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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 -
Child's Nervous System : ChNS :... Jan 2018A relationship between Zika virus (ZikV) infection in pregnancy and the occurrence of microcephaly was established during the Zika outbreak in Brazil (2015-2016).... (Review)
Review
BACKGROUND
A relationship between Zika virus (ZikV) infection in pregnancy and the occurrence of microcephaly was established during the Zika outbreak in Brazil (2015-2016). Neuropathological findings in congenital Zika syndrome helped to understand its pathogenetic mechanisms.
RESULTS
The most relevant postmortem findings in the central nervous system (CNS) of fetuses and neonates infected with ZikV early in gestation are microcephaly with ex-vacuo ventriculomegaly and large head circumference associated with obstructive hydrocephalus due to severe midbrain and aqueduct distortion. Babies with severe brain lesions are born with arthrogryposis. Histologically, there is extensive destruction of the hemispheric parenchyma, calcifications, various disturbances of neuronal migration, reactive gliosis, microglial hyperplasia and occasional perivascular cuffs of lymphocytes, also in the meninges. Hypoplastic lesions secondary to the lack of descending nerve fibers include small basis pontis, pyramids and spinal corticospinal tracts. Cerebellar hypoplasia is also common. Severe nerve motor nerve cell loss is observed in the anterior horn of the spinal cord.
CONCLUSION
A spectrum of neuropathological changes, from severe microcephaly to obstructive hydrocephalus was observed. The severity of the lesions is directly related to the gestational age, the most severe occurring when the mother is infected in the first trimester. Infection of progenitor cells at the germinal matrix was demonstrated. The lack of spinal motor neurons is responsible for fetal acynesia and consequent arthrogryposis.
Topics: Adult; Brain; Brazil; Female; Humans; Infant, Newborn; Microcephaly; Nervous System; Pregnancy; Zika Virus Infection
PubMed: 29167994
DOI: 10.1007/s00381-017-3651-3 -
American Journal of Medical Genetics.... Jun 2011Examples of white matter hyperintensities (wmh) on magnetic resonance images in a basis pontis are presented in two male carriers, each of whom carry a small CGG...
Examples of white matter hyperintensities (wmh) on magnetic resonance images in a basis pontis are presented in two male carriers, each of whom carry a small CGG expansion fragile X mental retardation (FMR1) allele. One carried a premutation (PM) allele of 85 CGG repeats and the other, a gray zone (GZ) allele of 47 repeats. Both were originally diagnosed with idiopathic Parkinson's disease (iPD). Similar changes are also shown in one PM carrier of 99 repeats affected with mild tremor and imbalance, who was ascertained through a fragile X syndrome family. These examples draw attention to the occurrence of wmh in a basis pontis in the carriers of small CGG expansions presenting with tremor and ataxia. Moreover, the presence of this change in GZ, as well as PM, allele carriers originally diagnosed with iPD supports our earlier suggestion that both these alleles may contribute to the neurodegenerative changes in this disorder which, in the examples presented, have been reflected by wmh, most prominent in the cerebellar peduncles and/or pontine area.
Topics: Adolescent; Alleles; Case-Control Studies; Child; Child, Preschool; Female; Fragile X Mental Retardation Protein; Heterozygote; Humans; Magnetic Resonance Imaging; Male; Nerve Fibers, Myelinated; Parkinsonian Disorders; Trinucleotide Repeat Expansion; Young Adult
PubMed: 21445959
DOI: 10.1002/ajmg.b.31189 -
Stroke Apr 2002This study investigated whether the topography of hypertensive pontine microhemorrhages (hPMHs) resembles that of larger primary pontine hemorrhages. (Clinical Trial)
Clinical Trial Comparative Study
BACKGROUND AND PURPOSE
This study investigated whether the topography of hypertensive pontine microhemorrhages (hPMHs) resembles that of larger primary pontine hemorrhages.
METHODS
Sixty-nine consecutive patients with small-vessel disease underwent imaging with gradient-echo MRI, and 27 patients with hPMH were detected. Lesion size and location along the rostrocaudal (longitudinal), lateral (coronal), and anteroposterior (sagittal) axes were determined.
RESULTS
A total of 52 hPMHs were identified in the 27 patients (mean, 1.93+/-2.4 per patient). The lesions showed a nonrandom distribution, with a propensity to occur in the middle pons in the rostrocaudal axis, posterior half of the basis pontis in the anteroposterior axis, and central subdivision within the lateral axis. The area of hPMH ranged from 1.3 to 19.0 mm2 (mean, 5.06+/-3.72 mm2). The size of hPMH did not vary as a function of lesion location.
CONCLUSIONS
Previous studies reported that primary pontine hemorrhages tend to occur in the middle pons and at the junction of basis pontis and tegmentum. Therefore, topographical correspondences between large and small pontine hemorrhages may provide evidence that the 2 lesions share some etiological basis. Further investigation may determine whether hPMHs portend future symptomatic primary pontine hemorrhages.
Topics: Aged; Aged, 80 and over; Cognition Disorders; Dementia, Vascular; Demography; Echo-Planar Imaging; Female; Humans; Hypertension; Intracranial Hemorrhages; Magnetic Resonance Imaging; Male; Microcirculation; Middle Aged; Neuropsychological Tests; Pons; Prevalence; Risk Factors; Severity of Illness Index
PubMed: 11935038
DOI: 10.1161/01.str.0000013563.73522.cb -
Advances in Experimental Medicine and... 2018Spinocerebellar ataxia type 3 (SCA-3)/Machado-Joseph disease (MJD), the most common autosomal dominant ataxia, affects many regions of the brain and spinal cord. Similar... (Review)
Review
Spinocerebellar ataxia type 3 (SCA-3)/Machado-Joseph disease (MJD), the most common autosomal dominant ataxia, affects many regions of the brain and spinal cord. Similar to SCA-1, SCA-2, SCA-6, SCA-7, and SCA-17, the mutation consists of a pathogenic translated cytosine-adenine-guanine (CAG) trinucleotide repeat expansion. Almost invariably, the substantia nigra and the dentate nucleus of the cerebellum bear the brunt of the disease, and these lesions account for the Parkinsonian and ataxic phenotypes. Lesions of motor nuclei in the brain stem cause the complex disturbance of ocular motility and weakness of the tongue. Atrophy of the basis pontis is common, and polyglutamine-positive neuronal intranuclear inclusion bodies are most readily found in the pontine gray. Abnormalities of basal ganglia, thalamus, spinal cord, dorsal root ganglia, and sensory peripheral nerves are more variable. This report of the main neuropathological lesions is based on the study of 12 genetically confirmed autopsy cases of SCA-3/MJD. In the cerebellum, all layers of the cortex remain normal, but the dentate nucleus exhibits neuronal loss and a peculiar proliferation of synaptic terminals termed grumose regeneration. The clusters surrounding residual neuronal cell bodies and dendrites are interpreted as a response to loss of γ-aminobutyric acid (GABA)-A-receptors and lack of gephyrin, a protein that accomplishes the proper positioning of GABA-A- and glycine receptors. At the spinal level, dorsal root ganglia reveal proliferation of satellite cells, active neuronal destruction, and residual nodules. The spinal cord shows total or subtotal loss of neurons in the dorsal nuclei, anterior horn cell atrophy, and variable long tract degeneration. While misfolding of ataxin-3 due to overly long polyglutamine stretches is a critical contributor to the pathogenesis of SCA-3/MJD, the great neuropathological complexity of the disorder remains largely unexplained.
Topics: Animals; Ataxin-3; Brain; Carrier Proteins; Ganglia, Spinal; Humans; Machado-Joseph Disease; Membrane Proteins; Receptors, GABA-A; Spinal Cord
PubMed: 29427106
DOI: 10.1007/978-3-319-71779-1_11 -
The Journal of Comparative Neurology Oct 2004Motor corticopontine studies suggest that the pons is topographically organized, but details remain unresolved. We used physiological mapping in rhesus monkey to define... (Comparative Study)
Comparative Study
Motor corticopontine studies suggest that the pons is topographically organized, but details remain unresolved. We used physiological mapping in rhesus monkey to define subregions in precentral motor cortex (M1), injected isotope tracers into M1 and the supplementary motor area (SMA), and studied projections to the basis pontis. Labeled fibers descend in the internal capsule (SMA in anterior limb and genu; M1 in posterior limb) and traverse the midsection of the cerebral peduncle, where SMA fibers are medial, and face, arm, and leg fibers are progressively lateral. Each motor region has unique terminations in the ipsilateral basis pontis and nucleus reticularis tegmenti pontis. Projections are topographically organized, preferentially in the caudal half of the pons, situated in close proximity to traversing corticofugal fibers. In nuclei that receive multiple inputs, terminations appear to interdigitate. Projections from the SMA-face region are most medial and include the median pontine nucleus. M1-face projections are also medial but are lateral to those from SMA-face. Hand projections are in medially placed curved lamellae in mid- and caudal pons. Dorsal trunk projections are in medial and ventral locations. Ventral trunk/hip projections encircle the peduncle in the caudal pons. Foot projections are heaviest caudally in laterally placed, curved lamellae. These results have relevance for anatomical clinical correlations in the human basis pontis. Furthermore, the dichotomy of motor-predominant caudal pons projections to cerebellar anterior lobe, contrasted with associative-predominant rostral pons projections to cerebellar posterior lobe, is consistent with new hypotheses regarding the cerebellar contribution to motor activity and cognitive processing.
Topics: Animals; Autoradiography; Brain Mapping; Cerebral Cortex; Face; Foot; Functional Laterality; Hand; Macaca mulatta; Motor Neurons; Neural Pathways; Pons; Radioisotopes
PubMed: 15368534
DOI: 10.1002/cne.20286 -
Current Opinion in Neurobiology Dec 2005Smooth-pursuit eye movements are used to stabilize the image of a moving object of interest on the fovea, thus guaranteeing its high-acuity scrutiny. Such movements are... (Review)
Review
Smooth-pursuit eye movements are used to stabilize the image of a moving object of interest on the fovea, thus guaranteeing its high-acuity scrutiny. Such movements are based on a phylogenetically recent cerebro-ponto-cerebellar pathway that has evolved in parallel with foveal vision. Recent work has shown that a network of several cerebrocortical areas directs attention to objects of interest moving in three dimensions and reconstructs the trajectory of the target in extrapersonal space, thereby integrating various sources of multimodal sensory and efference copy information, as well as cognitive influences such as prediction. This cortical network is the starting point of a set of parallel cerebrofugal projections that use different parts of the dorsal pontine nuclei and the neighboring rostral nucleus reticularis tegmenti pontis as intermediate stations to feed two areas of the cerebellum, the flocculus-paraflocculus and the posterior vermis, which make mainly complementary contributions to the control of smooth pursuit.
Topics: Animals; Cerebellum; Cerebral Cortex; Efferent Pathways; Humans; Learning; Pons; Pursuit, Smooth
PubMed: 16271460
DOI: 10.1016/j.conb.2005.10.013