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Case Reports in Neurology 2021Isolated pontine infarction accounts for 7% of all ischemic strokes. Millard-Gubler syndrome is a clinical syndrome which occurs following lesions involving the ventral...
Millard-Gubler Syndrome Associated with Cerebellar Ataxia in a Patient with Isolated Paramedian Pontine Infarction - A Rarely Observed Combination with a Benign Prognosis: A Case Report.
Isolated pontine infarction accounts for 7% of all ischemic strokes. Millard-Gubler syndrome is a clinical syndrome which occurs following lesions involving the ventral portion of the caudal pons, resulting in classic clinical features such as ipsilateral abducens and facial nerve palsy and contralateral hemiparesis. We report the case of a 55-year-old male patient having presented to the Yehuleshet Specialty Clinic 6 years back with sudden-onset dysarthria and appendicular ataxia of 10 days duration. He reported having right hemibody weakness and blurred vision, which have significantly improved since then. He had a history of smoking of 30 pack-years. However, he quit smoking 8 years ago. There was no history of prior stroke, transient ischemic attack, diabetes, hypertension, head trauma, or dyslipidemia. On examination, he had horizontal left gaze palsy with horizontal nystagmus suggesting left-sided 6th cranial nerve palsy. He had mild left-sided facial palsy causing dysarthric speech. Right upper limb dysmetria was observed during examination; otherwise, motor, sensory, fundus, and gait examination results were normal. He had low serum vitamin D. Brain magnetic resonance imaging examination showed a 25 × 10 mm segmental lesion in the left median pons involving the basis pontis and tegmentum section. The lesion had T2 and T1 abnormal prolongation with no diffusion restriction, suggesting a subacute pontine infarct. The patient was managed with aspirin 325 mg, atorvastatin 80 mg, physical therapy, and vitamin D supplementation, and advised on behavioral risk factors. Six years after his isolated pontine infarction, the patient is fully recovered from dysarthria, facial palsy, hemiparesis, right-sided appendicular ataxia, and horizontal nystagmus, and the follow-up brain MRI showed radiological evidence of chronic paramedian pontine perforator infarction. Millard-Gubler syndrome may present with cerebellar ataxia if the paramedian pontine infarction area slightly extends laterally, affecting the middle cerebellar peduncles. Isolated pontine infarction may have a good prognosis if diagnosed and managed early.
PubMed: 33976662
DOI: 10.1159/000515330 -
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 -
The British Journal of Radiology Apr 2021To set age-specific normal reference values for brainstem, cerebellar vermis, and peduncles measurements and characterize values' variations according to gender, age,...
OBJECTIVES
To set age-specific normal reference values for brainstem, cerebellar vermis, and peduncles measurements and characterize values' variations according to gender, age, and age by gender interaction.
METHODS
565 normal brain magnetic resonance examinations with normal anatomy and signal intensity of the supra- and infratentorial structures were categorized into six age groups (infant, child, adolescent, young adult, middle-age adult, and old aged adults). Patients with congenital malformations, gross pathology of the supra- or infratentorial brain, brain volume loss, developmental delay, metabolic disorders, and neuropsychological disorders ( = 2.839) were excluded. On midsagittal weighted and axial weighted images specific linear diameters and ratios of the brainstem, cerebellar vermis, and peduncles were attained. Two observers assessed a random sample of 100 subjects to evaluate the inter- and intraobserver reproducibility. Intraclass correlation coefficients, means ± standard deviation, one and two-way analysis of variance tests were used in the statistical analysis.
RESULTS
Good to excellent inter- and intraobserver measurements' reproducibility were observed, except for the transverse diameter of the midbrain, the anteroposterior diameter of the medulla oblongata at the pontomedullary and cervicomedullary junctions, cerebellar vermis anteroposterior diameter, and thickness of the superior cerebellar peduncle. Age-specific mean values of the investigated measurements were established. A significant gender-related variation was recorded in the anteroposterior diameter of the basis pontis ( = 0.044), the anteroposterior diameter of the medulla oblongata at the cervicomedullary junction ( = 0.044), and cerebellar vermis height ( = 0.018). A significant age-related change was detected in all measurements except the tectal ratio. Age by gender interaction had a statistically significant effect on the tectal ratio, inferior, and middle cerebellar peduncles' thickness ( = 0.001, 0.022, and 0.028, respectively).
CONCLUSION
This study provides age-specific normal mean values for various linear dimensions and ratios of the posterior fossa structures with documentation of measurements' variability according to gender, age, and their interaction.
ADVANCES IN KNOWLEDGE
It provides a valuable reference in the clinical practice for easier differentiation between physiological and pathological conditions of the posterior fossa structures especially various neurodegenerative diseases and congenital anomalies.
Topics: Adolescent; Adult; Age Factors; Aged; Brain Mapping; Brain Stem; Cerebellar Vermis; Cerebral Peduncle; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Magnetic Resonance Imaging; Male; Middle Aged; Middle Cerebellar Peduncle; Olfactory Cortex; Reference Values; Reproducibility of Results; Young Adult
PubMed: 33571018
DOI: 10.1259/bjr.20201353 -
BMC Microbiology May 2024Spontaneous fermentation of cereals like millet involves a diverse population of microbes from various sources, including raw materials, processing equipment, fermenting...
Spontaneous fermentation of cereals like millet involves a diverse population of microbes from various sources, including raw materials, processing equipment, fermenting receptacles, and the environment. Here, we present data on the predominant microbial species and their succession at each stage of the Hausa koko production process from five regions of Ghana. The isolates were enumerated using selective media, purified, and phenotypically characterised. The LAB isolates were further characterised by 16S rRNA Sanger sequencing, typed using (GTG) repetitive-PCR, and whole genome sequencing, while 28S rRNA Sanger sequencing was performed for yeast identification. The pH of the millet grains ranged from mean values of 6.02-6.53 to 3.51-3.99 in the final product, depending on the processors. The mean LAB and yeast counts increased during fermentation then fell to final counts of log 2.77-3.95 CFU/g for LAB and log 2.10-2.98 CFU/g for yeast in Hausa koko samples. At the various processing stages, the counts of LAB and yeast revealed significant variations (p < 0.0001). The species of LAB identified in this study were Limosilactobacillus pontis, Pediococcus acidilactici, Limosilactobacillus fermentum, Limosilactobacillus reuteri, Pediococcus pentosaceus, Lacticaseibacillus paracasei, Lactiplantibacillus plantarum, Schleiferilactobacillus harbinensis, and Weissella confusa. The yeasts were Saccharomyces cf. cerevisiae/paradoxus, Saccharomyces cerevisiae, Pichia kudriavzevii, Clavispora lusitaniae and Candida tropicalis. The identification and sequencing of these novel isolates and how they change during the fermentation process will pave the way for future controlled fermentation, safer starter cultures, and identifying optimal stages for starter culture addition or nutritional interventions. These LAB and yeast species are linked to many indigenous African fermented foods, potentially acting as probiotics in some cases. This result serves as the basis for further studies into the technological and probiotic potential of these Hausa koko microorganisms.
Topics: Fermentation; Ghana; Yeasts; Food Microbiology; Fermented Foods; Millets; Lactobacillales; RNA, Ribosomal, 16S; Phylogeny; Hydrogen-Ion Concentration; Edible Grain
PubMed: 38745280
DOI: 10.1186/s12866-024-03317-1 -
Internal Medicine (Tokyo, Japan) May 2022We herein report a 46-year-old man presenting with locked-in syndrome secondary to meningovascular syphilis. Brain magnetic resonance imaging (MRI) demonstrated multiple... (Review)
Review
We herein report a 46-year-old man presenting with locked-in syndrome secondary to meningovascular syphilis. Brain magnetic resonance imaging (MRI) demonstrated multiple acute infarctions in the left ventromedial pons, right basis pontis, and left basal ganglia. His locked-in syndrome was hypothesized to have been caused by thrombosis of the small paramedian branches of the basilar artery due to syphilitic arteritis. This is a unique case of bilateral ventromedial pontine infarction caused by meningovascular syphilis that presented as locked-in syndrome. Meningovascular syphilis should be included in the differential diagnosis of uncommon stroke, particularly in young men.
Topics: Basilar Artery; Humans; Locked-In Syndrome; Magnetic Resonance Imaging; Male; Middle Aged; Neurosyphilis; Syphilis
PubMed: 34670896
DOI: 10.2169/internalmedicine.8269-21 -
BMC Endocrine Disorders May 2023Central pontine myelinolysis (CPM) is a rare demyelinating disorder caused by the loss of myelin in the center of the basis pontis. CPM typically occurs with rapid...
BACKGROUND
Central pontine myelinolysis (CPM) is a rare demyelinating disorder caused by the loss of myelin in the center of the basis pontis. CPM typically occurs with rapid correction of severe chronic hyponatremia and subsequent disturbances in serum osmolality. Although hyperglycaemia is recognized as a pathogenetic factor in serum osmolality fluctuations, CPM is rarely seen in the context of diabetes.
CASE PRESENTATION
A 66-year-old Chinese male presented with a history of gait imbalance, mild slurred speech and dysphagia for two weeks. MRI showed the mass lesions in the brainstem, and laboratory examinations showed high blood glucose and HbA1c, as well as increased serum osmolality. The patient was diagnosed with CPM secondary to hyperosmolar hyperglyceamia and received insulin treatment as well as supportive therapy. After six weeks of followup, the patient had fully recovered to a normal state.
CONCLUSION
CPM is a potentially fatal neurological condition and can occur in uncontrolled diabetes mellitus. Early diagnosis and timely treatment are crucial for improving the prognosis.
Topics: Male; Humans; Aged; Myelinolysis, Central Pontine; Hyperglycemia; Hyponatremia; Magnetic Resonance Imaging
PubMed: 37165361
DOI: 10.1186/s12902-023-01361-y -
Life (Basel, Switzerland) Oct 2023Alterations in cerebral glucose metabolism can be indicative of both normal and pathological aging processes. In this retrospective study, we evaluated global and...
Alterations in cerebral glucose metabolism can be indicative of both normal and pathological aging processes. In this retrospective study, we evaluated global and regional neurological glucose metabolism in 73 healthy individuals (mean age: 35.8 ± 13.1 years; 82.5% female) using 18F-Fluorodeoxyglucose (FDG) positron emission tomography/computed tomography (PET/CT). This population exhibited a low prevalence of comorbidities associated with cerebrovascular risk factors. We utilized F-FDG-PET/CT imaging and quantitative regional analysis to assess cerebral glucose metabolism. A statistically significant negative correlation was found between age and the global standardized uptake value mean (SUVmean) of FDG uptake ( = 0.000795), indicating a decrease in whole-brain glucose metabolism with aging. Furthermore, region-specific analysis identified significant correlations in four cerebral regions, with positive correlations in the basis pontis, cerebellar hemisphere, and cerebellum and a negative correlation in the lateral orbital gyrus. These results were further confirmed via linear regression analysis. Our findings reveal a nuanced understanding of how aging affects glucose metabolism in the brain, providing insight into normal neurology. The study underscores the utility of F-FDG-PET/CT as a sensitive tool in monitoring these metabolic changes, highlighting its potential for the early detection of neurological diseases and disorders related to aging.
PubMed: 37895426
DOI: 10.3390/life13102044 -
The Journal of Comparative Neurology Feb 2015Despite its critical importance to global brain function, the postnatal development of the human pons remains poorly understood. In the present study, we first performed...
Despite its critical importance to global brain function, the postnatal development of the human pons remains poorly understood. In the present study, we first performed magnetic resonance imaging (MRI)-based morphometric analyses of the postnatal human pons (0-18 years; n = 6-14/timepoint). Pons volume increased 6-fold from birth to 5 years, followed by continued slower growth throughout childhood. The observed growth was primarily due to expansion of the basis pontis. T2-based MRI analysis suggests that this growth is linked to increased myelination, and histological analysis of myelin basic protein in human postmortem specimens confirmed a dramatic increase in myelination during infancy. Analysis of cellular proliferation revealed many Ki67(+) cells during the first 7 months of life, particularly during the first month, where proliferation was increased in the basis relative to tegmentum. The majority of proliferative cells in the postnatal pons expressed the transcription factor Olig2, suggesting an oligodendrocyte lineage. The proportion of proliferating cells that were Olig2(+) was similar through the first 7 months of life and between basis and tegmentum. The number of Ki67(+) cells declined dramatically from birth to 7 months and further decreased by 3 years, with a small number of Ki67(+) cells observed throughout childhood. In addition, two populations of vimentin/nestin-expressing cells were identified: a dorsal group near the ventricular surface, which persists throughout childhood, and a parenchymal population that diminishes by 7 months and was not evident later in childhood. Together, our data reveal remarkable postnatal growth in the ventral pons, particularly during infancy when cells are most proliferative and myelination increases.
Topics: Adolescent; Analysis of Variance; Basic Helix-Loop-Helix Transcription Factors; Cell Proliferation; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Ki-67 Antigen; Magnetic Resonance Imaging; Male; Myelin Sheath; Nerve Tissue Proteins; Oligodendrocyte Transcription Factor 2; Pons
PubMed: 25307966
DOI: 10.1002/cne.23690 -
AJNR. American Journal of Neuroradiology Aug 1995To report the MR and CT findings in a hereditary disease, infantile-onset spinocerebellar ataxia (IOSCA).
PURPOSE
To report the MR and CT findings in a hereditary disease, infantile-onset spinocerebellar ataxia (IOSCA).
METHODS
We studied the brains of 17 patients with infantile-onset spinocerebellar ataxia with CT and/or MR to determine the presence of cerebellar and brain stem atrophy and parenchymal lesions.
RESULTS
Cerebellar cortical atrophy was seen in 13 patients. The degree of atrophy correlated with increasing age and clinical deterioration. Brain stem atrophy was seen in 8 patients. It was never severe, and the basis pontis was not flattened even in the most severe cases. Hyperintense lesions were noted within the white matter of cerebellum, in the dentate nuclei, and in the middle cerebellar peduncles in 3 patients. The upper cervical cord was seen in 9 patients and showed mild to moderate atrophy in 4. The basal ganglia and cerebral hemispheres were normal, except in 2 patients transient cortical and subcortical lesions developed during episodes of status epilepticus; mild cortical brain atrophy subsequently developed.
CONCLUSION
The brain MR and CT findings of patients with infantile-onset spinocerebellar ataxia correspond to the neuropathologic entities of cerebellar cortical atrophy, olivopontocerebellar atrophy, and spinocerebellar atrophy. The appearance of the findings followed a uniform time sequence from cerebellar cortical atrophy in the early stage of the disease to olivopontocerebellar atrophy and spinocerebellar atrophy in the later stage. The severity of atrophy correlated with clinical deterioration.
Topics: Adolescent; Adult; Atrophy; Brain; Brain Stem; Cerebellar Cortex; Cerebellar Nuclei; Cerebral Cortex; Child; Child, Preschool; Dominance, Cerebral; Female; Follow-Up Studies; Humans; Infant; Magnetic Resonance Imaging; Male; Spinal Cord; Spinocerebellar Degenerations; Tomography, X-Ray Computed
PubMed: 7484627
DOI: No ID Found