scholarly journals Non epileptogenic Rasmussen`s encephalitis

2019 ◽  
Vol 98 (Suppl) ◽  
pp. 21-21
Author(s):  
Mario Minor Murakami Junior ◽  
Gabriel Bernardes Baron ◽  
Marcela Usberti Gutierre ◽  
Leandro José Guimarães Filho ◽  
Júlia Chartouni Rodrigues
Keyword(s):  
White Matter ◽  
Caudate Nucleus ◽  
Cerebral Hemisphere ◽  
Brain Inflammation ◽  
Cortical Atrophy ◽  
Current Evidence ◽  
Unknown Etiology ◽  
Left Cerebral Hemisphere ◽  
Rasmussen’S Encephalitis ◽  
The Brain

Introduction: Rasmussen's encephalitis (RE) is a very rare chronic inflammatory unilateral encephalopathy with still unknown etiology. It predominantly affects children under the age of 10, but can also affect adolescents and adults. Current evidence on possible etiologies are divided in two main theories. The first one is based on brain inflammation caused by a reaction to a foreign antigen. The second one is based on an autoimmune disease that is limited to a single hemisphere of the brain. The common presentation is intractable and frequent focal motor seizures, often associated with progressive neurological decline, and progressive unilateral focal cortical atrophy. Progression of the inflammatory process in MRI is considered a good biomarker in RE. Objectives: Report a rare case of a female infant presenting atypical manifestations of uncommon symptoms without seizures caused by Rasmussen's encephalitis. Case Report: A ten-year-old right-handed female presented to the emergency department with sudden onset of abnormal involuntary movements of the right upper and lower limbs, face paresia of the same side and loss of attention without loss of consciousness. The involuntary contractions were brief, random, irregular, and worsened with activity, along with a positive milkmaid grip sign. She also displayed reduced movement speed and hyperactive biceps, triceps, patellar and ankle jerk reflexes. Associated with the motor abnormalities, the girl presented with a recent but progressive decrease in cognitive functions, which caused a decline in school performance and impaired reasoning, and affective blunting. She had no sign of previous neuropsychomotor development abnormality. She was taken to a quaternary hospital where a MRI and a CT of the brain were performed. MRI showed atrophy of the left cerebral hemisphere, predominantly in the operculum and head of the caudate nucleus, hypersignal in the insular cortex and adjacent white matter. CT presented with hypoattenuation of the left frontal lobe and parieto-occipital white matter associated with atrophy of the head of the caudate nucleus of the same side and enlargement of the adjacent fissures and sulcus. A subsequent PET-CT showed a left cerebral hemisphere hypometabolism, predominantly in the basal ganglia, temporal, frontal and insular lobes. Functional evaluation detected diffuse unilateral disorganization of brain electric activity, but no epileptic paroxysmal discharges were found on the electroencephalogram. After hospitalization, human immunoglobulin at the dose 2g/kg/day was used for five days. The girl remained stable, with no complications. After sixteen days, she was discharged with a rehabilitation program and follow-up care.

A discussion of apraxia, aphasia, and gestural language

1984 ◽  
Vol 246 (6) ◽  
pp. R884-R887
Author(s):  
N. Helm-Estabrooks
Keyword(s):  
Sign Language ◽  
Cerebral Hemisphere ◽  
Communication Disorder ◽  
Left Cerebral Hemisphere ◽  
Language Behavior ◽  
Normal Language ◽  
Symbolic Gestures ◽  
The Many ◽  
The Relationship ◽  
The Brain

It is understood that damage to the left cerebral hemisphere in adulthood may result in syndromes of language disturbances called the aphasias. The study of these syndromes sheds light on normal language processes, the relationship between language behavior and the brain, and how best to treat aphasic individuals. Aphasia, for some, is a central communication disorder affecting all symbolic behavior in all modalities (i.e., speech, writing, and gesture). Difficulty producing symbolic gestures on command is called apraxia. Others view aphasia as a manifestation of a motor-sequencing disorder affecting all gestural systems including those required for speech movements. These divergent theories of the underlying nature of aphasia can be tested through examination of deaf individuals who use sign language before onset of aphasia. Poizner et al. [Am. J. Physiol. 246 (Regulatory Integrative Comp. Physiol. 15): R868-R883, 1984] studied three such patients with different aphasia syndromes: one patient had a nonsymbolic, motor-sequencing disorder; one had a gestural apraxia; and one had neither. These findings force the conclusion that neither the symbolic nor motor-sequencing theory of aphasia can account for the many varieties of that disorder.

scholarly journals Glutamine synthetase expression in the brain during experimental acute liver failure (immunohistochemical study)

2021 ◽  
Vol 11 (10) ◽  
pp. 342-356
Author(s):  
T. Shulyatnikova ◽  
V. Tumanskiy
Keyword(s):  
White Matter ◽  
Glutamine Synthetase ◽  
Liver Failure ◽  
Acute Liver Failure ◽  
Caudate Nucleus ◽  
Immunohistochemical Study ◽  
Clinical Signs ◽  
Brain Regions ◽  
Progressive Increase ◽  
The Brain

The aim of the study was to determine the immunohistochemical level of glutamine synthetase (GS) expression in different brain regions in the conditions of experimental acute liver failure in rats. Materials and methods. The study was conducted in Wistar rats: 5 sham (control) animals and 10 rats with acetaminophen induced liver failure model (AILF). The immunohistochemical study of GS expression in the sensorimotor cortex, white matter, hippocampus, thalamus, caudate nucleus/putamen was carried out in the period of 12-24 h after acetaminophen treatment. Results. Beginning from the 6th hour after acetaminophen treatment all AILF-animals showed the progressive increase in clinical signs of acute brain disfunction finished in 6 rats by comatose state up to 24 h - they constituted subgroup AILF-B, “non-survived”. 4 animals survived until the 24 h - subgroup AILF-A, “survived”. In the AILF-B group, starting from 16 to 24 hours after treatment, a significant (relative to control) regionally-specific dynamic increase in the level of GS expression was observed in the brain: in the cortex – by 307.33 %, in the thalamus – by 249.47%, in the hippocampus – by 245.53%, in the subcortical white matter – by 126.08%, from 12th hour – in the caudate nucleus/putamen, by 191.66 %; with the most substantive elevation of GS expression in the cortex: by 4.07 times. Conclusion. Starting from the 16th hours after the acetaminophen treatment (from the 12th h in the caudate nucleus/putamen region) and up to 24 h, it is observed reliable compared to control dynamic increase in GS protein expression in the cortex, white matter, hippocampus, thalamus, caudate nucleus/putamen of the rat brain with the most significant elevation in the cortex among other regions. The heterogeneity in the degree of GS expression rising in different brain regions potentially may indicate regions more permeable for ammonia and/or other systemic toxic factors as well as heterogeneous sensitivity of brain regions to deleterious agents in conditions of AILF. Subsequently, revealed diversity in the GS expression reflects the specificity of reactive response of local astroglia in the condition of AILF-encephalopathy during specific time-period. The dynamic increase in the GS expression associated with impairment of animal state, indicates involvement of increased GS levels in the mechanisms of experimental acute hepatic encephalopathy.

scholarly journals Microsurgical anatomy of the central core of the brain

2018 ◽  
Vol 129 (3) ◽  
pp. 752-769 ◽  
Author(s):  
Eduardo Carvalhal Ribas ◽  
Kaan Yağmurlu ◽  
Evandro de Oliveira ◽  
Guilherme Carvalhal Ribas ◽  
Albert Rhoton
Keyword(s):  
White Matter ◽  
Caudate Nucleus ◽  
Internal Capsule ◽  
Central Core ◽  
Surgical Approaches ◽  
Microsurgical Anatomy ◽  
Functional Importance ◽  
Posterior Portion ◽  
External Capsule ◽  
The Brain

OBJECTIVEThe purpose of this study was to describe in detail the cortical and subcortical anatomy of the central core of the brain, defining its limits, with particular attention to the topography and relationships of the thalamus, basal ganglia, and related white matter pathways and vessels.METHODSThe authors studied 19 cerebral hemispheres. The vascular systems of all of the specimens were injected with colored silicone, and the specimens were then frozen for at least 1 month to facilitate identification of individual fiber tracts. The dissections were performed in a stepwise manner, locating each gray matter nucleus and white matter pathway at different depths inside the central core. The course of fiber pathways was also noted in relation to the insular limiting sulci.RESULTSThe insular surface is the most superficial aspect of the central core and is divided by a central sulcus into an anterior portion, usually containing 3 short gyri, and a posterior portion, with 2 long gyri. It is bounded by the anterior limiting sulcus, the superior limiting sulcus, and the inferior limiting sulcus. The extreme capsule is directly underneath the insular surface and is composed of short association fibers that extend toward all the opercula. The claustrum lies deep to the extreme capsule, and the external capsule is found medial to it. Three fiber pathways contribute to form both the extreme and external capsules, and they lie in a sequential anteroposterior disposition: the uncinate fascicle, the inferior fronto-occipital fascicle, and claustrocortical fibers. The putamen and the globus pallidus are between the external capsule, laterally, and the internal capsule, medially. The internal capsule is present medial to almost all insular limiting sulci and most of the insular surface, but not to their most anteroinferior portions. This anteroinferior portion of the central core has a more complex anatomy and is distinguished in this paper as the “anterior perforated substance region.” The caudate nucleus and thalamus lie medial to the internal capsule, as the most medial structures of the central core. While the anterior half of the central core is related to the head of the caudate nucleus, the posterior half is related to the thalamus, and hence to each associated portion of the internal capsule between these structures and the insular surface. The central core stands on top of the brainstem. The brainstem and central core are connected by several white matter pathways and are not separated from each other by any natural division. The authors propose a subdivision of the central core into quadrants and describe each in detail. The functional importance of each structure is highlighted, and surgical approaches are suggested for each quadrant of the central core.CONCLUSIONSAs a general rule, the internal capsule and its vascularization should be seen as a parasagittal barrier with great functional importance. This is of particular importance in choosing surgical approaches within this region.

scholarly journals Immunohistochemical analysis of GFAP expression in the experimental sepsis-associated encephalopathy

Pathologia ◽  
2021 ◽  
Vol 18 (3) ◽  
pp. 295-302
Author(s):  
T. V. Shulyatnikova ◽  
V. O. Tumaskyi
Keyword(s):  
White Matter ◽  
Caudate Nucleus ◽  
Critical Role ◽  
Brain Regions ◽  
Subcortical White Matter ◽  
Control Group ◽  
Experimental Sepsis ◽  
Respiratory Impairment ◽  
Gfap Expression ◽  
The Brain

Pathophysiology of sepsis-associated encephalopathy (SAE) is linked to blood-brain barrier breakdown, neuroinflammation and neurotransmitter imbalance in the brain. Astroglia, the most abundant cell population within the brain, plays the critical role in control of all kinds of homeostatic processes, thereby regulating the adaptive reactions of the brain to various challenges. Astroglia are highly heterogenous across the brain regions, therefore, damaging factors stimulate heterogenous astroglial reactivity and response in different brain regions. The aim of this study was determining immunohistochemical features of GFAP expression in various brain regions in the model of rodent experimental sepsis. Materials and methods. The experiment was performed in Wistar rats: control group of 5 sham-operated rats and the main group of 20 rats subjected to cecum ligation and puncture (CLP) procedure. The immunohistochemical study of GFAP expression in the sensorimotor cortex, subcortical white matter, hippocampal, thalamic and caudate nucleus/putamen regions was performed from 20 to 48 hours of the postoperative period. Results. Starting from the 12th hour after CLP, animals began display progressive increase in signs of periorbital exudation, piloerection, fever-/hypothermia, diarrhea, social isolation, lethargy, and respiratory impairment. In the period of 20–38 hours, 9 animals showed expressed previously listed symptoms and were euthanized (CLP-B – lethal group), 11 rats survived until 48 hours of the experiment (CLP-A – survived group). In the lethal group, starting from 20 to 38 hours after the CLP procedure, a significant (relative to control) regionally-specific dynamic increase in the level of GFAP expression was observed in the brain: in the cortex – by 465 %, in the subcortical white matter – by 198 %, in the hippocampus – by 250 %, from the 23rd hour – in the caudate nucleus/putamen by 18 %. In the thalamus, no significant changes in the level of GFAP expression were observed. In the cortex and hippocampus of survived animals, 48 h after CLP, higher values of GFAP expression were observed comparing to the group of non-survived animals. Conclusions. Under conditions of the experimental SAE, an early dynamic increase in the astroglial reactivity was observed in the cortex, hippocampus, white matter, and caudate nucleus/putamen of the brain with the most significant increase of indicators in the cortex and hippocampus, which potentially indicates relatively more vulnerable areas of the brain to damaging factors, as well as places of the most active intercellular interaction in the condition of systemic inflammation. Higher values of GFAP expression in the cortex and hippocampus of survived animals at 48 hours of the experiment, compared with indicators of non-survived group, indicate increased astroglial reactivity in these brain regions at the noted time period, accompanied by relatively more favorable clinical course of the disease.  

The effects of probiotics and prebiotics on gastrointestinal and behavioural symptoms in autism spectrum disorder

2021 ◽  
Vol 16 ◽  
Author(s):  
José Guevara-Gonzaléz ◽  
José Guevara-Campos ◽  
Lucía González ◽  
Omar Cauli
Keyword(s):  
Gut Microbiota ◽  
Autism Spectrum ◽  
Current Evidence ◽  
Unknown Etiology ◽  
Behavioural Symptoms ◽  
Beneficial Effects ◽  
Behavioral Abnormalities ◽  
Spectrum Disorders ◽  
Different Strains ◽  
The Brain

Background: Autism spectrum disorders (ASDs) are a group of prevalent neuropsychiatric disorders. They present a complex and unknown etiology, which in most cases includes significant peripheral alterations outside the brain such as in the composition of gut microbiota. Because the gut microbiota is involved in modulating the gut–brain axis, several studies have suggested that the microbiome in the gut can modify metabolites which are able to cross the blood–brain barrier and modulate brain function. Methods: we reviewed the current evidence regarding microbiota alterations in patients with ASD and the effects of the administration of probiotics and prebiotics in these patients, both in terms of gastrointestinal and behavioural symptoms. Results: Administration of a probiotic formulation containing different strains of Lactobacillus (L. acidophilus, L. rhamnosus, and others) and Bifidobacteria had beneficial effects upon these aforementioned symptoms and their use is recommended in a subgroup of ASD patients that present gastrointestinal disturbances, Nonetheless, the types of gastrointestinal disturbances that most benefit from such interventions remains to be elucidated in order to personalize the medical approaches. Conclusion: Recent clinical studies have shown that probiotic treatments can regulate the gut microbiota and may result in improvements in some behavioral abnormalities associated with ASD. Trials using prebiotic fibers or synbiotics preparations are still lacking and necessary in order to deep in such therapeutic strategies in ASD with comorbid gastrointestinal disrturbances

Neuropsychiatric illness and neuropathological findings in a case of Klinefelter's syndrome

1970 ◽  
Vol 1 (1) ◽  
pp. 18-29 ◽  
Author(s):  
A. Jablensky ◽  
I. Janota ◽  
Michael Shepherd
Keyword(s):  
White Matter ◽  
Cortical Atrophy ◽  
Cerebral White Matter ◽  
Klinefelter’S Syndrome ◽  
Klinefelter's Syndrome ◽  
Genetic Abnormalities ◽  
The Brain ◽  
Neuropsychiatric Illness

SUMMARYA case of Klinefelter's syndrome with XXY karyotype and a dementing neuropsychiatric illness associated with a diffuse degeneration of the deep cerebral white matter and adrenal cortical atrophy is presented. The possible relationships between genetic abnormalities and disordered function and structure of the brain are discussed in the light of the clinical and pathological observations.

scholarly journals A Rare Form of Adult Onset Leukodystrophy: Orthochromatic Leukodystrophy with Pigmented Glia

1997 ◽  
Vol 24 (2) ◽  
pp. 146-150 ◽  
Author(s):  
P. Shannon ◽  
J.R. Wherrett ◽  
S. Nag
Keyword(s):  
White Matter ◽  
Rare Condition ◽  
Brain Biopsy ◽  
Hepatic Tissue ◽  
Cerebral White Matter ◽  
Unknown Etiology ◽  
Adult Onset ◽  
Ultrastructural Examination ◽  
Biopsy Material ◽  
The Brain

ABSTRACT:Background:Orthochromatic leukodystrophy with pigmented glia and scavenger cells is a rare leukodystrophy of unknown etiology. This report describes a 42-year-old man with a history of depression, dementia and parkinsonism having the pathological features of orthochromatic leukodystrophy with pigmented glia.Methods:We reviewed the clinical history and pathology of autopsy and brain biopsy material.Results:Imaging revealed bilateral cerebral white matter hypodensities. At autopsy, the brain demonstrated a leukodystrophy affecting predominantly the cerebral hemispheres and characterized by demyelination, and cytoplasmic pigment deposits in oligodendroglia and astrocytes. The pigment had the staining properties of ceroid-lipofuschin and on ultrastructural examination was composed of membrane-bound lipid and electron-dense inclusions which had a fingerprint-like pattern. Similar pigment inclusions were not observed on ultrastructural examination of renal, splenic or hepatic tissue obtained at autopsy. The brain biopsy contained cerebral cortex with sparse subcortical white matter in which a few oligodendroglia and fewer astrocytes at the grey/white junctions showed cytoplasmic pigmentary inclusions identical to those described above. However, due to the paucity of white matter in the specimen a definite diagnosis of orthochromatic leukodystrophy with pigmented glia was not made.Conclusions:The diagnosis of orthochromatic leukodystrophy with pigmented glia and scavenger cells can only be made antemortem if the brain biopsy contains adequate white matter and although a rare condition, it should be considered in the differential diagnosis of an adult onset leukodystrophy.

scholarly journals Clinical case of congenital CMV neuroinfection in a child with selective IgA deficiency

2021 ◽  
Vol 17 (6) ◽  
pp. 51-56
Author(s):  
D.V. Maltsev ◽  
L.I. Melnik ◽  
I.A. Maltseva
Keyword(s):  
White Matter ◽  
Corpus Callosum ◽  
Iga Deficiency ◽  
Cortical Atrophy ◽  
Cmv Infection ◽  
Selective Iga Deficiency ◽  
Temporal Lobes ◽  
Congenital Cmv Infection ◽  
The Brain ◽  
Congenital Cmv

Congenital CMV infection is thought to occur in at least 1 % of infants, although recent clinical studies indicate that these lesions account for 8 % of all neonates. The severity of clinical symptoms of nervous system damage of CMV-etiology depends on the duration of intrauterine infection. In early infection, during the first trimester of the fetal period, severe CNS malformations develop, including anencephaly, porencephaly, schizencephaly, lissencephaly, micropolygyria, and pachygyria. At later infection, during the 2nd–3rd trimesters of pregnancy, there are milder manifestations — ventriculomegaly, impaired myelination of the white matter of the brain, cysts in the poles of the temporal lobes, hypogenesis of the corpus callosum, periventricular calcifications, and lesions of the cochleovestibular nerves. The article presents the medical history of a 5-year-old boy with typical clinical and instrumental signs of congenital CMV infection. The child had deep spastic tetraparesis, severe mental retardation, refractory epileptic syndrome with polymorphic seizures, disorders of pelvic organs, inability to move independently. MRI of the brain showed typical radiological signs of congenital CMV infection: cortical atrophy, ventriculomegaly, periventricular gliosis, demyelination fields in the white matter of the hemispheres, cysts in the poles of the temporal lobes, hypoplasia of the corpus callosum. During the neonatal period, specific IgM to CMV in serum was observed. Blood leukocyte PCR revealed the CMV DNA in a 5-year-old child at the time of admission to the clinic. This infection led to genera-lized lymphadenopathy, hepatosplenomegaly, thrombocytopenia, and lymphomonocytosis. The assessment of immune status showed the presence of selective IgA deficiency, which was associated with the development of this opportunistic infection. Typical mistakes in the clinical management of children with congenital CMV infection and ways to avoid them are discussed.

Left-Cerebral Hemisphere Superiority in Tachistoscopic Word-Recognition Performances

1970 ◽  
Vol 30 (3) ◽  
pp. 763-766 ◽  
Author(s):  
Walter F. McKeever ◽  
Maurice D. Huling
Keyword(s):  
Word Recognition ◽  
Left Hemisphere ◽  
Cerebral Hemisphere ◽  
Right Hemisphere ◽  
Left Cerebral Hemisphere ◽  
Right Cerebral Hemisphere ◽  
Recognition Ability ◽  
Efficient Route ◽  
The Right ◽  
The Brain

Under conditions of monocular unihemispheric projection of word stimuli to the brain, 10 normal Ss uniformly showed superior word recognition ability of the left, as opposed to the right, cerebral hemisphere. Left-hemisphere recognitions were significantly more frequent than right-hemisphere recognitions for both eyes, but the extent of left-hemisphere superiority was significantly greater for the left eye. The results support the hypothesis that words projected to the right hemisphere traverse a less efficient route to the language centers of the left hemisphere.

Sign in / Sign up

Export Citation Format

Share Document