1,721,149 research outputs found
Sporadic CJD in a patient with relapsing-remitting multiple sclerosis on an immunomodulatory treatment
No full textCreutzfeld-Jacob disease (CJD) is a degenerative, invariably fatal brain disorder. Multiple sclerosis (MS) is a chronic, potentially disabling, immune-mediated inflammatory demyelinating disease of the central nervous system. Here, we report a 50-year-old woman who, two years after the diagnosis of relapsing remitting MS, developed altered consciousness, dystonic posture of the left hand and myoclonic jerks. Repeated brain MRI showed hyperintensities on T2 sequences in basal ganglia bilaterally and diffusion restriction in these areas, and, since typical EEG and CSF features were present, the diagnosis of CJD was made. To the best of our knowledge, this is the first report of a glatiramer acetate-treated MS patient who developed sporadic CJD. This combination is interesting in the light of recent data suggesting that CJD and MS may share similar mechanisms of "molecular mimicry" and autoimmunity. This case also emphasizes the importance of critically assessing every new symptom even in a patient with an established diagnosis of MS
Sporadic CJD in a patient with relapsing-remitting multiple sclerosis on an immunomodulatory treatment
No full textCreutzfeld-Jacob disease (CJD) is a degenerative, invariably fatal brain disorder. Multiple sclerosis (MS) is a chronic, potentially disabling, immune-mediated inflammatory demyelinating disease of the central nervous system. Here, we report a 50-year-old woman who, two years after the diagnosis of relapsing remitting MS, developed altered consciousness, dystonic posture of the left hand and myoclonic jerks. Repeated brain MRI showed hyperintensities on T2 sequences in basal ganglia bilaterally and diffusion restriction in these areas, and, since typical EEG and CSF features were present, the diagnosis of CJD was made. To the best of our knowledge, this is the first report of a glatiramer acetate-treated MS patient who developed sporadic CJD. This combination is interesting in the light of recent data suggesting that CJD and MS may share similar mechanisms of "molecular mimicry" and autoimmunity. This case also emphasizes the importance of critically assessing every new symptom even in a patient with an established diagnosis of MS
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Blood pressure variability is altered in secondary progressive multiple sclerosis but not in patients with a clinically isolated syndrome
No full textCilj je bio istražiti razlike u varijabilnosti sistoličkog krvnog tlaka (SBPV) mjerenog otkucaj po otkucaj kod osoba sa sekundarno progresivnom multiplom sklerozom (SPMS), klinički izoliranim sindromom (CIS) i zdravim kontrolama (HC). Ova retrospektivna studija slučajeva i kontrola uključivala je 46 osoba sa SPMS, 46 sa CIS i 44 HC. Za procjenu SBPV-a korišten je poluautomatizirani softver izrađen u MATLAB-u R2019b (The MathWorks, Inc.). Promatrane su karakteristike u frekvencijskom području: spektar snage u područjima niske frekvencije (LF) i visoke frekvencije (HF) te omjer LF/HF. Podaci su izraženi u apsolutnoj snazi (mmHg2) LF i HF spektra te omjeru (LF/HF) tijekom faza ispitivanja u ležećem položaju i tilt-up table testa. Studija je pokazala da nije bilo značajnih razlika u prosječnim vrijednostima sistoličkog (sBP) ili dijastoličkog krvnog tlaka (dBP) tijekom faza ispitivanja u ležećem položaju i tilt-up table testu između skupina. Tijekom faze ispitivanja u ležećem položaju, LF i LF/HF su značajno niži u grupi SPMS (4,17±5,38 i 3,52±2,34, redom) u usporedbi s CIS-om (5,42±3,59, p=0,015 i 5,92±4,63, p=0,029, redom) i HC grupom (6,03±4,55, p=0,011 i 6,52±5,09, p=0,010, redom), dok je tijekom faze ispitivanja u tilt-up table testu snaga LF značajno niža u usporedbi s obje skupine CIS i HC, a snaga HF je značajno niža samo u usporedbi s grupom CIS. Zaključno, SBPV je izmijenjen kod osoba sa SPMS-om u usporedbi s CIS i normalnim kontrolama. Daljnja istraživanja u području disautonomije povezane s multiplom sklerozom (MS) su opravdana ne samo zbog njihove važnosti vezane uz komorbiditete i simptome MS-a, već i zbog vjerojatne uloge u patofiziologiji MS-a.Objective was to investigate differences in beat-to-beat systolic blood pressure variability (SBPV) in people with secondary progressive multiple sclerosis (SPMS), clinically isolated syndrome (CIS) and healthy controls (HC). This retrospective, case-control study included 46 people with SPMS, 46 CIS and 44 HC. A semi-automated software made with MATLAB R2019b (The MathWorks, Inc.) was used for evaluation of SBPV. The frequency domain characteristics observed were the power spectrum in the low frequency (LF) and high frequency (HF) bands and the LF/HF ratio. Data is expressed in absolute power (mmHg2) of LF and HF and ratio (LF/HF) during both supine and tilt-up phases of testing. Study showed that there were no significant differences in mean systolic (sBP) or diastolic blood pressure (dBP) values during supine and tilt-up phases of testing between groups. During the supine phase of testing LF and LF/HF were significantly lower in SPMS group (4.17±5.38 and 3.52±2.34, respectively) compared to CIS (5.42±3.59, p=0.015 and 5.92±4.63, p=0.029, respectively) and HC group (6.03±4.55, p=0.011 and 6.52 ±5.09, p=0.010, respectively), while during tilt-up phase LF was significantly lower compared to both CIS and HC group, and HF was significantly lower only compared to CIS group. In conclusion SBPV is altered in people with SPMS compared to CIS and normal controls. Further research in the field of multiple sclerosis (MS) related dysautonomia is warranted not only because of its relevance to comorbidities and MS symptoms, but also because of its likely involvement in the pathophysiology of MS
Orthostatic hypotension
Full text linkOrtostatska hipotenzija se definira kao trajni pad sistoličkog tlaka za više od 20 mmHg i dijastoličkog za više od 10 mmHg unutar 3 minute od promjene položaja tijela iz ležećeg ili sjedećeg u uspravni. Posljedica je neadekvatnog fizološkog odgovora krvnog tlaka na promjenu položaja tijela, a može asimptomatska i simptomatska. Pojavljuje se u svakoj dobnoj skupini, ali prevalencija raste s dobi. U općoj populaciji prevalencija ortostatske hipotenzije je oko 0.5%, dok je kod osoba starijih od 65 godina je između 5 – 30%. Česti simptomi su vrtoglavica, zamagljen vid, slabost, iscrpljenost, mučnina, palpitacije i glavobolja. Ortostatska hipotenzija može biti prvi znak zatajivanja autonomnog živčanog sustava, pa je čest simptom u primarnim i sekundarnim bolestima autnomnog živčanog sustava (multisitemnoj atrofiji, Pariknsonovoj bolesti, dijabetičkoj autonomnoj neuropatiji), ali češće je nuspojava lijekova poput diuretika i antidepresiva, te posljedica hipovolemije. U obradi bolesnika s ortostatskom hipotenzijom bitno je tražiti potencijalno reverzibilan uzrok ili bolest u podlozi i ciljano ih liječiti. Iako je ortostatsku hipotenziju teško liječiti, cilj je smanjiti simptome i poboljšati kvalitetu života. Liječenje može biti farmakološko i nefarmakološko. Nefarmakološko podrazumijeva promjene životnog stila i preporuča se svim bolesnicima. Onima koji ne reagiraju daju se lijekovi poput fludrokortizona, midodrina i piridostigmina.Orthostatic hypotension is defined as a sustained decrease in systolic blood pressure of 20 mm Hg or a decrease in diastolic blood pressure of 10 mm Hg within three minutes of standing when compared with blood pressure from the sitting or supine position. It results from an inadequate physiologic response to postural changes in blood pressure. Orthostatic
hypotension may be symptomatic or asymptomatic. It occurs in all age groups, but the prevalence increases with age. In the general population the prevalence of orthostatic hypotension is 0.5%, whereas in patients older than 65 years is between 5-30%. It may be the first sign of dysfunction of the autonomic nervous system and is a common symptom in primary and secondary diseases of autonomic nervous system (multiple system atrophy, Parkinson's disease, diabetic autonomic neuropathy), but more often is a side effect of medications such as diuretics and antidepressants, as well as a consequence of hypovolemia. Common symptoms include dizziness, lightheadedness, blurred vision, weakness, fatigue, nausea, palpitations, and headache. Evaluation of suspected orthostatic hypotension begins by identifying reversible causes and underlying associated medical conditions. Although it is difficult to treat orthostatic hypotension, the goal is to reduce symptoms and improve quality of life. Treatment can be pharmacological and non-pharmacological. Non-pharmacological
involves lifestyle changes and is recommended for all patients. For patients who do not respond adequately to nonpharmacologic treatment, fludrocortisone, midodrine, and pyridostigmine are pharmacologic therapies proven to be beneficial
Benign paroxysmal positional vertigo
Full text linkBenigni paroksizmalni pozicijski vertigo (BPPV) najčešći je uzrok vrtoglavice. Većinom se javlja između 50. i 70. godine života, no može se javiti i u mlađoj populaciji, najčešće kao posljedica ozljede glave. Smatra se da su glavni uzrok nastanka BPPV-a male nakupine kalcijevih kristala, otokonije. One se fiziološki nalaze u labirintu unutarnjeg uha, točnije u utrikulusu. Kod BPPV-a,
otokonije migriraju u polukružne kanale, najčešće u stražnji polukružni kanal. Tamo pri normalnim pokretima glave uzrokuju abnormalnu raspodjelu endolimfe što dovodi do vrtoglavice. Pokreti koji najčešće uzrokuju pojavu BPPV-a jesu okretanje u krevetu te naginjanje glave pri pogledu prema gore ili dolje. Pacijenti se uglavnom žale na vrtoglavicu, mučninu, povraćanje i sinkopu te se uočava pojava nistagmusa. Iako BPPV nije maligno stanje, on može dovesti do ozbiljnih posljedica poput padova, ozljede glave te prijeloma kostiju. Dijagnoza se postavlja s pomoću Dix-Hallpikeova testa. Test se smatra pozitivnim ako se prilikom izvođenja jave vrtoglavica i nistagmus. Simptomi BPPV-a mogu se spontano povući ili mogu trajati danima, tjednima, mjesecima, ili čak i godinama. Pacijenti se liječe izvođenjem Epleyjeva zahvata koji je uspješan u 90 % slučajeva. To je jednostavan repozicijski zahvat kojim se otokonije pomiču natrag u utrikulus. Ukoliko pacijenti ne reagiraju dobro na zahvat ili imaju teže simptome, upućuje ih se na kiruršku metodu liječenja u vidu okluzije stražnjega kanala. Ostale mogućnosti liječenja podrazumijevaju Semontov tzv.
oslobađajući manevar, Brandt-Daroffove vježbe te antivertiginozne lijekove.Benign paroxysmal positional vertigo (BPPV) is the most common cause of vertigo. It generally affects patients 50 to 70 years of age but can also occur in younger patients, usually as a consequence of a head injury. Small collections of calcium crystals, known as otoconia, are believed to be the underlying cause of BPPV. Normally, they are positioned in the labyrinth of the inner ear, specifically in the utricle. In BPPV, otoconia migrate to semicircular canals, most commonly the posterior semicircular canal. There, during head movements in various directions, they cause abnormal fluid endolymph displacement, resulting in vertigo. The most common movements in which BPPV occurs are rolling in bed and looking up or under. Usual signs and symptoms include vertigo, nausea, vomiting, syncope and nistagmus. While BPPV is not a malign condition, it can lead to serious outcomes such as falls, head trauma and bone fractures. BPPV is diagnosed by performing the Dix-Hallpike maneuver, which is considered positive if nistagmus and vertigo occur during the maneuver. BPPV symptoms can resolve spontaneously or can last for days, weeks, months, or even years. Over 90 % of patients can be successfully treated performing the Epley“s maneuver, a simple repositioning maneuver that moves the otoconia back into the utricle. If patients do not respond well to the maneuver or have severe symptoms, they can undergo posterior canal occlusion surgery. Other possibilities of treatment include the Semont maneuver, Brandt-Daroff exercises and anti-vertigo medications
Postural orthostatic tachycardia syndrome
Full text linkOrtostatska intolerancija se može opisati kao nemogućnost toleriranja uspravnog stava uz olakšanje simptoma nakon zauzimanja ležećeg položaja. Sindrom posturalne ortostatske tahikardije (POTS) je oblik ortostatske intoleracije definiran kao kontinuiran porast srčane frekvencije za ≥30 otkucaja u minuti ili kao porast frekvencije na vrijednost od ≥120 otkucaja u minuti unutar 10 minuta od početka stajanja ili head-up tilt testa uz pojavu simptoma ortostatske intolerancije i odsutnost ortostatske hipotenzije. Pacijenti kojima je dijagnosticiran POTS su uglavnom žene, s omjerom žena prema muškarcima 4-5:1, te starosti između 15 i 50 godina. Nekoliko patofizioloških mehanizama je moguće uključeno u razvoj POTS-a. Neki od njih su: distalna periferna neuropatija, poremećaji centralne kontrole simpatičkog živčanog sustava, oštećenje sinaptičkih mehanizama ponovnog unosa norepinefrina, poremećaji renin-angiotenzin-aldosteron osovine, promjene u sintetičkom putu norepinefrina. Najčešći simptomi povezani s POTS-om su omamljenost, presinkopa, slabost i palpitacije. Pogoršanje simptoma sa stajanjem i olakšanje nakon zauzimanja ležećeg položaja je karakteristično obilježje POTS-a. Pri postavljanju dijagnoze koriste se aktivni test stajanja i pasivni head-up tilt test, zajedno s detaljnom povijesti bolesti i kliničkim pregledom. Nefarmakoterapijski pristup liječenju POTS-a podrazumijeva povećan unos soli i vode te vježbanje. Farmakoterapija je usmjerena prema povećanju volumena tekućine, povećanju periferne vakularne rezistencije i smanjenju centralne aktivnosti simpatičkog živčanog sustava. Velik broj pacijenata iskusi znatno poboljšanje nakon točno postavljene dijagnoze i pravilnog liječenja.Orthostatic intolerance(OI) can be defined as inability to tolerate upright posture relieved with recumbence. Postural orthostatic tachycardia syndrome(POTS) is a form of orthostatic intolerance defined as sustained increase in heart rate(HR) of ≥30 bpm or increase of HR to ≥120 bpm within 10 min of standing or head-up tilt associated with symptoms of orthostatic intolerance and absence of orthostatic hypotension. POTS patients are mostly female, with female to male ratio of 4-5:1, and age range from 15 to 50. Several pathophysiological mechanisms are thought to underly POTS. Some of possible mechanisms are distal peripheral neuropathy, abnormalities of central control of sympathetic nervous system, impaired synaptic norepinephrine (NE) reuptake, renin-angiotensin-aldosterone axis disturbance and altered NE synthetic pathway. The most common symptoms related to POTS are light-headedness, presyncope, weakness and palpitations. Exacerbation of symptoms with standing and symptoms relieved with recumbence is characteristical POTS feature. Active stand test and passive head-up tilt table (HUT) test are used in diagnosing POTS, along with detailed history and examination. Non-pharmacological therapy of POTS includes increase in daily salt and water intake, and exercise training. Pharmacological therapy is directed at expanding fluid volume, increasing peripheral vascular resistance and reducing central sympathetic activity. Majority of patients experience substantial improvement, after correct diagnosis and suitable therapy
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