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Marcin Pawłowski,1 Karolina Fila-Witecka,1 Julia Ewa Rymaszewska,1 Dorota Kamińska,2 Paweł Poznański,2 Hanna Augustyniak-Bartosik,2 Dorota Zielińska,2 Magdalena Krajewska,2 Piotr K Krajewski,3 Aleksandra Stefaniak,3 Jacek C Szepietowski,3 Anna Pokryszko-Dragan,4 Justyna Chojdak-Łukasiewicz,4 Sławomir Budrewicz,4 Tomasz Pawłowski,1 Dorota Szcześniak,1 Joanna Rymaszewska1 1Psychiatry Department, Wroclaw Medical University, Wroclaw, Poland; 2Nephrology and Transplantology Department, Wroclaw Medical University, Wroclaw, Poland; 3Department of Dermatology, Venereology and Allergology, Wroclaw Medical University, Wroclaw, Poland; 4Department of Neurology, Wroclaw Medical University, Wroclaw, PolandCorrespondence: Marcin Pawłowski, Email marcin.pawlowski@student.umw.edu.plIntroduction: The global severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) pandemic had a drastic psychological and economic impact on the global population. Having a chronic disease during the pandemic is associated with numerous limitations and challenges like regular hospital visits, access to health-care units and getting specialized treatment. In addition, chronically ill patients are at great risk of acquiring the SARS-CoV-2 virus and at experiencing a more severe course of illness, due to comorbid conditions as well as more frequent encounters with health-care workers and other patients in medical facilities. The aim of this study was to examine the psychological disturbances, during the pandemic in chronically ill patients.Methods: During the cross-sectional survey conducted between May and October 2020, 398 patients with four different chronic conditions (psoriasis, multiple sclerosis and patients who have undergone a kidney transplant or received dialysis). Study sample was examined regarding the occurrence of psychopathological symptoms (General Health Questionnaire 28) and their perceived stress levels (Perceived Stress Scale).Results: The highest scores were found in the MS group and the lowest scores were found in the kidney transplantation group in every subscale of the GHQ-28. Close to half of the studied population (48.74%, n = 193) patients scored above the cut-off for psychopathology.Conclusion: As the study was conducted during the SARS-CoV-2 pandemic in Poland, it stands to reason that the pandemic affected the psychological wellbeing of chronically ill patients. A COVID-19 infection, being quarantined and having had contact with a person who was infected, did not significantly affect the outcome measures; however, further research is needed to explore this topic.Keywords: pandemic, SARS-CoV 2, chronic kidney disease, psychopathology, multiple sclerosis, psoriasis

Parry-Romberg syndrome (PRS) is a rare disorder, described in the nineteenth century by Caleb Parry and Moritz Romberg, characterized by acquired and slowly progressive atrophy of one side of the face. The pathogenesis of PRS is still unclear. Immune-mediated processes are thought to be a basic factor in PRS etiology, but autonomic nervous system might also be impaired. A case of PRS in a 26-year-old woman with coexisting disturbances in the lower left limb is presented. The multimodal electrophysiological studies were done, including electroencephalography, visual, brain auditory, somatosensory and trigeminal somatosensory evoked potentials, blink reflex, standard neurographic and electromyographic examinations, quantitative sensory tests and autonomic tests. Neuroimaging studies consisted of brain MR, single voxel proton MR spectroscopy, diffusion tensor imaging with fiber tractography. Based on multimodal electrophysiological and neuroimaging studies, it was concluded that the impairment in PRS is multisystemic, i.e., motor, sensory, and autonomic. A cortical origin of the symptoms is possible. [ABSTRACT FROM AUTHOR]

Guillain-Barré syndrome (GBS) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) are the most common autoimmune polyneuropathies. Their aetiology is unclear. The pathomechanism includes damage mainly to the myelin sheath and, in the long-term process, secondary axonal loss. Both inflammatory polyneuropathies involve different combinations of motor, sensory and autonomic fibres in the peripheral nerves. The differential diagnosis should be based on clinical and neurophysiological features, and laboratory tests. Numerous studies aim to demonstrate the most common errors in the diagnosis of Guillain-Barré syndrome and acute-onset CIDP. Misdiagnosis can result in the wrong treatment. We still do not have reliable markers to help diagnose the disease or to monitor the effectiveness of the therapy., Competing Interests: Declaration of competing interest The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)

SIRTs were demonstrated to play an important role in inflammatory, degenerative, and metabolic alterations, constituting the background of the central nervous system. Thus, they seem to be an appropriate object of investigation (as potential biomarkers of disease activity and/or novel therapeutic targets) in multiple sclerosis (MS), which has a complex etiology that comprises a cross-talk between all these processes. The aim of this study was to evaluate the levels of SIRT1 and SIRT2 in the serum of patients with the relapsing-remitting type of MS (RRMS), as well as their relationships with various aspects of MS-related disability., Methods: A total of 115 patients with RRMS (78 women, 37 men, mean age 43 ± 9.9) and 39 healthy controls were included in the study. SIRT1 and SIRT2 were detected in the serum using the enzyme-linked immunoassay (ELISA) method. In the RRMS group, relationships were investigated between the SIRT 1 and 2 levels and the demographic data, MS-related clinical variables, and the results of tests evaluating fatigue, sleep problems, cognitive performance, autonomic dysfunction, and depression., Results: The levels of SIRT1 and SIRT2 in RRMS patients were significantly lower than in the controls (11.14 vs. 14. 23, p = 0.04; 8.62 vs. 14.2, p < 0.01). In the RRMS group, the level of both SIRTs was higher in men than in women (15.7 vs. 9.0; 11.3 vs. 7.3, p = 0.002) and showed a significant correlation with the degree of disability (R = -0.25, p = 0.018). No other relationships were found between SIRT levels and the analyzed data., Conclusions: The serum levels of SIRT1 and 2 were decreased in the RRMS patients (especially in the female ones) and correlated with the degree of neurological deficit. The role of SIRTs as biomarkers of disease activity or mediators relevant for "invisible disability" in MS warrants further investigation.

Background: The motor neuron survival protein performs numerous cellular functions; hence, spinal muscular atrophy (SMA) is considered to be a multi-organ disease with possible sensory system damage. The controversy surrounding the presence of sensory disturbances, prompted us to conduct standard electrophysiological studies and assess the sensory thresholds for different modalities in adults with SMA types 2 and 3. The study group consisted of 44 adult SMA patients (types 2 and 3). All patients underwent neurological examination using the Hammersmith Functional Motor Scale - Expanded (HFMSE). Standard sensory electrophysiological studies in the ulnar nerve and the estimation of vibratory, temperature, and warm- and cold-induced pain thresholds with temperature dispersion assessment were performed using quantitative sensory testing (QST)., Results: The most repeatable result was the high amplitude of the sensory nerve action potentials (SNAP) in SMA patients compared to controls. This was higher in type 2 patients compared to type 3a and 3b patients and patients with low HFSME scores. Patients with SMA, especially type 3b presented a longer sensory latency and slower conduction velocity than did controls. Cold pain threshold was higher and warm dispersion larger in SMA. The vibratory limit was higher in patients with high HFSME scores., Conclusions: A high SNAP amplitude suggests sensory fibre hyperactivity, which may be based on overactivation of metabolic pathways as an adaptive mechanism in response to SMN protein deficiency with additionally coexisting small C- and A-delta fibre damage. SMA patients seem to have a concomitant, mild demyelinating process present at the early SMA stage., (© 2024. The Author(s).)

Background: Research on amyotrophic lateral sclerosis (ALS) reveals that the disorder is not restricted to motor neurons., Objectives: This neuroimaging study aimed to investigate the presence of cerebellar damage in adult ALS patients., Material and Methods: The study retrospectively analyzed magnetic resonance imaging (MRI) examinations performed on a 1.5T MR unit of 33 patients (17 men and 16 women with a mean age of 59.3 years) diagnosed with ALS. Cerebellar and posterior fossa dimensions were calculated using plain MR images. In addition, diffusion tensor imaging (DTI) was used to obtain white matter integrity measurements, represented as fractional anisotropy (FA) values, in the posterior limbs of internal capsules (PLIC) and middle cerebellar peduncles (MCPs). These measurements were compared to 36 healthy volunteers (11 men and 25 women with a mean age of 55.3 years). The study also assessed clinical data for correlations with cerebellar imaging findings., Results: The linear measurements of the cerebellum did not differ between groups. However, the transverse cerebellar dimension (TCD) ratio to the maximum length of the posterior fossa (0.973 compared to 0.982, t = -2.76, p < 0.01) and FA value in both MCPs (0.67 compared to 0.65 and 0.69 compared to 0.67, p < 0.05) were significantly lower in ALS patients. No significant differences were found in FA value in the PLIC, and no significant correlations were observed between patient clinical characteristics and cerebellar damage., Conclusion: This study provides evidence of cerebellar damage in adult ALS patients. These findings contribute to ALS understanding and highlight the importance of considering cerebellar involvement in the disease process. The results suggest that measuring the TCD ratio and FA value in both MCPs could be potential biomarkers for cerebellar damage in ALS patients.

Introduction: Parkinson's disease (PD) presents with a progressive decline in manual dexterity, attributed to dysfunction in the basal ganglia-thalamus-cortex loop, influenced by dopaminergic deficits in the striatum. Recent research suggests that the motor cortex may play a pivotal role in mediating the relationship between striatal dopamine depletion and motor function in PD. Understanding this connection is crucial for comprehending the origins of manual dexterity impairments in PD. Therefore, our study aimed to explore how motor cortex activation mediates the association between striatal dopamine depletion and manual dexterity in PD., Materials and Methods: We enrolled 26 mildly affected PD patients in their off-medication phase to undergo [ 18 F]FDOPA PET/CT scans for evaluating striatal dopaminergic function. EEG recordings were conducted during bimanual anti-phase finger tapping tasks to evaluate motor cortex activity, specifically focusing on Event-Related Desynchronization in the beta band. Manual dexterity was assessed using the Purdue Pegboard Test. Regression-based mediation analysis was conducted to examine whether motor cortex activation mediates the association between striatal dopamine depletion and manual dexterity in PD., Results: Mediation analysis revealed a significant direct effect of putamen dopamine depletion on manual dexterity for the affected hand and assembly tasks (performed with two hands), with motor cortex activity mediating this association. In contrast, while caudate nucleus dopamine depletion showed a significant direct effect on manual dexterity, motor cortex mediation on this association was not observed., Conclusion: Our study confirms the association between striatum dopamine depletion and impaired manual dexterity in PD, with motor cortex activity mediating this relationship., Competing Interests: Declaration of competing interest The author declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (Copyright © 2024 The Authors. Published by Elsevier Ltd.. All rights reserved.)

Background: Cranial autonomic symptoms (CASs) include lacrimation, conjunctival injection, rhinorrhea, nasal congestion, facial flushing or sweating, ptosis, and myosis. These symptoms may be associated with trigeminal autonomic cephalalgias (TACs) and migraine., Objectives: The aim of the study was to assess whether CASs are also reported by patients with frequent episodic tension-type headache (eTTH)., Material and Methods: A cross-sectional online survey of a large Polish population was conducted between August 2021 and June 2022. The analysis assessed diagnostic criteria for migraine and eTTH, as well as the presence of allodynia, headache-related disability and symptoms of depression., Results: The survey involved 3,225 respondents (age: 13-80 years, mean (M) = 38.9 years; 87.1% female). A total of 166 individuals met the diagnostic criteria for isolated frequent eTTH without migraine or probable migraine with or without aura. Allodynia was present during the majority of attacks in 40 (24.1%) eTTH subjects, while 86 (51.8%) eTTH respondents reported at least 1 CAS during their headache attacks. The presence of at least 1 CAS was more prevalent in migraine than in eTTH (p = 0.001). The respondents with at least 1 CAS during eTTH attacks reported a higher burden associated with pain (p = 0.024) and higher Patient Health Questionnaire-9 (PHQ-9) scores (p = 0.016)., Conclusions: The prevalence of retrospectively reported CASs was high among individuals with eTTH, which may potentially contribute to diagnostic errors. Cranial autonomic symptoms in eTTH do not appear to be caused by severe pain or central sensitization.

Background: Tension-type headache (TTH) is the most common primary headache. Obstructive sleep apnea (OSA) and sleep bruxism (SB) are two of the most common sleep disorders; however, the relationship between TTH, OSA, and SB has not been conclusively proved in the literature. The objective of our study was to estimate potential associations with OSA and SB in TTH subjects. Methods : 108 adult individuals who underwent polysomnography (vPSG) were included, and the group was divided into two subgroups: TTH ( n = 34) and control ( n = 74). The International Classification of Headache Disorders (ICHD-3) guidelines were used to diagnose TTH. OSA and SB diagnoses were based on vPSG examination with electromyographic (EMG) recordings and the American Academy of Sleep Medicine (AASM) criteria. The results were analyzed, where p < 0.05 was considered to be statistically significant. Results : In the TTH group, the incidence of SB was more than two times lower than the control (OR = 0.41, 95% CI: 0.17-0.96, p < 0.05). However, the incidence of severe SB (BEI > 4) was similar in the TTH and control groups (OR = 0.54, 95% CI: 0.21-1.35, p > 0.05). Additionally, phasic and tonic SB episodes were less frequent in the TTH group compared to the controls ( p < 0.05). The mean apnea-hypopnea index (AHI) was not significantly different between the TTH and control groups ( p > 0.05). The sleep architecture and respiratory disturbances did not differ between the examined groups ( p > 0.05). Conclusions : SB is not a risk factor for TTH. Moreover, severe SB is not connected with TTH. OSA is not a risk factor for TTH. Sleep quality did not differ between both groups during PSG; therefore, TTH may not change sleep structure. The mechanism of these findings is still unclear, and further studies should explain in detail the association between TTH and OSA.

Obstructive sleep apnea (OSA) is one of the most common sleep disorders; however, there are inconsistent results about the connection and occurrence of primary and secondary headaches in OSA. Therefore, the primary objectives were to estimate the prevalence and potential relationship between all types of headaches and OSA. A systematic review was conducted according to PRISMA 2020 guidelines. Studies were searched in PubMed, Embase, and Web of science up to July 2023. The Joanna Briggs Institute tool assessed the risk of bias. 1845 articles were identified, and 23 studies describing 15,402 patients were included. Pooled prevalence of all headaches in OSA was 33% (95% CI: 0.25-0.41), 33% for morning headaches (95% CI: 0.24-0.45), 25% for sleep apnea headaches (95% CI: 0.18-0.34), 19% for tension-type headache (95% CI: 0.15-0.23), and 16% for migraine (95% CI: 0.09-0.26). Relative risk for the occurrence of headache in OSA patients compared to the non-OSA people was 1.43 (95% CI: 0.92-2.25). OSA females and males had morning headaches with similar frequency. The prevalence of headaches in OSA was moderate. OSA did not increase the risk of headache. There is a need to conduct further studies focused on bidirectional connections between sleep disorders and headaches., Competing Interests: Declaration of competing interest All authors declare that they have no conflict of interest., (Copyright © 2023 Elsevier Ltd. All rights reserved.)

Objective: The current research aimed to analyze the alterations within the motor cortex and pyramidal pathways and their association with the degree of damage within the peripheral nerve fibers in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). To achieve that goal, we investigated the microstructural changes within the pyramidal white matter tracts using diffusion tensor imaging (DTI) parameters, evaluated metabolic alterations in both precentral gyri using magnetic resonance spectroscopy (MRS) ratios, and correlated them with the neurographic findings in patients with CIDP., Methods: The spectroscopic ratios of NAA/Cr, Cho/Cr, and mI/Cr from both precentral gyri and the values of fractional anisotropy (FA), axial diffusivity (AD), and mean diffusivity (MD) from both of the corticospinal tracts were correlated with the results of neurological and neurographic findings. The comparison of DTI parameters between the patients and controls was performed using Student's t -test or the Mann-Whitney U test. Due to the lack of normal distribution of most variables, Spearman's Rho rank coefficient was used to test all correlations. All analyses were performed at a significant level of alpha = 0.05 using STATISTICA 13.3., Results: Compared to the control group (CG), the patient group showed significantly lower ratios of NAA/Cr (1.66 ± 0.11 vs. 1.61 ± 0.15; p  = 0.022), higher ratios of ml/Cr in the right precentral gyrus (0.57 ± 0.15 vs. 0.61 ± 0.08; p  = 0.005), and higher levels of Cho/Cr within the left precentral gyrus (0.83 ± 0.09 vs. 0.88 ± 0.14, p  = 0.012). The DTI parameters of MD from the right CST and AD from the right and left CSTs showed a strong positive correlation (0.52-0.53) with the sural sensory nerve action potential (SNAP) latency of the right sural nerve. There were no other significant correlations between other DTI and MRS parameters and neurographic results., Significance: In our study, significant metabolic alterations were found in the precentral gyri in patients with CIDP without clinical symptoms of central nervous system involvement. The revealed changes reflected neuronal loss or dysfunction, myelin degradation, and increased gliosis. Our results suggest coexisting CNS damage in these patients and may provide a new insight into the still unknown pathomechanism of CIDP., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2024 Dziadkowiak, Koszewicz, Podgórski, Wieczorek, Budrewicz and Zimny.)

Background: Spinal adhesive arachnoiditis is a chronic inflammatory process of the leptomeninges and intrathecal neural elements. The possible causes of arachnoiditis are: infections, injuries of spinal cord, surgical procedures and intrathecal administration of therapeutic substances or contrast., Case Presentation: We present a case of 56-old woman with spinal muscular atrophy type 3 who developed a severe back pain in the lumbosacral region after the fifth dose of nusinersen given intrathecally. Magnetic resonance of lumbosacral spine showed spinal adhesive arachnoiditis. She received high doses of methylprednisolone intravenously, and later non-steroidal anti-inflammatory drugs, alpha lipoic acid, vitamins and rehabilitation with slight improvement., Conclusions: The authors summarize that scheduled resonance imaging of the lumbosacral spine may be an important element of the algorithm in the monitoring of novel, intrathecal therapy in patients with spinal muscular atrophy., (© 2024. The Author(s).)

Objectives: The motor neuron survival protein, which is deficient in spinal muscular atrophy (SMA), performs numerous cellular functions. Currently, SMA is believed to be a multi-organ disease, including lesion of various structures of the central and peripheral nervous systems. Motor nerve damage, especially in milder SMA types, is controversial. This prompted the conduct of the electrophysiological studies in adults with SMA types 2 and 3 presented in this paper., Methods: The study group consisted of 44 adult patients with SMA types 2 and 3. All patients underwent neurological examination with Hammersmith Functional Motor Scale-Expanded (HFMSE) assessment. Standard electrophysiological studies in the ulnar nerve and conduction velocity distribution (CVD) tests were performed in all patients and controls., Results: A prolongation of the distal latency and lowering of the motor potential amplitude with no changes in CVD were found in the whole patient group. There were no dependencies on the number of gene copies. Patients with low HFSME value had slower standard conduction velocity, CVD in upper and median quartiles, and narrower CVD spread; in milder SMA, CVD spread was greater than in controls., Interpretation: The significant reduction in motor response amplitude in SMA seems to be primarily related to motor neuron loss and directly proportional to its severity. The coexisting rearrangement in the peripheral nerve structure is present in SMA, and this could be partially caused by a coexisting demyelinating process. Nerve remodeling mainly affects large fibers and occurs in more severe SMA types with significant disability., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Koszewicz, Ubysz, Dziadkowiak, Wieczorek and Budrewicz.)

Multiple sclerosis (MS) causes long-lasting, multifocal damage to the central nervous system. The complex background of MS is associated with autoimmune inflammation and neurodegeneration processes, and is potentially affected by many contributing factors, including altered composition and function of the gut microbiota. In this review, current experimental and clinical evidence is presented for the characteristics of gut dysbiosis found in MS, as well as for its relevant links with the course of the disease and the dysregulated immune response and metabolic pathways involved in MS pathology. Furthermore, therapeutic implications of these investigations are discussed, with a range of pharmacological, dietary and other interventions targeted at the gut microbiome and thus intended to have beneficial effects on the course of MS.

Background: Migraine without aura (MwoA) is often mistaken for rhinosinusitis. The purpose of this study was to assess the prevalence of sinonasal symptoms, sinusitis-targeting medication use and burden of migraine in a large group of people with MwoA attacks accompanied by rhinologic symptoms., Methods: Data was collected in a cross-sectional online survey based on an adapted population-based study questionnaire. The analysis included the prevalence of rhinorrhea, mucopurulent nasal discharge, nasal congestion, facial pressure and tenderness to palpation, hyposmia/anosmia and osmophobia., Results: 1,679 (52.73%) MwoA people were identified among 3,225 respondents (women n  = 2,809, 87.10%) aged 13-80 years (median age 39; standard deviation 10.4). 1004/1679 (59.8%) migraine patients reported one or more rhinologic symptoms and 341/1679 (20.3%) MwoA respondents had symptoms that met rhinosinusitis clinical diagnostic criteria during their headache attacks. In migraine patients, osmophobia was associated with hyposmia [ n  = 141 (12.7%) vs. n  = 41 (7.2%); p  = 0.001] and a sensation of unpleasant smells [ n  = 216 (19.4%) vs. n  = 45 (8.5%); p  = 0.001], while facial tenderness to palpation was associated with facial allodynia [ n  = 532 (50.4%) vs. n  = 211 (33.9%); p  < 0.001]. People with migraine accompanied by rhinosinusitis-like symptoms experienced more disease burden and used 'sinus medications' more often., Conclusion: MwoA patients with rhinosinusitis-like symptoms during migraine attacks require cautious assessment, especially that some symptoms seem to have little value in distinguishing between these disorders (i.e., facial tenderness, hyposmia), while many of these patients have a greater disease burden and therefore often choose medications targeting rhinologic instead of neurologic mechanisms., Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest., (Copyright © 2023 Straburzynski, Nowaczewska, Czapinska-Ciepiela, Gryglas-Dworak, Budrewicz and Waliszewska-Prosół.)

Background: Understanding brain death is essential for progress in organ transplantation; however, it remains a challenging ethical matter. In 2019, Poland revised its legislation on brain death to align with international standards. This study aimed to evaluate the knowledge and worldview concerning brain death among Polish medical students, categorised according to their stage of education., Methods: An online questionnaire was administered to 169 medical students from four Polish medical universities. The participants were divided into preclinical (n = 94) and clinical (n = 75) groups. The questionnaire consisted of two parts, with the first part comprising 13 questions focusing on knowledge about brain death and the process of its determination. The second part contained six questions related to the participants' worldview regarding brain death, particularly concerning organ transplantation., Results: The average score obtained by the respondents was 7.53 (± 2.35; min. 1, max. 13) in knowledge checking part of the developed questionnaire (maximal score:13). Students in the clinical stage of their education achieved significantly higher scores compared to preclinical students (mean 8.84; ± 1.89 vs mean 6.49; ± 2.15; p < 0.001). Significant correlations were found between the results of the knowledge part of the questionnaire and responses to worldview questions., Conclusions: The stage of education influenced the knowledge of brain death among medical students, although the overall test scores were unsatisfactory. Higher test scores were associated with worldview responses indicating compliance with the current legislation in Poland and evidence-based medicine., (© 2023. BioMed Central Ltd., part of Springer Nature.)