Experience of joint management of patient with Huntington's chorea by neurological and dental specialists with use of tetrabenazine

2021 ◽  
pp. 51-54
Author(s):  
M. G. Sokolova ◽  
V. A. Shavurov ◽  
G. I. Shvartsman ◽  
D. A. Pitolenko ◽  
N. S. Sotnikov
Keyword(s):  
General Anesthesia ◽  
Dental Care ◽  
Hereditary Disease ◽  
Huntington's Chorea ◽  
Hyperkinetic Syndrome ◽  
Gene Encoding ◽  
Basal Nuclei ◽  
Frequent Use ◽  
Dental Intervention ◽  
The Central Nervous System

Huntington's chorea is a hereditary disease causing progressive degeneration of the central nervous system with the damage to extrapyramidal structures: basal nuclei, striatum, subthalamic nucleus with increased activity of the central dopaminergic pathways, with the development of neurological, psychiatric, and emotional/personality disorders [1, 17]. The inheritance pattern of the disorder is autosomal dominance. The prevalence of Huntington's disease ranges from 3 to 17 cases per 100,000 population, averaging 5–7 cases per 100,000 population in Russia [2]. The development of the disease is associated with the expansion of CAG (cytosine-adenine-guanine) trinucleotide repeats in the frst exon of the HTT gene encoding the huntingtin protein. This expansion of trinucleotides (long sections of glutamine residues) leads to the selective loss of neurons that connect the striatum and the globus pallidus. This leads to a loss of inhibitory activity and an increase in the excitation impulse, which leads to uncontrolled movements.Unfortunately, medical treatment does not slow down the progression of this disease (a lethal outcome occurs within 15–20 years). Improvement of the quality of life of people with Huntington's chorea, with the provision of medical services at an appropriate level, remains an urgent issue. This direction is especially relevant in providing dental care to patients with Huntington's chorea. Due to the pronounced hyperkinetic syndrome and compulsive movements in the muscles of the arms, trunk, neck and face, it is almost impossible to provide dental care for these patients. Currently, general anesthesia is used to enable dental intervention, but patients note that with frequent use of anesthesia, the patient's condition deteriorates, with an increase in hyperkinetic symptoms. Tetrabenazine is known to reduce the severity of hyperkinetic symptoms and is used in many countries [5].However, in our country many specialists are not familiar with it. During the follow-up of a patient with Huntington's chorea, with the selection of a therapeutic dose of tetrabenazine, it was possible to provide three stages of dental care for the patient without the use of general anesthesia. The material presented in the article can provide useful information on the use of tetrabenazine in patients with Huntington's chorea.

scholarly journals Acid-beta-glycerophosphatase reaction products in the central nervous system mitochondria following x-ray irradiation.

1975 ◽  
Vol 23 (6) ◽  
pp. 402-410 ◽  
Author(s):  
L Roizin ◽  
D Orlovskaja ◽  
J C Liu ◽  
A L Carsten
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Acid Phosphatase ◽  
Enzyme Reaction ◽  
Reaction Products ◽  
Huntington's Chorea ◽  
X Ray ◽  
System A ◽  
Chemical Lesion ◽  
The Central Nervous System

A survey of the literature to date on the enzyme histochemistry of intracellular organelles has not yielded any reference to the presence of acid phosphatase reaction products in the mammalian mitochondria of the central nervous system. A combination of Gomori's acid phosphatase mehtod, however, with standard electron microscopy has disclosed the presence of enzyme reaction products in the mitochondria of the central nervous system of rats from 2 hr to 22 weeks after x-ray irradiation, as well as in a cerebral biopsy performed on a patient affected by Huntington's chorea. No enzyme reaction products, on the other hand, were observed in serial sections that had been incubated in substrates either containing sodium fluoride or lacking in beta-glycerophosphate. The abnormal mitochondrial enzyme reaction (chemical lesion) is considered to be the consequenco of the pathologic process affecting the ultrastructural-chemical organization of the organelle.

scholarly journals Expression of a Drosophila melanogaster acetylcholine receptor-related gene in the central nervous system.

1988 ◽  
Vol 8 (2) ◽  
pp. 778-785 ◽  
Author(s):  
S C Wadsworth ◽  
L S Rosenthal ◽  
K L Kammermeyer ◽  
M B Potter ◽  
D J Nelson
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Drosophila Melanogaster ◽  
Acetylcholine Receptor ◽  
Optic Lobe ◽  
Cdna Probe ◽  
Amino Acid Sequence Homology ◽  
Gene Encoding ◽  
Salivary Gland Polytene Chromosome ◽  
The Central Nervous System

We isolated Drosophila melanogaster genomic sequences with nucleotide and amino acid sequence homology to subunits of vertebrate acetylcholine receptor by hybridization with a Torpedo acetylcholine receptor subunit cDNA probe. Five introns are present in the portion of the Drosophila gene encoding the unprocessed protein and are positionally conserved relative to the human acetylcholine receptor alpha-subunit gene. The Drosophila genomic clone hybridized to salivary gland polytene chromosome 3L within region 64B and was termed AChR64B. A 3-kilobase poly(A)-containing transcript complementary to the AChR64B clone was readily detectable by RNA blot hybridizations during midembryogenesis, during metamorphosis, and in newly enclosed adults. AChR64B transcripts were localized to the cellular regions of the central nervous system during embryonic, larval, pupal, and adult stages of development. During metamorphosis, a temporal relationship between the morphogenesis of the optic lobe and expression of AChR64B transcripts was observed.

FEATURES OF THE PRODUCTION TECHNOLOGY LOW-PROTEIN FOODS

2021 ◽  
Vol 14 (3(53)) ◽  
pp. 98-104
Author(s):  
Alexey Iosifovich Grigel
Keyword(s):  
Amino Acids ◽  
Central Nervous System ◽  
Nervous System ◽  
Life Support ◽  
Hereditary Disease ◽  
Bakery Products ◽  
Severe Damage ◽  
Low Protein ◽  
The Central Nervous System ◽  
Manufacturing Technologies

Phenylketonuria is a hereditary disease associated with a violation of the metabolism of amino acids, in particular phenylalanine. It is accompanied by the accumulation of phenylalanine and its toxic products in the tissues, which leads to severe damage to the central nervous system, manifested, in particular, in a violation of mental development. An important component of the life support of such patients is the observance of a low-protein diet. The article describes the features of manufacturing technologies for lowprotein products, including such as low-protein pasta and cereals, low-protein dry mixes, small-piece bakery products. The technological process and equipment for the production of low-protein products are described, as well as the characteristics of the intake range of products are given.

Inductive interactions direct early regionalization of the mouse forebrain

Development ◽  
1997 ◽  
Vol 124 (14) ◽  
pp. 2709-2718 ◽  
Author(s):  
K. Shimamura ◽  
J.L. Rubenstein
Keyword(s):  
Sonic Hedgehog ◽  
Bone Morphogenetic Proteins ◽  
Molecular Mechanisms ◽  
Neural Plate ◽  
Gene Encoding ◽  
Prechordal Plate ◽  
Optic Vesicles ◽  
The Central Nervous System ◽  
Anterior Neural Plate ◽  
Anterior Posterior

The cellular and molecular mechanisms that regulate regional specification of the forebrain are largely unknown. We studied the expression of transcription factors in neural plate explants to identify tissues, and the molecules produced by these tissues, that regulate medial-lateral and local patterning of the prosencephalic neural plate. Molecular properties of the medial neural plate are regulated by the prechordal plate perhaps through the action of Sonic Hedgehog. By contrast, gene expression in the lateral neural plate is regulated by non-neural ectoderm and bone morphogenetic proteins. This suggests that the forebrain employs the same medial-lateral (ventral-dorsal) patterning mechanisms present in the rest of the central nervous system. We have also found that the anterior neural ridge regulates patterning of the anterior neural plate, perhaps through a mechanism that is distinct from those that regulate general medial-lateral patterning. The anterior neural ridge is essential for expression of BF1, a gene encoding a transcription factor required for regionalization and growth of the telencephalic and optic vesicles. In addition, the anterior neural ridge expresses Fgf8, and recombinant FGF8 protein is capable of inducing BF1, suggesting that FGF8 regulates the development of anterolateral neural plate derivatives. Furthermore, we provide evidence that the neural plate is subdivided into distinct anterior-posterior domains that have different responses to the inductive signals from the prechordal plate, Sonic Hedgehog, the anterior neural ridge and FGF8. In sum, these results suggest that regionalization of the forebrain primordia is established by several distinct patterning mechanisms: (1) anterior-posterior patterning creates transverse zones with differential competence within the neural plate, (2) patterning along the medial-lateral axis generates longitudinally aligned domains and (3) local inductive interactions, such as a signal(s) from the anterior neural ridge, further define the regional organization.

Behavioral and neurobiological alterations induced by chronic use of crack cocaine

2019 ◽  
Vol 31 (1) ◽  
pp. 59-75
Author(s):  
Bárbara dos Anjos Rosário ◽  
Maria de Fátima Santana de Nazaré ◽  
Débora Estadella ◽  
Daniel Araki Ribeiro ◽  
Milena de Barros Viana
Keyword(s):  
Crack Cocaine ◽  
Crystal Form ◽  
Anterior Cingulate ◽  
Attention And Memory ◽  
Morphological Alterations ◽  
Biochemical Alterations ◽  
Frequent Use ◽  
The Central Nervous System ◽  
Behavioral Sequelae ◽  
Chronic Use

Abstract Crack cocaine is the crystal form of cocaine and can be smoked, and rapidly absorbed, and, in part for this reason, is potently addictive. It is hypothesized that crack cocaine is able to induce important changes in different tissues and organs, and thus dramatically alter behavior. Nevertheless, which alterations in the central nervous system are related to its frequent use is still a matter of discussion. The present study is a literature review of articles published between the years 2008 and 2018 on the theme ‘crack cocaine and brain’ available in PUBMED, MEDLINE, EMBASE, and Google scholar databases. The results show that the use of crack cocaine induces important behavioral, neuroanatomical, and biochemical alterations. The main behavioral sequelae include cognitive and emotional changes, such as increased anxiety and depressive symptoms, attention and memory deficits, and hyperactivity. Among the neurobiological alterations are reductions in the activity of the prefrontal, anterior cingulate cortex, and nucleus accumbens. Molecular changes include decreases in neurotrophic factors and increases in oxidative stress and inflammatory cytokines, which may be responsible for the morphological alterations observed. It is also hypothesized that these neurobiological changes might explain the emotional and cognitive dysfunctions experienced by crack cocaine addicts.

The efficacy of comprehensive dental care for children under general anesthesia

BDJ ◽  
1991 ◽  
Vol 171 (2) ◽  
pp. 56-58 ◽  
Author(s):  
E A O'Sullivan ◽  
M E Curzon
Keyword(s):  
General Anesthesia ◽  
Dental Care ◽  
Dental Care For Children

scholarly journals A dominant connexin43 mutant does not have dominant effects on gap junction coupling in astrocytes

2010 ◽  
Vol 6 (4) ◽  
pp. 213-223 ◽  
Author(s):  
Sameh Wasseff ◽  
Charles K. Abrams ◽  
Steven S. Scherer
Keyword(s):  
Gap Junction ◽  
Lucifer Yellow ◽  
Brain Slices ◽  
Gene Encoding ◽  
Transfected Cells ◽  
Dominant Phenotype ◽  
Sulforhodamine B ◽  
Junction Protein ◽  
The Central Nervous System ◽  
Gap Junction Protein

Dominant mutations in GJA1, the gene encoding the gap junction protein connexin43 (Cx43), cause oculodentodigital dysplasia (ODDD), a syndrome affecting multiple tissues, including the central nervous system (CNS). We investigated the effects of the G60S mutant, which causes a similar, dominant phenotype in mice (Gja1Jrt/+). Astrocytes in acute brain slices from Gja1Jrt/+ mice transfer sulforhodamine-B comparably to that in their wild-type (WT) littermates. Further, astrocytes and cardiomyocytes cultured from Gja1Jrt/+ mice showed a comparable transfer of lucifer yellow to those from WT mice. In transfected cells, the G60S mutant formed gap junction (GJ) plaques but not functional channels. In co-transfected cells, the G60S mutant co-immunoprecipitated with WT Cx43, but did not diminish GJ coupling as measured by dual patch clamp. Thus, whereas G60S has dominant effects, it did not appreciably reduce GJ coupling.

A retrospective review of a service to provide comprehensive dental care under general anesthesia

1995 ◽  
Vol 15 (3) ◽  
pp. 97-101 ◽  
Author(s):  
June H. Nunn ◽  
Gaynor Davidson ◽  
Peter H. Gordon ◽  
John Storrs
Keyword(s):  
General Anesthesia ◽  
Dental Care ◽  
Retrospective Review
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