Evaluating atrophy on neuroimaging in patients experiencing memory decline, ventricular atrophy presents as a more trustworthy marker compared to sulcal atrophy. We anticipate that the overall score on the scale will provide valuable guidance for our clinical work.
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Despite the reduced rate of mortality linked to transplantation, those receiving hematopoietic stem cell transplants frequently experience short-term and long-term health problems, impaired quality of life, and difficulties in their psychosocial adaptation. Numerous studies have delved into the variations in post-transplant quality of life and emotional profiles among patients who have undergone autologous and allogeneic hematopoietic stem cell transplants. There are studies detailing similar or worse quality of life experiences among patients who receive allogeneic hematopoietic stem-cell transplants, but the results found are not uniform. We explored the correlation between hematopoietic stem-cell transplant types and the subsequent effects on the patients' quality of life and emotional well-being.
The study's patient population included 121 individuals with diverse hematological disorders who underwent hematopoietic stem cell transplantation at St. István and St. László Hospitals in Budapest. Epigallocatechin molecular weight The study was conducted using a cross-sectional approach. Using the Hungarian version of the Functional Assessment of Cancer Therapy-Bone Marrow Transplant (FACT-BMT) scale, quality of life was determined. Assessments of anxiety and depressive symptoms involved the application of the Spielberger State-Trait Anxiety Inventory (STAI) and the Beck Depression Inventory (BDI), respectively. Fundamental sociodemographic and clinical data were additionally recorded. When variables showed a normal distribution, a t-test was used to analyze comparisons between autologous and allogeneic recipients; otherwise, a Mann-Whitney U test was employed. A stepwise multiple linear regression analysis was used to identify the risk factors impacting quality of life and emotional symptoms in each group.
No significant divergence was observed in quality of life (p=0.83) or affective symptoms (pBDI=0.24; pSSTAI=0.63) when comparing the autologous and allogeneic transplant groups. The BDI scores of allogeneic transplant patients suggested a mild depressive state, yet their STAI scores were comparable to those of the general population. Patients undergoing allogeneic transplants exhibiting graft-versus-host disease (GVHD) symptoms encountered significantly more severe clinical presentations (p=0.001), demonstrating a markedly reduced functional capacity (p<0.001), and necessitating a higher dosage of immunosuppressive therapies (p<0.001), compared to those without the condition. Depression (p=0.001) and consistent anxiety (p=0.003) were more prevalent in patients who suffered from graft-versus-host disease when compared to those without the condition. Psychiatric comorbidity, alongside depressive and anxiety symptoms, negatively impacted the quality of life metrics for both the allo- and autologous groups.
Patients undergoing allogeneic transplantation experienced a decrease in quality of life due to severe somatic symptoms linked to graft-versus-host disease, often resulting in depressive and anxiety disorders.
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The most common focal dystonia, cervical dystonia (CD), presents a challenge in identifying the appropriate muscles for treatment, deciding on the right botulinum neurotoxin type A (BoNT-A) dosage for each muscle, and precisely aiming each injection. Epigallocatechin molecular weight The present study's focus is on comparing local center data with international counterparts, uncovering underlying population and methodological variations, and thereby further optimizing care for Hungarian patients with CD.
A cross-sectional, retrospective review of data from all consecutive CD patients treated with BoNT-A at the botulinum neurotoxin outpatient clinic within the University of Szeged's Department of Neurology, spanning from August 11, 2021 to September 21, 2021, was undertaken. The collum-caput (COL-CAP) concept was used to determine the frequencies of the involved muscles; these frequencies, and the parameters of the ultrasound (US)-guided BoNT-A formulations, were then calculated and compared with international data.
The current study involved 58 patients, 19 male and 39 female, with a mean age of 584 years (standard deviation ± 136, and ranging from 24 to 81 years). Torticaput constituted the dominant subtype, with a prevalence of 293%. Tremor affected a substantial 241 percent of the patient cohort. The injection rate for trapezius muscles stood at 569% of all instances, outpacing other muscles including levator scapulae (517%), splenius capitis (483%), sternocleidomastoid (328%), and semispinalis capitis (224%). The following data represents the mean doses per patient for three different substances: onaBoNT-A, incoBoNT-A, and aboBoNT-A. onaBoNT-A doses averaged 117 units, with a standard deviation of 385 units, and ranged between 50 and 180 units. IncoBoNT-A displayed a mean dose of 118 units, a standard deviation of 298 units, and a range of 80 to 180 units. Lastly, aboBoNT-A exhibited a mean dose of 405 units, with a standard deviation of 162 units, and a range of 100 to 750 units.
Although the results of the current and multicenter studies, both utilizing the COL-CAP approach and US-guided BoNT-A injections, showed some similarities, more precise identification of different forms of torticollis and a greater injection frequency, especially into the obliquus capitis inferior muscle, is essential, mainly in cases without no-no tremor.
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Stem cell transplantation, specifically hematopoietic stem cell transplantation (HSCT), stands as one of the most effective therapeutic approaches for a wide array of malignant and non-malignant ailments. Our research focused on early identification of EEG abnormalities in patients who received both allogeneic and autologous hematopoietic stem cell transplantation (HSCT) and were requiring treatment for potentially life-threatening non-convulsive seizures.
A total of 53 individuals were included in the study's cohort. A comprehensive record was maintained regarding patient age, gender, hematopoietic stem cell transplantation (HSCT) type (allogeneic or autologous), and the applied treatment protocols preceding and following HSCT. As part of the standard protocol, all patients underwent two EEG monitoring sessions: the initial session on the first day of hospitalization, and the subsequent session one week after the commencement of conditioning regimens and the completion of HSCT.
Evaluating the pre-transplant electroencephalograms (EEGs), 34 patients (64.2 percent) had normal EEGs, and 19 patients (35.8 percent) had abnormal EEGs. In a post-transplant analysis of EEG findings, 27 (509%) patients exhibited normal results, 16 (302%) presented with a basic activity disorder, 6 (113%) displayed focal anomalies, and 4 (75%) displayed generalized anomalies. The allogeneic group demonstrated a markedly higher rate of EEG anomalies following transplantation compared to the autologous group (p<0.05).
A critical component of the clinical follow-up for HSCT patients involves evaluating the risk factors related to epileptic seizures. Non-convulsive clinical manifestations require timely diagnosis and treatment, making EEG monitoring essential.
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The relatively newly identified chronic autoimmune disorder, IgG4-related (IgG4-RD) disease, has the capacity to affect any organ system. This medical condition is not common. Generally, the condition presents systemically; nonetheless, isolated cases within a single organ have been documented. An elderly male patient's case, reported herein, exhibits IgG4-related disease (IgG4-RD), characterized by diffuse meningeal inflammation and hypertrophic pachymeningitis, extending to one cranial nerve and the intraventricular regions.

Spinocerebellar ataxias (SCA), also termed autosomal dominant cerebellar ataxias (ADCA), present as a group of progressively debilitating neurodegenerative diseases, marked by noteworthy clinical and genetic variations. Over the past decade, 20 genes have been discovered within the genetic context of SCAs. One of these genes, STUB1 (STIP1 homology and U-box containing protein 1, NM 0058614 on chromosome 16p13), encodes a multifunctional E3 ubiquitine ligase, specifically CHIP1. STUB1's role as a causative gene for autosomal recessive spinocerebellar ataxia 16 (SCAR16) was identified in 2013. However, Genis et al. (2018) later published that heterozygous mutations in STUB1 can also result in the autosomal dominant inheritance pattern of spinocerebellar ataxia 48, as noted in reference 12. So far, reports indicate 28 French, 12 Italian, 3 Belgian, 2 North American, 1 Spanish, 1 Turkish, 1 Dutch, 1 German, and 1 British SCA48 families have been documented from studies 2-9. These publications suggest that SCA48 is a late-onset, progressive disorder featuring cerebellar problems, cognitive decline, psychiatric manifestations, difficulty swallowing, exaggerated reflexes, urinary difficulties, and movement disorders like parkinsonism, chorea, dystonia, and, occasionally, tremor. The brain MRI findings in all SCA48 patients consistently demonstrated atrophy of both the cerebellar vermis and hemispheres, with a greater degree of atrophy in the posterior cerebellar areas, specifically lobules VI and VII, in most subjects. 2-9 T2-weighted imaging (T2WI) hyperintensity of the dentate nuclei (DN) was reported as a feature in a portion of Italian patients, beyond the previously mentioned details. Additionally, the most recent publication highlighted modifications to DAT-scan imaging in certain French families. The neurophysiological examinations performed did not uncover any abnormalities within the central or peripheral nervous systems, which is consistent with the reported findings in references 23 and 5. Epigallocatechin molecular weight Cerebellar atrophy and cortical shrinkage, demonstrating variability in severity, were ascertained through neuropathological findings. Purkinje cell loss, p62-positive neuronal intranuclear inclusions in some cases, and tau pathology in one patient were noted in the histopathological assessment. The clinical and genetic profile of the first Hungarian SCA48 case, featuring a novel heterozygous missense mutation in the STUB1 gene, is described in this paper.