Pramipeksol — zastosowanie w leczeniu choroby Parkinsona

Pramipeksol należy do grupy nieergotaminowych agonistów receptorów dopaminy D2, D3 i D4. Lek w minimalnym stopniu jest metabolizowany w wątrobie i w postaci niezmienionej wydalany przez nerki, co powoduje, że nie wchodzi w interakcje z lekami metabolizowanymi w wątrobie i jest zalecany u pacjentów ze schorzeniami wątroby przyjmujących takie leki (np. doustne antykoagulanty). Pramipeksol można podawać w dawkach podzielonych 3 razy/dobę, ale stosowanie formy leku o przedłużonym uwalnianiu jest wygodniejsze w dawkowaniu, łatwiejsze do aplikacji dla pacjenta oraz charakteryzuje się stałym i stabilnym stężeniem we krwi. Niezależnie od stadium choroby można z dnia na dzień i nie zmieniając dawki, zmienić postać IR pramipeksolu na postać SR. Wnioski z badań typu evidence-based medicine wskazują, że pramipeksol o standardowym uwalnianiu (IR) oraz o przedłużonym uwalnianiu (ER) jest skuteczny w monoterapii objawów ruchowych we wczesnym okresie choroby Parkinsona (PD, Parkinson’s disease), w ograniczaniu objawów ruchowych PD w terapii dodanej do lewodopy, prewencji i opóźnieniu występowania fluktuacji ruchowych i dyskinez. Wprowadzenie do leczenia pramipeksolu jest rekomendowane w zaawansowanym okresie PD u pacjentów z efektem końca dawki, nieprzewidywalnymi stanami on–off, a także dyskinezami szczytu dawki, przy jednoczesnym zmniejszeniu dawki lewodopy. Pramipeksol cechuje również dodatkową korzyść dla pacjenta — efekt przeciwdepresyjny, który jest możliwy dzięki wiązaniu pramipeksolu z receptorami D3 w układzie limbicznym. Podsumowując, pramipeksol jest lekiem o udokumentowanej skuteczności zarówno we wczesnym, jak i zaawansowanym stadium PD ze szczególną rekomendacją dla chorych z towarzyszącą depresją, apatia oraz schorzeniami wątroby

A case report of patient with cerebellar variant of stiff person syndrome

Stiff person syndrome (SPS) is a rare autoimmune neurological disorder with antibodies against antigens involved in neurotransmission of gamma-aminobutyric acid (GABA). About 10% of patients with SPS may develop ataxia. This cerebellar variant is a distinct subset of SPS with more severe and complex clinical phenotype. We report the clinical, neuropsychological and neuroradiological findings in a 39-year-old female with cerebellar variant of SPS

A case report of patient with cerebellar variant of stiff person syndrome

Stiff person syndrome (SPS) is a rare autoimmune neurological disorder with antibodies against antigens involved in neurotransmission of gamma-aminobutyric acid (GABA). About 10% of patients with SPS may develop ataxia. This cerebellar variant is a distinct subset of SPS with more severe and complex clinical phenotype. We report the clinical, neuropsychological and neuroradiological findings in a 39-year-old female with cerebellar variant of SPS

Eye movements in essential tremor patients with parkinsonian and cerebellar signs

Apart from intention tremor essential tremor (ET) patients may display other cerebellar signs, like dysmetria or tandem gait disturbances as well as parkinsonian signs like resting tremor, cogwheel sign, subtle bradykinesia. Previous reports claimed the occurrence of the eye movement abnormalities characteristic for dysfunction of cerebellar dorsal vermis in ET patients with concomitant cerebellar signs. There are no previous reports evaluating the eye movement abnormalities in ET patients with concomitant parkinsonian signs. The objective of this study was to determine the relationship between the occurrence of parkinsonian and cerebellar signs and the oculomotor abnormalities in ET patients. Method Fifty ET patients including 6 (12.0%) patients with concomitant parkinsonian signs (ET-P), 20 (40.0%) patients with cerebellar signs (ET-C), 7 (14.0%) with mixed parkinsonian and cerebellar signs (ET-M), 17 (34.0%) patients with the only tremor (ET-T) together with 42 healthy controls were included to the study. Reflexive, pace-induced and cued saccades were recorded using Saccadometer Advanced. Smooth pursuit and fixation were tested using EOG. Results Latency of pace-induced saccades was significantly longer in ET-C and ET-M patients compared to ET-T and ET-P patients. Latency of cued saccades was significantly longer in ET-M patients compared to ET-T. There were no significant differences of the eye movement parameters between ET-P patients compared to ET-T patients. Conclusion In ET patient with concomitant cerebellar signs prolonged volitional saccades latency was detected. There are no particular differences in the eye movements in ET patients with concomitant parkinsonian signs compared to ET patients without concomitant signs

The effect of the rehabilitation program on balance, gait, physical performance and trunk rotation in Parkinson's disease

BACKGROUND: Parkinson’s disease (PD) is a progressive, neurodegenerative disease which leads to postural and gait disorders, limitation in mobility, activities of daily living and disability. AIMS: The aim of the study is to assess the effects of the rehabilitation program on balance, gait, motor performance and trunk rotations in PD patients. METHODS: Sixty-four patients with 1.5–3.0 stage PD in the Hoehn and Yahr scale were randomly allocated to rehabilitation and control groups. Sixty-one patients completed the study. Patients were assessed three times, at month intervals. Between the first and second assessments, the rehabilitation group participated in a rehabilitation training program focused on mobility, balance and gait exercises, consisting of 28 sessions. Balance was assessed with tandem stance and the Pastor test (shoulder tug). Gait was assessed with a 10 m walk at preferred speed and 360° turn. Motor performance was evaluated by means of the Physical Performance Test (PPT) and timed motor activities. The trunk rotations were measured in the lumbar and thoraco-lumbar spine with a tape measure. RESULTS: The rehabilitation group significantly improved (p < 0.05) in balance and gait outcomes, PPT score, timed activities and trunk rotations both in comparison to the control group and baseline results. The positive effects of the exercise program maintained for at least 1 month. CONCLUSION: The 4-week rehabilitation training program focused on mobility, balance and gait exercises improved balance, gait, physical performance and trunk rotations in patients with PD

Eye movements in essential tremor patients with parkinsonian and cerebellar signs

Apart from intention tremor essential tremor (ET) patients may display other cerebellar signs, like dysmetria or tandem gait disturbances as well as parkinsonian signs like resting tremor, cogwheel sign, subtle bradykinesia. Previous reports claimed the occurrence of the eye movement abnormalities characteristic for dysfunction of cerebellar dorsal vermis in ET patients with concomitant cerebellar signs. There are no previous reports evaluating the eye movement abnormalities in ET patients with concomitant parkinsonian signs. The objective of this study was to determine the relationship between the occurrence of parkinsonian and cerebellar signs and the oculomotor abnormalities in ET patients. Method: Fifty ET patients including 6 (12.0%) patients with concomitant parkinsonian signs (ET-P), 20 (40.0%) patients with cerebellar signs (ET-C), 7 (14.0%) with mixed parkinsonian and cerebellar signs (ET-M), 17 (34.0%) patients with the only tremor (ET-T) together with 42 healthy controls were included to the study. Reflexive, pace-induced and cued saccades were recorded using Saccadometer Advanced. Smooth pursuit and fixation were tested using EOG. Results: Latency of pace-induced saccades was significantly longer in ET-C and ET-M patients compared to ET-T and ET-P patients. Latency of cued saccades was significantly longer in ET-M patients compared to ET-T. There were no significant differences of the eye movement parameters between ET-P patients compared to ET-T patients. Conclusion: In ET patient with concomitant cerebellar signs prolonged volitional saccades latency was detected. There are no particular differences in the eye movements in ET patients with concomitant parkinsonian signs compared to ET patients without concomitant signs

Zmiany w obrazie ultrasonograficznym i obrazie rezonansu magnetycznego mózgu u noworodków leczonych metodą selektywnej hipotermii

Introduction: Hypoxic ischemic insults during labor remain an important cause of brain injury in term and near-term neonates. Selective cerebral hypothermia is a potentially neuroprotective rescue therapy. Ultrasonography (US) andnmagnetic resonance imaging (MRI) are routinely used to visualize intracranial changes in neonatal hypoxic-ischemic injuries. Aim of the study: We attempted to describe all pathological findings on US and MRI in the brains of our patients following selective cerebral hypothermia. Materials and methods: Twenty-nine neonates with hypoxic-ischemic encephalopathy (HIE) following therapeutic cooling were assessed with cranial ultrasound (US) and magnetic resonance imaging (MRI). The findings were compared with the clinical outcome. Results: Over one-fourth (27.6%) of the examined infants had a normal brain on MRI (with only 17.2% on US). Involvement of the basal ganglia and thalami was one of the most frequent findings in our material (9/29 = 31% on MRI, and 7/29-24.1% on US). Cerebral parenchymal hemorrhage was detected on MRI in as many as 7 (24.1%) and cerebellar parenchymal hemorrhage in 4 (13.8%) infants. The loss in the gray-white matter differentiation (‘fuzzy brain’), usually transient on US, was observed in 79.3% of the neonates. Diffusion restriction in the callosal splenium (13.8%) and hyperechoic thalami and basal ganglia were strictly correlated to a significantly higher incidence of severe developmental delay. Conclusion: Abnormalities on MRI and US were observed in 75% of newborns with hypoxic-ischemic encephalopathy treated with therapeutic hypothermia.Wstęp: Uraz niedotlenieniowo-niedokrwienny jest częstą przyczyną okołoporodowego uszkodzenia mózgu u noworodków, u których potencjalną, neuroprotekcyjną metodą leczenia jest selektywna hipotermia. Ultrasonografia (USG) i rezonans magnetyczny (MRI) są rutynowymi metodami obrazowania mózgu u noworodków ze zmianami niedotlenieniowo-niedokrwiennymi. Cel pracy: Podjęłyśmy próbę opisania wszystkich zmian patologicznych wykrytych w badaniu USG i MRI mózgu u noworodków, które leczone były metodą selektywnej hipotermii. Materiał i metoda: Przezciemiączkowe badanie ultrasonograficzne (USG) oraz badanie metodą rezonansu magnetycznego (MR) wykonano u 29 noworodków z encefalopatią niedotlenienieniowo-niedokrwienną (ENN) po zastosowaniu terapeutycznej hipotermii. Wyniki badań porównano z oceną kliniczną. Wyniki: U ponad jednej czwartej badanych dzieci (27,6%) badanie MR mózgu było prawidłowe (w badaniu USG tylko u 17,2%). Zajęcie jąder podstawy i wzgórz było jednym z najczęstszych znalezisk w naszym materiale (9/29= 31% w MR i 7/29 = 24.1% w USG). Krwawienie śródmózgowe wykryto w MR aż w 7 przypadkach (24,1%), a śródmóżdżkowe w 4 (13,8%). Brak zróżnicowaniu istoty szarej i białej (“fuzzy brain”), zwykle przemijające w obrazie USG, obserwowano u 79,3% noworodków. Restrykcja dyfuzji wody w płacie ciała modzelowatego (13,8%) oraz hiperechogeniczność wzgórz i jąder podstawy mózgu ściśle korelowały z istotnie częstszym występowaniem znacznego opóźnienia rozwoju. Wnioski: Nieprawidłowy obraz MRI i US występuje u 75% noworodków z encefalopatią niedotlenieniowoniedokrwienną leczonych metodą selektywnej hipotermii