In the neuroimaging assessment of patients with memory decline, ventricular atrophy emerges as a more reliable indicator of atrophy than sulcal atrophy. We anticipate that the overall score on the scale will provide valuable guidance for our clinical work.
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Though transplant-related mortality has declined, patients who receive hematopoietic stem-cell transplants continue to experience considerable short-term and long-term health problems, compromised quality of life, and psychosocial impairments. Comparisons of post-transplant quality of life and affective symptoms have been made across autologous and allogeneic hematopoietic stem cell transplant recipients in several studies. Although some research has indicated similar or heightened difficulties in quality of life for individuals receiving allogeneic hematopoietic stem cell transplants, the observed outcomes have varied significantly. Our investigation focused on evaluating the relationship between hematopoietic stem-cell transplant type and the quality of life and emotional status of our subjects.
A cohort of 121 patients, diagnosed with diverse hematological conditions, underwent hematopoietic stem cell transplantation at St. István and St. László Hospitals in Budapest. see more The study's approach was structured around a cross-sectional design. The Hungarian version of the Functional Assessment of Cancer Therapy-Bone Marrow Transplant scale (FACT-BMT) served as the instrument for evaluating quality of life. Assessments of anxiety and depressive symptoms involved the application of the Spielberger State-Trait Anxiety Inventory (STAI) and the Beck Depression Inventory (BDI), respectively. Essential sociodemographic and clinical details were also noted. 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.
Within both the autologous and allogeneic transplant groups, a similar pattern was observed regarding quality of life (p=0.83) and affective symptoms (pBDI=0.24; pSSTAI=0.63). While allogeneic transplant patients exhibited mild depressive tendencies, as indicated by their BDI scores, their STAI scores aligned with those of the general population. In allogeneic transplant recipients, the presence of graft-versus-host disease (GVHD) symptoms correlated with a more severe clinical picture (p=0.001), decreased functional capacity (p<0.001), and an increased requirement for immunosuppressive therapy (p<0.001) in comparison to patients without GVHD. 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. Depressive symptoms, anxiety, and psychiatric comorbidity were detrimental to quality of life in both the allo- and autologous cohorts.
Allogeneic transplant recipients' quality of life was affected by the severe somatic symptoms of graft-versus-host disease, frequently causing significant depressive and anxiety symptoms.
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Focal dystonias, of which cervical dystonia (CD) is the most prevalent, often present difficulties in pinpointing the affected muscles, administering the optimal dose of botulinum neurotoxin type A (BoNT-A) per injection site, and precisely targeting the necessary sites. see more This research project intends to compare local center data with international standards, pinpointing population and methodological factors influencing variations, thereby contributing to the enhanced care of Hungarian patients with CD.
Between August 11th, 2021, and September 21st, 2021, the Department of Neurology, University of Szeged, retrospectively collected and analyzed data from all consecutive CD patients treated with BoNT-A at their botulinum neurotoxin outpatient clinic, employing a cross-sectional methodology. By applying the collum-caput (COL-CAP) concept, the frequency of involved muscles was established; additionally, parameters of the ultrasound (US)-guided BoNT-A formulations were calculated and contrasted against international data.
The current study encompassed 58 patients, featuring 19 males and 39 females, and an average age of 584 years (standard deviation ± 136, and age range from 24 to 81 years). Torticaput demonstrated the highest frequency among subtypes, at a rate of 293%. A tremor was found to affect 241 percent of the patients examined. Of all the muscles injected, trapezius muscles were the most frequent target, showing a high rate of 569% of all cases, followed by the 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.
While the multicenter and current studies shared certain similarities, all leveraging the COL-CAP paradigm and US-guided BoNT-A injections, researchers should prioritize clearer differentiation of torticollis forms and increased injection frequency, particularly of the obliquus capitis inferior muscle, especially in instances presenting with benign essential tremor.
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The use of hematopoietic stem cell transplantation (HSCT) proves to be one of the most efficacious treatment modalities for a wide spectrum of malignant and non-malignant diseases. This investigation sought to identify early electroencephalographic (EEG) abnormalities in allogeneic and autologous hematopoietic stem cell transplant (HSCT) recipients managing potentially life-threatening non-convulsive seizures.
A total of 53 individuals were included in the study's cohort. Patient characteristics, including age, gender, type of HSCT (allogeneic or autologous), and the treatment regimens administered prior to and subsequent to HSCT, were meticulously recorded. EEG monitoring was conducted on all patients twice: initially on the first day of hospitalization, and subsequently one week after the commencement of conditioning regimens and HSCT procedures.
A detailed analysis of pre-transplant EEG findings indicated that 34 patients (64.2%) displayed normal EEG readings and 19 patients (35.8%) demonstrated abnormal EEG readings. Following transplantation, 27 (509%) patients exhibited normal EEG readings, while 16 (302%) demonstrated a basic activity disorder, 6 (113%) showed focal anomalies, and 4 (75%) displayed generalized anomalies. Anomalies in post-transplant EEGs were found to be considerably more common in the allogeneic group than in the autologous group, a statistically significant difference (p<0.05).
A critical component of the clinical follow-up for HSCT patients involves evaluating the risk factors related to epileptic seizures. EEG monitoring plays a vital part in the early identification and management of such non-convulsive clinical presentations.
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IgG4-related (IgG4-RD) disease, a relatively newly discovered, chronic autoimmune condition, has the capability of impacting any organ system. This medical condition is not common. The characteristic presentation is systemic, yet it can sometimes appear in an isolated form confined to a single organ. In our report, we detail a case study of an elderly male patient, exhibiting IgG4-related disease (IgG4-RD) manifest as diffuse meningeal inflammation and hypertrophic pachymeningitis, accompanied by involvement of a single cranial nerve and intraventricular structures.
A group of progressive neurodegenerative disorders, spinocerebellar ataxias (SCA), synonymous with autosomal dominant cerebellar ataxias (ADCA), display striking clinical and genetic heterogeneity. Twenty genes associated with SCAs were detected during the previous ten-year period. One of these genes, STUB1 (STIP1 homology and U-box containing protein 1, located on chromosome 16p13, NM 0058614), encodes a multifaceted E3 ubiquitin ligase, also known as CHIP1. The causative role of STUB1 in autosomal recessive spinocerebellar ataxia 16 (SCAR16) was established in 2013. A contrasting discovery, published by Genis et al. in 2018, showed that heterozygous mutations in STUB1 can also cause the autosomal dominant form of spinocerebellar ataxia, specifically SCA48, as found in reference 12. According to studies 2 through 9, a total of 28 French, 12 Italian, 3 Belgian, 2 North American, 1 Spanish, 1 Turkish, 1 Dutch, 1 German, and 1 British SCA48 families have been observed. These publications describe SCA48 as a progressive, late-onset condition presenting with cerebellar dysfunction, cognitive impairment, psychiatric symptoms, difficulties swallowing, heightened reflexes, urinary complications, and movement disorders including parkinsonism, chorea, dystonia, and, in exceptional cases, tremor. MRI scans of the brains of all SCA48 patients revealed cerebellar atrophy, both in the vermis and the hemispheres. This atrophy was particularly prominent in the posterior parts of the cerebellum, including lobules VI and VII, in the majority of cases.2-9 Hyperintensity within the dentate nuclei (DN) was a finding in some Italian patients' T2-weighted imaging (T2WI) scans, in addition to other observed features. In addition, the new publication documented alterations in DAT-scan images among some families of French origin. Studies 23 and 5, utilizing neurophysiological examinations, documented no central or peripheral nervous system abnormalities. see more Neuropathological investigation uncovered unequivocal cerebellar atrophy and cortical shrinkage, the intensity of which varied. The histopathological examination displayed a characteristic pattern including Purkinje cell loss, p62-positive neuronal intranuclear inclusions in some cases, and tau pathology noted in one patient. We present herein the clinical and genetic characteristics of the first Hungarian SCA48 patient, encompassing a novel heterozygous missense mutation in the STUB1 gene.