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Methylcathinone is a psychostimulant that increases the release of dopamine and norepinephrine in the brain. Ephedrone - methylcathinone obtained using "home" methods - is produced by the oxidation of drugs containing ephedrine. Methylcathinone itself is relatively low toxic, while the side effect of ephedrone is dementia, and parkinsonian and pyramidal syndromes. Case report: After 3 months of intravenous use of ephedrone, a 25-year-old woman developed gait disturbances with falls, followed by dysphagia, dysarthria, symptoms of multifocal dystonia and parkinsonian syndrome. Despite discontinuing the use of ephedrone, the symptoms gradually worsened, which led to the patient's immobilization in a wheelchair and her death 8 years later. Microscopic examination of the patient's brain was performed using routine histopathological methods, TUNEL method, and immunohistochemical reactions with antibodies against: ubiquitin, β-amyloid, α-synuclein, tau protein, GFAP ferritin, C4β complement, and CD3, CD45, CD68 antigens. Histopathological examinations revealed changes already described previously with manganese poisoning, such as: loss of neurons in the globus pallidus, reactive gliosis in the substan-tia nigra and excessive accumulation iron deposits in astroglia in the basal ganglia. In addition, abnormalities not occurring in manganism were also found, such as: generalized inflammation of small cerebral vessels of moderate severity and the presence of unknown material in the lumen of microvessels. Some astrocytes and neurons showed immunoreactivity in the TUNEL method. No signs of neurodegenerative process were found in the structures of the nigro-striatal system and striatum. Conclusions: Ephedrone's neurotoxicity is only partially due to manganese poisoning and it may also cause vasculitis, possibly following intravenous administration. [ABSTRACT FROM AUTHOR]

Vascular malformations usually develop as a result of influence of teratogenic factor(s) acting in the defined embryonic/ fetal period. However, in the case examined by us, various types of vascular malformations formed in different periods of the ontogenic development were found. They were seen in all parts of the central nervous system and clinically mimicked multiple sclerosis. On the background of generalized ischemic lesions of the CNS, certain kinds of vascular malformations were seen: cavernous or fetallike vessels within meninges, superficially located capillary angioma penetrating into the brain and spinal cord white matter, and arterio-venous pathological conglomerates forming meningeal angiomatosis. In pathological Vessels, immunocytochemical assessment of vascular endothelium with antibodies against antigens CD31, CD34, von Willebrand factor and lectin Ulex europaeus was normal but examination of the vascular basal membrane compounds revealed poor immunoreactivity to laminin and fibronectin. There were no disturbances in expression of angiopoietin, platelet-derived growth factor, transforming growth factor β and vascular endothelial growth factor receptors Tie-1/2, PDGFR-α/β, endoglin and Flk-1, respectively. The presence of various types of pathological vessels originating from different ontogenic periods indicates remittent or prolonged influence of teratogenic factor(s) in all periods of fetal vessel development. [ABSTRACT FROM AUTHOR]

Enteropathies are a serious concern in racing pigeons as they significantly impair performance in races and their training, and viruses are suspected to be one of the main factors. Astroviruses are well-known to be responsible for causing enteric disease in humans and various other animals including birds, although their prevalence and pathogenicity in pigeons is poorly understood. In this study, we investigated 2 groups of young racing pigeons (sick-study group and healthy-control group) to assess the correlation between the number of astrovirus genome copies in cloacal swabs and the occurrence of enteropathy. To determine this, we developed a novel TaqMan quantitative PCR (qPCR) and digital droplet PCR (ddPCR) methods for astrovirus detection and absolute quantitative analysis. We also performed high-throughput sequencing to obtain the complete genome sequences and establish the genetic similarity of the obtained strains to known astroviruses of poultry and other avian species. Two new complete genome sequences of pigeon astroviruses in the Avastrovirus genus were identified, representing 2 new species. These were found most closely related to astroviruses identified in Columbidae species and chickens. They share an average of 75.8% genome-wide pairwise identity and 57.6% and 64.6% capsid protein sequence identity with other unclassified columbid avastrovirus sequences in GenBank. Although the difference in prevalence of astrovirus in the study and control group was found statistically insignificant, there was a significant difference between the number of genome copies in positive samples from both groups. These unambiguous results leave the role of astroviruses as enteropathogenic factors in pigeons still undetermined., Competing Interests: DISCLOSURES 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.)

This study was aimed to investigate the frequency of PiCV recombination, the kinetics of PiCV viremia and shedding and the correlation between viral replication and host immune response in young pigeons subclinically infected with various PiCV variants and kept under conditions mimicking the OLR system. Fifteen racing pigeons originating from five breeding facilities were housed together for six weeks. Blood and cloacal swab samples were collected from birds every seven days to recover complete PiCV genomes and determine PiCV genetic diversity and recombination dynamics, as well as to assess virus shedding rate, level of viremia, expression of selected genes and level of anti-PiCV antibodies. Three hundred and eighty-eight complete PiCV genomes were obtained and thirteen genotypes were distinguished. Twenty-five recombination events were detected. Recombinants emerged during the first three weeks of the experiment which was consistent with the peak level of viremia and viral shedding. A further decrease in viremia and shedding partially corresponded with IFN-γ and MX1 gene expression and antibody dynamics. Considering the role of OLR pigeon rearing system in spreading infectious agents and allowing their recombination, it would be reasonable to reflect on the relevance of pigeon racing from both an animal welfare and epidemiological perspective., (© 2024. The Author(s).)

Viruses from Picornaviridae family are known pathogens of poultry, although the information on their occurrence and pathogenicity in pigeons is scarce. In this research, efforts are made to broaden the knowledge on Megrivirus B and Pigeon picornavirus B prevalence, phylogenetic relationship with other avian picornaviruses and their possible connection with enteric disease in racing pigeons. As a result of Oxford Nanopore Sequencing, five Megrivirus and two pigeon picornavirus B-like genome sequences were recovered, among which three recombinant strains were detected. The recombinant fragments represented an average of 10.9% and 25.5% of the genome length of the Pigeon picornavirus B and Megrivirus B reference strains, respectively. The phylogenetic analysis revealed that pigeons are carriers of species-specific picornaviruses. TaqMan qPCR assays revealed 7.8% and 19.0% prevalence of Megrivirus B and 32.2% and 39.7% prevalence of Pigeon picornavirus B in the group of pigeons exhibiting signs of enteropathy and in the group of asymptomatic pigeons, respectively. In turn, digital droplet PCR showed a considerably higher number of genome copies of both viruses in sick than in asymptomatic pigeons. The results of quantitative analysis leave the role of picornaviruses in enteropathies of pigeons unclear.

While disease control in racing pigeons and the potential role of pigeons as vectors transmitting viruses to poultry are of importance, there is still a paucity of data concerning the occurrence of coronaviruses in pigeons. In this study, 215 domestic pigeons were tested for the presence of coronaviral genetic material using the nested PCR method, which revealed 57 positive samples (26.51%). The difference in coronavirus prevalence between young and adult pigeons (34.34% and 19.83%, respectively) has been found statistically significant. In contrast, no statistically significant difference has been demonstrated between the prevalence in symptomatic and asymptomatic birds, leaving the influence of coronavirus presence on pigeon health uncertain. Phylogenetic analysis of the RdRp gene fragment allowed us to assign all the obtained strains to the Gammacoronavirus genus and Igacovirus subgenus. The phylogenetic tree plotted using the ML method revealed that those sequences formed a group most similar to pigeon coronavirus strains from China, Finland, and Poland, and to a single strain from a common starling from Poland, which suggests wide geographical distribution of the virus and its possible transmission between various species.

This short overview recalls the basic principles and technical aspects of skin and skeletal muscle biopsies in humans with paying special attention to the stages of these procedures essential for further correct morphological diagnosis. Some of these principles may also be useful in animal experimental studies. The authors emphasize the important role of proper thickness of the skin fragment, proper orientation of muscle fibres and a scalpel during skin biopsy, and proper concentration of fixatives. They recommend avoiding anaesthesia of the skeletal muscle itself and using forceps carefully so as not to crush the epidermis.

Animal production is identified as one of the main sources of high concentrations of odours, which are related to air pollution, health problems of living organisms and indirect negative impact on production results. One common method for reducing emissions of ammonia is using preparations containing probiotics and hygroscopic or disinfecting compounds. This study was undertaken in order to determine the impact of innovative mineral-microbial deodorizing preparation, which reduces odorous gases, applying to the litter once a week in poultry houses on the physiological status of breeder chickens, broiler chickens and turkeys. Samples were collected after slaughter and analyzed using ELISA tests, flow cytometry and biochemical methods. Biochemical markers of the liver and kidney profile (ALT, AST, LDH, ALP, CK, TP, CALC, PHOS) and the titers of specific antibodies against AEV, aMPV, AAvV-1, IBDV, HEV, BA were analyzed in serum samples. The percentage contribution of T and B lymphocyte subpopulations was determined in the samples of tracheal mucosa, blood, and spleen. No significant differences were found between the control and experimental group with regard to all the analyzed parameters, with some exceptions for biochemistry. The results of our study indicated that mineral-microbial deodorizing preparation did not affect the physiological status of birds.

Salmonellosis is one of the most important bacterial diseases in pigeons. This study aimed to estimate the prevalence of Salmonella spp. in domestic pigeons (Columba livia f. domestica) in Poland, its antimicrobial susceptibility (both phenotypic and genotypic), and its capability for biofilm formation. The presence of selected virulence genes, nucleotide homology of selected genes, and susceptibility to bacteriophages were investigated as well. From the 585 pigeons tested, 5.47% turned out positive. All isolated strains were recognized as Salmonella enterica ser. Typhimurium. The asymptomatic pigeons were carriers of 37.5% of the isolates. The dominant variants were as follows: 1,4,[5],12,:i:1,2 (53.13%) and 1,4,[5],12,:-:- (31.25%). Most of the strains analysed showed the ability to produce biofilm after 24 and 48 hr of incubation (59.38% and 53.13%, respectively). Over 90% of the strains were confirmed for lpfA, agafA, invA, sivH, and avrA virulence genes. Also, of the thirteen antimicrobial susceptibility genes, the following were confirmed: sul1, tet(A), bla TEM-1 , floR, strA, and strB. The most common were the strB (18%) and tet(A) (12%) genes that are responsible for coding resistance to aminoglycosides and tetracyclines, respectively. Most of the strains were phenotypically resistant to oxytetracycline (46.88%), neomycin (53.13%) and tylosin (100%). The susceptibility of the investigated Salmonella strains to the bacteriophages was between 33% and 100%. MLST, PCR MP and ERIC PCR analyses indicated a very high genetic similarity of the investigated strains (over 99%). Results of our study indicate that Salmonella enterica ser. Typhimurium is still an important agent in domestic pigeons and that its antimicrobial resistance increases. Alarming is also the confirmation of a single-phase variant 1,4,[5],12:i,-, which could have increased virulence and multi-drug resistance encoded on the plasmid. Most importantly, however, such strains have been isolated from humans with clinical symptoms of Salmonella infection., (© 2020 The Authors. Transboundary and Emerging Diseases published by Wiley-VCH GmbH.)

Pigeon circovirus (PiCV) infects pigeon populations worldwide and has been associated with immunosuppression in younger pigeons. Recombination is a common mechanism of evolution that has previously been shown in various members of the Circoviridae family, including PiCV. In this study, three groups of pigeons acquired from separate lofts were screened for PiCV, and their genome sequence was determined. Following this, they were housed in a single loft for 22 days, during which blood and cloacal swab samples were taken. From these blood and cloacal swabs, PiCV genomes were determined with the aim to study the spread and recombination dynamics of PiCV in the birds. Genome sequences of PiCV were determined from seven pigeons (seven tested PiCV positive) before they were housed together in a loft ( n = 58 sequences) and thereafter from the ten pigeons from blood and cloacal swabs ( n = 120). These 178 PiCV genome sequences represent seven genotypes (98% pairwise identity genotype demarcation), and they share >88% genome-wide pairwise identity. Recombination analysis revealed 13 recombination events, and a recombination hotspot spanning the 3' prime region, the replication-associated protein ( rep ) gene and the intergenic region. A cold spot in the capsid protein-coding region of the genome was also identified. The majority of the recombinant regions were identified in the rep coding region. This study provides insights into the evolutionary dynamics of PiCV in pigeons kept under closed rearing systems.

Avian metapneumoviruses (aMPV) are a causative agent of turkey rhinotracheitis (TRT). Despite vaccination, cases of TRT outbreaks are frequently reported. Considering that there are aMPV-free areas, a part of turkey poults possess (MDA+) or do not possess (MDA-) maternally derived antibodies (MDA) in the first weeks of life, which is the time of TRT vaccination. Study was undertaken to establish the level of protection against homologue aMPV/A infection, in MDA+ and MDA- turkeys, vaccinated against TRT at the 0 or 14th d of life and infected 14 d later. With the use of ELISA test and qPCR techniques, we have established the level of immune system stimulation after the vaccination and how does it correlate with the level of protection against the aMPV infection. Vaccination of MDA+ turkeys (especially at 0 d of life) resulted in weaker IgA production in upper respiratory tract. In addition, we have demonstrated differences in both humoral and cell-mediated immunity stimulation after infection of vaccinated turkeys. Despite these differences, we have shown that all vaccinated birds were protected against the disease which was determined based on the clinical and histopathological scoring, as well as the level of aMPV/A replication and shedding. Nonvaccinated groups of turkeys displayed typical signs of TRT after infection which indicates that MDA alone are incapable of preventing the disease. Differences in TRT course were recorded between different age groups of nonvaccinated birds. Birds infected at the 28th d of life (especially MDA- birds) developed more severe signs, and the level of aMPV replication was higher than that in birds infected on the 14th d of life. Despite the minor role in alleviating TRT course, MDA seems not to interfere with the vaccination efficacy. It is hard to predict whether the observed immune system stimulation differences between MDA+ and MDA- birds after vaccination can influence the outcome of vaccination efficacy under the field conditions., (Copyright © 2021 The Authors. Published by Elsevier Inc. All rights reserved.)

The complete genome sequence of a bacteriophage in the genus Phapecoctavirus (family Myoviridae ) isolated from a cloacal swab specimen from a domestic pigeon ( Columba livia f. domestica ) was identified using a high-throughput sequencing approach. The genome is 150,892 bp with a GC content of 39.1%, containing 269 open reading frames and 11 tRNA genes., (Copyright © 2021 Khalifeh et al.)

Introduction: Turkey histomonosis poses a serious threat to poultry production due to the ban on the use of effective drugs. The aim of the study was to evaluate the influence of a phytoncidal feed supplement on the course of histomonosis. The preparation was also analysed for immunomodulatory properties., Material and Methods: Clinical observations and production monitoring were conducted in a flock of turkeys with histomonosis from their 11 th to 56 th weeks of life which were treated with the adiCox SOL PF soluble supplement in a dose of 2.5 mL/L water. Later the preparation was used in a preventive dose (1 mL/L). The influence on the immune system was evaluated in broiler turkeys having been given adiCox SOL PF for 3 days in doses of 1 or 3 mL/L. The T and B lymphocyte percentages in turkey blood and spleen tissue were analysed with flow cytometry. ELISA was implemented to evaluate antibody titres after Ornithobacterium rhinotracheale vaccination, and biochemical analyses were performed to evaluate the supplement's safety., Results: AdiCox SOL PF was found effective in therapy and prevention of histomonosis. Additionally, adiCox SOL PF stimulated both humoral and cell-mediated immune mechanisms, without impairing the functions of internal organs. The treated turkeys also yielded better production results (eggs/hen, fertility, and hatchability)., Conclusion: AdiCox SOL PF possesses immunomodulatory properties and it can be used successfully in the prevention and therapy of histomonosis in turkeys., Competing Interests: Conflict of Interest Conflict of Interests Statement: The authors declare that there is no conflict of interests regarding the publication of this article., (© 2021 B. Tykałowski et al. published by Sciendo.)

Introduction: Migraine is considered not only as a separate clinical entity but also as a symptom of various brain disorders, including cerebral small vessel diseases. Since cerebral small vessel diseases are usually general angiopathies, evaluation of biopsy material other than brain tissue may help in their diagnosis in vivo. In patients with migraine, brain magnetic resonance imaging (MRI) often shows hyperintense changes in the cerebral white matter. Such changes may indicate the symptomatic nature of migraine and coexisting structural or biochemical vascular abnormalities., Material and Methods: To verify the hypothesis of the symptomatic nature of migraine in patients with abnormal brain neuroimaging, we performed an ultrastructural examination of skin and skeletal muscle vessels in biopsy material from 40 patients with clinically diagnosed migraine and hyperintense white matter lesions on MRI., Results: In 80% of the examined patients, ultrastructural examination showed various pathological changes in the microvessels including abnormalities characteristic of cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) and elastin disorders, as well as less specific changes such as thickening of the basal lamina, narrowing of the vessel lumen, degeneration of the vessel wall cells, endothelial activation, oncosis-like changes, and the presence of various types of deposits in the vessel wall. In 20% of the examined cases, ultrastructural examination of the vessels was normal., Conclusions: Patients with migraine and hyperintense cerebral white matter changes on MRI have an increased risk of concomitant microangiopathy. In this group of patients, skin-muscle biopsy allows the identification of cases with coexisting vessel abnormalities.

Transmissible viral proventriculitis (TVP) of chickens is manifested in decreased body weight gains, poor feed conversion and weight diversity. Although TVP etiology has not been defined, a Birnaviridae family member, named chicken proventricular necrosis virus (CPNV) is considered as a potential factor of a disease. This study was undertaken in order to reproduce TVP and to evaluate its etiology. Broiler chickens of the TVP-infected group were inoculated with TVP positive proventriculi homogenate on the 24th day of life. Samples were collected, on infection day and 14 days post-infection (dpi). The 14 dpi anatomo- and histopathological evaluation, revealed that we have succeeded to reproduce TVP. TVP-infected birds gained 30.38% less body weight. In the TVP-infected group a seroconversion against picornaviruses, fowl adenoviruses (FAdV) and infectious bursal disease viruses (IBDV) was recorded with an ELISA test. Using RT-PCR and PCR, CPNV was detected in proventriculi and FAdV in spleens and livers of infected birds, 14 dpi. Our study supports that CPNV is involved in the development of TVP. We did not record the presence of IBDV in TVP or control birds, despite our recording of a seroconversion against IBDV in TVP infected birds. CPNV and IBDV belong to the same family, which allows us to assume serological cross-reactivity between them. The role of FAdV needs further evaluation.

Background: A number of cases of severe parkinsonism-dystonia have been recognized and reported following the illicit use of ephedrone prepared from pseudoephedrine and potassium permanganate. The pathology associated with ephedrone neurotoxicity has not been described yet in the scientific literature., Objectives: To report the first neuropathological study of ephedrone toxicity., Methods: The brain of a 33-year-old Ukrainian female ex-ephedrone addict with a long history of l-dopa-unresponsive parkinsonism with dysarthria, dystonia, profound postural instability, cock-gait, and frequent falls, and on antiretroviral treatment, was examined using routine stains and immunohistochemistry., Results: Neuropathological findings included diffuse pallidal astrogliosis without neuronal depletion. There was also widespread vascular pathology with small vessels occluded by foreign material, associated with giant cell response without any evidence of consequent focal infarction and a cerebellar abscess., Conclusions: Clinical findings of l-dopa-unresponsive parkinsonism with dystonia, caused by illicit use of ephedrone, are fully consistent with neuropathological changes in the pallidum, lack of change in the SN, and preserved tyrosine hydroxylase activity. The findings in the basal ganglia are compatible with manganese toxicity. The vascular pathology is likely a joint effect of infection and the ephedrone toxicity on the vessels. © 2020 International Parkinson and Movement Disorder Society., (© 2020 International Parkinson and Movement Disorder Society.)

The pathology of pigeon circovirus (PiCV) is still unknown, but it is regarded as an immunosuppressant. This study aimed to find a correlation between PiCV natural infection and immunosuppression. The study was conducted with 56 pigeons divided into the following groups: PiCV-positive but showing (group S) or not (group I) non-specific clinical symptoms and asymptomatic pigeons negative for PiCV (group H). The percentage and apoptosis of T CD3 + and B IgM + splenocytes; the expression of CD4, CD8, and IFN-γ genes in splenic mononuclear cells; the number of PiCV viral loads in the bursa of Fabricius; and the level of anti-PiCV antibodies were analyzed. The results showed that the percentage of B IgM + cells was almost two-fold lower in group S than in group H, and that ca. 20% of the lymphocytes were apoptotic. No increased apoptosis was detected in TCD3 + subpopulation. The PiCV viral loads were approximately one thousand and ten thousand times higher in group S than in groups I and H, respectively. Our results indicate a possible correlation between the number of PiCV viral loads and severity of PiCV infection and confirm that PiCV infection leads to the suppression of humoral immunity by inducing B lymphocyte apoptosis.

Introduction: Despite vaccination against avian metapneumoviruses (aMPV), cases of turkey rhinotracheitis (TRT) caused by aMPV field strains are frequently reported. Differences have been shown in the level of immune system stimulation after aMPV vaccination between turkeys that do and do not possess specific anti-aMPV maternally derived antibodies (MDA). The article describes the influence of MDA on the production of IFNγ in the spleen of aMPV-vaccinated turkeys., Material and Methods: MDA+ or MDA- turkeys were vaccinated against TRT after hatching or on the 14 th day of life. Spleen samples were collected 3, 7, and 14 days post vaccination for mononuclear cell isolation. Real-time PCR, flow cytometry, and the enzyme-linked immunospot assay were used to evaluate the levels of IFNγ gene expression, production, and secretion by cells within the spleen samples., Results: Increased IFNγ gene expression was noticed after vaccination only in birds that did not possess MDA or possessed MDA at relatively low level (MDA+ birds vaccinated at 14 DOL). In all birds, an increased percentage of T lymphocytes producing IFNγ was recorded. The proportion of anti-aMPV IFNγ-secreting cells was increased only in MDA- birds., Conclusion: Besides having a protective role, MDA are known to interfere with vaccination efficacy. The analysis of our results confirms that MDA can decrease the level of immune system stimulation after aMPV vaccination of turkeys., Competing Interests: Conflict of Interest Conflict of Interests Statement: The authors declare that there is no conflict of interests regarding the publication of this article., (© 2020 M. Śmiałek et al. published by Sciendo.)

Considering the lack of research on the local immunity of the reproductive tract in other poultry species than chickens, the aim of this study was to determine the contribution of T and B cell subpopulations in different parts of breeder turkey oviduct mucous membrane with the use of flow cytometry. In addition, the study aimed to establish an impact of bird age and different stages of the egg production cycle on the systemic and local oviduct-related immune system structure. Our study results demonstrated a lower percentage of T lymphocytes in 32-wk turkey hens followed by a successively increasing population of these cells up to the 38th week of bird's life. The results of our study have also shown a similar dependency between birds' age and number of B lymphocytes. In addition, we demonstrated a decrease in the number of immune system cells in the oviduct, blood, and spleen of turkey hens in the late and end laying period. The differences reported in the number of lymphocyte subpopulations in the reproductive system of laying turkey hens at various stages of the production cycle may, to some extent, explain the frequency and periods of increased predilection to the incidence of infectious diseases in birds under field conditions., (Copyright © 2020 The Authors. Published by Elsevier Inc. All rights reserved.)

Background: Studies show that dysphagia is a common problem in patients with demyelinating diseases. However, there are no published studies on dysphagia in this group of patients, which would include the individual phases or the safety and effectiveness of the swallowing process., Objective: The main objective of this study was to assess the prevalence of swallowing disorders and to characterize them based on subjective assessment by the study subjects with multiple sclerosis and Devic's syndrome., Method: The study included 72 patients (47 F, 25 M). Patients at risk of dysphagia were identified using the DYMUS, EAT-10 and SDQ questionnaires. To assess the type of oral- and pharyngeal-stage dysphagia, questions in the questionnaires were classified into groups according to symptoms typical of each stage., Results: The risk of dysphagia and the need for instrumental examination were identified in 37.5% of the study subjects. Pharyngeal-stage dysphagia (repeated swallowing, increased effort of swallowing, cough, a feeling of food sticking in the throat) was reported to occur at a significantly higher frequency. However, no differences were found between difficulty in swallowing liquids and difficulty in swallowing solid food., Conclusion: There is a need for further research, which should include a detailed dysphagia-oriented diagnosis, with a view to gaining a detailed insight into the pathophysiology of deglutition in this group of patients., Competing Interests: Declaration of Competing Interest None declared., (Copyright © 2019 The Author(s). Published by Elsevier B.V. All rights reserved.)

Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a form of cerebellar ataxia related to mutations in the SACS gene on chromosome 13q12.12 encoding sacsin protein. Characteristic clinical features are ataxia, spasticity, distal muscle wasting, neuropathy, dysarthria, nystagmus, and finger or feet deformities. The presented case concerns a 32-year-old man with clinical diagnosis of ARSACS. Magnetic resonance imaging (MRI) scans of the brain revealed cerebellar atrophy typical of the disease while neuroimaging of the C1-C3 and C6-Th12 segments showed only the thin thoracic spinal cord. The patient died suddenly and a gross examination of the spinal cord revealed extraspinal tumour at the C4-C5 levels, which turned out to be an additional spinal cord. Microscopic examination showed an extensive ischemic necrosis involving C6-Th5 segments of the proper spinal cord, and disturbed intrinsic structure containing many pathological vessels of the extra spinal cord. Lack of visualization of C4-C5 spinal cord segments on MRI scans made diagnosis of diplomyelia in vivo impossible. However, diplomyelia does not exclude coexistence of ARSACS because of the occurrence of such clinical symptoms as dysarthria or nystagmus which cannot be explained by the presence of the spinal cord defect. The possibility of congenital malformations of the spinal cord in adults should be remembered as their early identification and surgical correction can improve neurological symptoms.

Introduction: Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a hereditary, progressive ischemic disease of small vessels of the brain characterized by migraine with aura (MA), recurrent subcortical ischemic episodes, cognitive decline and psychiatric disorders. CADASIL is caused by mutations in the NOTCH3 gene. We identified the NOTCH3 Y189C mutation as a genetic cause of CADASIL in a Polish family and provided its first clinical manifestation., Material and Methods: The study included twelve subjects from one family. The NOTCH3 mutation, APOE and MTHFR polymorphisms were determined by high-resolution melting analyses (HRMA) and Sanger sequencing. Neuroimaging included CT and MRI. Ultrastructural examination of skin-muscle biopsy material of the proband was performed., Results: The NOTCH3 Y189C mutation was present in a 36-year-old woman and her two sisters (aged 40 and 27) from 6 siblings. The MA was found in all of them, and started or became more severe after childbirth. The numerous T2/FLAIR hyperintense lesions were shown in the brain MRI. The deposition of granular osmiophilic material in the wall of small vessels of the proband observed in histopathological analysis confirmed the high degree of CADASIL severity., Conclusions: Patients with the Y189C mutation of NOTCH3 from the same family display a similar phenotype of CADASIL.

Immunoglobulins, which are passed vertically from hens to their progeny, are first present in the eggs but with time also in the developing embryos and eventually in the serum of hatching chicks, and have protective function during embryogenesis and in the first few weeks of birds' life, before the immune system becomes fully efficient. Considering the above fact, the aim of this study was to determine total levels of IgM and IgY as well as specific IgY antibody titers against selected pathogens in the serum of breeder turkeys and their progeny, as well as in egg yolks and egg whites. Study results demonstrated that the level of IgY antibodies in the serum of turkey breeder hens reached 22.04 mg/mL on average in the whole egg laying cycle. In addition, the mean transfer percentage of IgY antibodies from turkey layers to their progeny reached approximately 31.4%, but the level of this transfer differed depending on pathogen character and accounted for 33.2%, 51.9%, 45.1%, and 44.3% in the case of antibodies against avian metapneumoviruses, Newcastle disease virus, Ornithobacterium rhinortacheale, and Pasteurella multocida, respectively. Antibody percentage transfer differed also as affected by the stage of the egg production cycle. Study results confirmed the earlier observed dependency concerning the class of antibodies transferred to eggs from laying hens, and while the IgY were mainly detected in the egg yolk extracts, the IgM were found only in egg white extracts; in comparison to IgY, the IgM antibodies were not transferred to the serum of turkey poults. To our best knowledge, this is the first study that describes in detail the phenomenon of maternal antibody transfer in turkeys., (© 2019 Poultry Science Association Inc.)

Infections with immunosuppressive pigeon circovirus (PiCV) pose the most severe health problem to the global pigeon breeding. The vaccination with immunogenic PiCV recombinant capsid protein (PiCV rCP) is a potential tool for disease control. Because of the high prevalence of PiCV asymptomatic infections, the subclinically infected pigeons will be vaccinated in practice. The aim of this study was to answer a question if vaccination of asymptomatic, infected with PiCV pigeons induces a similar immune response to PiCV rCP as in uninfected birds. One hundred and twenty 6-week-old carrier pigeons were divided into 4 groups (2 groups of naturally infected and uninfected with PiCV individuals). Birds from groups V and V1 were vaccinated twice with PiCV rCP mixed with an adjuvant, whereas pigeons from groups C and C1 were immunized with an adjuvant only. The expression of genes encoding IFN-γ, CD4, and CD8 T lymphocyte receptors; the number of anti-PiCV rCP IgY-secreting B cells (SBC) and anti-PiCV rCP IgY were evaluated 2, 21, 39 and 46 days post vaccination (dpv). Study results showed that the expression of CD8 and IFN-γ genes was higher in both groups of infected pigeons than in the uninfected birds, irrespective of vaccination. In the uninfected birds, the expression of these genes was insignificantly higher in the vaccinated pigeons. The anti-PiCV rCP IgY-SBC were detected on 2 and 23 dpv and seroconversion was noted on 23 and 39 dpv in V and V1 groups, respectively. In the light of the results obtained, it could be concluded that pigeon circovirus recombinant capsid protein elicits the immune response in both naturally infected and uninfected pigeons, but its rate varies depending on PiCV infectious status. The infection with PiCV masks the potential cellular immune response to the vaccination with PiCV rCP and leads to the suppression of humoral immunity., Competing Interests: The authors have declared that no competing interests exist.

This paper is the first record describing the molecular analysis of Eimeria species occurring in capercaillie (Tetrao urogallus) and black grouse (Tetrao tetrix) which inhabit northern Eurasia and are species critically endangered of extinction. Actions undertaken to protect endangered species, such as breeding individuals in closed aviaries, could allow saving those birds, but they also pose risk of accidental healing of invasive diseases, like coccidiosis. Therefore, an investigation was conducted on fecal samples collected from the capercaillies and black grouse originating from the Kirov region (Russia) and breeding centers located in Poland. Results indicate that the average prevalence of Eimeria revealed 72% (average OPG = 3548) and 80% (average OPG = 5220) in capercaillies and black grouse respectively. Most of the Eimeria spp. oocysts were non-sporulated; however, two different morphological types were observed. The phylogenetic analysis of cox-1 and 18S rRNA genes revealed the analyzed Eimeria sequences to belong to two species. In addition, it showed some similarities between both analyzed genes. Most of the sequences obtained from both grouse species coccidia belonged to one species partially homologous to the Eimeria spp. isolated from ring-necked pheasant (approx. 94 and 96% for cox-1 and 18S rRNA genes, respectively). Two strains isolated from capercaillies imported from Russia were related to turkey coccidia: E. innocua and E. dispersa (97-99% homology) in the cox-1 gene analysis and only one of them was related to those Eimeria species in the 18S rRNA gene analysis (98-99% homology).

Cerebral autosomal-dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a stroke and dementia syndrome with degeneration and loss of vascular smooth muscle cells (VSMCs). The disease is due to mutations in NOTCH3 playing an important role in VSMC differentiation, proliferation and apoptosis. Searching for a possible cause of VSMC dysfunction in CADASIL, we investigated morphology and proliferative activity the affected myocytes. In material from autopsy brains and skin-muscle biopsies of patients with CADASIL diagnosis, assessment of VSMCs in arterial vessels at the level of light and electron microscopy was performed. Proliferative activity of VSMCs was evaluated in immune reactions to proliferative markers: proliferating cell nuclear antigen, and cyclins B1 and D. In CADASIL, abnormal morphology of VSMC nuclei was observed in 18.1%, 11.5%, and 6.9% of the cerebral, skin, and skeletal muscle vessels, respectively. The affected myocytes showed variability in nuclear size, irregularity in nuclear shape, and abnormal chromatin appearance. Frequently, double nuclei of equal size or micronuclei were observed. Sometimes, even multinuclear myocytes were found. In some of the nuclei immune reactions to the examined proliferative markers were positive. Aberrant structure and number of VSCM nuclei, and their immunoreactivity to proliferative markers suggest mitotic instability of vascular myocytes in CADASIL. We speculate that mutant NOTCH3 which is unable to control properly VSMC proliferation, and may be responsible for their premature or inappropriate entry into mitosis, irreversible arrest of the cell cycle, senescence or degeneration and loss., (© 2018 Japanese Society of Neuropathology.)

Aims: Familial hemiplegic migraine type 1 (FHM1) due to mutations in the CACNA1A gene is known as functional vascular disorder with cerebellar atrophy. We describe a case of a FHM1 family in which pathological changes occurred in both brain neuroimaging and skin and muscle biopsy., Materials and Methods: In 5 of 18 affected family members, brain MRI scans revealed hyperintense changes in the cerebral white matter. In 2 of these 5 patients, skin and muscle biopsies were performed at the interictal period of the disease and examined under light and transmission electron microscopy., Results: Ultrastructural examination of the biopsy samples revealed abnormal appearance of microvessels resembling oncosis. In the affected vessels, endothelial cells and myocytes/pericytes showed clear cytoplasm, distended endoplasmic reticulum, enlarged mitochondria, and numerous intracytoplasmic vesicular structures. Swollen endothelial cells often significantly narrowed vessel lumen., Conclusion: The morphological changes described for the first time in FHM1 suggest that the disease may not only be a functional, but also a structural vascular disorder. We suggest that the presence of these vascular abnormalities can interfere with microcirculation causing damage to the cerebral white matter, visible in MRI scans as hyperintense changes.
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Pigeon circovirus (PiCV) is the most frequently diagnosed virus in pigeons and is thought to be one of the causative factors of a complex disease called the young pigeon disease syndrome (YPDS). The development of a vaccine against this virus could be a strategy for YPDS control. Since laboratory culture of PiCV is impossible, its recombinant capsid protein (rCP) can be considered as a potential antigen candidate in sub-unit vaccines. The aim of this basic research was to evaluate the immune response of pigeons to PiCV rCP. Sixty six-week-old carrier pigeons were divided into two groups (experimental immunized with PiCV rCP mixed with an adjuvant, and control immunized with an adjuvant only), and immunized twice in a 21-day interval. On the day of immunization and on two, 23, 39, and 46 days post first immunization (dpv), samples of blood, spleen, and bursa of Fabricius were collected from six birds from each group to examine anti-PiCV rCP IgY, anti-PiCV rCP IgY-secreting B cells (SBC), IFN-γ gene expression, and percentage of T CD3⁺, CD4⁺, CD8⁺, and B IgM⁺ lymphocytes. The results indicated a correct immune response to PiCV rCP both in humoral and cell-mediated immunity, which was manifested by seroconversion since 23 dpv, by a significantly higher anti-PiCV rCP IgY-SBC number on two and 23 dpv, and significantly higher IFN-γ gene expression since two dpv. There were no significant differences or trends noted between particular T and B lymphocyte subpopulations. To conclude, PiCV rCP may be deemed immunogenic and could be considered as an antigen candidate in sub-unit vaccines against PiCV infections in pigeons.

Background: The aim of the study was to evaluate the impact of herbal extracts on selected immunity mechanisms in clinically healthy pigeons and pigeons inoculated with the pigeon paramyxovirus type 1 (PPMV-1). For the first 7 days post-inoculation (dpi), an aqueous solution of Aloe vera or licorice extract was administered daily at 300 or 500 mg/kg body weight (BW). The birds were euthanized at 4, 7 and 14 dpi, and spleen samples were collected during necropsy. Mononuclear cells were isolated from spleen samples and divided into two parts: one part was used to determine the percentage of IgM + B cells in a flow cytometric analysis, and the other was used to evaluate the expression of genes encoding IFN-γ and surface receptors on CD3 + , CD4 + and CD8 + T cells., Results: The expression of the IFN-γ gene increased in all birds inoculated with PPMV-1 and receiving both herbal extracts. The expression of the CD3 gene was lowest at 14 dpi in healthy birds and at 7 dpi in inoculated pigeons. The expression of the CD4 gene was higher in uninoculated pigeons receiving both herbal extracts than in the control group throughout nearly the entire experiment with a peak at 7 dpi. A reverse trend was observed in pigeons inoculated with PPMV-1 and receiving both herbal extracts. In uninoculated birds, increased expression of the CD8 gene was noted in the pigeons receiving a lower dose of the Aloe vera extract and both doses of licorice extracts. No significant differences in the expression of this gene were found between inoculated pigeons receiving both herbal extracts. The percentage of IgM + B cells did not differ between any of the evaluated groups., Conclusions: This results indicate that Aloe vera and licorice extracts have immunomodulatory properties and can be used successfully to prevent viral diseases, enhance immunity and as supplementary treatment for viral diseases in pigeons.

Circoviruses are circular single-stranded DNA (ssDNA) viruses that infect a variety of animals, both domestic and wild. Circovirus infection in birds is associated with immunosuppression and this in turn predisposes the infected animals to secondary infections that can lead to mortality. Farmed geese ( Anser anser ) in many parts of the world are infected with circoviruses. The majority of the current genomic information for goose circoviruses (GoCVs) ( n = 40) are from birds sampled in China and Taiwan, and only two genome sequences are available from Europe (Germany and Poland). In this study, we sampled 23 wild and 19 domestic geese from the Gopło Lake area in Poland. We determined the genomes of GoCV from 21 geese; 14 domestic Greylag geese ( Anser anser) , three wild Greylag geese ( A. anser ), three bean geese ( A. fabalis ), and one white fronted goose ( A. albifrons ). These genomes share 83-95% nucleotide pairwise identities with previously identified GoCV genomes, most are recombinants with exchanged fragment sizes up to 50% of the genome. Higher diversity levels can be seen within the genomes from domestic geese compared with those from wild geese. In the GoCV capsid protein ( cp ) and replication associated protein ( rep ) gene sequences we found that episodic positive selection appears to largely mirror those of beak and feather disease virus and pigeon circovirus. Analysis of the secondary structure of the ssDNA genome revealed a conserved stem-loop structure with the G-C rich stem having a high degree of negative selection on these nucleotides., Competing Interests: The authors declare no conflict of interest. The funders had no role in the design of the study; in the collection, analyses, or interpretation of data; in the writing of the manuscript, and in the decision to publish the results.

We describe an 86-year-old woman with a history of hypertension who presented sudden disturbances of consciousness and left hemiparesis. Brain magnetic resonance imaging (MRI) revealed diffused hyperintensive changes on T2-weighted images localized subcortically in the white matter of both cerebral hemispheres, corresponding to acute vasogenic edema, causing moderate mass effect. Posterior reversible encephalopathy syndrome was initially diagnosed. After implementation of anti-edema intravenous steroid treatment and hypotensive therapy the symptoms began to retire, till the total regression. The successive hospitalizations took place two and eight months later due to the occurrence of seizures, motor deficits and the development of mild cognitive impairment. Brain MRI revealed progression of the white matter changes and diffused subcortical microhemorrhages. Each time pulse steroid therapy was implemented and the symptoms improved significantly after several days. Chronic oral steroid treatment resulted in the stabilization of neurological status. The long-term observation of clinical symptoms, remission after immunosuppressive therapy and white matter changes with subcortical microhemorrhages in brain MRI leaded to the diagnosis of cerebral amyloid angiopathy-related inflammation., (Copyright © 2018. Published by Elsevier Urban & Partner Sp. z o.o.)

Lower motoneurons (MNs) show varied vulnerability in amyotrophic lateral sclerosis (ALS): those of non-ocular brainstem nuclei and most of those of the spinal cord are highly vulnerable, while those of extraocular brainstem nuclei are quite resistant. Results of our former study on the immunoexpression of the survival of motor neuron protein (SMN) and Gemins 2-4 in cervical spinal cord anterior horn -MNs of sporadic ALS patients suggested that a relative deficit in Gemin2 may play some role in the pathomechanism of the disease. Here, we tested this idea further by comparing immunoexpression patterns of SMN and Gemins 2-8 between MNs of the oculomotor nucleus and -MNs of the cervical spinal cord anterior horns in autopsy material from sALS patients and controls. In the latter, no considerable difference in any studied protein was found between these structures except that SMN expression was slightly but significantly lower (p < 0.01) in the oculomotor MNs. In the sporadic ALS patients, the expression of SMN, Gemin4 and Gemin7 was significantly weaker (p < 0.05, p < 0.05 and p < 0.01, respectively), while that of Gemin8 was stronger (p < 0.001) in the MNs of the oculomotor nucleus than in the examined cervical spinal cord anterior horn -MNs. The immunoexpression of Gemin3 and Gemin6 in the spinal cord correlated strongly negatively with ALS duration (Spearman's correlation coefficient: RS = -0.84, p < 0.001, and RS = -0.86, p = 0.002, respectively). In the oculomotor nucleus MNs, no studied protein immunoexpression correlated significantly with ALS duration, but there was a tendency for such negative correlation for Gemin2 (RS = -0.56, p = 0.07). There was an apparent relative deficit of Gemin2 and Gemin8 in the spinal cord -MNs and of Gemins 2, 4 and 7 in the oculomotor nucleus MNs. These data do not support the hypothesis that the diverse ALS vulnerability of the two MN subsets is related to their disparate expression patterns of SMN and Gemins 2-8. The differences in these patterns may result from ALS-related epiphenomena, or from intrinsic differences in the structure and function between the MN subsets, or both.

Introduction: Migraine is a common neurological disorder characterized by a particular phenotype, complex pathophysiology and a heterogeneous genetic background. Among several heritable forms, familial hemiplegic migraine is the best described one. In the majority of cases it is caused by mutations in one of three different genes., Case Report: Clinical symptoms of a 47 year old proband (and independently described in his 20 year old son) as well as differential diagnosis are discussed in the presented report. The most characteristic were recurrent attacks of blurred vision, paresthesias and hemiparesis often accompanied by speech disturbances and followed by severe headache with vomiting. Advanced morphological and genetic procedures were required to exclude MELAS, CADASIL and Call-Fleming syndrome. Finally, the definite diagnosis was possible after the application of the whole exome sequencing technique. It confirmed, for the first time in the Polish population, a heterozygous T666M mutation (c.1997C>T; p.Thr666Met) in the CACNA1A gene in the proband, the proband's son and in several other family members., Conclusion: The presented report provides clinical and genetic insight into familial hemiplegic migraine 1 resulting from a mutation in the CACNA1A gene., (Copyright © 2017 Polish Neurological Society. Published by Elsevier Urban & Partner Sp. z o.o. All rights reserved.)

Background: One of the most commonly applied protectotype vaccination protocol against infectious bronchitis (IB) in broiler chickens in the EU is simultaneous or alternate use of Ma5 and 4/91 vaccine strains. After IB vaccination and infection, systemic and upper respiratory tract (URT), humoral and cell-mediated immunity (CMI), are stimulated. The level of this stimulation correlates with the level of protection against IB., Results: We've investigated the development of URT and systemic, cell-mediated and humoral immunity in commercial broiler chickens vaccinated with Ma5 and/or 4/91 strains at hatch day. We've demonstrated that the group vaccinated with Ma5 and 4/91 strain simultaneously developed the most desirable immunity which reflects the level of CD8 + T cells stimulation in spleen and Harderian gland, as well as the level of IgA and IgY in URT washings and serum and their cross-reactivity with 7 IBV strains., Conclusions: Although we did not demonstrate directly why Ma5 + 4/91 protocol is so efficient it seems that it combines the benefits of monovalent vaccination with either Ma5 or 4/91 and while Ma5 seems to stimulate CMI more efficiently, the 4/91 strain generates a wider spectrum of immune system cross-reactivity and higher URT IgA production.

We report patients from a Polish family with cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) who possess a novel heterozygous R110C mutation in exon 3 of the NOTCH3 gene leading to stereotypical cysteine loss. The proband had only seizure attacks and her magnetic resonance imaging (MRI) showed very numerous hyperintense foci in the cerebral white matter in a location characteristic of CADASIL. Distinctive ultrastructural assessment of vessels from skin-muscle biopsy revealed only mild degenerative changes but relatively numerous homogeneous deposits of granular osmiophilic material (GOM). In the other symptomatic family members with the same mutation ischaemic strokes were present but not epilepsy. In the proband's affected brother at a similar age, the brain MRI was normal but vessels showed pronounced degenerative changes and irregular GOM deposits. The present report not only extends the list of known pathogenic mutations responsible for CADASIL but also emphasizes clinical and morphologic variability among family members with the same NOTCH3 mutation, suggesting that probably additional factors, not only mutations, may influence the disease phenotype. .

An excessive glutamate level can result in excitotoxic damage and death of central nervous system (CNS) cells, and is involved in the pathogenesis of many CNS diseases. It may also be related to a failure of the blood-spinal cord barrier (BSCB). This study was aimed at examining the effects of extended administration of monosodium glutamate on the BSCB and spinal cord cells in adult male Wistar rats. The glutamate was delivered by subarachnoidal application of glutamate-carrying electrospun nanofiber mat dressing at the lumbar enlargement level. Half of the rats with the glutamate-loaded mat application were treated systemically with the histone deacetylase inhibitor valproic acid. A group of intact rats and a rat group with subarachnoidal application of an 'empty' (i.e., carrying no glutamate) nanofiber mat dressing served as controls. All the rats were euthanized three weeks later and lumbar fragments of their spinal cords were harvested for histological, immunohistochemical and ultrastructural studies. The samples from controls revealed normal parenchyma and BSCB morphology, whereas those from rats with the glutamate-loaded nanofiber mat dressing showed many intraparenchymal microhemorrhages of variable sizes. The capillaries in the vicinity of the glutamate-carrying dressing (in the meninges and white matter alike) were edematous and leaky, and their endothelial cells showed degenerative changes: extensive swelling, enhanced vacuo-lization and the presence of vascular intraluminal projections. However, endothelial tight junctions were generally well preserved. Some endothelial cells were dying by necrosis or apoptosis. The adjacent parenchyma showed astrogliosis with astrocytic hypertrophy and swelling of perivascular astrocytic feet. Neurons in the parenchyma revealed multiple symptoms of degeneration, including, inter alia, perikaryal, dendritic and axonal swelling, and destruction of organelles. All the damage symptoms were slightly less severe in the rats given valproic acid treatment, and were absent from both the intact rats and the rats with 'empty' nanofiber mat dressing. These results demonstrate that glutamate-loaded nanofiber mat dressing can locally create glutamate levels capable of damaging BSCB and that the resulting damage can be mitigated with concurrent systemic valproate treatment.

CADASIL (cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy) is an inherited angiopathy characterized by degeneration and loss of vascular smooth muscle cells (VSMCs) of still unknown pathomechanism. Many functions of VSMCs, such as adhesion, apoptosis, contraction, differentiation, migration, and proliferation are determined by integrins - surface adhesion receptors involved in binding and interactions between cells and extracellular matrix (ECM). Since integrins play such an important role in VSMCs biology, disturbances in their expression may influence myocytes behavior and fate in CADASIL. In this study, we focused on the most important compounds of VSMCs integrins: subunits α 4 , β1, and β 3 in an attempt to elucidate their immune expression in the arterial media of CADASIL patients. The immunohistochemistry revealed a decreased expression of integrin β1 subunit (p < 0.001) but similar to the control expression of integrin subunits α 4 and β 3 . Decreased β1 immunoreactivity was observed in capillary vessels, arterioles, and small arteries. The abnormal immune expression of integrin β1 subunit was found even in microvessels without microscopically noted degenerative changes, which suggests that this is an early phenomenon in CADASIL. Since integrin β1 subunit is a compound of 10 heterodimer integrin receptors, its disturbed expression may significantly influence VSMCs biology leading to myocytes degeneration and loss via anoikis - a type of apoptotic cell death due to loss or inappropriate cell adhesion to ECM.