[Tremor-dominant form of Parkinson's disease]. / Tremor-dominantnaya forma bolezni Parkinsona.

The article is of a review nature and is devoted to tremor, one of the maladaptive and difficult-to-treat symptoms of Parkinson's disease (PD). Along with the classic rest tremor, patients with PD may experience tremor of other modalities: postural tremor, kinetic tremor, which reflects a multimodal mechanism of tremor formation involving multiple neurotransmitter systems. The unpredictable response to therapeutic options, the ambiguous response to levodopa, also reflects the role of multiple underlying pathophysiological processes. Among the drug methods of tremor correction, preference is given to dopamine receptor agonists - due to the spectrum of their pharmaceutical action, high efficiency in relation to all leading motor and a number of non-motor manifestations. The evidence for advanced neurosurgical, non-invasive modalities is mixed, and there are insufficient comparative studies to assess their efficacy in patients with tremor-dominant forms of PD.

[Postural stability and walking in Parkinson's disease]. / Postural'naya ustoichivost' i khod'ba pri bolezni Parkinsona.

The article is of an overview nature and is devoted to movement disorders in Parkinson's disease. The article discusses the existing problems according to the latest literature data, a review on the treatment and rehabilitation of postural instability. Special attention in the article is paid to dopamine receptor agonists - namely, piribedil, prescribed for the correction of these disorders.

[Tardive dyskinesia]. / Tardivnaya diskineziya.

Tardive dyskinesia (TD) is a delayed, often irreversible iatrogenic movement disorder caused by long-term use of that dopamine receptors blocking drugs. Prevention of TD is paramount, and clinicians should follow best practice recommendations for prescribing antipsychotics, as well as reduction the using of dopamine receptor blocking drugs for non-psychiatric prescriptions. Replacement of antipsychotics with lower affinity for D2 receptors drugs, addition of VMAT2 (tetrabenazine), botulinum therapy, amantadine may be effective. In detection and incurable cases, the possibility of neuromodulation of brain structures should be considered. Most methods for testing TD currently have an insufficient level of evidence, although they include recommendations from professional communities. There is a great need for new clinical trials.

[Orthostatic hypotension in patients with Parkinson's disease]. / Ortostaticheskaya gipotenziya u patsientov s bolezn'yu Parkinsona.

Orthostatic hypotension (OH) is a common vegetative symptom of Parkinson's disease (PD), which is predominantly neurogenic in nature. Detection and treatment of OH is of great importance, since it affects daily activity and increases the risk of falls. In the long term it damages target organs - the heart, kidneys and brain. In this regard, the review discusses the issues of classification, pathogenesis of OH, stages of diagnosis and correction of blood pressure, as well as measures for lifestyle changing, non-drug and drug treatment of orthostasis. Strategies for the management of patients with postprandial hypotension, hypertension in the supine position and nocturnal hypertension are considered separately. Despite modern combined methods of treatment, the burden of OH in patients with PD remains heavy, and fluctuations in blood pressure due to concomitant hypertension in the supine position are a significant problem. This highlights the need to initiate scientific research and new therapeutic approaches.

[Frontotemporal dementia with cortico-basal syndrome]. / Lobno-visochnaya dementsiya s kortikobazal'nym sindromom.

FTD is a group of neurodegenerative diseases with progressive deterioration of behavioral and speech disorders, morphologically associated with pathology of the frontal or temporal lobes. International clinical trials have made it possible to define modern diagnostic criteria for various subtypes of clinically «possible/probable¼ FTD. Our article is devoted to one of the rare subtypes of frontotemporal dementia (FTD), corticobasal syndrome (CBD), in which we presented a review of current data with a demonstration of clinical observation. A clinical case of a patient with a patient with speech disorders and memory impairment is presented. A 60-year-old man at the time of the outpatient visit had been complaining of speech impairment for two years, a slight decrease in memory for current events. Neurological and neuropsychological studies revealed two leading clinical syndromes in the patient: «frontal¼ syndrome with impaired higher cortical functions in the form of efferent motor aphasia, impaired writing and reading with visual-spatial agnosia and dysgraphia, «frontal¼ signs (positive «palm-mouth «and¼ grasping «reflexes); «Corticobasal syndrome¼ with pronounced dynamic, optic-kinesthetic dyspraxia, dermolexia, apraxia of closing the eyes, «alien¼ hand syndrome with symptoms of levitation and intermanual conflict. MRI diagnostics revealed changes characteristic of neurodegeneration of the frontotemporal type (atrophy of the frontal and temporal lobes prevails). Taking into account complaints, anamnesis of the disease, identified clinical syndromes and structural changes according to MRI data, the patient was diagnosed with a clinically «probable¼ FTD. Determination and accurate diagnosis of FTD subtypes will help the neurologist in managing these patients with the appointment of the correct pharmacologic treatment. In FTD, in contrast to AD patients, the administration of cholinesterase inhibitors does not lead to a positive therapeutic effect a positive therapeutic effect and, therefore, is not advisable. The standards of patient therapy should include recommendations for antipsychotic therapy, the use of antidepressants (SSRIs) and anxiolytics with nootropic effects for the correction of affective and behavioral disorders.

[Risk and course of COVID-19 in patients with Parkinson's disease]. / Risk razvitiya i techenie COVID-19 u patsientov s bolezn'yu Parkinsona.

The article provides an overview of the data on the impact of Parkinson's disease on the risk of infection and the course of COVID-19, and also assesses the possible pathogenetic relationship between the SARS-CoV-2 virus, COVID-19 and PD. By penetrating the central nervous system, SARS-CoV-2 can cause not only neurological symptoms, but also exacerbate the course of an existing neurological disease. The impact of Parkinson's disease on the risk of infection and the course of COVID-19 is controversial. However, a number of authors support the opinion that PD is an anti-risk factor for the development of COVID-19, which is associated both with the pathogenesis of the disease and with the used antiparkinsonian drugs, in particular amantadines. There are no clear data indicating higher risk of infection and higher severity of COVID-19 in patients with PD. On the contrary, experimental and clinical data suggest a possible modifying role of α-synuclein and antiparkinsonian drugs.

[Hemifacial spasm]. / Gemifatsial'nyi spazm.

Hemifacial spasm (HFS), or facial hemispasm, is a paroxysmal hyperkinetic disorder involving muscles innervated by the facial nerve, mainly on the one hand. The development of HFS is based on neurovascular conflict. However, it is impossible to explain the clinical manifestations of HFS only by nerve compression. Both peripheral and central mechanisms are involved in the generation of HFS, with the formation of ephaptic transmission, antidromic excitation, primary or secondary hyper-excitability of the nuclear and supranuclear level of innervation. Two treatment methods are pathogenetically justified: microvascular decompression (MVD) and botulinum toxin (BTX) injections. The effectiveness of MVD is 95.37% with full or partial recovery. The recurrence rate does not exceed 2.4%. Facial nerve paralysis (2.7-22.5%) and hearing loss (1.9-20%) are the most common complications of treatment with the use of the MVD for HFS with partial or complete cure in most cases. Botulinum toxin injection chemo-denervation is a first-line treatment of primary and secondary HFS. HFS is an officially registered indication for the drug dysport (abobotulotoxin) (ABO) in the Russian Federation. Total doses of ABO ranged from 25 to 150 units for one side depending on the severity of the clinical manifestations. Studies demonstrate the statistically significant benefits of HFC treatment with ABO. ABO is generally well-tolerated. Adverse events (up to 3.6%) are transient and include ptosis, lacrimation, blurred vision, double vision, dry eyes and weak facial muscles.

[Salivation after stroke]. / Sliunotechenie posle insul'ta.

Salivation after stroke (sialorrhea, hypersalivation, ptyalism) can be caused by the overproduction or a lack of utilization of saliva. Patients after stroke often have sialorrhea as a result of dysphagia. Neurogenic dysphagia in acute period after stroke occurs in 25-65% of patients. The mortality rate among people with post-stroke dysphagia and tube feeding ranges from 20 to 24%. Patients who suffering from profuse salivation have difficulties with articulation and swallowing, bad breath, irritation and maceration of perioral area, contamination of clothing and bed linen. Profuse salivation increases caregiver burden as well. Psychologically, profuse salivation leads to lower self-esteem and social isolation. Pulmonary aspiration is the most serious complication of sialorrhea. Injections of botulinum toxins in the large salivary glands and the m. cricopharyngeus are the most effective method of treatment.

[Levodopa-carbidopa intestinal gel in the treatment of patients with Parkinson disease: results of a 12-month open study]. / Levodopa-karbidopa intestinal'nyi gel' v terapii bol'nykh s razvernutymi stadiyami bolezni Parkinsona: rezul'taty 12-mesyachnogo otkrytogo issledovaniya.

AIM: To evaluate the long-term safety and efficacy of intrajejunal levodopa-carbidopa intestinal gel (LCIG) infusion in the treatment of patients with severe stages of Parkinson disease (PD) who did not respond adequately to treatment with oral drugs. MATERIAL AND METHODS: A large-scale international prospective open-label 54-week study of LCIG in patients with PD with severe motor fluctuations was carried out. A total of 48 patients were enrolled in Russia, 46 patients (95.8%) had PEG-J inserted, and 43 of them completed the study. The safety, including adverse events (AEs), infusion system and pump failures analysis, number of patients completely terminated the study, and efficacy (duration of "off" periods, "on" periods with or without troublesome dyskinesias, UPDRS scores, Clinical Global Impression, Quality of Life (PDQ-39, EQ-5D и EQ-VAS) dynamics, an analysis of patient's diaries) were assessed throughout the whole study. RESULTS: The majority of AEs were mild or moderate with most AEs connected with infusion system application (28.3% patients) including procedure pain. Serious AEs were registered in 8 patients (16.7%). 3 patients (6.3%) discontinued their participation in the study due to AEs. Mean duration of "off" periods by the end of the study decreased by 5.35±2.59 hours (p<0.001), duration of "on" periods without troublesome dyskinesia increased by 5.74±3.91 hours (p<0.001), reduction of "on" periods duration with troublesome dyskinesia became statistically significant by week 36 (p=0.020). The statistically significant improvement of UPDRS (generally and in respect to sub-scales), Clinical Global Impression, and Quality of Life scores was observed throughout the study. Levodopa dose remained stable throughout the 54 treatment weeks. Forty-three patients (93.5%) received LCIG monotherapy throughout the whole study. CONCLUSION: LCIG intrajejunal infusion during 54 weeks showed the favorable safety profile, high tolerability, and efficacy in PD motor symptoms correction.

[Pediatric acute-onset neuropsychiatric syndrome: clinical/laboratory characteristics]. / Kliniko-laboratornaia kharakteristika pediatricheskogo ostro voznikshego neiropsikhiatricheskogo sindroma.

AIM: Based on the current conceptions on the genesis of hyperkinetic syndromes in children and adolescents, to single out a group of patients with suggestive PANS, compare clinical and laboratory results and determine clinical/laboratory characteristics of this syndrome. MATERIAL AND METHODS: Sixty-nine patients with tics were studied using neurological examination, questionnaires and international scales for assessment of tics, obsessive-compulsive disorders and attention deficit hyperactivity disorder (ADHD). Laboratory tests included general blood tests, antistreptolysin O test, determination of rheumatoid factor, C-reactive protein, circulating immune complexes, nasopharyngeal wash for ß-hemolytic streptococcus, antineuronal antibodies and immunoglobulins A, M, G, E, CD4, CD8-lymphocytes. The same tests were performed in the control group. RESULTS AND CONCLUSION: Clinical symptoms were different by the severity and phenomenology of tic hyperkineses in patients with PANS compared to the patients with tics without immune disorders. Most of the patients were diagnosed with Tourette syndrome. ADHD was the most common diagnosis in the PANS group. Its frequency was 2.5 higher in the male patients. A chronic focus of infection did no predict the development of PANS. Based on the laboratory results, one can assume that ß-hemolytic streptococcus A infection, lower JgM levels and an elevated CD8+ lymphocytes predict the development of autoimmune mental and neurological disorders in the group of PANS patients.

[Ratio of transcription factor PHF10 splice variants in lymphocytes as a molecular marker of Parkinson's disease].

Parkinson's disease (PD) is the second most common neurodegenerative disorder and causes degeneration of dopaminergic neurons in the nigrostriatal system of the brain. PHF10 is one of the most important regulatory subunits of the SWI/SNF chromatin-remodeling protein complex, which controls the gene function and chromatin state in neurons. Two alternative RHF10 isoforms, PHF10-P and PHF10-S, replace each other in the complex to change the target gene pattern. Expression of the PHF10-P and PHF10-S transcripts in the nigrostriatal system and their ratio in blood lymphocytes were found to change in a mouse model of early clinical stage of PD as compared with control mice. Changes in PHF10-S level were also observed in peripheral blood lymphocytes from patients with early clinical stage of PD. A ratio of the PHF10-P and PHF10-S transcripts in peripheral blood cells was assumed to provide a potential marker of early stage PD.

[Pain fluctuations in Parkinson's disease].

OBJECTIVE: To specify the character of pain syndromes and determine their relationship with main symptoms of ON and OFF periods in patients with complications of long-term treatment with levodopa medications. MATERIAL AND METHODS: Authors examined 40 patients with Parkinson's disease (27 women and 13 men), mean age 69.2±8 years (from 62 to 85 years), illness duration 9.2±1.2 years (from 7 to 11 years), duration of treatment with levodopa 6.9±1.2 years (from 5 to 9 years). The severity of disease course, character and intensity of pain syndrome were assessed. RESULTS AND CONCLUSION: We described pain syndromes that had differences in the pathogenesis and localization. Adjustment of the antiparkinsonian treatment resulted in the decrease in their severity. Motor fluctuations and drug-induced dyskinesia that was accompanied by pain sensations were the most frequent signs of Parkinson's disease during its progression. The pain syndrome was related to main symptoms of the ON and OFF period in patients with complications of long-term treatment with levodopa. The pain fluctuations had both nociceptive and central neuropathic pain phenotypes.

[Meige's syndrome or segmental craniocervical dystonia: terminology, history and contemporary view].

The term "Meige's syndrome" or "Breughel's syndrome" is used by specialists for the description of blepharospasm with the involuntary movements in the lower part of the face and/or masseter muscle. These eponyms "Meige's syndrome" and "Breughel's syndrome" make some mess in the terminology. The term "segmental craniocervical dystonia" joins different blepharospam-plus phenotypes and reflects contemporary perceptions about its genetic and pathophysiological community. Botulinotherapy is in fact the only way of symptomathic treatment of craniocervical dystonias.