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Neben den durch die Pharmakokinetik der Dopaminersatzmedikation bedingten Wirkungsfluktuationen können bei der Parkinson-Krankheit verschiedene Zustandsschwankungen auftreten, die nicht oder nur partiell auf die Wirkung der Medikamente zurückzuführen sind. Das Spektrum motorischer Zustandsschwankungen reicht von kurzen Episoden von Gangblockaden oder Tremordurchbrüchen bis hin zu akinetischen Krisen. Zu den nichtmotorischen Phänomenen zählen insbesondere diskontinuierliche Veränderungen von Kognition und Vigilanz. Als Ursachen dieser Zustandsschwankungen kommen systemische oder krankheitsimmanente Komplikationen wie Demenz oder orthostatische Dysregulation in Betracht. Zusätzlich können externe Reize oder intrinsische Faktoren zu vorübergehenden Änderungen der Symptomausprägung führen. Die korrekte ätiologische Zuordnung dieser Zustandsschwankungen und die Abgrenzung von medikamentös bedingten Wirkungsfluktuationen sind für das korrekte Management entscheidend.

In advanced Parkinson disease (PD), immediate-release pramipexole, taken 3 times daily, improves symptoms and quality of life. A once-daily extended-release formulation may be an effective and simple alternative therapy.

To assess the clinical efficacy of a novel once-daily extended-release (ER) formulation of the dopamine agonist pramipexole as monotherapy in patients with early Parkinson disease (PD) and establish its noninferiority vs standard immediate-release (IR) pramipexole.

A consensus conference on multiple system atrophy (MSA) in 1998 established criteria for diagnosis that have been accepted widely. Since then, clinical, laboratory, neuropathologic, and imaging studies have advanced the field, requiring a fresh evaluation of diagnostic criteria. We held a second consensus conference in 2007 and present the results here.

The present study sought to investigate lower urinary tract symptoms and urodynamic and cystometric findings in Parkinson disease (PD), dementia with Lewy bodies (DLB), and Alzheimer disease (AD).

Die Fähigkeit Entscheidungen zu treffen ist im täglichen Leben von zentraler Bedeutung. Patienten mit kognitiven Einschränkungen sind oft mit schwierigen Entscheidungen konfrontiert. Auch Patienten mit Morbus Parkinson (MP) weisen häufig kognitive Leistungsminderungen, insbesondere der exekutiven Funktionen, auf. Zahlreiche Arbeiten berichteten in den letzten Jahren über Impulskontrollstörungen (z. B. pathologisches Spielen) bei MP Patienten. Im Vergleich dazu gibt es wenige Studien, welche das Entscheidungsverhalten von MP Patienten ohne Impulskontrollstörungen anhand von Entscheidungsaufgaben untersucht haben. Der Artikel gibt eine Übersicht über in der neueren Forschung häufig verwendete experimentelle Verfahren zum Entscheidungsverhalten und diskutiert den Zusammenhang zwischen Entscheidungsverhalten und exekutiven Funktionen. In einem Überblick zu Entscheidungen unter Unsicherheit und unter Risiko bei MP Patienten zeigen sich heterogene Ergebnisse im Hinblick auf die Gesamtleistung in Entscheidungsaufgaben. Diskutiert wird der Zusammenhang mit anderen kognitiven Leistungen, sowie der Einfluss dopaminerger Medikation. Zusätzlich widmet sich ein Abschnitt Impulskontrollstörungen bzw. dem dopaminergen Dysregulationssyndrom.

To estimate rates, predictors, and prognostic importance of recanalization in an unselected series of patients with stroke treated with IV thrombolysis.

In der vorliegenden Übersicht soll deutlich gemacht werden, dass ein vaskuläres Parkinson-Syndrom (VPS) im engeren Sinne eine seltene und klinisch nicht immer von der idiopathischen Parkinson abgrenzbare Erkrankung ist. Im klinischen Sprachgebrauch wird der Terminus VPS allerdings auch für andere Störungsbilder verwendet, die nicht zuletzt aufgrund der unterschiedlichen therapeutischen Implikationen von einem VPS differenziert werden sollten. Die Differenzialdiagnose des VPS umfasst in erster Linie die subkortikale arteriosklerotische Enzephalopathie, bei der eine Parkinson-artige Gangstörung nicht mit weiteren Kardinalsymptomen eines Parkinson-Syndroms verbunden ist sowie die Koexistenz eines neurodegenerativen Parkinson-Syndroms mit einer subkortikalen vaskulären Enzephalopathie. Ein akutes oder subakutes VPS entsteht in der Regel kontralateral zu Hirninfarkten, die den externen Teil des Globus pallidus, den ventrolateralen Teil des Thalamus und (seltener) die Substantia nigra betreffen. Chronische VPS mit schleichendem Beginn entstehen auf dem Boden bilateraler lakunärer subkortikaler Infarkte mit Beeinträchtigung thalamokortikaler Projektionen. Progressionsneigung und Dopa-Responsivität kommen bei akuten und chronischen VPS vor und erschweren die Differenzialdiagnose zu einem degenerativen Parkinson-Syndrom.

Zusammenfassung Die spinale fibrokartilaginäre Embolie (FKE) ist eine seltene Ursache für ischämische Rückenmarksschädigungen. In der vorliegenden Arbeit wird der Fall eines 13-jährigen Patienten mit wahrscheinlicher posttraumatischer FKE in Anamnese, Klinik, differenzialdiagnostischer Aufarbeitung und Verlauf geschildert. Die Diagnose einer FKE als Ursache eines akuten Querschnittssyndroms ist in der Regel nur per exclusionem möglich. Ein möglicher Hinweis auf das Vorliegen einer Rückenmarksischämie ist neben einem Trauma in der Anamnese der Nachweis eines Wirbelkörperödems angrenzend an die spinale Läsion in der Magnetresonanztomographie. Zudem ist der Ausschluss systemischer Emboliequellen sowie anderer Rückenmarksschädigungen notwendig, um die Verdachtsdiagnose einer FKE stellen zu können. Des Weiteren werden mögliche Pathomechanismen, durchzuführende Untersuchungen, Therapie und Prognose in diesem Artikel besprochen.

We studied cortical activation patterns by functional MRI in a patient who received bilateral hand transplantation after amputation 6 years ago and in a patient who had received unilateral hand replantation within 2 hours after amputation. In the early postoperative period, the patient who had had the hand transplantation revealed strong activation of a higher motor area, only weak activation of the primary sensorimotor motor cortex and no activation of the primary somatosensory cortex. At 1-year follow-up, a small increase in primary sensorimotor motor cortex activation was observed. Activation of the primary somatosensory cortex was only seen at the 2 year follow-up. By contrast, after hand replantation, the activation pattern was similar to that of the uninjured hand within 6 weeks. This included activation of the primary sensorimotor motor cortex, higher motor areas and primary somatosensory cortex. Transplantation after long-standing amputation results in cortical reorganization occurring over a 2-year period. In contrast, hand replantation within a few hours preserves a normal activation pattern.

To assess the prevalence and severity of restless legs syndrome (RLS) in the general community and to investigate its potential relationship with iron metabolism and other potential risk factors.

The parkinson variant of multiple system atrophy (MSA-P) and progressive supranuclear palsy (PSP) present with atypical parkinsonism, which may be misdiagnosed as PD, particularly in early disease stages. It was previously shown that diffusion-weighted MRI (DWI) is a sensitive tool to discriminate MSA-P from PD based on increased apparent diffusion coefficients (ADCs) in the putamen. In this study DWI was evaluated in 10 patients with PSP compared with 13 patients with PD and 12 with MSA-P.

To assess the therapeutic efficacy of modafinil in the treatment of increased daytime sleepiness in patients with Parkinson's disease (PD).

Objective: To delineate the frequency and nature of dystonia in multiple system atrophy (MSA). Methods: a cohort of 24 patients with clinically probable MSA over the past 10 years were prospectively followed up. Motor features were either dominated by parkinsonism (MSA-P subtype, n=18) or cerebellar ataxia (MSA-C, n=6). Classification of dystonic features and their changes with time was based on clinical observation during 6-12 monthly follow up visits. Parkinsonian features and complications of drug therapy were assessed. Most patients (22/24) died during the observation period. Neuropathological examination was confirmatory in all of the five necropsied patients. Results: At first neurological visit dystonia was present in 11 (46%) patients all of whom had been levodopa naive at this time point. Six patients (25%) exhibited cervical dystonia (antecollis) (MSA-P n=4, MSA-C n=2), five patients (21%) showed unilateral limb dystonia (MSA-P n=4; MSA-C n=1). A definite initial response to levodopa treatment was seen in 15/18 patients with MSA-P, but in none of the six patients with MSA-C. A subgroup of 12 patients with MSA-P developed levodopa induced dyskinesias 2.3 years (range 0.5-4) after initiation of levodopa therapy. Most patients had peak dose craniocervical dystonia; however, some patients experienced limb or generalised dystonia. Isolated peak dose limb chorea occurred in only one patient. Conclusion: The prospective clinical study suggests that dystonia is common in untreated MSA-P. This finding may reflect younger age at disease onset and putaminal pathology in MSA-P. Levodopa induced dyskinesias were almost exclusively dystonic affecting predominantly craniocervical musculature. Future studies are required to elucidate the underlying pathophysiology of dystonia in MSA.

Routine MRI as well as MR volumetry and MRS have been shown to contribute to the differential diagnosis of the Parkinson variant of multiple system atrophy (MSA-P) and PD. However, it is currently unknown whether diffusion-weighted imaging (DWI) discriminates these disorders.

Zusammenfassung: Strungen des Gleichgewichtes fhren hufig zu wesentlicher Behinderung von Patienten mit idiopathischer Parkinson-Erkrankung (IPE). In der vorliegenden Studie wurden bei 40 Patienten mit IPE und 30 gesunden Probanden Messungen auf einer statischen Posturografie-Plattform durchgefhrt. Der Vergleich des spontanen Schwankens im Stand (sway) zeigte keinen statistisch signifikanten Unterschied zwischen Patienten und gesunden Probanden. Der Einfluss verschiedener Faktoren (Reaktion auf den Pull-Test, Nachweis einer Mikroangiopathie im CCT, Demenz, Alter) auf den sway innerhalb der Patientengruppe wurde mittels multipler linearer Regressionsanalyse berprft. Als einziger signifikanter Faktor fr einen erhhten sway wurde dabei das Vorliegen einer subkortikalen arteriosklerotischen Enzephalopathie identifiziert. Die Patienten (n=20) mit zerebrovaskulrer Komorbiditt zeigten auerdem hufiger Strze und pathologische Reaktionen im Pull-Test. Aus den Ergebnissen dieser Untersuchung kann gefolgert werden, dass eine koinzidierende zerebrale Mikroangiopathie zu verstrkter Gleichgewichtsstrung bei IPE fhrt. Vermehrtes spontanes Schwanken im ruhigen Stand kann bei IPE als Hinweis auf Komorbiditt gewertet werden und Anlass zu weiterfhrender Diagnostik geben.

The objective of this study was to investigate cerebellar metabolism in patients with autosomal dominant cerebellar ataxia type 1 (ADCA‐I) carrying two distinct mutations of spinocerebellar ataxia (SCA). Non‐invasive image‐guided proton magnetic resonance spectroscopy imaging (1H‐MRSI) was performed in 4 patients with SCA2, and 3 patients carrying the SCA6 mutation. For MRSI, we employed a spin‐echo sequence (TR = 1500 msec, TE = 135 msec, slice thickness = 15 mm, FOV = 240 mm) and a stimulated‐echo sequence (TR = 1500 msec, TE = 20 msec, slice thickness = 15 mm, FOV = 240 mm). Measures included the peak integral ratios of neuronal and glial markers [N‐acetylaspartate (NA) to creatine (Cr), choline‐containing compounds (CHO) to Cr, and lactate (LAC) to Cr]. We found NA:Cr ratios were significantly lower in patients with SCA2 (40.4% lower) compared to patients carrying the SCA6 mutation. CHO:Cr ratios differed between the two mutations using short echo time (30.8% lower in SCA2), but not when applying long echo time 1H‐MRSI. Measurements using long echo time revealed LAC peaks in all SCA2 patients. 1H‐MRSI revealed metabolic differences between SCA2 and SCA6 patients. NA:Cr ratios were significantly lower in patients with the SCA2 mutation compared to the SCA6 mutation, and LAC signals were obtained in the cerebella of SCA2 patients. In addition, CHO:Cr ratios showed different behavior using short and long TE, indicating differences in relaxation times of choline compounds in SCA2. J. Magn. Reson. Imaging 2001;13:553–559. © 2001 Wiley‐Liss, Inc.

Zusammenfassung: Die Demenz mit Lewy-Körperchen (DLK) ist die zweithäufigste neuropathologisch diagnostizierte degenerative dementielle Erkrankung. Ihre klinischen Charakteristika sind progrediente Demenz, Parkinson-Symptome, Schwankungen der Hirnleistungen und Vigilanz, detaillierte wiedergegebene visuelle Halluzinationen, Depression, REM-Schlaf-Verhaltensstörungen, Überempfindlichkeit auf die Dosen üblicher Neuroleptika, Stürze, Synkopen, systemisierter Wahn und Halluzinationen anderer Art. Die klinischen Kriterien haben eine hohe Spezifität (95%) und eine deutlich geringere Sensitivität. Die Erkrankung beginnt im Schnitt zwischen dem 60. und 68. Lebensjahr, betrifft Männer häufiger als Frauen und dauert 6–8 Jahre. Differenzialdiagnosen sind Demenz vom Alzheimer-Typ (DAT), Parkinson-Krankheit (PK), subkortikale arteriosklerotische Enzephalopathie, progressive supranukleäre Parese, Multisystematrophie und bisweilen die Creutzfeldt-Jakob-Erkrankung. Der genetische Hintergrund der Erkrankung ist ungeklärt. Bildgebende Diagnostik (Magnetresonanz-Imaging, Single-Photon-Emission-Tomographie) kann zur Differenzialdiagnose beitragen. Systematische Therapiestudien liegen noch nicht vor. Die Erkrankung wird mit L-Dopa, atypischen Neuroleptika, Azetylcholinesterase-Hemmern, blutdrucksteigernden Medikamenten, sowie zur Verbesserung der neurogenen Blasenstörungen mit peripheren Anticholinergika und α-Rezeptorenblockern behandelt.

L‐Dopa‐induced dyskinesias constitute a challenge to the management of advanced Parkinson's disease. According to recent reports, treatment with the NMDA receptor antagonist amantadine may significantly diminish L‐dopa‐induced dyskinesias. In the present study, the effect of amantadine on L‐dopa‐induced dykinesias was assessed in a 5‐week, double‐blind crossover trial. Dyskinesia severity as assessed following oral L‐dopa challenges and by self‐scoring dyskinesia diaries were reduced approximately 50% after amantadine treatment compared with baseline or placebo phases. Similarly, dyskinesia assessments on the Unified Parkinson's Disease Rating Scale, part IV (items 32 and 33) also revealed significant improvement after treatment with amantadine. The magnitude of the L‐dopa motor response to oral challenges was not different after amantadine or placebo treatment, and there was no significant reduction of daily off‐time when patients received active treatment. These results confirm previous observations concerning the antidyskinetic potential of amantadine.

The progression to different Hoehn and Yahr (HY) stages was evaluated in 81 pathologically confirmed patients with parkinsonism. Patients with PD (n 18) showed significantly longer latencies to each HY stage than patients with atypical parkinsonian disorder (APD). While no patient with PD developed a HY-III stage within 1 year of onset of motor symptoms, 72 of those with APDs did. In fact, development of a HY-III within 1 year of motor onset accurately predicted an APD. However, the progression to each HY stage was unhelpful in distinguishing the APDs from each other. Once patients with PD and APD became wheelchair-bound, both had equally short survival times.

ObjectivesRepetitive speech phenomena are morphologically heterogeneous iterations of speech which have been described in several neurological disorders such as vascular dementia, progressive supranuclear palsy, Wilson's disease, and Parkinson's disease, and which are presently only poorly understood. The present, prospective study investigated repetitive speech phenomena in Parkinson's disease to describe their morphology, assess their prevalence, and to establish their relation with neuropsychological and clinical background data.MethodsTwenty four patients with advanced Parkinson's disease and 29 subjects with mid-stage, stable idiopathic disease were screened for appearance, forms, and frequency of repetitive speech phenomena, and underwent a neuropsychological screening procedure comprising tests of general mental functioning, divergent thinking and memory. Patients with advanced Parkinson's disease had a significantly higher disease impairment, longer disease duration, and an unstable motor response to levodopa with frequent on-off fluctuations. Both groups were well matched as to their demographical, clinical, and cognitive background. Perceptual speech evaluation was used to count and differentiate forms of repetitive speech phenomena in different speech tasks. To compare the effect of the motor state, the appearance of repetitive speech phenomena was also assessed in a subgroup of patients with advanced Parkinson's disease during the on versus the off state.ResultsSpeech repetitions emerged mainly in two variants, one hyperfluent, formally resembling palilalia, and one dysfluent, stuttering-like. Both forms were present in each patient producing repetitive speech phenomena. The repetitive speech phenomena appeared in 15 patients (28.3 %), 13 of whom belonged to the advanced disease group, indicating a significant preponderance of repetitive speech phenomena in patients with a long term, fluctuating disease course. Repetitive speech phenomena appeared with almost equal frequency during the on and the off state of patients with advanced Parkinson's disease. Their distribution among different variants of speech was disproportional, with effort demanding speech tasks producing a significantly higher number of repetitive speech phenomena over semiautomatic forms of speech.ConclusionsIn idiopathic Parkinson's disease repetitive speech phenomena seem to emerge predominantly in a subgroup of patients with advanced disease impairment; manifest dementia is not a necessary prerequisite. They seem to represent a deficit of motor speech control; however, linguistic factors may also contribute to their generation. It is suggested that repetitions of speech in Parkinson's disease represent a distinctive speech disorder, which is caused by changes related to the progression of Parkinson's disease.

To determine the value of vascular endothelial growth factor (VEGF) in CSF as a marker for carcinomatous meningitis (CM).

To compare the long-term efficacy and safety of ropinirole with bromocriptine over 3 years in patients with early PD with limited or no previous dopaminergic therapy.

Quantitative gait analysis has been used to elucidate characteristic features of neurological gait disturbances. Although a number of studies compared single patient groups with controls, there are only a few studies comparing gait parameters between patients with different neurological disorders affecting gait. In the present study, gait parameters were compared between control subjects, patients with parkinsonian gait due to idiopathic Parkinson's disease, subjects suffering from cerebellar ataxia and patients with gait disturbance due to subcortical arteriosclerotic encephalopathy. In addition to recording of baseline parameters during preferred walking velocity, subjects were required to vary velocity from very slow to very fast. Values of velocity and stride length from each subject were then used for linear regression analysis. Whereas all patient groups showed slower walking velocity and reduced step length compared with healthy controls when assessed during preferred walking, patients with ataxia and subcortical arteriosclerotic encephalopathy had, in addition, increased variability of amplitude and timing of steps. Regression analysis showed that with changing velocity, subjects with Parkinson's disease changed their stride length in the same proportion as that measured in controls. In contrast, patients with ataxia and subcortical arteriosclerotic encephalopathy had a disproportionate contribution of stride length when velocity was increased. Whereas the findings in patients with Parkinson's disease can be explained as a reduction of force gain, the observations for patients with ataxia and subcortical arteriosclerotic encephalopathy reflect an altered spatiotemporal gait strategy in order to compensate for instability. The similarity of gait disturbance in subcortical arteriosclerotic encephalopathy and cerebellar ataxia suggests common mechanisms.

Objective To quantitatively assess control of balance under static and dynamic conditions in patients with tonic and phasic cervical dystonia. Methods Ten patients with purely tonic cervical dystonia with fixed postural deviation and 20 patients with cervical dystonia with phasic head movements were investigated at least 3 months after botulinum toxin injections. Seventeen age matched volunteers served as controls. Static posturography was performed on a force platform; dynamic equilibrium was studied on a stabilometer, which requires the subject to continuously adapt upright posture to an unstable tilting surface. Measurements of maximum amplitude and linear displacement of the pivot were taken with open and closed eyes. Results Sway path values in static posturography were not significantly different between patients with cervical dystonia and controls. On dynamic posturography, patients with phasic cervical dystonia showed significantly higher platform measures (maximum amplitude and linear displacement of the pivot) with eyes open and closed By contrast, none of the dynamic platform measures differed significantly between patients with tonic cervical dystonia and controls. Conclusions Normal measures of dynamic equilibrium in tonic cervical dystonia argue against a primary abnormality of balance control in cervical dystonia. Impaired dynamic equilibrium in phasic cervical dystonia is likely to reflect a disruption of vestibular input due to repetitive, involuntary head oscillations.

Thirteen patients with idiopathic Parkinson's disease of recent onset (mean age 63·2 years) and a group of 10 young healthy volunteers (mean age 26·1 years) underwent a series of neuropsychological tests for assessment of memory, learning ability and mental processing speed before and during treatment with trihexyphenidyl. Retesting after anticholinergic exposure (mean of 2 weeks for patients and 1 week for controls) revealed in young healthy controls the same pattern and magnitude of decline in memory function as in Parkinson patients. Non-demented subjects with Parkinson's disease of recent onset thus do riot seem to be selectively vulnerable to cognitive side-effects of anticholinergic treatment.

This paper deals with signs, symptoms and course in spasmodic torticollis (ST). Two hundred and fifty-six patients were included in the study, 59.3% women, 40.7% men. The mean age was 49.1 years. Rotating torticollis out-numbered latero- and antero-retrocollis. A family history of ST occurred in 3.1% of the total sample. First degree relatives were affected in 2.3%. Thirty-four per cent of the patients had additional dystonic symptoms. Most frequently these affected the upper extremities (13%), and less often the legs. Of the patients 19.1% had experienced a period of complete remission. The correlations between the severity of the signs and the neurological symptoms are surprisingly weak.

The GSI (General Symptom Index) of the Symptom Checklist 90 R (SCL 90 R) (as a global indicator of the severity of psychiatric symptoms) of 27% of the spasmodic torticollis (ST) sample fell outside the 95% range of the normal control group (two standard deviations). Patients with a higher GSI were younger, more functionally disabled and subject to higher psychosocial stress due to the illness. The highest scores were reached on the subscales of somatization, interpersonal sensitivity and depression. On the depression scale, 23% of the patients' scores were abnormal. This scale correlated significantly with the neurological signs, particularly the TSUI-index and laterocollis. A statistically significant correlation also existed between psychiatric morbidity and a family history of mental disorder. More than 50% of the patients reported that stressful life events had triggered their illness. In order of frequency, a death came first, followed by marital strife, changes in employment and family arguments. The data suggest that psychopathology in ST should generally be considered as a result of a variety of interacting factors, biological, psychological and social.

Psychosocial changes in spasmodic torticollis (ST) affect predominantly social life, professional life and psychological well-being. Concerning social life, 84.6% of the patients felt that they attracted considerable public attention due to their neurological illness and 65% had reduced participation in social events. A substantial number of patients had retired from professional activities; others felt severely impaired in their working capacity. The psychosocial sequelae in ST, however, seemed to depend less on the neurological signs per se than on the physical symptoms and on coping. Depressive coping in particular emerged as a predictor of psychosocial distress.