Blood-brain barrier breakdown and myeloperoxidase activity in silver catfish experimentally infected with Pseudomonas aeruginosa.

Central nervous system (CNS) infections continue to be an important cause of morbidity and mortality, and microbial invasion of the blood-brain barrier (BBB) is considered a prerequisite for CNS infections, which contribute to behavioural abnormalities and disease pathogenesis. Based on this information, the aim of this study was to evaluate whether Pseudomonas aeruginosa causes disruption of the BBB, and to investigate the involvement of cerebral myeloperoxidase (MPO) activity in this process in experimentally infected silver catfish. The permeability of the BBB to Evans blue dye increased in the infected animals on days three and six post-infection (PI) compared to the control group. Moreover, cerebral MPO activity and reactive oxygen species (ROS) levels also increased in the infected animals on days three and six PI compared to the control group. Based on this evidence, we concluded that P. aaeruginosa causes a disruption of the BBB, which may contribute to disease pathogenesis in the CNS. Moreover, the increase in cerebral MPO activity and ROS levels may be considered a pathway involved in BBB breakdown, allowing the passage of bacteria to the CNS.

Involvement of xanthine oxidase inhibition with the antioxidant property of nanoencapsulated Melaleuca alternifolia essential oil in fish experimentally infected with Pseudomonas aeruginosa.

Nanoencapsulated Melaleuca alternifolia essential oil (tea tree oil, TTO) is a natural alternative treatment, with 100% therapeutic efficacy in fish experimentally infected with Pseudomonas aeruginosa, and has also potent protective effects linked with antioxidant properties. However, the pathways responsible for the antioxidant capacity remain unknown. Thus, this study evaluated whether the inhibition of seric xanthine oxidase (XO) activity can be considered a pathway involved in the antioxidant capacity of nanoencapsulated TTO in fish experimentally infected with P. aeruginosa. Seric samples from fish infected with P. aeruginosa showed increased XO activity, as well as increased uric acid and reactive oxygen species (ROS) levels. In contrast, the prophylactic treatment with nanoencapsulated TTO prevented these infection-induced alterations. Based on the evidence obtained, the upregulation of seric XO activity induced pro-oxidative effects in the serum of fish experimentally infected with P. aeruginosa, due to excessive formation of uric acid, which stimulates the release of ROS. This treatment was able to prevent the upregulated seric XO activity and, consequently, the excessive formation of uric acid and ROS. In summary, inhibition of seric XO activity can be considered a pathway involved in the antioxidant capacity of nanoencapsulated TTO in fish experimentally infected with P. aeruginosa.

Serum adenosine deaminase and xanthine oxidase activities in silver catfish naturally infected with Ichthyophthirius multifiliis: The influence of these enzymes on inflammatory and oxidative status.

Adenosine deaminase (ADA) activity, through adenosine (Ado) levels, as well as xanthine oxidase (XO) activity through uric acid levels exerts an essential role on immune and inflammatory responses during infectious diseases. Thus, the aim of this study was to evaluate the involvement of seric ADA and XO activities in the inflammatory and oxidative status of silver catfish naturally infected with Ichthyophthirius multifiliis. Seric ADA activity decreased, while Ado levels increased in infected animals compared to uninfected animals. Moreover, the seric XO activity increased in infected animals compared to uninfected animals, alongside the seric levels of uric acid, metabolites of nitric oxide (NOx) and reactive oxygen species (ROS). Based on this evidence, the downregulation of seric ADA activity exerts an anti-inflammatory profile, contributing to restricting the inflammatory process. The most important finding is that upregulation of seric XO activity leads to an excessive formation of uric acid, which contributes to oxidative and inflammatory processes. Moreover, uric acid induces the release of pro-inflammatory and pro-oxidative mediators, such NOx and ROS, which contribute directly to disease pathogenesis. In summary, the upregulation of XO activity may be considered a pathway involved in NOx and ROS production in silver catfish infected with I. multifiliis.

Xanthine oxidase activity exerts pro-oxidative and pro-inflammatory effects in serum of silver catfish fed with a diet contaminated with aflatoxin B1.

Several studies have associated the involvement of xanthine oxidase (XO) activity, a source of uric acid and reactive oxygen species (ROS), to pro-oxidative and pro-inflammatory effects during pathological conditions. Considering this, the aim of this study was to evaluate whether upregulation on seric XO activity may be a pathway involved in the oxidative stress in fish exposed to a diet contaminated with aflatoxin B1 (AFB1 ), as well as whether it may be considered a pathway involved in ROS and NOx production. Xanthine oxidase activity, as well as the uric acid, ROS and NOx levels increased in serum of fish fed with a AFB1 -contaminated diet on days 14 and 21 post-feeding compared to fish fed with a basal diet. Based on these evidences, upregulation of seric XO activity induces pro-oxidant and pro-inflammatory profiles in serum of fish fed with a AFB1 -contaminated diet due to excessive formation on uric acid. Also, the excessive uric acid induces the release of pro-oxidant and pro-inflammatory mediators, as ROS and NOx, also contributing to oxidative and inflammatory profiles. In summary, the upregulation on seric XO activity may be considered a pathway involved in the oxidative stress of fish exposed to a diet contaminated with AFB1 .

Purinergic signalling displays an anti-inflammatory profile in the spleen of fish experimentally infected with Aeromonas caviae: Modulation of the immune response.

Extracellular adenosine triphosphate (ATP) and its metabolite adenosine (Ado) are recognized as key mediators of immune and inflammatory responses. Depending on its concentration, ATP may act as an immunostimulant or immunodepressant, while Ado levels display an anti-inflammatory profile. The aim of this study was to evaluate whether splenic purinergic signalling is capable of modulating immune and inflammatory responses in fish experimentally infected with Aeromonas caviae. Triphosphate diphosphohydrolase (NTPDase) and 5'-nucleotidase activities increased in the spleen of silver catfish (Rhamdia quelen) experimentally infected with A. caviae compared with the uninfected control group. Moreover, splenic Ado levels increased in the infected animals relative to the uninfected control group. Based on these lines of evidence, our findings revealed that adenine nucleotide hydrolysis is modified in the spleen of fish infected with A. caviae attempting to restrict the inflammatory process through the upregulation of NTPDase and 5'-nucleotidase activities, which occurs in an attempt to hydrolyse the excessive ATP in the extracellular environment and rapidly hydrolyse AMP to form Ado. In summary, purinergic signalling can modulate immune and inflammatory responses during A. caviae infection.

Involvement of cholinergic and adenosinergic systems on the branchial immune response of experimentally infected silver catfish with Streptococcus agalactiae.

It has been recognized that the cholinergic and adenosinergic systems have an essential role in immune and inflammatory responses during bacterial fish pathogens, such as the enzymes acetylcholinesterase (AChE) and adenosine deaminase (ADA), which are responsible for catalysis of the anti-inflammatory molecules acetylcholine (ACh) and adenosine (Ado) respectively. Thus, the aim of this study was to investigate the involvement of the cholinergic and adenosinergic systems on the immune response and inflammatory process in gills of experimentally infected Rhamdia quelen with Streptococcus agalactiae. Acetylcholinesterase activity decreased, while ACh levels increased in gills of infected animals compared to uninfected animals. On the other hand, a significant increase in ADA activity with a concomitant decrease in Ado levels was observed in infected animals compared to uninfected animals. Based on this evidence, we concluded that infection by S. agalactiae in silver catfish alters the cholinergic and adenosinergic systems, suggesting the involvement of AChE and ADA activities on immune and inflammatory responses, regulating the ACh and Ado levels. In summary, the downregulation of AChE activity exerts an anti-inflammatory profile in an attempt to reduce or prevent the tissue damage, while the upregulation of ADA activity exerts a pro-inflammatory profile, contributing to disease pathophysiology.

Aeromonas caviae inhibits hepatic enzymes of the phosphotransfer network in experimentally infected silver catfish: Impairment on bioenergetics.

Several studies have been demonstrated that phosphotransfer network, through the adenylate kinase (AK) and pyruvate kinase (PK) activities, allows for new perspectives leading to understanding of disease conditions associated with disturbances in energy metabolism, metabolic monitoring and signalling. In this sense, the aim of this study was to evaluate whether experimental infection by Aeromonas caviae alters hepatic AK and PK activities of silver catfish Rhamdia quelen. Hepatic AK and PK activities decreased in infected animals compared to uninfected animals, as well as the hepatic adenosine triphosphate (ATP) levels. Also, a severe hepatic damage was observed in the infected animals due to the presence of dilation and congestion of vessels, degeneration of hepatocytes and loss of liver parenchyma architecture and sinusoidal structure. Therefore, we have demonstrated, for the first time, that experimental infection by A. caviae inhibits key enzymes linked to the communication between sites of ATP generation and ATP utilization. Moreover, the absence of a reciprocal compensatory mechanism between these enzymes contributes directly to hepatic damage and for a severe energetic imbalance, which may contribute to disease pathophysiology.

Accessory subscapularis muscle - A forgotten variation?

The quadrangular space is a space in the axilla bounded by the inferior margin of the teres minor muscle, the superior margin of the teres major muscle, the lateral margin of the long head of the triceps brachii muscle and the surgical neck of the humerus, medially. The axillary nerve (C5-C6) and the posterior circumflex humeral artery and veins pass through this space in order to supply their territories. The subscapularis muscle is situated into the scapular fossa and inserts itself into the lesser tubercle of the humerus, thus helping stabilize the shoulder joint. A supernumerary muscle known as accessory subscapularis muscle originates from the anterior surface of the muscle and usually inserts itself into the shoulder joint. It is a rare variation with few reports of its existence and incidence. We present a case of the accessory subscapularis muscle in a male cadaver fixated with a 10% formalin solution. The muscle passed anteriorly to the axillary nerve, thus, predisposing an individual to quadrangular space compression syndrome. We perform a review of the literature and address its clinical, anthropological and anatomical significance.

Trypanosomatid essential metabolic pathway: new approaches about heme fate in Trypanosoma cruzi.

Trypanosoma cruzi, the causal agent of Chagas disease, has a complex life cycle and depends on hosts for its nutritional needs. Our group has investigated heme (Fe-protoporphyrin IX) internalization and the effects on parasite growth, following the fate of this porphyrin in the parasite. Here, we show that epimastigotes cultivated with heme yielded the compounds α-meso-hydroxyheme, verdoheme and biliverdin (as determined by HPLC), suggesting an active heme degradation pathway in this parasite. Furthermore, through immunoprecipitation and immunoblotting assays of epimastigote extracts, we observed recognition by an antibody against mammalian HO-1. We also detected the localization of the HO-1-like protein in the parasite using immunocytochemistry, with antibody staining primarily in the cytoplasm. Although HO has not been described in the parasite's genome, our results offer new insights into heme metabolism in T. cruzi, revealing potential future therapeutic targets.

HLA and MICA genes in patients with tuberculosis in Brazil.

Major histocompatibility complex (MHC) genes have been investigated because of their crucial role in the defense against pathogens and their high degree of polymorphism. We performed a case-control study to assess a genetic association of MHC genes with susceptibility to tuberculosis (TB). The allelic lineages HLA-A*02 and B*18 were significantly less frequent in TB patients (n = 112, 44.6% women) than in controls (n = 224, 51.5% women): 18.8% vs 26.5%; odds ratio (OR) = 0.64; P = 0.037 and 2.7% vs 6.9%; OR = 0.37; P = 0.041. The negative association with haplotype HLA-B*18-MICA*018 (2.3% patients vs 6.4% controls; OR = 0.34; P = 0.035) was significant as a consequence of strong linkage disequilibrium (D' = 0.827 for patients and 0.923 for controls). These findings suggest a trend toward protection of the HLA-A*02 and HLA-B*18 alleles.

Heme-induced Trypanosoma cruzi proliferation is mediated by CaM kinase II.

Trypanosoma cruzi, the etiologic agent of Chagas disease, is transmitted through triatomine vectors during their blood-meal on vertebrate hosts. These hematophagous insects usually ingest approximately 10mM of heme bound to hemoglobin in a single meal. Blood forms of the parasite are transformed into epimastigotes in the crop which initiates a few hours after parasite ingestion. In a previous work, we investigated the role of heme in parasite cell proliferation and showed that the addition of heme significantly increased parasite proliferation in a dose-dependent manner [1]. To investigate whether the heme effect is mediated by protein kinase signalling pathways, parasite proliferation was evaluated in the presence of several protein kinase (PK) inhibitors. We found that only KN-93, a classical inhibitor of calcium-calmodulin-dependent kinases (CaMKs), blocked heme-induced cell proliferation. KN-92, an inactive analogue of KN-93, was not able to block this effect. A T. cruzi CaMKII homologue is most likely the main enzyme involved in this process since parasite proliferation was also blocked when Myr-AIP, an inhibitory peptide for mammalian CaMKII, was included in the cell proliferation assay. Moreover, CaMK activity increased in parasite cells with the addition of heme as shown by immunological and biochemical assays. In conclusion, the present results are the first strong indications that CaMKII is involved in the heme-induced cell signalling pathway that mediates parasite proliferation.

Identification of a novel UMOD mutation (c.163G>A) in a Brazilian family with autosomal dominant tubulointerstitial kidney disease.

Autosomal dominant tubulointerstitial kidney disease (ADTKD) is characterized by autosomal dominant inheritance, progressive chronic kidney disease, and a bland urinary sediment. ADTKD is most commonly caused by mutations in the UMOD gene encoding uromodulin (ADTKD-UMOD). We herein report the first confirmed case of a multi-generational Brazilian family with ADTKD-UMOD, caused by a novel heterozygous mutation (c.163G>A, GGC→AGC, p.Gly55Ser) in the UMOD gene. Of 41 family members, 22 underwent genetic analysis, with 11 individuals found to have this mutation. Three affected individuals underwent hemodialysis, one peritoneal dialysis, and one patient received a kidney transplant from a family member later found to be genetically affected. Several younger individuals affected with the mutation were also identified. Clinical characteristics included a bland urinary sediment in all tested individuals and a kidney biopsy in one individual showing tubulointerstitial fibrosis. Unlike most other reported families with ADTKD-UMOD, neither gout nor hyperuricemia was found in affected individuals. In summary, we report a novel UMOD mutation in a Brazilian family with 11 affected members, and we discuss the importance of performing genetic testing in families with inherited kidney disease of unknown cause.

WITHDRAWN: Recommendations for evaluation and management of pain in patients with mucopolysaccharidosis in Latin America.

The Publisher regrets that this article is an accidental duplication of an article that has already been published, https://doi.org/10.1016/j.jpainsymman.2018.03.023. The duplicate article has therefore been withdrawn. The full Elsevier Policy on Article Withdrawal can be found at https://www.elsevier.com/about/our-business/policies/article-withdrawal.

Pilot Study of Early Monitoring Using Urinary Screening for BK Polyomavirus as a Strategy for Prevention of BKV Nephropathy in Kidney Transplantation.

BACKGROUND: Urine monitoring programs represent an important strategy for early diagnosis of reactivation of BK polyomavirus (BKV) in kidney transplant recipients. This study analyzes a BKV urine screening model in kidney transplant patients. METHODS: Urinary screening for BKV reactivation was performed by urinary decoy cell and polymerase chain reaction (PCR) tests in samples from 32 consecutive kidney transplant patients, collected in a 6-month follow-up period. PCR in plasma samples and BKV immunohistochemical studies to assess BKV renal disease, if a kidney biopsy was indicated, were performed. RESULTS: The urinary screening for BKV among 32 renal receptors was positive in 18 patients (56%) by the concomitant use of the decoy cells and/or qualitative PCR at some time during the study period. Transfusion before transplantation was significantly associated with urinary decoy cell positive screening (odds ratio = 11; 95% confidence interval = 1.47 to 82.4; P < .05); and so was male sex (odds ratio = 2.02; 95% confidence interval = 1.07 to 3.83; P < .05). The clinical management of screening positive cases consisted of decreasing or changing the immunosuppression regimen. Sixteen renal biopsies were performed. Immunohistochemistry for SV40 T antigen was negative in all biopsies. After 1 year of follow-up, no patient developed BKV-associated nephropathy, and there was no difference in renal function between patients positive and negative for BKV urinary screening. CONCLUSIONS: Early urinary monitoring is effective in detection of BKV replication and represents a good strategy to minimize the deleterious effects caused by the presence of the virus on preservation of graft function.

Mucosal leishmaniasis ("espundia") responsive to low dose of N-methyl glucamine (Glucantime) in Rio de Janeiro, Brazil.

Response to treatment with antimonial drugs varies considerably depending on the parasite strain involved, immune status of the patient and clinical form of the disease. Therapeutic regimens with this first line drug have been frequently modified both, in dose and duration of therapy. A regimen of 20 mg/kg/day of pentavalent antimony (Sb5+) during four weeks without an upper limit on the daily dose is currently recommended for mucosal disease ("espundia"). Side-effects with this dose are more marked in elderly patients, more commonly affected by this form of leishmaniasis. According to our experience, leishmaniasis in Rio de Janeiro responds well to antimony and, in cutaneous disease, high cure rates are obtained with 5 mg/kg/day of Sb5+ during 30 to 45-days. In this study a high rate of cure (91.4%) employing this dose was achieved in 36 patients with mild disease in this same geographic region. Side-effects were reduced and no antimony refractoriness was noted with subsequent use of larger dose in patients that failed to respond to initial schedule.

Neuronal ceroid lipofuscinoses: a clinical and morphological study of 17 patients from southern Brazil.

The neuronal ceroid lipofuscinoses (NCL) are a group of inherited progressive neurodegenerative disorders with presentation from infancy to adulthood. Three main childhood forms can be established on the basis of age of onset, clinical course, and ultrastructural morphology: infantile (INCL), late infantile (LINCL), and juvenile (JNCL). Several variant subtypes have been described. Genetic and biochemical analysis are helping to better understand, diagnose and classify these disorders. We report on clinical, neurophysiological, neuroradiological, and morphological data from 17 patients with different forms (infantile, late infantile, and juvenile ) of neuronal ceroid lipofuscinoses (NCL) evaluated at Hospital de Clínicas de Porto Alegre, Southern Brazil, during 6 years (1992-1997). Seven cases were infantile; 5 were late infantile; and 5 were juvenile NCL. Gender ratio was male:female, 11:6. Age at presentation varied from 2-24 months for INCL; 2,5 to 5 years for LINCL; and 4-10 years for the JNCL cases. Seizures (6 patients) and psychomotor retardation (1 patient) were the initial symptoms in the INCL group. All the patients in the group of LINCL had the usual findings. JNCL patients manifested different initial symptoms, although tending to follow a similar clinical picture within familial cases. Epidemiological data on the prevalence of NCLs in Brazil are not available, we expect this series of cases to contribute to further research in our population.

Parâmetros reprodutivos de touros Nelore (Bos taurus indicus) criados a pasto, em de diferentes faixas etárias / Reproductive parameters of pasture-raised Nelore bulls (Bos taurus indicus) in different age

Este estudo teve o objetivo de demonstrar o efeito da idade sobre as características de circunferência escrotal, cor de pelagem e qualidade seminal, desde a puberdade até após a maturidade sexual. Foram utilizados dados de 6607 exames andrológicos de touros da raça Nelore criados a pasto. Os animais eram de diferentes faixas etárias, variando de 12 até 80 meses. O exame andrológico consistiu em exame clínico reprodutivo, perímetro escrotal (PE), avaliação do sêmen e nota para cor do pelame (COR; 1-4). Estabeleceram-se quatro faixas etárias, que foram comparadas pelo teste de Bonferroni. Os parâmetros seminais PE e COR variaram (P<0,05) conforme a faixa etária dos animais: A) 12-18m: COR=1,45±0,64a, PE=31,63±3,51cma, motilidade total (Mot)=67,73±17,99%a, total de defeitos espermáticos (TDE)=16,22±16,95%a; B) 18-24m: COR=1,50±0,57b, PE=32,00±3,47cma, Mot=69,60±29,13%a, TDE=14,49±15,00%b; C) 24-36m: COR=1,51±0,66b, PE=33,56±3,91cmb, Mot=69,46±15,52%a, TDE=12,29±12,92%c; D) 36-48m: COR=1,60±0,57c, PE=36,66±3,50cmc, Mot=71,04±16,19%b, TDE=10,87±12,97%d; E) >48m: COR=1,64±0,72c, PE=38,00±3,22d, Mot=71,54±15,30b, TDE=9,70±16,95d. Concluiu-se que a faixa etária influencia o tamanho testicular, a cor da pelagem e os parâmetros de qualidade seminal. Com o avançar da idade, ocorre escurecimento do pelo, aumento do perímetro escrotal, da motilidade e do vigor, e redução dos defeitos espermáticos de touros Nelores criados a pasto, avaliados a partir de 12 meses de idade.(AU)

This study aimed to demonstrate the effect of age on bull traits such as scrotal circumference, pelage color, and semen quality, from puberty to post sexual maturity. Data from 6607 breeding soundness examinations of pasture raised Nelore bulls were used. The animals presented different age groups ranging from 12 to 80 months. The andrological examination consisted in reproductive clinical evaluation, assessment of scrotal perimeter (PE). In addition, color of pelage (COR; 1-4) was recorded for each animal. Four age groups were established, which were compared by Bonferroni test. Semen parameters, scrotal circumference (PE) and color of the pelage (COR) varied (P< 0.05) according to the age range: A) 12-18m: COR=1.45±0.64 a , PE=31.63±3.51cm a , Total Motility (Mot)=67.73±17.99% a , Total os sperm defects (TDE)=16.22±16.95% a ; B) 18-24m: COR=1.50±0.57 b , PE=32.00±3.47cm a , Mot=69.60±29.13% a , TDE=14.49±15.00% b ; C) 24-36m: COR=1.51±0.66 b , PE=33.56±3.91cm b , Mot=69.46±15.52% a , TDE=12.29±12.92% c ; D) 36-48m: COR=1.60±0.57 c , PE=36.66±3.50cm c , Mot=71.04±16.19% b , TDE=10.87±12.97% d ; E) >48m: COR=1.64±0.72 c , PE=38.00±3.22 d , Mot=71.54±15.30 b , TDE=9.70±16.95 d . It was concluded that age influences testicular size, pelage color, and semen quality parameters. As the age progresses, there is an increase in scrotal perimeter, hair darkening, sperm motility and vigor, and reduction of sperm morphological defects of pasture raised Nelore bulls, assessed as from as 12 months of age.(AU)

Regeneration of skin tissue promoted by mesenchymal stem cells seeded in nanostructured membrane.

BACKGROUND: The mesenchymal stem cell therapy has proven to be an effective option in the treatment of skin injuries. The combination of these cells with nanostructured membranes seems to be the future for tissues recovery. The aim of this project was to use biomolecules of polysaccharides to be incorporated on regenerated cellulose membranes and to prospect the improvement as bioactive wound dressings with mesenchymal stem cells. METHODS: The biocomposites were obtained after defibrillation with the use of never-dried bacterial cellulose to form a pulp, and, after the films were regenerated, in the presence of gellan gum with or without fluconazole. Membrane atomic force microscopy was performed for comparison of their structures. RESULTS: Adipose-derived mesenchymal stem cells were obtained from human adipose tissue liposuction in accordance with Zuk et al. The flow cytometric analysis and induction tests for adipocytes and osteocytes were performed. In vitro assays were performed on different membranes to evaluate the ability of these cells to adhere at 2 hours and proliferate at 7 days; the results were obtained by use of the MTT cell counting technique. In vivo testing allowed us to observe cell migration and participation in wound-healing by fluorescence labeling of the cells with BrdU. The bioactive curative, seeded with cells, was tested in skin burned in a murine model. CONCLUSIONS: The bacterial cellulose with gelan gum membrane incorporated with fluconazole presented the best performance in adhesion and proliferation tests. The cells can be identified in burned host tissue after occurrence of the wound.

Inherited cerebellar ataxia in childhood: a pattern-recognition approach using brain MRI.

Ataxia is the principal symptom of many common neurologic diseases in childhood. Ataxias caused by dysfunction of the cerebellum occur in acute, intermittent, and progressive disorders. Most of the chronic progressive processes are secondary to degenerative and metabolic diseases. In addition, congenital malformation of the midbrain and hindbrain can also be present, with posterior fossa symptoms related to ataxia. Brain MR imaging is the most accurate imaging technique to investigate these patients, and imaging abnormalities include size, shape, and/or signal of the brain stem and/or cerebellum. Supratentorial and cord lesions are also common. This review will discuss a pattern-recognition approach to inherited cerebellar ataxia in childhood. The purpose is to provide a comprehensive discussion that ultimately could help neuroradiologists better manage this important topic in pediatric neurology.

Severity score system for progressive myelopathy: development and validation of a new clinical scale.

Progressive myelopathies can be secondary to inborn errors of metabolism (IEM) such as mucopolysaccharidosis, mucolipidosis, and adrenomyeloneuropathy. The available scale, Japanese Orthopaedic Association (JOA) score, was validated only for degenerative vertebral diseases. Our objective is to propose and validate a new scale addressing progressive myelopathies and to present validating data for JOA in these diseases. A new scale, Severity Score System for Progressive Myelopathy (SSPROM), covering motor disability, sphincter dysfunction, spasticity, and sensory losses. Inter- and intra-rater reliabilities were measured. External validation was tested by applying JOA, the Expanded Disability Status Scale (EDSS), the Barthel index, and the Osame Motor Disability Score. Thirty-eight patients, 17 with adrenomyeloneuropathy, 3 with mucopolysaccharidosis I, 3 with mucopolysaccharidosis IV, 2 with mucopolysaccharidosis VI, 2 with mucolipidosis, and 11 with human T-cell lymphotropic virus type-1 (HTLV-1)-associated myelopathy participated in the study. The mean ± SD SSPROM and JOA scores were 74.6 ± 11.4 and 12.4 ± 2.3, respectively. Construct validity for SSPROM (JOA: r = 0.84, P < 0.0001; EDSS: r = -0.83, P < 0.0001; Barthel: r = 0.56, P < 0.002; Osame: r = -0.94, P < 0.0001) and reliability (intra-rater: r = 0.83, P < 0.0001; inter-rater: r = 0.94, P < 0.0001) were demonstrated. The metric properties of JOA were similar to those found in SSPROM. Several clinimetric requirements were met for both SSPROM and JOA scales. Since SSPROM has a wider range, it should be useful for follow-up studies on IEM myelopathies.