Naming decline is one of the most common symptoms of primary progressive aphasia (PPA). Most studies on anomia in PPA are performed without taking into account PPA variants, especially for action naming. Only limited data are available for the neuroanatomical basis of anomia considering differences in the pathogenesis of PPAs. The aim of our study is to investigate the associations between anomia severity for both noun and verb naming and gray matter (GM) atrophy, as well as accompanying functional connectivity (FC) changes in three PPA variants. A total of 17 patients with non-fluent (nfvPPA), 11 with semantic (svPPA), and 9 with logopenic (lvPPA) PPA variants were included in the study and underwent cognitive/naming assessments and brain MRIs. Voxel-based morphometry was performed to evaluate GM volume. A resting-state functional MRI was applied to investigate FC changes in the identified GM areas. The study shows that different brain regions are involved in naming decline in each PPA variant with a predominantly temporal lobe involvement in svPPA, parietal lobe involvement in lvPPA, and frontal lobe involvement in nfvPPA. Separate data for object and action naming in PPA variants are provided. The obtained results mainly correspond to the current understanding of language processing and indicate that the evaluation of language impairments is preferable for each PPA variant separately. A further analysis of larger cohorts of patients is necessary to confirm these preliminary results.

Language is one of the higher brain functions and the primary method of communication, so it plays a key role in human social functioning. Primary progressive aphasia, as a slowly progressive neurodegenerative disease with a clinical predominance of different speech and language disorders, is a promising model for a more detailed study of topographic distribution of language disorders. This review presents data on different clinical variants of primary progressive aphasia and the corresponding clinical and neuroanatomical correlates that have significantly expanded the modern understanding of the neural network language organization.

Frontotemporal dementia (FTD) is a neurodegenerative disorder characterized by language and behaviour deficits, which is considered the second most common cause of early-onset dementia. Detection of brain atrophy patterns is important for FTD diagnosis. However, the visual assessment of magnetic resonance imaging data may not be sensitive enough requiring the use of objective gray matter (GM) volume determination method. The study was aimed to assess the GM atrophy pattern in patients with FTD compared to control group patients using voxel-based morphometry (VBM). The study included 16 patients with FTD (12 patients with nonfluent agrammatic variant primary progressive aphasia (nfvPPA), three patients with behavioral variant of FTD, and one patient with logopenic variant PPA) and 10 healthy volunteers. VBM of patients with FTD and healthy controls revealed three significant (pFWE-corr < 0.05) atrophy areas in the left inferior frontal, left fusiform, and left supramarginal gyri. Taking into account the predominance of patients with nfvPPA in the group of FTD patients, the additional VBM of this group and control group was carried out, which revealed a distinct atrophy pattern: the reduced GM volume was detected in the left inferior frontal and left middle frontal gyri (pFWE-corr < 0.05). The results obtained indicate that regardless of the clinical variant, there is a certain atrophy pattern characteristic of FTD, which involves both frontotemporal areas and parietal lobe. The example of nfvPPA shows that each variant of the disease is associated with distinct localization of atrophy.

Mitochondrial insufficiency does not always have vivid polysystemic manifestations that clinically reflect the dysfunction of these organelles. Diagnosis can be difficult both from a clinical and laboratory point of view. In this case, the authors describe a family with erased or not obvious corresponding clinical manifestations in 3 generations. Complete genomic assessment of mitochondrial DNA give enabled the authorsto identify inherited pathological changes. These findings are very important for understanding the molecular processes of mitochondrial disease development and the further development of specific therapy.

BACKGROUND: Primary progressive aphasia is a rare neurodegenerative disease with high clinical, genetic, and pathomorphological heterogeneity that greatly complicates its diagnosis. Voxel-based morphometry can be used to objectively assess structural gray matter changes and determine atrophy patterns in variants of primary progressive aphasia, which can improve the diagnosis and our understanding of its pathogenesis. AIMS: This study aimed to evaluate the patterns of atrophy in each of the primary progressive aphasia variants in comparison with the control group. MATERIALS AND METHODS: Patients diagnosed with one of the primary progressive aphasia variants, established in accordance with the current diagnostic criteria, were included in the main group. The control group consisted of healthy volunteers without any neurological symptoms or structural brain changes. All participants underwent brain magnetic resonance imaging. The obtained images were processed and used for voxel-based morphometry, which was performed by comparing the gray matter volume between each of the primary progressive aphasia variants and the control group. The study was adjusted for the sex, age, and intracranial volume of the participants. RESULTS: The study enrolled 25 patients with nonfluent, 11 with semantic, and 9 with logopenic variants of primary progressive aphasia, as well as 20 healthy volunteers. Voxel-based morphometry showed a specific atrophy pattern in each of the variants of primary progressive aphasia, with predominant involvement of the frontal and insular lobes in nonfluent, temporal lobe and hippocampus in semantic, and a more diffuse frontotemporal pattern in logopenic variants. CONCLUSIONS: The study revealed gray matter atrophy patterns specific to each variant of primary progressive aphasia. The obtained results mainly correspond to the clinical presentations of the disease. Moreover, some findings (e.g., absence of the posterior perisylvian atrophy and reduced motor cortex volume in the logopenic variant, atrophy of the orbitofrontal cortex and cerebellum in the nonfluent variant, and premotor cortex, precentral, and inferior frontal gyrus degeneration in the semantic variant) do not correlate with the usual understanding of primary progressive aphasia pathogenesis and require further study.

Background. Glioblastoma is the most common primary malignant brain tumor with an extremely unfavorable prognosis. The frequency of the “longevity” phenomenon (>3 years of overall survival – OS) in this disease is 5–10 %. The reasons for the more favorable prognosis in these patients are still unclear.Aim. To compare the clinical and MRI data as well as features of complex treatment of patients with supratentorial glioblastomas among the control group (OS 3 years).Material and methods. This study included 41 patients with hemispheric glioblastomas: 17 with the “longevity” phenomenon (study group – long-term survival (LTS), prospective set); 24 patients in the control group (retrospective set). Taking into account the average age of patients, the following MRI features were examined: tumor localization relative to the frontal lobe; invasion of deep brain structures; the ratio of contrastenhancing and non-contrast-enhancing parts of the glioma; tumor contrast intensity; localization of recurrent tumor (local / distant) in case of disease progression. Comparative analysis of complex treatment took into account the following parameters: the number of chemotherapy (ChT) courses and radiation regimens after the 1^st operation and after disease recurrence; the fact and number of repeated tumor resections after recurrence; the presence of IDH1 mutation.Results. The localization of the lesion relative to the frontal lobe, the number of affected lobes of the brain and the invasion of deep structures did not differ significantly in the examined groups. Patients of study group (LTS, prospective set) were significantly younger than the patients of the control group (p 0.05). The tumor contrast intensity was 1.5 in average compared to the intact cerebral hemisphere. Among patients of LTS group, 8 patients (47 %) were re-operated due to disease recurrence, while in the control group there were no repeated operations (p 0.05); the number of temozolomide courses in the LTS group were 9 (6–22), while in the control group it was 6 (3–10), p <0.05. The repeated radiotherapy (RT) in different regimens was applied in the LTS group in 52 % of patients, in the control group – 0 (p <0.05). The repeated ChT (17 (9–23) courses, mainly with bevacizumab) was applied in the LTS group in 65 % of patients, in the control group – 0 (p <0.05). The IDH1 mutation was studied only in 15 patients: positive – in 1 (LTS group); negative – in 14 patients (7 from the control group).Conclusion. The following significant differences were revealed: patients with supratentorial glioblastomas were younger in the LTS group. The important features of the complex treatment of patients in LTS group included statistically higher frequency of repeated resections in case of recurrence (47 %) and repeated sessions of radiotherapy (RT) in various modes (52 %); significantly more aggressive and prolonged ChT (with the predominance of temozolomide in the 1^st line of treatment and bevacizumab in case of recurrence). There were no significant differences between two examined groups in tumor localization, intensity of its contrasting, the ratio of contrasted and non-contrast parts of the tumor, invasion of deep structures of the brain, involvement of functionally significant areas (FSA).