Prognostička vrijednost praćenja razine serumskog proteina amiloid a u bolesnika s moždanim infarktom

Serum amyloid A protein (SAA) is an acute-phase plasma protein, which increases in response to tissue damage. The aim of the study was to investigate SAA in the blood of patients with cerebral infarction. Sixty subjects were studied, including 45 patients with cerebral infarction and 15 controls. SAA was determined using ELISA method on days 1, 3, 7 and 14 after ischemic stroke onset. SAA was found to increase by day 3, which was followed by a decrease if no infectious complication occurred. The concentration of SAA was statistically significantly increased on days 1, 3, 7 and 14 (p < 0.05); however, the increase was greater in patients with severe clinical manifestations. A statistically significant SAA increase by day 14 was recorded in patients with an accompanying infectious process. The study showed the concentration of SAA to depend on the clinical severity of ischemic stroke. SAA was found to be a sensitive early indicator of possible infectious complications.Serumski protein amiloid A (SAA) je plazmatski protein akutne faze koji raste u odgovoru na tkivno oštećenje. Cilj ovoga ispitivanja bio je istražiti SAA u krvi bolesnika s moždanim infarktom. Ispitivanje je obuhvatilo 60 osoba, od toga 45 bolesnika s cerebralnim infarktom i 15 kontrolnih osoba. SAA je mjeren pomoću metode ELISA, i to 1., 3., 7. i 14. dana nakon nastupa ishemijskog moždanog udara. Povišenje SAA zabilježeno je do 3. dana, nakon čega je uslijedilo snižavanje njegove razine ukoliko nije bilo infektivnih komplikacija. Koncentracija SAA bila je statistički značajno povišena 1., 3., 7. i 14. dana (p<0,05), međutim, taj je porast bio veći u skupini bolesnika s težim kliničkim manifestacijama bolesti. Statistički značajno povišenje SAA zabilježeno je do 14. dana u bolesnika s pratećim infektivnim procesom. Ispitivanje je pokazalo da koncentracija SAA ovisi o kliničkoj težini ishemijskog udara. SAA je osjetljiv rani pokazatelj mogućih infektivnih komplikacija

Analiza walidacyjna polskiej wersji Międzynarodowego Kwestionariusza Jakości Życia w Stwardnieniu Rozsianym (MusiQoL)

Background and purpose The aim of this study was to perform a validation analysis of the Polish adaptation of the Multiple Sclerosis International Quality of Life Questionnaire, MusiQoL. Material and methods Validation analysis included the translation of the original English version into Polish according to translation principles and the analysis of convergent validity, internal reliability and reproducibility of the Polish version of MusiQoL. The study included 150 randomly chosen patients (109 women and 41 men) with definite multiple sclerosis (MS) diagnosed according to McDonald criteria. Mean age of patients was 41 ± 10 years and mean disease duration was 11.7 ± 7.2 years. The patients completed the examined MusiQoL, the Functional Assessment of Multiple Sclerosis (FAMS) and the Multiple Sclerosis Impact Scale (MSIS-29). Data regarding sociodemographic status and MS history were collected. The disability of the patients was assessed according to the Expanded Disability Status Scale (EDSS). The examination was repeated after 28 ± 4 days. Results The internal reliability, convergent validity and reproducibility of MusiQoL were satisfactory. The dimensions of the scale exhibited high internal consistency (Cronbach's alpha from 0.67 to 0.90). The MusiQoL correlated with FAMS (positive correlations), EDSS and MSIS-29 (negatively). Conclusions Psychometric-statistical analysis showed that the Polish version of MusiQoL is a valuable measure to examine the health-related quality of life of Polish MS patients.Wstęp i cel pracy Celem pracy była analiza walidacyjna aspektów psychometrycznych polskiej adaptacji Międzynarodowego kwestionariusza jakości życia w stwardnieniu rozsianym (SR) – Multiple Sclerosis International Quality of Life Questionnaire, MusiQoL. Materiał i metody Analiza walidacyjna objęła tłumaczenie oryginalnej wersji angielskiej na język polski zgodnie z obowiązującymi zasadami translacyjnymi oraz analizę aspektów trafności i rzetelności skali MusiQoL. Do badań włączono 150 losowo wybranych pacjentów z rozpoznaniem SR według kryteriów McDonalda (109 kobiet i 41 mężczyzn). Średnia wieku badanych wynosiła 41 ± 10 lat, średni czas trwania choroby – 11,7 ± 7,2 roku. Pacjenci wypełniali badany kwestionariusz MusiQoL, Kwestionariusz do Oceny Jakości Życia w Stwardnieniu Rozsianym (FAMS) oraz Skalę Wpływu Stwardnienia Rozsianego na Jakość Życia Chorych (MSIS-29). Zebrano dane społeczno-demograficzne pacjentów i dotyczące historii przebiegu choroby. U wszystkich oceniono ponadto stopień niesprawności na podstawie Rozszerzonej Skali Niewydolności Ruchowej (EDSS). Badania przeprowadzono w dniu 0, a następnie powtórzono po 28 ± 4 dniach. Wyniki Rzetelność i badane aspekty trafności polskiej wersji skali MusiQoL są zadowalające. Wykazano również powtarzalność wyników badanej skali oraz dużą spójność wewnętrzną poszczególnych podskal (współczynnik alfa Cronbacha od 0,67 do 0,90). Stwierdzono korelacje skali MusiQoL ze skalą FAMS (korelacje dodatnie), EDSS i MSIS-29 (korelacje ujemne). Wnioski Analiza psychometryczno-statystyczna wykazała, że polska wersja skali MusiQoL jest wartościowym narzędziem do badania wpływu choroby na jakość życia polskich chorych na SR

Obustronny udar wzgórza z dysfunkcją podwzgórza

Unilateral thalamic lesions cause transient or permanent behavioral, sensory and oculomotor disturbances; bilateral lesions of thalamus result in more severe and longer lasting symptoms. We present an atypical case of bilateral paramedian thalamic infarct with concomitant hypothalamic dysfunction. The only risk factor of ischaemic stroke found in the patient was a short lasting episode of atrial fibrillation. Bilateral paramedian thalamic infarcts may result from occlusion of one paramedian thalamic artery, which arises from the posterior cerebral artery, either with separated or with a common trunk, thus supplying the thalamus bilaterally. Independently of anatomical variants of thalamus blood supply, the most probable cause of infarct in our patient was unilateral or bilateral occlusion of the posterior cerebral artery by cardioembolism, probably in the course of basilar artery occlusion. Hypothalamic dysfunction may accompany thalamic infarcts; thus hypothalamo-pituitary function should be routinely assessed in bithalamic infarcts.Jednostronne udary w obrębie wzgórza mogą być przyczyną przemijających lub trwałych zaburzeń zachowania, objawów czuciowych lub zaburzeń gałkoruchowych. Obustronne udary wzgórza skutkują zwykle bardziej nasilonymi i dłużej trwającymi objawami. W niniejszym artykule zaprezentowano przypadek obustronnego udaru wzgórza z jednoczesną dysfunkcją podwzgórza. Jedynym czynnikiem ryzyka udaru niedokrwiennego mózgu, jaki stwierdzono u pacjenta, był krótkotrwały epizod migotania przedsionków. Obustronne udary wzgórza mogą być wynikiem zamknięcia jednej tętnicy przyśrodkowej wzgórza, odchodzącej od tętnicy tylnej mózgu albo w postaci dwóch osobnych gałęzi, albo też jednego wspólnego pnia, zaopatrującego jednak wzgórze obustronnie. Niezależnie jednak od wariantów anatomicznych unaczynienia wzgórza najbardziej prawdopodobną przyczyną udaru niedokrwiennego u przedstawionego pacjenta było jednostronne lub też obustronne zamknięcie tętnicy tylnej mózgu przez materiał zatorowy, prawdopodobnie w przebiegu zamknięcia szczytu tętnicy podstawnej. Dysfunkcja podwzgórza może towarzyszyć udarom wzgórza, autorzy sugerują więc, aby funkcja układu podwzgórzowo-przysadkowe-go była rutynowo oceniana w przypadku obustronnych udarów wzgórza

Comparison of 99mTc-HEPIDA and 99mTc-MBrIDA from the standpoint of hepatic clearance determination - preliminary communication

BACKGROUND. In order to evaluate the functional capacity of the liver by means of clearance determination, the derivative of iminodiacetic acid (99mTc-HEPIDA) has been used in recent decades. Because of recent problems with manufacturing and delivery of 99mTc-HEPIDA, an investigation was undertaken with the aim of testing whether a more widely available 99mTc-MBrIDA could be used for clearance determination and whether hepatic clearance measured with the use of this compound provides a similarly useful test of hepatic function. MATERIAL AND METHODS. Comparative investigations were performed in 73 patients of both sexes. The state of the efficiency of liver parenchyma was determined based on seven widely used biochemical tests, i.e. levels of: bilirubin, albumin, and gamma globulin; activity of AST, ALT, GGTP, and prothrombin index. The clearances of both radiopharmaceuticals, 99mTc-HEPIDA and 99mTc-MBrIDA, were determined by means of multisample technique. The results of determination were correlated among themselves and with the results of biochemical tests. The set of results of all estimations allowed a factorial analysis to be performed to find a common factor and to compute the values of factor loadings in particular tests. RESULTS. Obvious correlation between plasma and hepatic clearances of both radiopharmaceuticals was obtained and between plasma clearance of 99mTc-MBrIDA and hepatic clearance of 99mTc-HEPIDA. Correlation coefficients of 99mTc-MBrIDA clearance and the biochemical test results attained somewhat lower values than for 99mTc-HEPIDA clearance. Similarly, values of &#967;2 test of independence of 99mTc-MBrIDA clearances and test results were also less close than for 99mTc-HEPIDA clearances. Factorial analysis showed that common factor loading is greatest for hepatic clearance of 99mTc-HEPIDA; the values of two loadings of 99mTc-MBrIDA clearances are very close, but somewhat lower than those for 99mTc-HEPIDA. CONCLUSIONS. From the performed investigations it is possible to conclude that 99mTc-MBrIDA clearances may be used for the evaluation of liver parenchyma performance, even if the results may not be as certain as those obtained using 99mTc-HEPIDA

Wybrane aspekty epidemiologiczne stwardnienia rozsianego w Polsce – wieloośrodkowe badanie pilotażowe

Background and purpose The aim was to conduct a pilot study of selected epidemiological aspects of multiple sclerosis (MS) in Poland. Material and methods Cross-sectional data were collected in 21 centres providing MS treatment. The demographic profile of the patients, medical history of MS, disability status, comorbidity, and diagnostic and treatment modalities were analysed. Results Data on 3581 patients were obtained, including 2494 women (69.6%) and 1030 men (28.8%) – sex ratio 2.4 : 1. The mean age was 40.7 ± 11.9 years. Monofocal onset was reported in 80.8% of cases – the most frequently reported location of lesions was supratentorial (36.1%), followed by optic nerves (26.5%) and spinal cord (20.1%). The mean disease duration was 10.2 ± 8.8 years (range 0.04–53 years), and the mean time from the first symptoms to MS diagnosis was 2.6 years. Relapsing-remitting MS was reported in 70.5% of patients, secondary progressive in 16.8%, primary progressive in 8.4%, and ‘benign MS’ in 2.5%. The mean EDSS score was 3.3 ± 2.2 (range 0–9.5). The family history of MS was positive in 6.4% of cases. Comorbidity mainly applied to the musculoskeletal system (6.5%), the urinary system (5.8%) and psychiatric disturbances (5.5%). Brain magnetic resonance studies were available in 96.3% of the patients, evoked potentials in 54%, and cerebrospinal fluid testing in 63.1% – of whom only 41.2% were tested for oligoclonal bands, with 84% of samples being positive. Immunomodulatory drugs were used in 842 patients (24%), predominandy interferon beta (81%) and glatiramer (13%). Mitoxantrone was the most commonly used immunosuppressant. Conclusions This project is the first countrywide large-scale MS survey, covering approximately 18% of patients, according to our estimates. The results identify the clinical condition of the patients, as well as diagnostic and treatment modalities.Wstęp i cel pracy Celem badania była pilotażowa analiza wybranych aspektów epidemiologicznych stwardnienia rozsianego (SR) w Polsce. Materiał i metody Przekrojowe dane zebrano w 21 ośrodkach prowadzących leczenie SR. Analizowano profil demograficzny chorych, wywiad chorobowy SR, stopień niepełnosprawności, choroby współwystępujące, metody diagnostyczne i stosowane leczenie. Wyniki Uzyskano dane 3581 pacjentów, w tym 2494 kobiet (69,6%) i 1030 mężczyzn (28,8%) – proporcja płci 2,4 : 1. Średni wiek wynosił 40,7 ± 11,9 roku. Początek jednoogniskowy odnotowano w 80,8% przypadków – najczęściej wskazywano na nadnamiotową lokalizację uszkodzeń (36,1%), w dalszej kolejności zajęte były nerwy wzrokowe (26,5%) i rdzeń kręgowy (20,1%). Choroba trwała średnio 10,2 ± 8,8 roku (zakres 0,04–53 lat), a od pierwszych objawów do ustalenia rozpoznania mijało średnio 2,6 roku. Postać nawra-cająco-zwalniającą SR miało 70,5% chorych, wtórnie postępującą – 16,8%, pierwotnie postępującą – 8,4%, u 2,5% rozpoznano zaś „łagodne” SR. Średni stopień niesprawności w skali EDSS wyniósł 3,3 ±2,2 (zakres 0–9,5). Rodzinne występowanie SR odnotowano w 6,4% przypadków. Wśród chorób współwystępujących najczęstsze były schorzenia narządu ruchu (6,5%), układu moczowego (5,8%) i zaburzenia psychiczne (5,5%). Rezonans magnetyczny mózgu wykonano u 96,3% pacjentów, potencjały wywołane u 54%, badanie płynu mózgowo-rdzeniowego u 63,1% – z czego zaledwie w 41,2% przypadków oznaczano prążki oligoklonalne (ich obecność stwierdzono w 84% próbek). Leki immunomodulujące otrzymywało 842 chorych (24%), najczęściej były to interferon β (81%) i glatiramer (13%). W immunosupresji najczęściej stosowano mitoksantron. Wnioski Zrealizowany projekt jest pierwszym ogólnopolskim badaniem populacji chorych na SR na tak szeroką skalę – wg naszych szacunków obejmującym ok. 18% populacji chorych. Zgromadzone wyniki informują o stanie klinicznym chorych, najczęstszych metodach diagnostyki i leczenia

Disappearance of white matter lesions on MRI and clinical recovery after initiating antiretroviral therapy in a case of HIV infection presenting as spastic paraparesis

We present a case of a 30-year-old Polish female who presented with increasing for about 2 years spastic paraparesis and urinary incontinence. She denied any risky sexual behaviors, drug abuse, there was no history of surgery or blood transfusions. MRI of the brain showed diffuse, hyperintensive in T2, poorly defined lesions in the white matter. About 3 months later paraparesis increased and control MRI showed progression of previously described lesions. She was then diagnosed with HIV infection. There was a suspicion of progressive multifocal leucoencephalopathy (PML) or vacuolar myelopathy in the course of HIV infection. Antiretroviral treatment was initiated leading, together with rehabilitation, to a progressive improvement of symptoms. Pathological lesions on brain MRI completely disappeared. In conclusion, HIV test should be done in every patient with neurological signs of unknown cause

Efficacy and safety of Privigen® in patients with chronic inflammatory demyelinating polyneuropathy: results of a prospective, single-arm, open-label Phase III study (the PRIMA study)

This prospective, multicenter, single-arm, open-label Phase III study aimed to evaluate the efficacy and safety of Privigen (R) (10% liquid human intravenous immunoglobulin [IVIG], stabilized with l-proline) in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Patients received one induction dose of Privigen (2g/kg body weight [bw]) and up to seven maintenance doses (1g/kg bw) at 3-week intervals. The primary efficacy endpoint was the responder rate at completion, defined as improvement of 1 point on the adjusted Inflammatory Neuropathy Cause and Treatment (INCAT) disability scale. The preset success criterion was the responder rate being 35%. Of the 31 screened patients, 28 patients were enrolled including 13 (46.4%) IVIG-pretreated patients. The overall responder rate at completion was 60.7% (95% confidence interval [CI]: 42.41%-76.43%). IVIG-pretreated patients demonstrated a higher responder rate than IVIG-naive patients (76.9% vs. 46.7%). The median (25%-75% quantile) INCAT score improved from 3.5 (3.0-4.5) points at baseline to 2.5 (1.0-3.0) points at completion, as did the mean (standard deviation [SD]) maximum grip strength (66.7 [37.24] kPa vs. 80.9 [31.06] kPa) and the median Medical Research Council sum score (67.0 [61.5-72.0] points vs. 75.5 [71.5-79.5] points). Of 108 adverse events (AEs; 0.417 AEs per infusion), 95 AEs (88.0%) were mild or moderate in intensity and resolved by the end of study. Two serious AEs of hemolysis were reported that resolved after discontinuation of treatment. Thus, Privigen provided efficacious and well-tolerated induction and maintenance treatment in patients with CIDP

Efficacy and safety of IVIG in CIDP : combined data of the PRIMA and PATH studies

Intravenous immunoglobulin (IVIG) is a potential therapy for chronic inflammatory demyelinating polyneuropathy (CIDP). To investigate the efficacy and safety of the IVIG IgPro10 (Privigen) for treatment of CIDP, results from Privigen Impact on Mobility and Autonomy (PRIMA), a prospective, open-label, single-arm study of IVIG in immunoglobulin (Ig)-naive or IVIG pre-treated subjects (NCT01184846, n = 28) and Polyneuropathy And Treatment with Hizentra (PATH), a double-blind, randomized study including an open-label, single-arm IVIG phase in IVIG pre-treated subjects (NCT01545076, IVIG restabilization phase n = 207) were analyzed separately and together (n = 235). Efficacy assessments included change in adjusted inflammatory neuropathy cause and treatment (INCAT) score, grip strength and Medical Research Council (MRC) sum score. Adverse drug reactions (ADRs) and ADRs/infusion were recorded. Adjusted INCAT response rate was 60.7% in all PRIMA subjects at Week 25 (76.9% in IVIG pre-treated subjects) and 72.9% in PATH. In the pooled cohort (n = 235), INCAT response rate was 71.5%; median time to INCAT improvement was 4.3 weeks. No clear demographic differences were noticed between early (responding before Week 7, n = 148) and late responders (n = 21). In the pooled cohort, median change from baseline to last observation was -1.0 (interquartile range -2.0; 0.0) point for INCAT score; +8.0 (0.0; 20.0) kPa for maximum grip strength; +3.0 (1.0; 7.0) points for MRC sum score. In the pooled cohort, 271 ADRs were reported in 105 subjects (44.7%), a rate of 0.144 ADRs per infusion. This analysis confirms the efficacy and safety of IgPro10, a recently FDA-approved IVIG for CIDP, in a population of mainly pre-treated subjects with CIDP [Correction added on 14 March 2019 after first online publication: the INCAT response rate has been corrected.]

Effect of natalizumab on disease progression in secondary progressive multiple sclerosis (ASCEND). a phase 3, randomised, double-blind, placebo-controlled trial with an open-label extension

Background: Although several disease-modifying treatments are available for relapsing multiple sclerosis, treatment effects have been more modest in progressive multiple sclerosis and have been observed particularly in actively relapsing subgroups or those with lesion activity on imaging. We sought to assess whether natalizumab slows disease progression in secondary progressive multiple sclerosis, independent of relapses. Methods: ASCEND was a phase 3, randomised, double-blind, placebo-controlled trial (part 1) with an optional 2 year open-label extension (part 2). Enrolled patients aged 18–58 years were natalizumab-naive and had secondary progressive multiple sclerosis for 2 years or more, disability progression unrelated to relapses in the previous year, and Expanded Disability Status Scale (EDSS) scores of 3·0–6·5. In part 1, patients from 163 sites in 17 countries were randomly assigned (1:1) to receive 300 mg intravenous natalizumab or placebo every 4 weeks for 2 years. Patients were stratified by site and by EDSS score (3·0–5·5 vs 6·0–6·5). Patients completing part 1 could enrol in part 2, in which all patients received natalizumab every 4 weeks until the end of the study. Throughout both parts, patients and staff were masked to the treatment received in part 1. The primary outcome in part 1 was the proportion of patients with sustained disability progression, assessed by one or more of three measures: the EDSS, Timed 25-Foot Walk (T25FW), and 9-Hole Peg Test (9HPT). The primary outcome in part 2 was the incidence of adverse events and serious adverse events. Efficacy and safety analyses were done in the intention-to-treat population. This trial is registered with ClinicalTrials.gov, number NCT01416181. Findings: Between Sept 13, 2011, and July 16, 2015, 889 patients were randomly assigned (n=440 to the natalizumab group, n=449 to the placebo group). In part 1, 195 (44%) of 439 natalizumab-treated patients and 214 (48%) of 448 placebo-treated patients had confirmed disability progression (odds ratio [OR] 0·86; 95% CI 0·66–1·13; p=0·287). No treatment effect was observed on the EDSS (OR 1·06, 95% CI 0·74–1·53; nominal p=0·753) or the T25FW (0·98, 0·74–1·30; nominal p=0·914) components of the primary outcome. However, natalizumab treatment reduced 9HPT progression (OR 0·56, 95% CI 0·40–0·80; nominal p=0·001). In part 1, 100 (22%) placebo-treated and 90 (20%) natalizumab-treated patients had serious adverse events. In part 2, 291 natalizumab-continuing patients and 274 natalizumab-naive patients received natalizumab (median follow-up 160 weeks [range 108–221]). Serious adverse events occurred in 39 (13%) patients continuing natalizumab and in 24 (9%) patients initiating natalizumab. Two deaths occurred in part 1, neither of which was considered related to study treatment. No progressive multifocal leukoencephalopathy occurred. Interpretation: Natalizumab treatment for secondary progressive multiple sclerosis did not reduce progression on the primary multicomponent disability endpoint in part 1, but it did reduce progression on its upper-limb component. Longer-term trials are needed to assess whether treatment of secondary progressive multiple sclerosis might produce benefits on additional disability components. Funding: Biogen