPM alone in 1 patient. Nine patients had a good outcome with mRS < 3.ConclusionParkinsonism and dystonia are important manifestations of ODS. Triphasic course with a delayed phase of worsening of movement disorders is probably due to the maladaptive neuronal repair. The concept of triphasic ODS is first being described in our series.
| S-EPMC8354067 | biostudies-literature
Project description:Rapid correction of chronic hyponatremia can lead to osmotic demyelination syndrome (ODS), a severe demyelination disease. The microglia that accumulate in the demyelinative lesions may play a detrimental role in the pathogenesis of ODS by producing proinflammatory cytokines, suggesting that they may be a target for therapeutic intervention. Here, we investigated whether minocycline, a selective and potent inhibitor of microglial activation, could protect against ODS in rats. We induced hyponatremia by liquid diet feeding and dDAVP infusion. Rapid correction of the hyponatremia 7 days later resulted in neurologic impairment with severe demyelinative lesions. Activated microglia accumulated at the site of demyelination. Treatment with minocycline within 24 hours of rapid correction, however, was protective: rats exhibited minimal neurologic impairment, and survival improved. Histologic analysis showed that minocycline inhibited demyelination and suppressed the accumulation of microglia at the site of demyelination. Real-time RT-PCR and immunohistochemical analyses showed that minocycline inhibited the activity of microglia and the expression of inflammatory cytokines (e.g. IL-1β, inducible nitric-oxide synthase, and TNF-α), monocyte chemoattractant protein-1, and matrix metalloproteinase-12 in microglia. These results demonstrate that minocycline can protect against ODS by inhibiting the activation and accumulation of microglia at the site of demyelinative lesions, suggesting its possible use in clinical practice.
| S-EPMC3014022 | biostudies-literature
Project description:Diabetic striatopathy (DS), also known as non-ketotic hyperglycemic hemichorea, is a rare condition that arises from uncontrolled hyperglycemia. It is characterized by new onset movement disorders, changes in the striatum on imaging, or both. DS can occur as a complication of long-standing non-ketogenic hyperglycemia or be the first presentation of previously undiagnosed diabetes mellitus (DM). Additionally, uncontrolled or rapidly corrected hyperglycemia can, in rare cases, lead to osmotic demyelination syndrome (ODS). Although ODS typically occurs after the rapid correction of hyponatremia, the same effects and symptoms can manifest in patients with diabetes when hyperglycemia is corrected too quickly. We present a 59-year-old male with a history of uncontrolled diabetes mellitus and cerebrovascular accident who was brought to the emergency department by EMS with a new onset movement disorder. This case demonstrates a rare example of a patient presenting with classic imaging findings of both DS and ODS. Specifically, the patient demonstrated unilateral basal ganglia hyperdensity on CT, indicative of DS, alongside central pontine diffusion restriction and T2/FLAIR hyperintensity, consistent with ODS. This report discusses a rare case of the simultaneous occurrence of diabetic striatopathy and osmotic demyelination syndrome in a patient with uncontrolled diabetes mellitus, highlighting the intricate neurological complications of hyperglycemia. These findings stress the importance of timely recognition and management of hyperglycemia-related conditions, with imaging playing a pivotal role in diagnosis.
| S-EPMC11915231 | biostudies-literature
Project description:Jaw clonus, a fascinating, yet uncommon clinical sign, is suggestive of supranuclear lesions of the trigeminal nerve. It has previously been reported in association with amyotrophic lateral sclerosis. Hereby, we report an index case of jaw clonus in a patient of neuromyelitis optica spectrum disorder with subsequent osmotic demyelination syndrome with pseudobulbar palsy due to the involvement of pontine corticobulbar fibres.
| S-EPMC7114005 | biostudies-literature
Project description:Rationale & objectiveOsmotic demyelination syndrome (ODS) is a rare but severe condition often attributed to the rate of sodium collection. We evaluated the association between the overly rapid sodium correction in adult hospitalized patients with ODS.Study designSystematic review and meta-analysis.Setting & study populationsAdults hospitalized hyponatremia patients.Selection criteria for studiesThe studies comparing the incidence of ODS with and without rapid sodium correction inception to January 2024.Data extractionTwo reviewers independently extracted data and assessed the risk of bias and the certainty of evidence.Analytic approachThe incidence of ODS following a rapid and nonrapid sodium correction was pooled using the random effects model. Subgroup and meta-regression analyses were performed for the robustness and the source of heterogeneity.ResultsEleven cohort studies were included with 26,710 hospitalized hyponatremia patients. The definition of hyponatremia varied from <116 to <130 mmol/L, and overly rapid sodium correction was defined as >8 to 12 mmol/L within 24 hours. The overall incidence of ODS was 0.23%. The incidence of ODS in rapid and nonrapid sodium correction was 0.73% and 0.10%, respectively. Meta-analysis demonstrated that a rapid rate of sodium correction was associated with a higher incidence of ODS (odds ratio 3.16, 95% CI, 1.54-6.49, I2 = 27%), whereas some patients with hyponatremia developed ODS without rapid sodium level correction. The sensitivity analysis based on the quality of the studies was consistent with the main result.LimitationVarious definition criteria for ODS diagnosis across studies, lack of potential electrolyte and treatment data that may affect the incidence of ODS.ConclusionsThe rapid rate of sodium correction had a statistical correlation with a higher incidence of ODS. Among ODS without rapid correction, further studies are recommended to evaluate and comprehend the relationship for better and proper management of hospitalized patients with hyponatremia.
| S-EPMC11833618 | biostudies-literature