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Biomedical subjects

W Poewe

Publications and source records attributed to W Poewe.

At least 73 records · Page 4Linked to original sources
PubMed2001

Clinical characteristics of the geste antagoniste in cervical dystonia.

The geste antagoniste (moving an arm to the face or head) is a well-known clinical feature in cervical dystonia (CD) to alleviate the abnormal posture. The clinical phenomenology of these manoeuvres has not so far been assessed systematically. Fifty patients with idiopathic CD aware of at least one geste antagoniste (60% women, mean age at onset 44.1 years, mean disease duration 7.5 years) were subjected to a standardized investigation including a semiquantitative clinical rating scale and polymyographic recordings of six cervical muscles. Twenty-seven patients (54%) demonstrated more than one geste antagoniste (range 2-5). A clinically significant (> or = 30%) reduction of head deviation was observed in 41 patients (82 %). Dystonic head posture improved by a mean of 60 % along all planes by the geste manoeuvre with a complete cessation of head oscillations in nine of 33 patients (27 %) with phasic CD. No significant laterality of the "geste-arm" or the facial target area was found. The duration of geste-effects depended significantly on disease duration and determined the patient's self-rating of the benefit of the manoeuvre. EMG-polygraphy revealed two types of geste-induced polymyographic changes: a decrease in recruitment density and amplitude in at least one dystonic muscle (66%), and an increased tonic muscle activation in the remaining patients. The remarkable efficacy of the geste antagoniste and the considerable variety in performance, duration, and EMG-pattern of these manoeuvres warrant further investigation of the therapeutic use of sensorimotor stimulation, in particular for those CD patients who experience limited or no effect from botulinum toxin therapy.

Adolescent↗
PubMed2001

No functional effects of embryonic neuronal grafts on motor deficits in a 3-nitropropionic acid rat model of advanced striatonigral degeneration (multiple system atrophy).

Intrastriatal injection of 3-nitropropionic acid results in secondary excitotoxic local damage and retrograde neuronal cell loss in substantia nigra pars compacta, thus mimicking salient features of striatonigral degeneration, the core pathology underlying Parkinsonism associated with multiple system atrophy. We used 3-nitropropionic acid to create a rat model of advanced striatonigral degeneration in order to assess the effects of embryonic allografts upon rotational and complex-motor behavioural abnormalities. Following stereotaxic intrastriatal administration of 500nmol 3-nitropropionic acid in male Wistar rats we observed consistent amphetamine- and apomorphine-induced ipsiversive rotation. Furthermore, there were marked deficits of contralateral paw reaching. Subsequently, animals received intrastriatal implantations of either E14 mesencephalic or striatal or mixed embryonic cell suspensions. In addition, one group received sham injections. Grafted rats were followed for up to 21 weeks and repeated behavioural tests were obtained during this period. Drug-induced rotation asymmetries and complex motor deficits measured by paw reaching tests were not compensated by embryonic grafts. Persistence of drug-induced rotations and of paw reaching deficits following transplantation probably reflects severe atrophy of adult striatum, additional nigral degeneration as well as glial demarcation of embryonic grafts. We suggest that dopamine rich embryonic grafts fail to induce functional recovery in a novel 3-nitropropionic acid rat model of advanced striatonigral degeneration (multiple system atrophy).

Animals↗
PubMed2001

Juvenile asymmetric segmental spinal muscular atrophy (Hirayama's disease): three cases without evidence of "flexion myelopathy".

Compression of the cervical spinal cord during neck movements ("flexion myelopathy") was proposed to be the main pathomechanism of juvenile asymmetric spinal muscular atrophy (JASSMA). We present 3 patients with the clinical appearance of JASSMA and typical high-intensity signals in the anterior horn cell region of the lower cervical spinal cord (T2-weighted magnetic resonance images) but without evidence of dynamic spinal cord compression. In all these patients pathomechanism distinct from mechanical damage must be assumed.

Adolescent↗
PubMed2001

Temporal and spatial profile of caspase 8 expression and proteolysis after experimental traumatic brain injury.

Recent studies have demonstrated that the downstream caspases, such as caspase 3, act as executors of the apoptotic cascade after traumatic brain injury (TBI) in vivo. However, little is known about the involvement of caspases in the initiation phase of apoptosis, and the interaction between these initiator caspases (e.g. caspase 8) and executor caspases after experimental brain injuries in vitro and in vivo. This study investigated the temporal expression and cell subtype distribution of procaspase 8 and cleaved caspase 8 p20 from 1 h to 14 days after cortical impact-induced TBI in rats. Caspase 8 messenger RNA levels, estimated by semiquantitaive RT-PCR, were elevated from 1 h to 72 h in the traumatized cortex. Western blotting revealed increased immunoreactivity for procaspase 8 and the proteolytically active subunit of caspase 8, p20, in the ipsilateral cortex from 6 to 72 h after injury, with a peak at 24 h after TBI. Similar to our previous studies, immunoreactivity for the p18 fragment of activated caspase 3 also increased in the current study from 6 to 72 h after TBI, but peaked at a later timepoint (48 h) as compared with proteolyzed caspase 8 p20. Immunohistologic examinations revealed increased expression of caspase 8 in neurons, astrocytes and oligodendrocytes. Assessment of DNA damage using TUNEL identified caspase 8- and caspase 3-immunopositive cells with apoptotic-like morphology in the cortex ipsilateral to the injury site, and immunohistochemical investigations of caspase 8 and activated caspase 3 revealed expression of both proteases in cortical layers 2-5 after TBI. Quantitative analysis revealed that the number of caspase 8 positive cells exceeds the number of caspase 3 expressing cells up to 24 h after impact injury. In contrast, no evidence of caspase 8 and caspase 3 activation was seen in the ipsilateral hippocampus, contralateral cortex and hippocampus up to 14 days after the impact. Our results provide the first evidence of caspase 8 activation after experimental TBI and suggest that this may occur in neurons, astrocytes and oligodendrocytes. Our findings also suggest a contributory role of caspase 8 activation to caspase 3 mediated apoptotic cell death after experimental TBI in vivo.

Animals↗
PubMed2001

[Treatment of focal dystonia with botulinum toxin A].

Local injections with Botulinum toxin A (BtxA) are safe and effective in the treatment of focal dystonia. In cervical dystonia and blepharospasm, BtxA injections have become the treatment of choice. However, good results have also been reported with oromandibular dystonia, spasmodic dysphonia and writer's cramp. In cervical dystonia, muscles for injection are selected by clinical presentation or in complex forms with EMG guidance. Several studies have shown that 500 units Dysport are safe and effective in the treatment of cervical dystonia. In blepharospasm, injections are performed in the periorbital part of the orbicularis oculi muscle with good results for 12-14 weeks. The most frequently employed starting dose is 120 units Dysport per eye, divided in three periorbital injection sites. In case of levator inhibition, the pretarsal part of the orbicularis oculi muscle should be injected in a lower dose. EMG guidance is not necessary. By contrast, BtxA treatment of spasmodic dysphonia and writer's cramp require EMG-guided injections in order to avoid side-effects. Dose recommendations for the various types of dystonia are given in the text. In up to 5% of patients with dystonia, the development of neutralising antibodies is reported following repetitive injections with BtxA. Patients with antibodies had a shorter interval between injections, more "boosters", a higher dose per 3-month interval, and a higher total dose injected. In case of neutralizing antibodies against the A toxin, the treatment with Botulinum toxin B (Neurobloc) is a possible alternative.

Biomechanical Phenomena↗
PubMed2000

Progression of Hoehn and Yahr stages in Parkinsonian disorders: a clinicopathologic study.

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.

Adult↗
PubMed2000

Vascular endothelial growth factor in CSF: a biological marker for carcinomatous meningitis.

OBJECTIVE: To determine the value of vascular endothelial growth factor (VEGF) in CSF as a marker for carcinomatous meningitis (CM). METHODS: The concentration of VEGF was measured by ELISA in matched samples of CSF and serum collected from 162 patients. These included patients with solid tumors with CM (n = 11) or brain metastases without concomitant CM (n = 12), paraneoplastic neurologic syndromes (n = 4), viral (n = 15) and bacterial (n = 20) meningitis, and a variety of non-neoplastic and noninfectious neurologic diseases (n = 100). Using CSF/serum albumin ratios, the VEGF index was calculated to estimate the proportion of intrathecally produced VEGF. Immunohistochemical staining for VEGF was performed in a brain metastasis from a mammary carcinoma associated with CM. RESULTS: High VEGF levels (median 6,794.8 pg/mL) were found in CSF of all patients with CM, whereas VEGF levels in matched sera were comparable to other disease groups. In patients with CM, the concentration of VEGF in CSF decreased significantly following antineoplastic treatment. In CSF samples from patients with brain metastases without concomitant CM, VEGF was not detectable. Median VEGF concentration in CSF from patients with acute bacterial meningitis was 38.6 pg/mL, with only 9 of these 17 patients showing detectable VEGF levels in CSF. The VEGF indices in patients with bacterial meningitis were significantly lower than in tumor patients with CM (<22.8 versus >62.3), suggesting that the proportion of intrathecally produced VEGF is much higher in patients with CM as compared with patients with bacterial meningitis. Patients without neoplastic or infectious neurologic disorders consistently showed VEGF levels in CSF below the assay detection limit of 25 pg/mL. Immunohistochemistry revealed strong cytoplasmic staining for VEGF in a metastatic lesion from breast cancer infiltrating the meninges. CONCLUSION: In patients with carcinomatous meningitis, significant amounts of VEGF are released into CSF. This study yields preliminary evidence that VEGF in CSF may be a useful biologic marker for both the diagnosis and evaluation of treatment response in carcinomatous meningitis.

Adolescent↗
PubMed2000

Toward a primate model of L-dopa-unresponsive parkinsonism mimicking striatonigral degeneration.

We developed a primate model of striatonigral degeneration (SND), the neuropathology underlying levodopa-unresponsive parkinsonism associated with multiple systemic atrophy (MSA-P), by sequential systemic administration of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) and 3-nitropropionic acid (3NP) in a Macaca fascicularis monkey. L-Dopa-responsive parkinsonian features emerged after MPTP injections. Subsequent chronic 3NP administration aggravated the motor symptoms and abolished the L-dopa response. In vivo magnetic resonance imaging revealed bilateral striatal lesions. Histopathologically, there was severe dopaminergic cell loss in the substantia nigra pars compacta compared with the control monkey. Furthermore, we observed circumscribed areas of severe neuronal degeneration in the motor striatum. These changes were absent in the control monkey, and they were associated with diffuse metabolic failure as demonstrated by cytochrome oxidase histochemistry. The striatal pathology predominantly involved output pre-pro-enkephalin A- and substance P-containing cells, whereas somatostatin (NADPH-diaphorase)-containing interneurons were relatively spared. Our model therefore reproduced levodopa-unresponsive parkinsonism and SND-like pathologic changes characteristic of MSA-P. The double-lesion primate model of SND may serve as a preclinical test-bed for the evaluation of novel therapeutic strategies in MSA-P.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗
PubMed2000

Progressive multifocal leukoencephalopathy presenting with an isolated focal movement disorder.

Progressive multifocal leukoencephalopathy (PML) is a rare but fatal papovavirus infection of the central nervous system predominantly affecting immunocompromised patients. Although the basal ganglia circuitry may be involved in the pathology of PML, movement disorders are exceedingly rare as presenting symptoms and have not been described as isolated features in such patients. We report a previously healthy, immunocompetent 24-year-old woman with histologically proven PML who presented with a focal movement disorder of the left arm as an isolated symptom for many months before diagnosis.

Adult↗
PubMed2000

Apomorphine: an underutilized therapy for Parkinson's disease.

Apomorphine was the first dopaminergic drug ever used to treat symptoms of Parkinson's disease. While powerful antiparkinsonian effects had been observed as early as 1951, the potential of treating fluctuating Parkinson's disease by subcutaneous administration of apomorphine has only recently become the subject of systematic study. A number of small scale clinical trials have unequivocally shown that intermittent subcutaneous apomorphine injections produce antiparkinsonian benefit close if not identical to that seen with levodopa and that apomorphine rescue injections can reliably revert off-periods even in patients with complex on-off motor swings. Continuous subcutaneous apomorphine infusions can reduce daily off-time by more than 50% in this group of patients, which appears to be a stronger effect than that generally seen with add-on therapy with oral dopamine agonists or COMT inhibitors. Extended follow-up studies of up to 8 years have demonstrated long-term persistence of apomorphine efficacy. In addition, there is convincing clinical evidence that monotherapy with continuous subcutaneous apomorphine infusions is associated with marked reductions of preexisting levodopa-induced dyskinesias. The main side effects of subcutaneous apomorphine treatment are related to cutaneous tolerability problems, whereas sedation and psychiatric complications play a lesser role. Given the marked degree of efficacy of subcutaneous apomorphine treatment in fluctuating Parkinson's disease, this approach seems to deserve more widespread clinical use.

Antiparkinson Agents↗
PubMed2000

Beneficial effects of amantadine on L-dopa-induced dyskinesias in Parkinson's disease.

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.

Aged↗
PubMed2000

Quantification of sensory trick impact on tremor amplitude and frequency in 60 patients with head tremor.

Head tremor with an obvious head deviation is the typical clinical picture of tremulous cervical dystonia (TCD), whereas head tremor without any significant head deviation allows for the differential diagnosis of dystonic head tremor (DHT) as well as essential head tremor (EHT). Clinical and polyelectromyographic (poly-EMG) studies have shown a suppression of dystonic muscle activity in patients with TCD performing a maneuver called geste antagonistique. The effect of these trick maneuvers on head tremor has not been investigated in patients with DHT and EHT. We studied the impact of sensory trick maneuvers on head tremor amplitude and frequency clinically by using the tremor subscore of the Tsui scale and by means of computer-based accelerometry in 60 patients with head tremor as their major disorder. Based on clinical data (modified Tsui scale: rating of spontaneous head deviation [rotation + lateroflexion + ante-/retroflexion]), pharmacologic response of tremor (propranolol, primidone, or alcohol), family history (postural hand tremor in first-degree relatives), and poly-EMG findings (reciprocal inhibition in neck muscles during voluntary head rotation), 34 patients were diagnosed as having TCD, 14 were classified as having DHT, and 12 patients were diagnosed as having EHT. Using a clinical rating scale, head tremor amplitudes showed a significant decrease compared with baseline during the performance of sensory trick maneuvers in patients with TCD and DHT, but not in patients with EHT. This clinically observed effect was accompanied by a significant reduction in the mean peak power of the dominant frequency in patients with TCD (decrease by 83%, p = 0.0001) and DHT (decrease by 90%, p = 0.01), but not in patients with EHT (decrease by 6%, p = 0.6). Head tremor frequencies showed no significant changes in relation to the trick maneuvers. We conclude that a significant reduction of head tremor amplitude during a sensory trick maneuver is a useful quantitative criterion to distinguish TCD and DHT from EHT.

Adrenergic beta-Antagonists↗
PubMed2000

Sociocultural differences in gait.

Transcultural differences in routine motor behavior and movement disorders have rarely been assessed. In the present study gait was studied in 47 healthy inhabitants of Tyrol living in rural or semi-urban (Innsbruck, Austria) settings and 43 healthy subjects residing in Berlin, Germany. In addition, gait was assessed in 23 patients in early stages of idiopathic Parkinson's disease (11 in Berlin, 12 in Innsbruck). Healthy subjects in Berlin showed faster gait velocity than their counterparts in Tyrol, and patients with Parkinson's disease were slightly slower than their respective healthy peers in both environments. Surprisingly, patients with Parkinson's disease from Berlin had significantly faster walking speeds than both patients and healthy control subjects from Tyrol. High gait tempo in parkinsonian patients from Berlin was characterized by fast step-rates and short strides. Differences in normal gait in different sociocultural settings are thus reflected in parkinsonian slowing of gait.

Aged↗