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European Neurology 2009In 1949, Victor and Adams observed an alcoholic patient who developed quadriplegia and pseudobulbar palsy, and inability to chew, talk or swallow. A post-mortem...
In 1949, Victor and Adams observed an alcoholic patient who developed quadriplegia and pseudobulbar palsy, and inability to chew, talk or swallow. A post-mortem confirmed their suspicion of 'a large, symmetrical, essentially demyelinative lesion occupying the greater part of the basis pontis.' This paper follows the historical evolution of central pontine myelinolysis and the changing concepts of its metabolic aetiology. Too rapid a rate of correction of hyponatraemia is the most common, but not invariable aetiology.
Topics: History, 20th Century; Humans; Hyponatremia; Myelinolysis, Central Pontine
PubMed: 19033724
DOI: 10.1159/000175124 -
WMJ : Official Publication of the State... Oct 2014Formerly known as central pontine myelinolysis, osmotic demyelination syndrome (ODS) is defined by a symmetrical destruction of myelin sheaths involving mainly the...
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.
Topics: Diagnosis, Differential; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Myelinolysis, Central Pontine
PubMed: 25739163
DOI: No ID Found -
Journal of Neurology, Neurosurgery, and... Sep 1975Attenuation of cerebral evoked responses after stimulation of the median nerve in the hemiplegic limbs suggested that an apparently pure motor hemiplegia in some...
Attenuation of cerebral evoked responses after stimulation of the median nerve in the hemiplegic limbs suggested that an apparently pure motor hemiplegia in some patients may not have pure involvement of the corticospinal system. Frontoparietal metastasis, infarction in basis pontis and medullary pyramid, and occlusion of internal carotid artery in the neck resulted in pure motor hemiplegia in some individuals.
Topics: Adult; Aged; Brain; Electroencephalography; Electromyography; Evoked Potentials; Female; Hemiplegia; Humans; Infarction; Male; Median Nerve; Middle Aged; Pyramidal Tracts; Somatosensory Cortex
PubMed: 1185228
DOI: 10.1136/jnnp.38.9.896 -
Journal of Neurology, Neurosurgery, and... Feb 1983Dissection of the basilar artery caused sudden coma and death in a 40-year-old man. Atypical clinical features were explained at necropsy. A ventral dissection of the...
Dissection of the basilar artery caused sudden coma and death in a 40-year-old man. Atypical clinical features were explained at necropsy. A ventral dissection of the artery within its outer layers resulted in destruction of the pontine tegmentum with sparing of the basis pontis. An unsuspected defect in the internal elastic lamina in the left internal carotid artery was also found suggesting a more generalised disorder of arterial walls. Basilar artery dissection should be considered in the diagnosis of coma in young people.
Topics: Adult; Aortic Dissection; Basilar Artery; Brain Stem; Cerebellum; Cerebral Angiography; Cerebral Infarction; Coma; Diagnosis, Differential; Humans; Intracranial Aneurysm; Male; Tomography, X-Ray Computed
PubMed: 6842215
DOI: 10.1136/jnnp.46.2.126 -
Indian Journal of Critical Care... Sep 2022Central pontine myelinolysis (CPM) classically occurs due to rapid rise in serum osmolarity. Most cases have been associated with a history of chronic alcohol abuse,...
UNLABELLED
Central pontine myelinolysis (CPM) classically occurs due to rapid rise in serum osmolarity. Most cases have been associated with a history of chronic alcohol abuse, malnutrition, diuretic abuse, and hyponatremia. The pathological process of CPM starts in the central pons near median raphe and spreads out "like a brush Fire" into the surrounding basis pontis. Extrapontine sites such as internal capsule, basal ganglia, cerebellum, and cerebrum can also be affected. We report a case of 60-year-old male with history of chronic alcoholism who presented to us with severe neurological deficits 10 days after his episode of severe hyponatremia.
HOW TO CITE THIS ARTICLE
Tiwari R, Kumari A. Central Pontine Myelinolysis: A Case Report. Indian J Crit Care Med 2022;26(9):1049-1051.
PubMed: 36213701
DOI: 10.5005/jp-journals-10071-24311 -
AJNR. American Journal of Neuroradiology Mar 2021Preterm birth interferes with regular brain development. The aim of this study was to investigate the impact of prematurity on the physical tissue properties of the...
BACKGROUND AND PURPOSE
Preterm birth interferes with regular brain development. The aim of this study was to investigate the impact of prematurity on the physical tissue properties of the neonatal brain stem using a quantitative MR imaging approach.
MATERIALS AND METHODS
A total of 55 neonates (extremely preterm [=30]: <28 + 0 weeks gestational age; preterm [=10]: 28 + 0-36 + 6 weeks gestational age; term [=15]: ≥37 + 0 weeks gestational age) were included in this retrospective study. In most cases, imaging was performed at approximately term-equivalent age using a standard MR protocol. MR data postprocessing software SyMRI was used to perform multidynamic multiecho sequence (acquisition time: 5 minutes, 24 seconds)-based MR postprocessing to determine T1 relaxation time, T2 relaxation time, and proton density. Mixed-model ANCOVA (covariate: gestational age at MR imaging) and the post hoc Bonferroni test were used to compare the groups.
RESULTS
There were significant differences between premature and term infants for T1 relaxation time (midbrain: < .001; pons: < .001; basis pontis: = .005; tegmentum pontis: < .001; medulla oblongata: < .001), T2 relaxation time (midbrain: < .001; tegmentum pontis: < .001), and proton density (tegmentum pontis: = .004). The post hoc Bonferroni test revealed that T1 relaxation time/T2 relaxation time in the midbrain differed significantly between extremely preterm and preterm (T1 relaxation time: < .001/T2 relaxation time: = .02), extremely preterm and term (T1 relaxation time/T2 relaxation time: < .001), and preterm and term infants (T1 relaxation time: < .001/T2 relaxation time: = .006).
CONCLUSIONS
Quantitative MR parameters allow preterm and term neonates to be differentiated. T1 and T2 relaxation time metrics of the midbrain allow differentiation between the different stages of prematurity. SyMRI allows for a quantitative assessment of incomplete brain maturation by providing tissue-specific properties while not exceeding a clinically acceptable imaging time.
Topics: Brain Stem; Female; Gestational Age; Humans; Image Processing, Computer-Assisted; Infant, Newborn; Infant, Premature; Magnetic Resonance Imaging; Male; Pregnancy; Retrospective Studies
PubMed: 33478940
DOI: 10.3174/ajnr.A6945 -
The American Journal of Pathology Sep 1986The central nervous system (CNS) has been examined at autopsy in 89 patients who died of the acquired immune deficiency syndrome (AIDS), including 14 patients who died... (Review)
Review
The central nervous system (CNS) has been examined at autopsy in 89 patients who died of the acquired immune deficiency syndrome (AIDS), including 14 patients who died primarily of neurologic complications of the disease. A total of 66 brains (74%) showed significant pathologic abnormalities, with opportunistic infections including cytomegalovirus (14) and cryptococcal (11) infections, progressive multifocal leukoencephalopathy (6), toxoplasmosis (6), and histoplasma microabscesses (1). Incidental Mycobacterium avium-intracellulare infection was found in 4 cases. Simultaneous CNS infection by more than one microorganism was encountered in 5 patients. Subacute (microglial nodule) encephalitis-related to cytomegalovirus infection or possibly brain infection by the causative agent of AIDS was present in 56 cases. Primary CNS lymphoma was noted in 3 patients. Secondary CNS deposits of lymphoma were found in 1 patient, and another patient had lymphomatoid granulomatosis. Vascular complications were not infrequently seen, and included infarcts secondary to vessel occlusion and disseminated intravascular coagulation in 4 patients and intracranial hemorrhage of variable severity in 13. White matter changes included vacuolar myelopathy (3 cases), central pontine myelinolysis (1 case), and foci of calcified, necrotizing leukoencephalopathy in pontocerebellar fibers of the basis pontis (2 cases). These findings highlight the variety of CNS complications in AIDS, some of which are not associated with clinical manifestations. Nevertheless, characterization of all lesions may be important in understanding the neurologic sequelae of AIDS.
Topics: Adult; Aged; Bacterial Infections; Central Nervous System Diseases; Cytomegalovirus Infections; Deltaretrovirus Infections; Female; Humans; Male; Middle Aged; Nervous System
PubMed: 2876640
DOI: No ID Found -
Ultrasound in Obstetrics & Gynecology :... Aug 2021To characterize spatiotemporal growth differences of prenatal brainstem substructures and cerebellum, using linear biometry and planimetry on fetal magnetic resonance...
OBJECTIVES
To characterize spatiotemporal growth differences of prenatal brainstem substructures and cerebellum, using linear biometry and planimetry on fetal magnetic resonance imaging (MRI).
METHODS
In this retrospective study, we included fetuses with normal brain and a precise midsagittal T2-weighted brain MRI sequence obtained between May 2003 and April 2019. The cross-sectional area, rostrocaudal diameter and anteroposterior diameter of the midbrain, pons (basis pontis and pontine tegmentum), medulla oblongata and cerebellar vermis, as well as the transverse cerebellar diameter, were quantified by a single observer. The diameters were also assessed by a second observer to test inter-rater variability.
RESULTS
We included 161 fetuses with normal brain and a precise midsagittal MRI sequence, examined at a mean ± SD gestational age of 25.7 ± 5.4 (range, 14 + 0 to 39 + 2) weeks. All substructures of the fetal brainstem and the cerebellum could be measured consistently (mean ± SD interobserver intraclass correlation coefficient, 0.933 ± 0.065). We provide reference data for diameters and areas of the brainstem and cerebellum in the second and third trimesters. There was a significant quadratic relationship between vermian area and gestational age, and all other measured parameters showed a significant linear growth pattern within the observed period (P < 0.001). A significant change in the relative proportions of the brainstem substructures occurred between the beginning of the second trimester and the end of the third trimester, with an increase in the area of the pons (P < 0.001) and a decrease in that of the midbrain (P < 0.001), relative to the total brainstem area.
CONCLUSIONS
The substructures of the fetal brainstem follow a distinct spatiotemporal growth pattern, characterized by a relative increase in the pons and decrease in the midbrain, between 15 and 40 weeks of gestation. Caution is needed when interpreting fetal brainstem appearance during the early second trimester, as the brainstem proportions differ significantly from the adult morphology. The reference data provided herein should help to increase diagnostic accuracy in detecting disorders of defective hindbrain segmentation. © 2020 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Brain Stem; Female; Fetus; Gestational Age; Humans; Magnetic Resonance Imaging; Pregnancy; Prenatal Diagnosis; Reference Values; Retrospective Studies
PubMed: 32730667
DOI: 10.1002/uog.22162 -
Acta Neuropathologica Communications Jul 2013Central pontine myelinolysis (CPM) is a demyelinating disorder of the central basis pontis that is often associated with osmotic stress. The aquaporin water channels...
BACKGROUND
Central pontine myelinolysis (CPM) is a demyelinating disorder of the central basis pontis that is often associated with osmotic stress. The aquaporin water channels (AQPs) have been pathogenically implicated because serum osmolarity changes redistribute water and osmolytes among various central nervous system compartments.
RESULTS
We characterized the immunoreactivity of aquaporin-1 and aquaporin-4 (AQP1 and AQP4) and associated neuropathology in microscopic transverse sections from archival autopsied pontine tissue from 6 patients with pathologically confirmed CPM. Loss of both AQP1 and AQP4 was evident within demyelinating lesions in four of the six cases, despite the presence of glial fibrillary acidic protein (GFAP)-positive astrocytes. Lesional astrocytes were small, and exhibited fewer and shorter processes than perilesional astrocytes. In two of the six cases, astrocytes within demyelinating lesions exhibited increased AQP1 and AQP4 immunoreactivities, and gemistocytes and mitotic astrocytes were numerous. Blinded review of medical records revealed that all four cases lacking lesional AQP1 and AQP4 immunoreactivities were male, whereas the two cases with enhanced lesional AQP1 and AQP4 immunoreactivities were female.
CONCLUSIONS
This report is the first to establish astrocytic AQP loss in a subset of human CPM cases and suggests AQP1 and AQP4 may be involved in the pathogenesis of CPM. Further studies are required to determine whether the loss of AQP1 and AQP4 is restricted to male CPM patients, or rather may be a feature associated with specific underlying precipitants of CPM that may be more common among men. Non-rodent experimental models are needed to better clarify the complex and dynamic mechanisms involved in the regulation of AQPs in CPM, in order to determine whether it occurs secondary to the destructive disease process, or represents a compensatory mechanism protecting the astrocyte against apoptosis.
Topics: Adult; Aged; Aquaporin 1; Aquaporin 4; Astrocytes; Cell Size; Female; Glial Fibrillary Acidic Protein; Humans; Immunohistochemistry; Male; Middle Aged; Myelinolysis, Central Pontine; Pons; Sex Characteristics; Young Adult
PubMed: 24252214
DOI: 10.1186/2051-5960-1-40 -
Annals of Neurology Mar 2016Succinate dehydrogenase-deficient leukoencephalopathy is a complex II-related mitochondrial disorder for which the clinical phenotype, neuroimaging pattern, and genetic...
OBJECTIVE
Succinate dehydrogenase-deficient leukoencephalopathy is a complex II-related mitochondrial disorder for which the clinical phenotype, neuroimaging pattern, and genetic findings have not been comprehensively reviewed.
METHODS
Nineteen individuals with succinate dehydrogenase deficiency-related leukoencephalopathy were reviewed for neuroradiological, clinical, and genetic findings as part of institutional review board-approved studies at Children's National Health System (Washington, DC) and VU University Medical Center (Amsterdam, the Netherlands).
RESULTS
All individuals had signal abnormalities in the central corticospinal tracts and spinal cord where imaging was available. Other typical findings were involvement of the cerebral hemispheric white matter with sparing of the U fibers, the corpus callosum with sparing of the outer blades, the basis pontis, middle cerebellar peduncles, and cerebellar white matter, and elevated succinate on magnetic resonance spectroscopy (MRS). The thalamus was involved in most studies, with a predilection for the anterior nucleus, pulvinar, and geniculate bodies. Clinically, infantile onset neurological regression with partial recovery and subsequent stabilization was typical. All individuals had mutations in SDHA, SDHB, or SDHAF1, or proven biochemical defect.
INTERPRETATION
Succinate dehydrogenase deficiency is a rare leukoencephalopathy, for which improved recognition by magnetic resonance imaging (MRI) in combination with advanced sequencing technologies allows noninvasive diagnostic confirmation. The MRI pattern is characterized by cerebral hemispheric white matter abnormalities with sparing of the U fibers, corpus callosum involvement with sparing of the outer blades, and involvement of corticospinal tracts, thalami, and spinal cord. In individuals with infantile regression and this pattern of MRI abnormalities, the differential diagnosis should include succinate dehydrogenase deficiency, in particular if MRS shows elevated succinate.
Topics: Female; Humans; Infant; Infant, Newborn; Leukoencephalopathies; Magnetic Resonance Imaging; Male; Pyramidal Tracts; Spinal Cord; Succinate Dehydrogenase; Thalamus
PubMed: 26642834
DOI: 10.1002/ana.24572