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

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.

Insertion of Occupational Therapy Means in the Complex Treatment of the Child with Hyperkinetic Syndrome

Hyperkinetic syndrome is a biological disorder, characterized by an imbalance in the brain, which prevents it from functioning at normal parameters. It is a disorder that affects many people and at maturity. The disorder has a negative effect on school learning and influences the social integration of the child. A person's mental, emotional and communication skills are severely impaired. The research methodology refers to the theoretical principles, methods and techniques of data collection, as well as to the instruments, the procedures for analyzing and evaluating the case. Following the use of therapy in the complex intervention process, it was found that the client became more friendly, cheerful and very keen when it comes to other activities both inside and outside the center. In the reassessment of the problems of occupational performance, very considerable changes have been registered both in the satisfaction of the client and in his performance.

Clinical and pathogenetic features and correction of hyperkinetic disorders in vegetative dysfunctions

One of the early syndromes in vascular pathology is motor disorders in the form of tremor. We examined 101 patients with cerebral dystonia on the background of chronic cerebral ischemia with compensated and subcompensated stages (n = 55) and autonomic dystonia syndrome (n = 46). Pathological mechanisms of formation of trembling hyperkinesis in cerebral angiodystonia of different etiology have been studied. Participation in these processes of the autonomic nervous system (ANS) is established. It is proved that the severity of this type of motor disorders depends on the condition of the ANS at different levels of its organization, especially on the background of sympathicotonic effects, as well as the vegetative characteristics of the patient. At the same time, signifi cant changes in electrophysiological parameters, as well as data of vegetative, emotional, psychometric testing, were obtained. For an objective evaluation of the severity of the tremor, a tremograph with the calculation of an integrative tremographic index was used. Developed a complex of cerebral angiodystonia with hyperkinetic syndrome and the use of antioxidants, nootropic drugs, which allowed to reduce or eliminate subjective experiences, to increase the adaptation capacity of the organism with the normalization of sympatho-parasympathetic relationships, to the normalization of the organism. also inhibition of the implementation of trembling hyperkinesis in both research groups. The average values of the tremographic index decreased towards an adequate distribution with sympathicolytic influence on the indices of vegetative testing and caused skin sympathetic potentials. At the same time, a greater effect was achieved when using the proposed method of treatment, with the maximum — in the syndrome of autonomic dystonia, as well as in the compensated stage of chronic brain ischemia. Keywords: cerebral angioedema, cerebral ischemia, autonomic disorders, tremor, treatment

Dolphin therapy

Dolphin therapy emerged during the 1950s. The work of Dr. John Lilly played an important role, since he investigated the impact of dolphins on disabled people. From 1978 onwards, the therapeutic aid of dolphins was further advanced, especially after the success of Dr Davis Nathanson with Down-syndrome children and Dr. Ludmila Lukina, who developed many therapeutic methods. The therapeutic method is based on the hypersonic stimulus transmitted by the dolphin sonar (a strong organ at the top of the head, which helps dolphins to produce and receive sounds) towards the central nervous system of humans, resulting in the synchronization of brain hemispheres for a certain period (brain waves are concerted in both hemispheres regarding frequency and phase), which is in turn contemplated as neurologically uncommon. The scientific experience of supplementary therapeutic approaches using dolphins reinforces the classical medical therapies and has provided solid proofs of positive effects of such programmes on: autism, Rett syndrome, Down syndrome, mental delay, brain paralysis, depression, cognitive difficulties, hyperkinetic syndrome. Therapeutic centres, such as Dolphin Assisted Therapy, provide various therapeutic programs of different duration, targeting to assist people with a variety of problems. Scientific results documented that dolphins affect the speech during interaction with humans, decrease the depression symptoms and reduce the pain in people suffering from multiple sclerosis. The present work provides up to date data regarding dolphin therapy in a worldwide context. It is actually a thorough literature review, which attempts to cover a gap in the field of therapeutic contribution of animals to human's health.

Abnormal Brain Connectivity Spectrum Disorders Following Thimerosal Administration

Background: Autism spectrum disorder (ASD), tic disorder (TD), and hyperkinetic syndrome of childhood (attention deficit disorder [ADD]/attention deficit hyperactivity disorder [ADHD]) are disorders recently defined as abnormal connectivity spectrum disorders (ACSDs) because they show a similar pattern of abnormal brain connectivity. This study examines whether these disorders are associated with exposure to thimerosal, a mercury (Hg)-based preservative. Methods: A hypothesis testing case-control study evaluated the Vaccine Safety Datalink for the potential dose-dependent odds ratios (ORs) for diagnoses of ASD, TD, and ADD/ADHD compared to controls, following exposure to Hg from thimerosal-containing Haemophilus influenzae type b vaccines administrated within the first 15 months of life. Febrile seizures, cerebral degeneration, and unspecified disorders of metabolism, which are not biologically plausibly linked to thimerosal, were examined as control outcomes. Results: On a per 25 μg Hg basis, cases diagnosed with ASD (OR = 1.493), TD (OR = 1.428), or ADD/ADHD (OR = 1.503) were significantly ( P < .001) more likely than controls to have received increased Hg exposure. Similar relationships were observed when separated by gender. Cases diagnosed with control outcomes were no more likely than controls to have received increased Hg exposure. Conclusion: The results suggest that Hg exposure from thimerosal is significantly associated with the ACSDs of ASD, TD, and ADD/ADHD.

Potential neurobiological ADHD biomarkers

ObjectivesPathogenetic mechanisms of hyperkinetic syndrome (HKS) or attention deficit hyperactivity disorder (ADHD) are not clear.AimTo elucidate some aspects of monoamine involvement in pathogenesis of disorder and response of monoaminergic systems to psychostimulant medication.MethodsLevels of different monoamines, their metabolites and N-methylnicotinamide (end product of kynurenine pathway) were measured in daily samples of urine from children (7–11 years old) with mild and severe HKS using fluorimetric and chromatographic methods as well as platelet monoamine oxidase (MAO) activity. Thirty children with mild HKS received psychostimulant Sydnocarb 5–15 mg daily for 1–1.5 months (for ethical reasons children with severe HKS were not included in study).ResultsHKS was accompanied by activation of dopaminergic and inhibition of noradrenergic systems. There were found metabolic differences between two forms of HKS. Compared with mild HKS, severe HKS was characterized by significant 2-fold increase of MAO activity and L-dopa, dopamine and adrenaline excretion. After sydnocarb treatment children's clinical status improved along with decrease of excretion of homovanillic, vanillylmandelic and 5-hydroxyindoleacetic acids and increase of N-methylnicotinamide.ConclusionsResults indicate that dopaminergic and noradrenergic systems play important role in pathogenesis of HKS. Clinical improvement of HKS children was accompanied by significant increase of N-methylnicotinamide excretion. It is proposed that increased urine excretion of kynurenine metabolite–N-methylnicotinamide and N-methylnicotinamide/5-hydroxyindoleacetic acid ratio can serve as potential biomarkers for evaluation of efficacy of psychostimulant medication. We hypothesize that kynurenine system plays significant role in pathogenesis of HKS/ADHD.Disclosure of interestThe authors have not supplied their declaration of competing interest.