BACKGROUND AND PURPOSE DYT1 dystonia is the most common form of inherited primary dystonia. The a... more BACKGROUND AND PURPOSE DYT1 dystonia is the most common form of inherited primary dystonia. The aim of the study was: 1) to evaluate the prevalence of the DYT1 mutation in a population of Polish patients with early-onset generalized dystonia and with other forms of familial dystonia, 2) to evaluate the frequency of the DYT1 mutation in patients with writer's cramp, 3) to characterize the phenotype of the DYT1 mutation in the Polish population, and 4) to define the group of patients in whom genetic testing is recommended. MATERIAL AND METHODS The following groups of patients were included in the study: 1) patients with early-onset (<30 years) generalized dystonia and those patients with onset after age 30 years who have relatives with early-onset dystonia, 2) patients with writer's cramp (focal or as part of segmental dystonia) independently of age of onset, 3) asymptomatic (adult only) relatives of the diagnosed DYT1 carriers. Genetic tests were performed in 63 subjects--...
Punkcja ledźwiowa (LP, lumbar puncture ) to podstawowy sposob pobrania plynu mozgowo-rdzeniowego ... more Punkcja ledźwiowa (LP, lumbar puncture ) to podstawowy sposob pobrania plynu mozgowo-rdzeniowego (CSF, cerebrospinal fluid ). Jest to zabieg bezpieczny dla pacjenta i obarczony bardzo niskim ryzykiem powaznych powiklan, jezeli zostanie wykonany zgodnie z obowiązującymi zasadami. Przed LP nalezy wykonac badanie dna oka, a w przypadkach wątpliwych rowniez badanie obrazowe, w celu wykluczenia wzmozonego ciśnienia wewnątrzczaszkowego. Nalezy rowniez ustalic, czy nie wystepują przeciwwskazania hematologiczne do LP. Punkcje ledźwiową nalezy wykonywac u pacjenta znajdującego sie w pozycji siedzącej lub lezącej na boku, ale pomiar ciśnienia CSF przeprowadza sie wylącznie w pozycji lezącej. Optymalna średnica igly punkcyjnej to 25 G; preferuje sie igly atraumatyczne. Zalecane jest pobieranie CSF swobodnie wyplywającego z igly, a nie aspirowanie strzykawką. Pobranie do 30 ml CSF jest bezpieczne i dobrze tolerowane. W przypadku nieudanego pobrania CSF nie nalezy podejmowac wiecej niz czterech ...
Ropinirol jest nieergotaminowym agonistą receptorow dopaminowych. Wykazuje wysokie powinowactwo d... more Ropinirol jest nieergotaminowym agonistą receptorow dopaminowych. Wykazuje wysokie powinowactwo do receptorow dopaminowych D2 i D 3. Obecnie stosowany jest w postaci o kontrolowanym uwalnianiu (ropinirol CR [ controlled release ]), umozliwiającej przyjmowanie leku raz dziennie. Ropinirol zmniejsza nasilenie objawow choroby Parkinsona i jest stosowany w leczeniu w jej wczesnym okresie. Rozpoczecie leczenia choroby Parkinsona od monoterapii ropinirolem opoźnia wystąpienie dyskinez. Skutecznie redukuje nasilenie objawow ruchowych u chorych wcześniej nieleczonych lub leczonych lekami niedopaminergicznymi oraz w przypadku braku satysfakcjonującej kontroli objawow u chorych bez fluktuacji, u ktorych wcześniej stosowano monoterapie lewodopą. U pacjentow z fluktuacjami ruchowymi ropinirol umozliwia skrocenie lącznego czasu trwania stanow off bez spowodowania czestszego wystepowania uciązliwych dyskinez.
BACKGROUND In 2008, the Movement Disorders Society published the Unified Dyskinesia Rating Scale ... more BACKGROUND In 2008, the Movement Disorders Society published the Unified Dyskinesia Rating Scale (UDysRS). This has become the established tool for assessing the severity and disability associated with dyskinesia in patients with Parkinson's Disease (PD). We translated and validated the Polish version of the UDysRS, explored its dimensionality, and compared it to the Spanish version, which is the Reference Standard for UDysRS translations. MATERIAL AND METHODS The UDysRS was translated into Polish by a team led by JS and GO. The back-translation, completed by colleagues fluent in both Polish and English who were not involved in the original translation, was reviewed and approved by the Executive Committee of the MDS Rating Scales Programme. Then the translated version of the UDysRS underwent cognitive pretesting, and the translation was modified based on the results. The approved version was considered to be the Official Working Document of the Polish UDysRS and was tested on 25...
Nuclear medicine review. Central & Eastern Europe, 2008
The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differenti... more The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differential diagnostics of extrapyramidal diseases. Neuroimaging with the use of presynaptic tracers of the dopaminergic system provides evidence of nigrostriatal degeneration and may support the clinical diagnosis of Parkinsonism. During the last two years (2007-2008) we tried to elaborate the optimal methodology of SPECT/CT examination with the use of DaTSCAN ((123)I-Ioflupane), and we sought to evaluate the effect of the reconstruction and attenuation correction method on semi-quantitative measures of relative uptake in the striatum. In a present study, we retrospectively studied DaTSCAN scans of 44 consecutive patients with clinical indications of Parkinson's disease or uncertain Parkinsonian syndromes. The quality of DaTSCAN images reconstructed with the use of ordered-subset expectation maximization reconstruction technique (OSEM) with attenuation correction based on CT maps was found t...
The pathomechanism of the migraine aura remains unclear. The most probable cause of the aura is c... more The pathomechanism of the migraine aura remains unclear. The most probable cause of the aura is cortical spreading depression with associated hypoperfusion. Both the cortical spreading depression and hypoperfusion begin in the occipital lobes and spread forward slowly (2-3 mm/min) in a wave-like mode along the brain convolutions and cross territories of brain arteries. We present a 24-year-old female patient with a combination of aura symptoms. Each migraine attack began with a bright scintillating zig-zag, which crossed the visual field. It was followed by left sided hemiparesthesiae marching from the face to the hand. The last symptom of aura was motor aphasia. Later a unilateral, pulsating headache developed with associated photo- and phonophobia. The stable pattern and duration of aura symptoms in the presented case suggest that the cortical spreading depression plays an important role in the pathomechanism of migraine with aura.
Dyskinezy, określane takze mianem dyskinez wywolanych przez lewodope, są objawem charakterystyczn... more Dyskinezy, określane takze mianem dyskinez wywolanych przez lewodope, są objawem charakterystycznym dla zaawansowanej choroby Parkinsona. Glowne czynniki odpowiedzialne za rozwoj dyskinez to czas trwania choroby oraz leczenie lewodopą. Praktyczny, uwzgledniający relacje czasowe z przyjmowanymi dawkami lewodopy oraz stanami on i off, podzial dyskinez obejmuje: 1) dyskinezy szczytu dawki, 2) dyskinezy stanow off oraz 3) dyskinezy dwufazowe. Leczenie dyskinez obejmuje miedzy innymi modyfikacje leczenia dopaminergicznego, w tym przede wszystkim stosowanie doustnych agonistow dopaminergicznych. Rozpoczecie leczenia od monoterapii agonistą dopaminergicznym opoźnia wystąpienie dyskinez. W grupie chorych z pierwszymi fluktuacjami ruchowymi, ale jeszcze bez dyskinez, prawdopodobienstwo ich wystąpienia jest mniejsze w przypadku dolączenia agonisty niz w przypadku zwiekszania dawek lewodopy. U pacjentow z fluktuacjami ruchowymi i dyskinezami szczytu dawki wlączenie agonisty dopaminergicznego p...
We reexamined the database of some 20,000 electron micrographs from the Echigo-1, the 263K-strain... more We reexamined the database of some 20,000 electron micrographs from the Echigo-1, the 263K-strain or the 22C-H of scrapie-infected hamsters to look for the cytoplasmic clearance. We reevaluated the largest database in the world of photographed dystrophic neurites for the presence of cytoplasmic clearance as shown in transgenic fruit flies transfected with Aβ-42. In several neurites, we found electron-lucent areas not bound by any membranes or only partially bound; thus, they were not autophagic vacuoles as the latter are membrane-bound and contain cargo. Those changes were not observed in every examined neurite and no correlation with any other changes were noticed. In some neurites, which could be traced over several sections, the electron-lucent areas were evident to change size, i.e. to expand.
The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differenti... more The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differential diagnostics of extrapyramidal diseases. Neuroimaging with the use of presynaptic tracers of the dopaminergic system provides evidence of nigrostriatal de-generation and may support the clinical diagnosis of Parkin-sonism. During the last two years (2007–2008) we tried to elab-orate the optimal methodology of SPECT/CT examination with the use of DaTSCAN (123I-Ioflupane), and we sought to evaluate the effect of the reconstruction and attenuation correction meth-od on semi-quantitative measures of relative uptake in the stria-tum. In a present study, we retrospectively studied DaTSCAN scans of 44 consecutive patients with clinical indications of Parkinson’s disease or uncertain Parkinsonian syndromes. Impact of CT based attenuation correction on quantitative assessment of DaTSCAN (123I-Ioflupane) imaging in diagnosis of extrapyramidal diseases
We report here on the ultrastructure of amyloid plaques in chronic wasting disease (CWD) transmit... more We report here on the ultrastructure of amyloid plaques in chronic wasting disease (CWD) transmitted to Tg20 transgenic mice overexpressing prion protein (PrPc). We identified three main types of amyloid deposits in mCWD: large amyloid deposits, unicentric plaques similar to kuru plaques in human prion diseases and multicentric plaques reminiscent of plaques typical of GSS. The most unique type of plaques were large subpial amyloid deposits. They were composed of large areas of amyloid fibrils but did not form „star-like” appearances of unicentric plaques. All types of plaques were totally devoid of dystrophic neuritic elements. However, numerous microglial cells invaded them. The plaques observed by confocal laser microscope were of the same types as those analyzed by electron microscopy. Neuronal processes surrounding the plaques did not show typical features of neuroaxonal dystrophy.
BACKGROUND AND PURPOSE DYT1 dystonia is the most common form of inherited primary dystonia. The a... more BACKGROUND AND PURPOSE DYT1 dystonia is the most common form of inherited primary dystonia. The aim of the study was: 1) to evaluate the prevalence of the DYT1 mutation in a population of Polish patients with early-onset generalized dystonia and with other forms of familial dystonia, 2) to evaluate the frequency of the DYT1 mutation in patients with writer's cramp, 3) to characterize the phenotype of the DYT1 mutation in the Polish population, and 4) to define the group of patients in whom genetic testing is recommended. MATERIAL AND METHODS The following groups of patients were included in the study: 1) patients with early-onset (<30 years) generalized dystonia and those patients with onset after age 30 years who have relatives with early-onset dystonia, 2) patients with writer's cramp (focal or as part of segmental dystonia) independently of age of onset, 3) asymptomatic (adult only) relatives of the diagnosed DYT1 carriers. Genetic tests were performed in 63 subjects--...
Punkcja ledźwiowa (LP, lumbar puncture ) to podstawowy sposob pobrania plynu mozgowo-rdzeniowego ... more Punkcja ledźwiowa (LP, lumbar puncture ) to podstawowy sposob pobrania plynu mozgowo-rdzeniowego (CSF, cerebrospinal fluid ). Jest to zabieg bezpieczny dla pacjenta i obarczony bardzo niskim ryzykiem powaznych powiklan, jezeli zostanie wykonany zgodnie z obowiązującymi zasadami. Przed LP nalezy wykonac badanie dna oka, a w przypadkach wątpliwych rowniez badanie obrazowe, w celu wykluczenia wzmozonego ciśnienia wewnątrzczaszkowego. Nalezy rowniez ustalic, czy nie wystepują przeciwwskazania hematologiczne do LP. Punkcje ledźwiową nalezy wykonywac u pacjenta znajdującego sie w pozycji siedzącej lub lezącej na boku, ale pomiar ciśnienia CSF przeprowadza sie wylącznie w pozycji lezącej. Optymalna średnica igly punkcyjnej to 25 G; preferuje sie igly atraumatyczne. Zalecane jest pobieranie CSF swobodnie wyplywającego z igly, a nie aspirowanie strzykawką. Pobranie do 30 ml CSF jest bezpieczne i dobrze tolerowane. W przypadku nieudanego pobrania CSF nie nalezy podejmowac wiecej niz czterech ...
Ropinirol jest nieergotaminowym agonistą receptorow dopaminowych. Wykazuje wysokie powinowactwo d... more Ropinirol jest nieergotaminowym agonistą receptorow dopaminowych. Wykazuje wysokie powinowactwo do receptorow dopaminowych D2 i D 3. Obecnie stosowany jest w postaci o kontrolowanym uwalnianiu (ropinirol CR [ controlled release ]), umozliwiającej przyjmowanie leku raz dziennie. Ropinirol zmniejsza nasilenie objawow choroby Parkinsona i jest stosowany w leczeniu w jej wczesnym okresie. Rozpoczecie leczenia choroby Parkinsona od monoterapii ropinirolem opoźnia wystąpienie dyskinez. Skutecznie redukuje nasilenie objawow ruchowych u chorych wcześniej nieleczonych lub leczonych lekami niedopaminergicznymi oraz w przypadku braku satysfakcjonującej kontroli objawow u chorych bez fluktuacji, u ktorych wcześniej stosowano monoterapie lewodopą. U pacjentow z fluktuacjami ruchowymi ropinirol umozliwia skrocenie lącznego czasu trwania stanow off bez spowodowania czestszego wystepowania uciązliwych dyskinez.
BACKGROUND In 2008, the Movement Disorders Society published the Unified Dyskinesia Rating Scale ... more BACKGROUND In 2008, the Movement Disorders Society published the Unified Dyskinesia Rating Scale (UDysRS). This has become the established tool for assessing the severity and disability associated with dyskinesia in patients with Parkinson's Disease (PD). We translated and validated the Polish version of the UDysRS, explored its dimensionality, and compared it to the Spanish version, which is the Reference Standard for UDysRS translations. MATERIAL AND METHODS The UDysRS was translated into Polish by a team led by JS and GO. The back-translation, completed by colleagues fluent in both Polish and English who were not involved in the original translation, was reviewed and approved by the Executive Committee of the MDS Rating Scales Programme. Then the translated version of the UDysRS underwent cognitive pretesting, and the translation was modified based on the results. The approved version was considered to be the Official Working Document of the Polish UDysRS and was tested on 25...
Nuclear medicine review. Central & Eastern Europe, 2008
The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differenti... more The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differential diagnostics of extrapyramidal diseases. Neuroimaging with the use of presynaptic tracers of the dopaminergic system provides evidence of nigrostriatal degeneration and may support the clinical diagnosis of Parkinsonism. During the last two years (2007-2008) we tried to elaborate the optimal methodology of SPECT/CT examination with the use of DaTSCAN ((123)I-Ioflupane), and we sought to evaluate the effect of the reconstruction and attenuation correction method on semi-quantitative measures of relative uptake in the striatum. In a present study, we retrospectively studied DaTSCAN scans of 44 consecutive patients with clinical indications of Parkinson's disease or uncertain Parkinsonian syndromes. The quality of DaTSCAN images reconstructed with the use of ordered-subset expectation maximization reconstruction technique (OSEM) with attenuation correction based on CT maps was found t...
The pathomechanism of the migraine aura remains unclear. The most probable cause of the aura is c... more The pathomechanism of the migraine aura remains unclear. The most probable cause of the aura is cortical spreading depression with associated hypoperfusion. Both the cortical spreading depression and hypoperfusion begin in the occipital lobes and spread forward slowly (2-3 mm/min) in a wave-like mode along the brain convolutions and cross territories of brain arteries. We present a 24-year-old female patient with a combination of aura symptoms. Each migraine attack began with a bright scintillating zig-zag, which crossed the visual field. It was followed by left sided hemiparesthesiae marching from the face to the hand. The last symptom of aura was motor aphasia. Later a unilateral, pulsating headache developed with associated photo- and phonophobia. The stable pattern and duration of aura symptoms in the presented case suggest that the cortical spreading depression plays an important role in the pathomechanism of migraine with aura.
Dyskinezy, określane takze mianem dyskinez wywolanych przez lewodope, są objawem charakterystyczn... more Dyskinezy, określane takze mianem dyskinez wywolanych przez lewodope, są objawem charakterystycznym dla zaawansowanej choroby Parkinsona. Glowne czynniki odpowiedzialne za rozwoj dyskinez to czas trwania choroby oraz leczenie lewodopą. Praktyczny, uwzgledniający relacje czasowe z przyjmowanymi dawkami lewodopy oraz stanami on i off, podzial dyskinez obejmuje: 1) dyskinezy szczytu dawki, 2) dyskinezy stanow off oraz 3) dyskinezy dwufazowe. Leczenie dyskinez obejmuje miedzy innymi modyfikacje leczenia dopaminergicznego, w tym przede wszystkim stosowanie doustnych agonistow dopaminergicznych. Rozpoczecie leczenia od monoterapii agonistą dopaminergicznym opoźnia wystąpienie dyskinez. W grupie chorych z pierwszymi fluktuacjami ruchowymi, ale jeszcze bez dyskinez, prawdopodobienstwo ich wystąpienia jest mniejsze w przypadku dolączenia agonisty niz w przypadku zwiekszania dawek lewodopy. U pacjentow z fluktuacjami ruchowymi i dyskinezami szczytu dawki wlączenie agonisty dopaminergicznego p...
We reexamined the database of some 20,000 electron micrographs from the Echigo-1, the 263K-strain... more We reexamined the database of some 20,000 electron micrographs from the Echigo-1, the 263K-strain or the 22C-H of scrapie-infected hamsters to look for the cytoplasmic clearance. We reevaluated the largest database in the world of photographed dystrophic neurites for the presence of cytoplasmic clearance as shown in transgenic fruit flies transfected with Aβ-42. In several neurites, we found electron-lucent areas not bound by any membranes or only partially bound; thus, they were not autophagic vacuoles as the latter are membrane-bound and contain cargo. Those changes were not observed in every examined neurite and no correlation with any other changes were noticed. In some neurites, which could be traced over several sections, the electron-lucent areas were evident to change size, i.e. to expand.
The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differenti... more The quality of visually and semi-quantitatively assessed DaTSCAN images is crucial for differential diagnostics of extrapyramidal diseases. Neuroimaging with the use of presynaptic tracers of the dopaminergic system provides evidence of nigrostriatal de-generation and may support the clinical diagnosis of Parkin-sonism. During the last two years (2007–2008) we tried to elab-orate the optimal methodology of SPECT/CT examination with the use of DaTSCAN (123I-Ioflupane), and we sought to evaluate the effect of the reconstruction and attenuation correction meth-od on semi-quantitative measures of relative uptake in the stria-tum. In a present study, we retrospectively studied DaTSCAN scans of 44 consecutive patients with clinical indications of Parkinson’s disease or uncertain Parkinsonian syndromes. Impact of CT based attenuation correction on quantitative assessment of DaTSCAN (123I-Ioflupane) imaging in diagnosis of extrapyramidal diseases
We report here on the ultrastructure of amyloid plaques in chronic wasting disease (CWD) transmit... more We report here on the ultrastructure of amyloid plaques in chronic wasting disease (CWD) transmitted to Tg20 transgenic mice overexpressing prion protein (PrPc). We identified three main types of amyloid deposits in mCWD: large amyloid deposits, unicentric plaques similar to kuru plaques in human prion diseases and multicentric plaques reminiscent of plaques typical of GSS. The most unique type of plaques were large subpial amyloid deposits. They were composed of large areas of amyloid fibrils but did not form „star-like” appearances of unicentric plaques. All types of plaques were totally devoid of dystrophic neuritic elements. However, numerous microglial cells invaded them. The plaques observed by confocal laser microscope were of the same types as those analyzed by electron microscopy. Neuronal processes surrounding the plaques did not show typical features of neuroaxonal dystrophy.
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Papers by Agata Gajos