Vol 20, No 3 (2026)

Cover Page

Original articles

Circadian-induced hypothermia of the cerebral cortex in rehabilitation of patients after ischemic stroke

Shevelev O.A., Petrova M.V., Fedin A.I., Torshin D.V., Khodorovich N.A., Mengistu E.M., Borisov I.V.

Abstract

Introduction. The role of circadian (nocturnal) cerebral hypothermia in brain recovery following daytime activity is well recognized.

The study aim is to investigate the clinical effects of craniocerebral hypothermia (CCH) administered in the evening in patients during the early post-stroke recovery period.

Materials and methods. The study included 95 patients in the early recovery period after IS, of whom 70 comprised the main group and 25 — the comparison group. Patients in both groups received pharmacotherapy, physiotherapy (magnetotherapy, electrotherapy), therapeutic exercise, and massage. Treatment in the main group was supplemented with a course of CCH (10 daily sessions of 90 minutes each, administered between 20:00 and 22:00).

Results. Patients in the main group showed significantly greater improvements in cognitive and motor functions, increased daily activity, and enhanced self-care ability and functional independence compared to the comparison group. In addition, patients in the main group showed improved sleep quality.

Conclusion. This study indicates the feasibility of incorporating CCH into comprehensive rehabilitation programs for patients after IS, taking into account the physiological circadian rhythmicity of brain temperature changes, and provides a rationale for considering CCH as a nature-inspired (biomimetic) technology.

Annals of Clinical and Experimental Neurology. 2026;20(3):5-12
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Limbic system changes in Huntington's disease: diffusion tensor MRI data with ROI analysis

Anikin G.A., Filatov A.S., Liaskovik A.A., Klyushnikov S.A., Abramycheva N.Y., Illarioshkin S.N.

Abstract

Introduction. Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder in which early affective and cognitive symptoms may be associated with limbic system involvement; however, the pattern of microstructural changes in its major white matter pathways remains insufficiently understood.

The aim of the study was to assess the state of limbic system tracts (cingulum, fornix, and inferior longitudinal fasciculus) in patients at manifest and premanifest stages of HD using diffusion tensor MRI (DT-MRI) and region-of-interest (ROI) analysis.

Materials and methods. A total of 64 individuals were examined: 24 patients with manifest HD, 20 premanifest mutation carriers, and 20 healthy volunteers matched for sex and age. All subjects underwent DT-MRI on a Siemens Magnetom Prisma 3T scanner (64 diffusion directions, b-values 1000 and 2500 s/mm2), followed by data adjustment and normalization to MNI space. ROI analysis of the cingulum, fornix, and inferior longitudinal fasciculus was performed using DSI Studio software, extracting fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD). Correlations with clinical scales (UHDRS, MoCA, FAB, HADS, Schulte tables) and genetic markers (CAG repeat length, CAP score) were assessed.

Results. In manifest patients, the cingulum showed decreased FA and increased RD, indicating predominant demyelination, whereas the inferior longitudinal fasciculus exhibited concurrent increases in both AD and RD, suggesting more severe axonal and myelin damage. In premanifest mutation carriers, the earliest changes were detected in the fornix crura (decreased FA) and in the anterior portion of the inferior longitudinal fasciculus (increased RD and MD). Significant correlations were found between diffusion metrics and anxiety and depression scores (HADS), cognitive performance (MoCA, FAB), and genetic burden (CAG repeat length, CAP score).

Conclusion. These findings confirm the heterogeneous and stage-dependent nature of limbic tract involvement in HD, supporting the potential use of DT-MRI as a tool for preclinical diagnosis and monitoring of non-motor manifestations of the disease.

Annals of Clinical and Experimental Neurology. 2026;20(3):13-28
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Comorbidity in amyotrophic lateral sclerosis: structure and prognostic aspects

Pervushina E.V., Kutlubaev M.A.

Abstract

Introduction. The course of amyotrophic lateral sclerosis (ALS) is characterized by high variability. Factors influencing the rate of progression and disease outcome remain insufficiently studied. Comorbidity in ALS is of particular interest; however, its structure and impact on disease progression are underexplored.

Objective: To study the structure of comorbid pathology, injuries, and surgical interventions in patients with ALS and to assess their impact on disease course.

Results. Data from 336 patients with ALS were analyzed. The most common comorbid conditions were diseases of the circulatory system (58%), with hypertension being predominant (41%). Thyroid diseases were identified in 6.8% of patients, mainly in those with bulbar-onset ALS. Approximately 25% of patients reported significant psychoemotional stress within one year prior to disease onset. A negative impact of previous kidney surgeries on overall survival was established. Patients with spinal-onset ALS were significantly more likely to have undergone surgical interventions in general and spinal surgeries in particular within 10 years before symptom onset compared to patients with bulbar-onset ALS. The Charlson Comorbidity Index demonstrated a statistically significant association with both the rate of ALS progression and patient survival.

Conclusion. Comorbid pathology is widespread among patients with ALS. Concomitant diseases and surgical interventions may exert a differential influence on various forms of the disease. The most substantial negative impact on ALS progression is exerted by the cumulative burden of somatic disorders, highlighting the importance of comprehensive assessment of comorbidity in the management of this patient group.

Annals of Clinical and Experimental Neurology. 2026;20(3):29-37
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Multiparametric quantitative magnetic resonance neurography of the lumbosacral plexus in chronic demyelinating polyneuropathies

Tsaregorodtsev D.S., Morozova S.N., Grishinа D.A., Murtazina A.F., Subbotin D.M., Shchagina O.A., Krotenkova M.V., Suponeva N.A.

Abstract

Introduction. Chronic demyelinating polyneuropathies, including chronic inflammatory demyelinating polyneuropathy (CIDP) and hereditary motor and sensory neuropathy type 1 (HMSN1), are characterized by clinical heterogeneity and diagnostic challenges. Electrophysiological methods do not always reliably distinguish between hereditary and acquired forms, highlighting the need for additional objective biomarkers.

The study aim was to evaluate the diagnostic potential of multiparametric quantitative MR neurography of the lumbosacral plexus in patients with chronic demyelinating polyneuropathies of different etiologies.

Materials and methods. Data were acquired from 30 patients with genetically confirmed HMSN1, 31 patients with CIDP (fulfilling EAN/PNS 2021 electrodiagnostic criteria), and 27 healthy volunteers. Imaging was performed on a 3T MRI scanner using quantitative MR techniques: diffusion tensor imaging (fractional anisotropy — FA; mean, axial, and radial diffusivity — MD, AD, RD), T2 relaxometry (apparent T2 relaxation time — T2app; proton density — PD), and magnetization transfer imaging (MTR). Quantitative parameters were analyzed at the level of the L5–S2 nerve roots bilaterally.

Results. Both patient groups showed significantly reduced FA and increased MD and RD compared to controls, reflecting a demyelinating pattern of injury. AD did not differ significantly. Patients with HMSN1 showed more pronounced changes in PD and MTR compared to the CIDP group.

Conclusion. Multiparametric quantitative MR neurography is associated with differences in microstructural changes between patient groups with chronic demyelinating polyneuropathies and may serve as a promising tool for differentiating hereditary from acquired forms.

Annals of Clinical and Experimental Neurology. 2026;20(3):38-49
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Modeling of malignant middle cerebral artery infarct in rabbits

Sehweil S.M., Sufianov A.A., Prokofiev V.N., Goncharova Z.A., Kirichenko E.Y., Logvinov A.K., Deribas V.Y.

Abstract

Introduction. Malignant ischemic stroke (MIS) in the middle cerebral artery (MCA) territory is characterized by extensive hemispheric infarction leading to massive edema, ultimately resulting in brain herniation. The study of pathophysiological mechanisms and the development of novel treatments for this condition have become possible through experimental stroke models. However, existing experimental models are often not translatable to humans.

The study aim is to develop an experimental model of MIS in the MCA territory that closely mimics the clinical setting.

Materials and methods. The study was conducted on 40 male California rabbits weighing 2 kg at 2 months of age, using casting of the common carotid artery with polymerizing colored silicone. Carotid territory anatomy was studied in 10 rabbits. MIS modeling in the MCA territory was performed in 30 rabbits divided into 2 groups. In Group 1 (n = 15), MIS was modeled by occluding the MCA with two 6-0 Prolene monofilaments. In Group 2 (n = 15), in addition to MCA occlusion with two monofilaments, decompressive craniectomy and coagulation of cortical MCA branches were performed.

Results. Two variants of the internal carotid artery (ICA) were identified based on the occipital artery origin. The first, more common variant (80%), has the occipital artery arising from the external carotid artery. In the second variant, the occipital artery is a branch of the ICA. Inserting two 6-0 Prolene monofilaments through the common carotid artery into the MCA occludes its proximal segment. Decompressive craniectomy followed by coagulation of cortical arteries produces cortical ischemia.

Conclusion. We identified two ICA variants based on occipital artery origin. The first variant is more common (80%), with the occipital artery arising from the external carotid artery. The second variant is characterized by a carotid-occipital trunk common to both the occipital and internal carotid arteries. Additional coagulation of cortical arteries produces a larger hemispheric infarct compared to isolated proximal MCA occlusion with two monofilaments, providing a model of MIS that closely approximates the clinical scenario.

Annals of Clinical and Experimental Neurology. 2026;20(3):50-64
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Reviews

Parkinson's disease and cardiovascular abnormalities: clinical and pathogenetic aspects

Sayfitdinkhuzhaev Z.F., Zhukova N.G., Guseva O.V., Zhukova I.A.

Abstract

Parkinson's disease (PD) is a multisystem neurodegenerative disorder characterized by progressive loss of dopaminergic neurons in the substantia nigra. Cardiovascular dysfunction is an important non-motor component of PD, yet it remains underrecognized in clinical practice. Accumulating evidence indicates a high prevalence of orthostatic hypotension, coronary artery disease, and cardiac arrhythmias in patients with PD, significantly impacting morbidity and mortality. The aim of this review is to examine current data on the pathogenetic mechanisms and clinical features of cardiovascular disease in PD. We conducted a search for full-text publications in Russian and English over the past 20 years in the eLIBRARY.RU, PubMed, Google Scholar, and Web of Science databases using relevant keywords and phrases. The review demonstrates that cardiovascular disease in PD forms a continuum, sharing common autonomic and neurodegenerative mechanisms. Systematic screening, including blood pressure measurement in the standing position, prolonged ECG monitoring, assessment of the QT interval and heart rate variability, together with active management of traditional risk factors, is essential to reduce morbidity and mortality.

Annals of Clinical and Experimental Neurology. 2026;20(3):65-72
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Clinical utility of intravascular ultrasound in brachiocephalic artery stenting

Annushkin V.A., Raskurazhev A.A., Sinitsyn I.A., Shchipakin V.L., Lukyanchikov V.A., Tanashyan М.M.

Abstract

Intravascular ultrasound (IVUS) during stenting of brachiocephalic arteries is a promising tool for optimizing endovascular procedures. The method allows precise assessment of plaque morphology and calcification, selection of optimal stent size, and intraoperative monitoring of stent expansion and wall apposition. Current evidence shows that integrating IVUS into carotid stenting protocols significantly reduces periprocedural complications, minimizes the risk of early restenosis, and improves long-term clinical outcomes. This technology enables a personalized approach and underscores the need for its routine use to enhance both safety and long-term efficacy in carotid and, potentially, vertebral artery stenting. Technical aspects and limitations of the method are also discussed.

Annals of Clinical and Experimental Neurology. 2026;20(3):73-79
pages 73-79 views

Clinical analysis

Clinical case of Zhu–Tokita–Takenouchi–Kim syndrome

Belykh N.A., Krukovskaya M.D., Krukovsky G.B., Piznyur I.V., Zhdankina A.S.

Abstract

Zhu–Tokita–Takenouchi–Kim syndrome is an extremely rare hereditary disorder belonging to the group of nuclear speckleopathies with an autosomal dominant inheritance pattern. To date, only approximately 87 confirmed cases have been described in the global medical literature, underscoring the exceptional rarity of this condition. Its pathogenesis is driven by mutations in the SON gene, which plays a critical role in RNA splicing and the regulation of embryonic development. The clinical presentation of the syndrome is highly polymorphic and manifests from birth. Key features include significant motor and psychomotor and speech developmental delay, generalized muscle hypotonia, and intellectual disability of varying severity. Characteristic facial dysmorphism includes macrocephaly or dolichocephaly, hypertelorism, a broad nasal bridge, and a thin upper lip. Central nervous system abnormalities often include structural malformations such as hypoplasia of the corpus callosum and cortical dysplasia. In addition to neurological impairments, patients may present with musculoskeletal anomalies (scoliosis, pectus carinatum, joint contractures) and ocular disorders (optic atrophy, strabismus, microcornea). This article presents our own clinical observation of the disease in an 8-year-old child, contributing to the description of the natural history and long-term outcomes of the syndrome. Analysis of such cases is fundamental to better understanding the pathophysiology of the disease and developing palliative care strategies aimed at improving patients' quality of life.

Annals of Clinical and Experimental Neurology. 2026;20(3):80-85
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Familial case of rapid-onset dystonia-parkinsonism (DYT/PARK-ATP1A3, DYT12)

Zaripova L.R., Evdokimov K.M., Gromova K.A., Sadekova M.A., Nuzhnyi E.P., Abramycheva N.Y., Fedotova E.Y., Illarioshkin S.N.

Abstract

Rapid-onset dystonia-parkinsonism (RDP) is one of the best-known and most frequently encountered phenotypes of ATP1A3-associated disorders. It is characterized by acute or subacute onset of dystonia, parkinsonism, and bulbar symptoms, typically triggered by provoking factors such as fever, infections, or physical exertion. In clinical practice, RDP is challenging to recognize due to atypical symptoms that do not fit the established diagnostic criteria, overlap with other phenotypes within this group of disorders, and the frequent absence of a positive family history. Only two clinical cases of RDP have been described in the Russian literature. This article presents two new patients from a single family with the RDP phenotype and a likely pathogenic variant in the ATP1A3 gene, c.2974G>A (p.Asp992Asn). A literature review covering pathogenesis, diagnosis, and treatment of the group of disorders caused by various ATP1A3 variants is provided.

Annals of Clinical and Experimental Neurology. 2026;20(3):86-97
pages 86-97 views