Management of dystonia in Europe: a survey of the European network for the study of the dystonia syndromes.

BACKGROUND AND PURPOSE: Dystonia is difficult to recognize due to its large phenomenological complexity. Thus, the use of experts in dystonia is essential for better recognition and management of dystonia syndromes (DS). Our aim was to document managing strategies, facilities and expertise available in various European countries in order to identify which measures should be implemented to improve the management of DS. METHODS: A survey was conducted, funded by the Cooperation in Science and Technology, via the management committee of the European network for the study of DS, which is formed from representatives of the 24 countries involved. RESULTS: Lack of specific training in dystonia by general neurologists, general practitioners as well as other allied health professionals was universal in all countries surveyed. Genetic testing for rare dystonia mutations is not readily available in a significant number of countries and neurophysiological studies are difficult to perform due to a lack of experts in this field of movement disorders. Tetrabenazine is only readily available for treatment of dystonia in half of the surveyed countries. Deep brain stimulation is available in three-quarters of the countries, but other surgical procedures are only available in one-quarter of countries. CONCLUSIONS: Internationally, collaboration in training, advanced diagnosis, treatment and research of DS and, locally, in each country the creation of multidisciplinary teams for the management of dystonia patients could provide the basis for improving all aspects of dystonia management across Europe.

Cognitive impairment in early-stage non-demented Parkinson's disease patients.

OBJECTIVES: In Parkinson's disease (PD), Parkinson's disease dementia (PDD) and Parkinson's disease-mild cognitive impairment (PD-MCI) are common. PD-MCI is a risk factor for developing PDD. Knowledge of cognition in early-stages PD is essential in understanding and predicting the dementia process. MATERIALS AND METHODS: We describe the cognitive profile in early-stage PD patients with no prior clinical suspicion of cognitive impairment, depression or psychiatric disturbances, and investigate possible features distinguishing patients with cognitive deficits, defining a PD-MCI risk-profile. Single Photon Emission Computerized Tomography (SPECT) DaT-scan and neurological examination confirmed the diagnosis. Mini-mental state examination-, Addenbrooke's Cognitive Examination, Unified Parkinson's Disease Rating Scale scoring, Hoehn &Yahr/Activity of Daily Living staging and a neuropsychological test battery were applied. Mild cognitive impairment patients were identified according to modified criteria by Troster necessarily omitting subjective cognitive complaints. 80 patients, mean age 61.0 years (SD 6.6), mean duration of disease 3.4 years (SD 1.2) were included. 76 patients were neuropsychologically tested. RESULTS: 26 (34%) patients fulfilled modified PD-MCI criteria, 18 (69%) of these showed episodic memory deficits, 14 (54%) executive dysfunction, 13 (50%) language/praxis deficits, 12 (46%) visuospatial/constructional deficits and 9 (35%) attention/working memory deficits. Cognitive impairment was associated with higher Unified Parkinson's Disease Rating scale (UPDRS)-, bradykinesia- and rigidity scores and more symmetric distribution of symptoms, but not tremor scores. Patients with cognitive impairment were less educated. Other demographic and clinical variables were comparable. CONCLUSIONS: 34% of early-stage PD patients without prior clinical suspicion of cognitive impairment exhibit cognitive impairment, which is associated to disease severity, especially bradykinesia, rigidity, axial symptoms and less asymmetry of motor symptoms, even at early disease stages and when cognitive symptoms are mild.

Anal sphincter EMG in the diagnosis of parkinsonian syndromes.

BACKGROUND: The role of electromyography (EMG) recorded from the external anal sphincter (EAS) in the diagnosis of atypical parkinsonian syndromes is a matter for continuous debate. Most studies addressing this issue are retrospective. METHODS: In this study, we prospectively investigated six patients with Parkinson's Disease (IPD), 14 patients with multiple system atrophy (MSA) and eight with progressive supranuclear palsy (PSP) using EMG of the EAS, motor-evoked potential (MEP) to the EAS and EMG of m. gastrocnemius and nerve conduction velocity measured at the sural nerve. Patients were followed up for 2 years to secure correct diagnosis. RESULTS: The mean duration of motor unit potentials (MUPs) recorded from the EAS was significantly longer in patients with MSA and PSP compared with MUPs recorded from patients with PD (P < 0.005 for both). There were no signs of diffuse loss of motor neurons or peripheral neuropathy. MEP revealed signs of supranuclear affection in patients with MSA, whereas in patients with PSP the mechanism is a focal loss of motor neurons in Onuf's nucleus. CONCLUSION: Abnormal EMG of the EAS is strongly suggestive of atypical parkinsonism and the pathophysiology may be different in patients with MSA and PSP.

Dopamine transporter imaging and the effects of deep brain stimulation in patients with Parkinson's disease.

PURPOSE: Single-photon emission computed tomography (SPECT) with [123I]FP-CIT is a marker for loss of presynaptic dopamine transporters in the striatum in Parkinson's disease (PD). We used [123I]FP-CIT SPECT in order to evaluate binding to the dopamine transporter before and after neurosurgical treatment with bilateral stimulation in the subthalamic nucleus (STN). METHODS: Thirty-five patients with levodopa-responsive PD were examined with [123I]FP-CIT SPECT pre-operatively (baseline scan: mean 3 months before surgery), and 3 and 12 months after surgery. RESULTS: Pre-operatively, all patients already had substantial signs of severe nigrostriatal neuronal loss as determined from the [123I]FP-CIT SPECT scans. One year after surgery the specific [123I]FP-CIT binding to the striatum was significantly reduced by 10.3% compared with the pre-operative baseline scan. The mean time span from the baseline scan before surgery to the follow-up scan 1 year after surgery was 16.2 months. Hence, the rate of reduction equals a mean annual reduction of 7.7%. A comparable control group of patients with PD who did not undergo surgery was also examined longitudinally. In this group the specific binding of [123I]FP-CIT was reduced by 6.7% per year. CONCLUSION: The specific binding of [123I]FP-CIT was reduced equally in the STN-stimulated patients and a group of non-operated PD patients with advanced disease. Our study does not support the notion that electrode implantation and STN stimulation exert a neuroprotective effect by themselves.

Stereological estimates of number and length of capillaries in subdivisions of the human hippocampal region.

The hippocampal formation is a neuroanatomically well-defined region of the brain involved in memory processes. In view of the functional importance of the region and its involvement in a number of brain pathologies, including Alzheimer's disease and temporal lobe epilepsy, a quantitative description of its vascular supply represents an important first step in evaluating the involvement of vascular changes in these phenomena. Unbiased estimates of the length and connectivity of the vascular supply of brain regions have not been described previously. The total number, total length, and distribution of the diameters of capillaries were estimated in the five major subdivisions of the hippocampal formation (fascia dentata, hilus, CA3-2, CA1, and subiculum) in 5 normal males, 52-84 years of age. These estimates were used to derive several other structural parameters. Both the primary and the derived parameters were used to make inter- and intra-individual comparisons. For each of the five major subdivisions from each individual, the volume was estimated using the Cavalieri principle. The total capillary length was estimated on 3-microm-thick plastic isotropic uniform random sections. Using a topological definition of a capillary unit and the optical disector, total capillary number was estimated in 40-microm-thick plastic sections. Length-and number-weighted three-dimensional diameter distributions were obtained from the thin and thick plastic sections, respectively. In each subdivision the total length of capillaries was correlated with previously obtained data on the number of neurons in the same subdivisions of the same individuals. Intersubdivisional differences were observed, in that the hilus of the dentate gyrus had fewer capillaries per unit volume than the other four subdivisions. Interindividual comparisons indicate that the interindividual variances are of a magnitude suitable for sensitive group comparisons. The design-based stereological methods that were used in the analyses can provide a basis for a new unbiased approach to the estimation of vascular parameters in well-defined regions of the brain.