scholarly journals Diagnostic imaging in patients with mucopolysaccharidosis: important imaging patterns.

2021 ◽  
Author(s):  
Igor Yarmola ◽  
Anatoly Anikin ◽  
Lyubov Fomina
Keyword(s):  
Structural Changes ◽  
Craniovertebral Junction ◽  
Diagnostic Methods ◽  
Imaging Method ◽  
Perivascular Spaces ◽  
Focal Lesions ◽  
Brain Substance ◽  
Structured Information ◽  
Analysis Of The Images ◽  
The Brain

Background: the need for systematization, generalization and analysis of structural changes in various organs and systems that occur in patients with mucopolysaccharidosis (MPS). MPS is a rare disease, therefore, there is a lack of structured information in the field of radiology among Russian publications. Aims: The puspose of the study is to summarize our own experience, compare it with the literature data, and describe the most significant changes and their causes in MPS. Methods: Retrospectively, 303 children with MPS were examined (the sample included 70 cases verified by laboratory and molecular genetics) of different types, revision of tomograms and radiographs was carried out among studies from 2015 to 2021. All patients underwent MRI of the brain and cervical spine, X-ray of the bones of the skeleton. Results: Analysis of the images obtained revealed the most common changes, such as dysostosis (in 100%, 70 patients), stenosis of the spinal canal at the craniovertebral level (73%, 51 patients), atrophy (47%, 33 patients) and focal lesions of the brain substance ( 67%, 47 patients), hydrocephalus (28%, 20 patients), expansion of the perivascular spaces (70%, 58 patients). The pathophysiological mechanisms of the occurrence of structural changes have been analyzed and described. Conclusions: The assessment and comparison of various diagnostic methods for different organs and systems was carried out: for the assessment of the craniovertebral junction, the most informative imaging method is MRI. Given the lower radiation exposure compared to computed tomography, it is preferable to use digital radiography for examining the bones of the extremities.

scholarly journals Special aspects of the course and diagnosis of neurosyphilis on the example of a clinical case

2021 ◽  
Keyword(s):  
Cerebrospinal Fluid ◽  
Clinical Case ◽  
Structural Changes ◽  
Treponema Pallidum ◽  
State Institution ◽  
Neurological Status ◽  
Diagnostic Methods ◽  
Cerebrospinal Fluid Analysis ◽  
Fluid Analysis ◽  
The Brain

Neurosyphilis is an infectious disease caused by Treponema pallidum and characterized by damage of the central nervous system. This disease may be asymptomatic or have an atypical clinical course, which leads to late diagnosis. The most informative diagnostic methods for this disease are specific serological reactions to syphilis, MRI of the brain and cerebrospinal fluid analysis. Aim. To show the features of the course, treatment and diagnosis of neurosyphilis using the example of a clinical case. Materials and methods. Patient S., born in 1963, complained about significant memory impairment, difficulties with orientation in time and space, mood swings, verbosity and exaggeration, and was hospitalized at the State Institution “Institute of Neurology, Psychiatry and Narcology of the National Academy of Medical Sciences of Ukraine”. Neurological status: eye slits and pupils were uniform. The movements of the eyeballs were painless. Insufficiency of the act of convergence was found. Corneal reactions were reduced. The patient felt pain after the palpation of supra- and infraorbital points. There was an asymmetry in the facial innervation. The tongue was on the midline, swollen, with tooth imprints. There were no pathological signs, sensitive violations. Shaking movements were noticed during the Romberg test. During the examination of the cognitive function using the Mini-Mental State Examination (MMSE) scale, the patient scored 21 points, which corresponds to mild dementia. Results. 1. According to the results of MRI examination of the brain, there was an MR-picture of areas of cystic-gliosis transformation of the poles of the temporal lobes and structural changes of the hippocampal gyrus (most likely, caused by the chronic inflammatory process); vascular foci of the brain as manifestations of dyscirculatory changes, moderate external hydrocephalus. 2. A serological examination for the presence of the antigen of the Treponema pallidum pathogen was performed, the result was positive. 3. Cerebrospinal fluid analysis revealed the following results. Cytosis was 1x106/l, protein was 0.21 g/l, glucose 3.4 mmol/l, Pandy test positivity. Based on the obtained data, the patient was diagnosed with neurosyphilis. The patient underwent etiopathogenetic treatment with benzylpenicillin sodium. After treatment the patient's condition gradually improved. Conclusions. Specific serological reactions to syphilis, MRI of the brain and cerebrospinal fluid analysis are mandatory tests for the diagnosis of neurosyphilis. Early detection of Treponema pallidum and rational therapy can prevent the development of severe consequences and improve the patient's condition. Syphilis is a multidisciplinary problem today and needs the attention of general practitioners, dermatologists and neurologists.

scholarly journals Links of pathogenesis of pre- and perinatal damage of the children’s brain

2019 ◽  
Vol 16 (1) ◽  
pp. 88-92
Author(s):  
M. G. Devyaltovskaya
Keyword(s):  
Calcium Binding ◽  
Nervous Tissue ◽  
Structural Damage ◽  
Structural Changes ◽  
Periventricular Leukomalacia ◽  
S100 Protein ◽  
Brain Substance ◽  
Autoimmune Processes ◽  
Destructive Processes ◽  
The Brain

A correlation analysis was made of the relationships between the structural changes in the brain and the content of idiotypic, anti-idiotypic antibodies to the soluble calcium-binding protein of the nervous tissue S100 in the serum of 318 children of the frst year of life with consequences of pre- and perinatal brain damage. We established the conjugation between the structural pathology of the brain, represented by cystic-atrophic changes, calcifcations, glia in the brain substance, periventricular leukomalacia, periventricular cysts, expansion of the ventricular system, congenital malformations of the brain, and the content of idiotypic, anti-idiotypic antibodies to the S100 protein in the serum blood of children 3, 6, 9, 12 months old. The concentration of idiotypic and anti-idiotypic antibodies to the protein of the nervous tissue S100 in the serum reflects the severity of destructive processes in the brain substance. Autoimmune processes are one of the mechanisms that lead to the structural damage to the brain in children with adverse pre- and perinatal factors.

Сauses of Dizziness in Patients with Suspected Stroke

2018 ◽  
Vol 7 (3) ◽  
pp. 217-221
Author(s):  
E. V. Shevchenko ◽  
G. R. Ramazanov ◽  
S. S. Petrikov
Keyword(s):  
Magnetic Resonance Imaging ◽  
Magnetic Resonance ◽  
Demyelinating Disease ◽  
Heart Rhythm ◽  
Diagnostic Methods ◽  
Deficiency Anemia ◽  
Resonance Imaging ◽  
Peripheral Vestibulopathy ◽  
Suspected Stroke ◽  
The Brain

Background Acute dizziness may be the only symptom of stroke. Prevalence of this disease among patients with isolated dizziness differs significantly and depends on study design, inclusion criteria and diagnostic methods. In available investigations, we did not find any prospective studies where magnetic resonance imaging, positional maneuvers, and Halmagyi-Curthoys test had been used to clarify a pattern of diseases with isolated acute dizziness and suspected stroke.Aim of study To clarify the pattern of the causes of dizziness in patients with suspected acute stroke.Material and methods We examined 160 patients admitted to N.V. Sklifosovsky Research Institute for Emergency Medicine with suspected stroke and single or underlying complaint of dizziness. All patients were examined with assessment of neurological status, Dix-Hollpike and Pagnini-McClure maneuvers, HalmagyiCurthoys test, triplex scans of brachiocephalic arteries, transthoracic echocardiography, computed tomography (CT) and magnetic resonance imaging (MRI) of the brain with magnetic field strength 1.5 T. MRI of the brain was performed in patients without evidence of stroke by CT and in patients with stroke of undetermined etiology according to the TOAST classification.Results In 16 patients (10%), the cause of dizziness was a disease of the brain: ischemic stroke (n=14 (88%)), hemorrhage (n=1 (6%)), transient ischemic attack (TIA) of posterior circulation (n=1 (6%)). In 70.6% patients (n=113), the dizziness was associated with peripheral vestibulopathy: benign paroxysmal positional vertigo (n=85 (75%)), vestibular neuritis (n=19 (17%)), Meniere’s disease (n=7 (6%)), labyrinthitis (n=2 (1,3%)). In 6.9% patients (n=11), the cause of dizziness was hypertensive encephalopathy, 1.9% of patients (n=3) had heart rhythm disturbance, 9.4% of patients (n=15) had psychogenic dizziness, 0.6% of patients (n=1) had demyelinating disease, and 0.6% of patients (n=1) had hemic hypoxia associated with iron deficiency anemia.Conclusion In 70.6% patients with acute dizziness, admitted to hospital with a suspected stroke, peripheral vestibulopathy was revealed. Only 10% of patients had a stroke as a cause of dizziness.

Native Ubiquitin Structural Changes Resulting from Complexation with β-methylamino-L-alanine (BMAA)

2020 ◽  
Author(s):  
Katie Mae Wilson ◽  
Aurora Burkus-Matesevac ◽  
Samuel Maddox ◽  
Christopher Chouinard
Keyword(s):  
Structural Changes ◽  
Noncovalent Complex ◽  
Unfolded Protein ◽  
Protein Size ◽  
High Concentrations ◽  
The Unfolded Protein Response ◽  
Critical Binding ◽  
Protein Response ◽  
The Brain ◽  
Lateral Sclerosis

β-methylamino-L-alanine (BMAA) has been linked to the development of neurodegenerative (ND) symptoms following chronic environmental exposure through water and dietary sources. The brains of those affected by this condition, often referred to as amyotrophic lateral sclerosis-parkinsonism-dementia complex (ALS-PDC), have exhibited the presence of plaques and neurofibrillary tangles (NFTs) from protein aggregation. Although numerous studies have sought to better understand the correlation between BMAA exposure and onset of ND symptoms, no definitive link has been identified. One prevailing hypothesis is that BMAA acts a small molecule ligand, complexing with critical proteins in the brain and reducing their function. The objective of this research was to investigate the effects of BMAA exposure on the native structure of ubiquitin. We hypothesized that formation of a Ubiquitin+BMAA noncovalent complex would alter the protein’s structure and folding and ultimately affect the ubiquitinproteasome system (UPS) and the unfolded protein response (UPR). Ion mobility-mass spectrometry revealed that at sufficiently high concentrations BMAA did in fact form a noncovalent complex with ubiquitin, however similar complexes were identified for a range of additional amino acids. Collision induced unfolding (CIU) was used to interrogate the unfolding dynamics of native ubiquitin and these Ubq-amino acid complexes and it was determined that complexation with BMAA led to a significant alteration in native protein size and conformation, and this complex required considerably more energy to unfold. This indicates that the complex remains more stable under native conditions and this may indicate that BMAA has attached to a critical binding location.

Native Ubiquitin Structural Changes Resulting from Complexation with β-methylamino-L-alanine (BMAA)

2020 ◽  
Author(s):  
Katie Mae Wilson ◽  
Aurora Burkus-Matesevac ◽  
Samuel Maddox ◽  
Christopher Chouinard
Keyword(s):  
Structural Changes ◽  
Noncovalent Complex ◽  
Unfolded Protein ◽  
Protein Size ◽  
High Concentrations ◽  
The Unfolded Protein Response ◽  
Critical Binding ◽  
Protein Response ◽  
The Brain ◽  
Lateral Sclerosis

β-methylamino-L-alanine (BMAA) has been linked to the development of neurodegenerative (ND) symptoms following chronic environmental exposure through water and dietary sources. The brains of those affected by this condition, often referred to as amyotrophic lateral sclerosis-parkinsonism-dementia complex (ALS-PDC), have exhibited the presence of plaques and neurofibrillary tangles (NFTs) from protein aggregation. Although numerous studies have sought to better understand the correlation between BMAA exposure and onset of ND symptoms, no definitive link has been identified. One prevailing hypothesis is that BMAA acts a small molecule ligand, complexing with critical proteins in the brain and reducing their function. The objective of this research was to investigate the effects of BMAA exposure on the native structure of ubiquitin. We hypothesized that formation of a Ubiquitin+BMAA noncovalent complex would alter the protein’s structure and folding and ultimately affect the ubiquitinproteasome system (UPS) and the unfolded protein response (UPR). Ion mobility-mass spectrometry revealed that at sufficiently high concentrations BMAA did in fact form a noncovalent complex with ubiquitin, however similar complexes were identified for a range of additional amino acids. Collision induced unfolding (CIU) was used to interrogate the unfolding dynamics of native ubiquitin and these Ubq-amino acid complexes and it was determined that complexation with BMAA led to a significant alteration in native protein size and conformation, and this complex required considerably more energy to unfold. This indicates that the complex remains more stable under native conditions and this may indicate that BMAA has attached to a critical binding location.

scholarly journals The association between biomarkers in cerebrospinal fluid and structural changes in the brain in patients with Alzheimer's disease

2013 ◽  
Vol 275 (4) ◽  
pp. 418-427 ◽  
Author(s):  
X. Li ◽  
T.-Q. Li ◽  
N. Andreasen ◽  
M. K. Wiberg ◽  
E. Westman ◽  
...  
Keyword(s):  
Alzheimer’S Disease ◽  
Alzheimer's Disease ◽  
Cerebrospinal Fluid ◽  
Structural Changes ◽  
The Brain

Differential diagnosis of spontaneous intracranial hemorrhage in young

2021 ◽  
Vol 22 (1) ◽  
pp. 83-86
Author(s):  
O. A. Kicherova ◽  
◽  
L. I. Reikhert ◽  
O. N. Bovt ◽  
◽  
...  
Keyword(s):  
Hemorrhagic Stroke ◽  
Young Patients ◽  
Vascular Diseases ◽  
Cerebrovascular Diseases ◽  
Intracerebral Hematoma ◽  
Toxic Substances ◽  
Brain Substance ◽  
Secondary All ◽  
Visualization Techniques ◽  
The Brain

In recent years, cerebral vascular diseases have been increasingly detected in young patients. It is due not only to better physicians’ knowledge about this pathology, but also to the improvement of its diagnosis methods. Modern neuroimaging techniques allow us to clarify the nature of hemorrhage, to determine the volume and location of intracerebral hematoma, and to establish the degree of concomitant edema and dislocation of the brain. However, despite the high accuracy of the research, it is not always possible to establish the cause that led to a brain accident, which greatly affects the tactics of management and outcomes in this category of patients. A special feature of the structure of cerebrovascular diseases of young people is the high proportion of hemorrhagic stroke, the causes of which are most often arterio-venous malformations. Meanwhile, there are a number of other causes that can lead to hemorrhage into the brain substance. These include disorders of blood clotting, and various vasculitis, and exposure to toxic substances and drugs, and tumor formations (primary and secondary). All these pathological factors outline the range of diagnostic search in young patients who underwent hemorrhagic stroke. Diagnosis of these pathological conditions with the help of modern visualization techniques is considered to be easy, but this is not always the case. In this article, the authors give their own clinical observation of a hemorrhagic stroke in a young patient, which demonstrates the complexity of the diagnostic search in patients with this pathology.

Physiology and Clinical Relevance of Enlarged Perivascular Spaces in the Aging Brain

Neurology ◽  
2021 ◽  
pp. 10.1212/WNL.0000000000013077
Author(s):  
Corey W Bown ◽  
Roxana O Carare ◽  
Matthew S Schrag ◽  
Angela L Jefferson
Keyword(s):  
Fluid Transport ◽  
Small Vessel Disease ◽  
Treatment Strategies ◽  
Small Vessel ◽  
Aging Brain ◽  
Perivascular Spaces ◽  
Vessel Disease ◽  
Clinical Consequences ◽  
The Brain

Perivascular spaces (PVS) are fluid filled compartments that are part of the cerebral blood vessel wall and represent the conduit for fluid transport in and out of the brain. PVS are considered pathologic when sufficiently enlarged to be visible on magnetic resonance imaging. Recent studies have demonstrated that enlarged PVS (ePVS) may have clinical consequences related to cognition. Emerging literature points to arterial stiffening and abnormal protein aggregation in vessel walls as two possible mechanisms that drive ePVS formation. In this review, we describe the clinical consequences, anatomy, fluid dynamics, physiology, risk factors, and in vivo quantification methods of ePVS. Given competing views of PVS physiology, we detail the two most prominent theoretical views and review ePVS associations with other common small vessel disease markers. As ePVS are a marker of small vessel disease and ePVS burden is higher in Alzheimer’s disease, a comprehensive understanding about ePVS is essential in developing prevention and treatment strategies.

Ultrastructural changes of Basolateral Amygdaloid nuclei in the Sprague Dawley rats brain following exposure to naphthalene balls

2021 ◽  
Vol 12 (2) ◽  
pp. 1272-1275
Author(s):  
Angu Bala Ganesh K S V ◽  
Sujeet Shekhar Sinha ◽  
Kesavi Durairaj ◽  
Abdul Sahabudeen K
Keyword(s):  
Structural Changes ◽  
Corn Oil ◽  
Chromatin Condensation ◽  
Ultrastructural Changes ◽  
High Dose ◽  
Model Group ◽  
Sprague Dawley ◽  
Sprague Dawley Rats ◽  
The Brain ◽  
Amygdaloid Nuclei

Naphthalene is a bicyclic aromatic constituent commonly used in different domestic and marketable applications comprising soil fumigants, lavatory scent disks and mothballs. Accidentally, workers, children and animals are exposed to naphthalene mothballs, so there is a need to study the pathology behind this chemical toxicity. The current study was carried out to assess the ultra structural changes of basolateral amygdaloid nuclei in the Sprague Dawley rats brain in association to naphthalene toxicity. The toxicity model group was administered with naphthalene (200 and 400mg) using corn oil as a vehicle for 28 days. The post delayed toxicity of naphthalene high dose ingestion was also assessed in rats. After the experimental period, the brain tissue was processed to observe the ultra structural changes using a transmission electron microscope. The alterations in cell organelles, nuclei damage, mitochondrial swelling, chromatin condensation suggested naphthalene induced damage in the neurons of the basolateral amygdala of the brain in the toxicity model group. These experimental trials provide information about the alert of mothball usage in the home and identify risks linked with accidental exposure and misuse.

Perivascular (Virchow–Robin) spaces

2021 ◽  
pp. 86-89
Keyword(s):  
Blood Vessels ◽  
Shape Change ◽  
Lymphatic Drainage ◽  
Brain Regions ◽  
Mechanical Trauma ◽  
Normal Brain ◽  
Perivascular Spaces ◽  
Mr Images ◽  
Lenticulostriate Artery ◽  
The Brain

Perivascular spaces; also known as the Virchow-Robin Spaces, they are pleurally lined, interstitial fluid-filled areas that surround certain blood vessels in various organs, especially the perforating arteries in the brain, with an immunological function. Dilated perivascular spaces are divided into three types. The first of these is on the lenticulostriate artery, the second is in the cortex following the path of the medullary artery, and the third is in the midbrain. Perivascular spaces can be detected as areas of dilatation on MR images. Although a limited number of perivascular spaces can be seen in a normal brain, the increase in the number of these spaces has been associated with the incidence of various neurodegenerative diseases. Different theories have been suggested about the tendency of the perivascular spaces to expand. Current theories include mechanical trauma due to cerebrospinal fluid pulsing, elongation of penetrating blood vessels, unusual vascular permeability, and increased fluid exudation. In addition, the brain tissue atrophy that occurs with aging; It is thought to contribute to the widening of perivascular spaces by causing shrinkage of arteries, altered arterial wall permeability, obstruction of lymphatic drainage pathways and vascular demyelination. It is assumed that the clinical significance of the dilation tendencies of the perivascular spaces is based on shape change rather than size. These spaces have been mostly observed in brain regions such as corpus callosum, cingulate gyrus, dentate nucleus, substantia nigra and various arterial basins including lenticulostriate artery and mesencephalothalamic artery. In conclusion, when sections are taken on MR imaging, it is possible that perivascular spaces may be confused with microvascular diseases and some neurodegenerative changes. In addition, perivascular spaces can be seen without pathological significance. Therefore, it would be appropriate to investigate the etiological relationship by evaluating the radiological findings and clinical picture together.

Sign in / Sign up

Export Citation Format

Share Document