Central Pontine Myelinolysis: A Metabolic Disorder of Myelin

Hurley, Robin A.; Filley, Christopher M.; Taber, Katherine H.

doi:10.1176/jnp.23.4.jnp369

Cited by 38 publications

(45 citation statements)

References 52 publications

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“…Using serial ADC mapping at different time points after the onset of symptoms showed lower ADC values early after the onset of symptoms and a gradual increase to normal or above-normal values thereafter. 77 This finding corresponded to the emergence of cytotoxic edema (lower ADC) and subsequent vasogenic edema (higher ADC) at different time intervals. It appears that coupling of DWI and ADC yields higher specificity for ODS lesions, in which lower ADC values correlate better with ODS.…”

Section: Cerebral Volume Changesmentioning

confidence: 82%

Clinical Semiology and Neuroradiologic Correlates of Acute Hypernatremic Osmotic Challenge in Adults: A Literature Review

Ismail¹,

Szóllics

Szólics

et al. 2013

AJNR Am J Neuroradiol

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SUMMARY:The complex interplay between hypernatremic osmotic disturbances and cerebral lesions is yet to be clarified. In this review, we discuss, on the basis of the reported data of hypernatremic CNS challenge in the adult population, the clinical and radiologic features of the condition. Our search captured 20 case studies and 1 case series with 30 patients in total who acquired acute hypernatremia due to different etiologies and developed CNS lesions. We explored the associations between premorbid conditions, clinical presentation, hypernatremic state, correction rate, and radiologic appearance, including the localization of brain lesions and the outcomes. The results revealed that altered mental status was the most commonly reported symptom and osmotic demyelination syndrome in the form of extrapontine myelinolysis was the prevailing radiologic pattern. Finally, we contrasted, when appropriate, clinical and experimental data related to hypernatremic and hyponatremic osmotic insults to aid the understanding of the pathophysiology of CNS osmotic brain injury.ABBREVIATIONS: BG ϭ basal ganglia; CPM ϭ central pontine myelinolysis; EPM ϭ extrapontine myelinolysis; GCS ϭ Glasgow Coma Scale; ODS ϭ osmotic

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Section: Cerebral Volume Changesmentioning

confidence: 82%

Clinical Semiology and Neuroradiologic Correlates of Acute Hypernatremic Osmotic Challenge in Adults: A Literature Review

Ismail¹,

Szóllics

Szólics

et al. 2013

AJNR Am J Neuroradiol

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“…Blood vessels are patent, and both axons and neuronal cell bodies are generally spared. Since both the pontine and extrapontine sites involved have a rich grey and white matter interface, it has been hypothesized that rapid osmotic changes result in endothelial injury in the more vascular grey and white matter, inducing the release of myelinolytic factors that damage the adjacent white matter [1]. MRI is more accurate than CT for the detection of CPEPM; lesions are identified as hyperintense on T2-weighted (including FLAIR) and hypointense on T1-weighted MRI sequences with no mass effect.…”

Section: Discussionmentioning

confidence: 99%

“…It is a clinically heterogeneous disease, originally thought to occur only in the central pons area [1, 2], and later shown to affect areas outside the pons [3]. When demyelination involves areas outside the pons, the disorder is referred to as either extrapontine myelinolysis (EPM) or central and extrapontine myelinolysis (CPEPM).…”

Section: Introductionmentioning

confidence: 99%

Diffuse large B-cell lymphoma presenting with central pontine myelinolysis: a case report

et al. 2015

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IntroductionThe most common cause of central pontine myelinolysis is an overly rapid correction of hyponatremia, although it can also occur in patients with any condition leading to nutritional or electrolyte stress. We report a case of diffuse large B-cell lymphoma with central pontine myelinolysis developing at the onset of disease. To the best of our knowledge, hematological malignancies presenting with central pontine myelinolysis have been rarely reported, especially in previously untreated patients, as in our case report.Case presentationA 78-year-old Japanese woman presented to a neighborhood clinic with persistent high fever, edema, and general weakness. Despite the absence of specific neurological findings, brain magnetic resonance imaging showed an abnormal lesion in the central pons area of her brain (hyperintense on T2-weighted and hypointense on T1-weighted sequences), compatible with central pontine myelinolysis. She was admitted to our emergency department in a state of shock one month later. The results of her blood tests showed greatly elevated C-reactive protein and lactate dehydrogenase levels. She had severe hypoalbuminemia and mild hyponatremia, and showed signs of disseminated intravascular coagulation. Mild bilateral pleural effusion, prominent subcutaneous edema, and splenomegaly were detected on her systemic computed tomography scan. Her body fluid cultures did not show signs of infection and her spinal aspiration did not show pleocytosis or abnormal cells. A diagnosis of diffuse large B-cell lymphoma was made based on the results of her bone marrow examination. As she was critically ill before the diagnosis was made, she was treated with methylprednisolone pulse therapy, followed by systemic chemotherapy (rituximab with modified THP-COP regimen, including cyclophosphamide, pirarubicin, vindesine, and prednisolone), which resulted in complete remission and recovery without any neurological defects, and resolution of her abnormal findings on magnetic resonance imaging.ConclusionsCentral pontine myelinolysis is a serious condition that may result in neuropathological sequelae and mortality, and clinicians should be aware of its potential presence in patients with malignancies.

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“…It has been proposed that, in CPM, oligodendrocytes in close proximity to gray matter are exposed to a myelinotoxic substance as a result of osmotic stress. 7 Since both the pontine and extra pontine sites involved have a rich grey and white matter interface, it has been hypothesized that a rapid osmotic change causes an endothelial injury in the more vascular grey and white matter which induces the release of myelinolytic factors that damage the adjacent white matter. 8 The term osmotic myelinolysis is currently favored for this condition.…”

Section: Discussionmentioning

confidence: 99%