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Biomedical subjects

R Benecke

Publications and source records attributed to R Benecke.

At least 19 recordsLinked to original sources
PubMed2006

Transcallosal inhibition in amyotrophic lateral sclerosis.

OBJECTIVE: Assessment of upper motor neuron (UMN) involvement is essential for the diagnosis of amyotrophic lateral sclerosis (ALS). In a number of ALS cases, mirror movements (MM) suggest an involvement of transcallosal fibre tracts in conjunction with UMN involvement. The present study analysed whether deficient transcallosal inhibition (TI) tested by TMS enables detection of cortical affection in ALS, even at early stages of the disease. METHODS: In three patients with definite ALS and 12 patients with early ALS (aged 64.1+/-7.8 years) TMS investigation included analysis of contralateral (cMEP) and ipsilateral (iMEP) motor evoked potentials as well as measurement of TI (latency, duration) with recording from both first dorsal interosseus muscles. RESULTS: Clinical UMN signs were present in four patients. 83.3% of patients showed a pathological TI (prolongation or loss of TI). Five out of eight ALS patients showing a pathological TI had no clinical UMN signs. Two of these patients showed MM. One patient displayed also pathological findings in TI investigation. CONCLUSIONS: Our findings suggest a functional deficit of transcallosal fibre tracts even at early stages of the disease still lacking clinical UMN signs. SIGNIFICANCE: Measurement of TI tested by TMS can detect an involvement of the cortical output system in ALS and may be helpful in an early assessment of the diagnosis.

Aged↗
PubMed2006

Modulation of transcallosally mediated motor inhibition in children with attention deficit hyperactivity disorder (ADHD) by medication with methylphenidate (MPH).

Motor hyperactivity is one of the most outstanding symptoms of attention deficit hyperactivity disorder (ADHD) which might be caused by a disturbed inhibitory motor control. Using focal transcranial magnetic stimulation (TMS) we tested the cortico-callosal inhibition (duration and latency of the ipsilateral Silent Period, iSP) in 23 children with ADHD (mean age 11+/-2.6 years) before and on treatment with methylphenidate (MPH). iSP latency was age correlated, whereas iSP duration as well as Conners scores were age independent. Analyses of mean differences revealed a significant prolongation of iSP duration (p=0.001), shortening of iSP latency (p=0.027) and reduction of Conners score (p=0.001) under medication. Increase of iSP duration and reduction of Conners score under medication were significantly correlated (t=-9.87, p=0.016). Reduced iSP duration and prolonged iSP latency in ADHD children could be the result of a disturbed transcallosally mediated inhibition, most probable due to a combination of maturation deficits of callosal fiber tracts as well as neuronal synaptical transmission within the neuronal network between ipsilaterally stimulated cortex layer III--the origin of transcallosal motor-cortical fibers--and contralateral layer V, the origin of the pyramidal tract. MPH may indirectly improve the dysbalance between excitatory and inhibitory interneuronal activities of this neuronal network via dopaminergic modulatory effects of the striato-thalamo-cortical loop.

Adolescent↗
PubMed2006

Overactive bladder in Parkinson's disease: alteration of brainstem raphe detected by transcranial sonography.

Urinary dysfunction is very common in idiopathic Parkinson's disease (PD) and manifests primarily with symptoms of overactive bladder (OAB). Affection of central serotonergic systems has been suggested to play a role in OAB. The objective of this study was to evaluate whether in PD patients with OAB symptoms a specific alteration of the brainstem raphe (BR), which contains serotonergic neurons, can be detected with transcranial sonography (TCS). Of 116 PD patients enrolled, 19 had PD-related OAB symptoms (OAB+) unlike remaining 97 patients (OAB-). Patients were examined by a sonographer blinded to the clinical data. Reduced echogenicity of BR was found in 12 (63%) OAB+ patients but only in 18 (19%) of 93 assessable OAB- patients (Mann-Whitney U-test, P < 0.001). In OAB+ patients, lower raphe echogenicity score was associated with longer duration of OAB symptoms (anova, P = 0.033). Other TCS findings such as echogenicity of substantia nigra, thalami, lenticular and caudate nuclei, and widths of third and lateral ventricles did not differ between OAB+ and OAB- patients. TCS findings suggest a pathogenetic role of BR in OAB related to PD. Alterations may reflect disturbance of its central serotonergic system.

Adult↗
PubMed2005

Concurrent chemoradiotherapy and adjuvant chemotherapy with Topotecan for patients with glioblastoma multiforme.

PURPOSE: The prognosis for patients with glioblastoma multiforme remains poor. Phase II studies, meta-analyses and a phase III study show that concurrent chemoradiotherapy has an advantage over irradiation alone. In this study the effectiveness of concurrent chemoradiotherapy with Topotecan and an adjuvant chemotherapy with Topotecan was investigated. PATIENTS AND METHOD: Forty-two patients with predominantly unfavourable prognosis factors were included in the study and treated as follows: hyperfractionated accelerated radiotherapy (2x1.75Gy to 45.5 + 12.25 Gy (RP)) with a concurrent, continuous infusion of Topotecan (0.5 mg/m(2)/d, days 1-21). On day 28 the adjuvant chemotherapy (three courses) was begun according to the same scheme. RESULT: Haematological toxicities were 13/42 (30%) grade III leucopenia, 2/42 (4%) grade IV, as well as 5/42 (10%) grade III thrombopenias and 1/42 (2%) grade IV. 30/42 (71%) patients showed improvement or stabilisation of an existing neurological symptomatic complex. The median time to progression was 7.2 (+/- 0.8) months, the median total survival was 10 (+/- 1.2) months, the 2 year survival rate 4.7 (+/- 0.3)%. Prognostic factors were age, surgical radicality, performance status and the tumour volume before therapy. SUMMARY: Concurrent chemoradiotherapy and an adjuvant chemotherapy with Topotecan is feasible at acceptable toxicity levels also for patients with a moderate performance status. The patients benefit from the improvement of the clinical symptomatic complex and, even with unfavourable prognosis factors, have a higher median survival in comparison to data published on similar groups of patients given only radiotherapy.

Adult↗
PubMed2005

Diagnosis and management of acute movement disorders.

Most movement disorders, reflecting degenerative disorders, develop in a slowly progressive fashion. Some movement disorders, however, manifest with an acute onset. We wish to give an overview of the management and therapy of those acute-onset movement disorders.Drug-induced movement disorders are mainly caused by dopamine-receptor blockers (DRB) as used as antipsychotics (neuroleptics) and antiemetics. Acute dystonic reactions usually occur within the first four days of treatment. Typically, cranial pharyngeal and cervical muscles are affected. Anticholinergics produce a prompt relief. Akathisia is characterized by an often exceedingly bothersome feeling of restlessness and the inability to remain still. It is a common side effect of DRB and occurs within few days after their initiation. It subsides when DRB are ceased. Neuroleptic Malignant Syndrome is a rare, but life-threatening adverse reaction to DRB which may occur at any time during DRB application. It is characterised by hyperthermia, rigidity, reduced consciousness and autonomic failure. Therapeutically immediate DRB withdrawal is crucial. Additional dantrolene or bromocriptine application together with symptomatic treatment may be necessary. Paroxysmal dyskinesias are childhood onset disorders characterised by dystonic postures, chorea, athetosis and ballism occurring at irregular intervals. In Paroxysmal Kinesigenic Dyskinesia they are triggered by rapid movements, startle reactions or hyperventilation. They last up to 5 minutes, occur up to 100 times per day and are highly sensitive to anticonvulsants. In Paroxysmal Non-Kinesiogenic Dyskinesia they cannot be triggered, occur less frequently and last longer. Other paroxysmal dyskinesias include hypnogenic paroxysmal dyskinesias, paroxysmal exertional dyskinesia, infantile paroxysmal dystonias, Sandifer's syndrome and symptomatic paroxysmal dyskinesias. In Hereditary Episodic Ataxia Type 1 attacks of ataxia last for up to two minutes, may be accompanied by dysarthria and dystonia and usually respond to phenytoin. In Type 2 they can last for several hours, may be accompanied by vertigo, headache and malaise and usually respond to acetazolamide. Symptomatic episodic ataxias can occur in a number of metabolic disorders, but also in multiple sclerosis and Behcet's disease.

Adolescent↗
PubMed2005

A new botulinum toxin type A free of complexing proteins for treatment of cervical dystonia.

A new botulinum toxin type A free of complexing proteins (NT 201) was compared with BOTOX in patients with cervical dystonia by means of a double-blind noninferiority trial. Four hundred sixty-three patients received IM injections of 70 to 300 U of NT 201 or BOTOX and were followed up over 16 weeks. The study clearly shows that NT 201 is at least as effective and safe as BOTOX.

Antibodies↗
PubMed2005

Sonographic detection of basal ganglia lesions in asymptomatic and symptomatic Wilson disease.

OBJECTIVE: To investigate whether transcranial brain parenchyma sonography (TCS) detects basal ganglia abnormalities in asymptomatic and symptomatic patients with Wilson disease (WD) and whether findings correlate with disease severity. METHODS: Twenty-one patients with WD with (n = 18) or without (n = 3) neurologic symptoms were investigated. Disease severity was assessed by three independent neurologists using a WD rating scale (WDRS) with the items dysarthria, akinesia, ataxia, tremor, and dystonia; the raters' median score was used for further analysis. Basal ganglia TCS was performed according to a standardized protocol. RESULTS: TCS revealed lenticular nucleus (LN) hyperechogenicity in all assessable neurologically symptomatic and in two of the three asymptomatic patients. Size of LN hyperechogenic area correlated with the WDRS score (Spearman correlation, rho = 0.604, p = 0.006), as did the size of thalamus hyperechogenic area (n = 7, rho = 0.891, p = 0.007), the width of third ventricle (n = 21, rho = 0.613, p = 0.003), and the width of lateral ventricles (n = 20, rho = 0.642, p < 0.001). Substantia nigra hyperechogenicity, detected in 10 patients, did not correlate with disease severity. There was no correlation between age at disease onset or disease duration and any TCS finding. Of the 19 patients with LN hyperechogenicity, only 12 showed abnormal LN on MRI. CONCLUSIONS: Transcranial brain parenchyma sonography (TCS) detects lenticular nucleus hyperechogenicity, likely to be caused by copper accumulation, in neurologically symptomatic and asymptomatic Wilson disease (WD). TCS findings correlate with disease severity. TCS appears a promising tool for disease monitoring in WD.

Adult↗
PubMed2005

[Diagnosis and therapy of Parkinson's disease].

The clinical diagnosis of Parkinson's disease is mainly based upon the principal symptoms bradykinesia, rigidity and tremor at rest. The differential diagnosis of idiopathic Parkinson's disease (IPD) from other parkinsonisms is from the prognostic and therapeutic standpoint of considerable importance because the Parkinson-Plus syndrome generally does not or only poorly responds to a dopaminergic therapy. The treatment of IPD comprises, in addition to, dopaminergic medications such as L-dopa or dopamine receptor agonists, operative therapeutic options such as deep brain stimulation and nondrug therapies.

Antiparkinson Agents↗
PubMed2004

Sonographic discrimination of corticobasal degeneration vs progressive supranuclear palsy.

OBJECTIVE: To study the use of brain parenchyma sonography (BPS) in discriminating between patients with corticobasal degeneration (CBD) and progressive supranuclear palsy (PSP). METHODS: Thirteen patients with PSP and eight with CBD were studied with BPS according to a standardized protocol. RESULTS: Seven (88%) of the eight CBD patients showed marked hyperechogenicity of the substantia nigra (SN) but none of eleven PSP patients (Mann-Whitney U test, p < 0.001). This finding indicated CBD with a positive predictive value of 100%. Marked dilatation of the third ventricle (width > 10 mm) was found in 10 (83%) of 12 PSP patients, but in none of the CBD patients (p < 0.005). BPS measurements of ventricle widths closely matched MRI measurements (Pearson correlation, r = 0.90, p < 0.001). The presence of at least one of the BPS findings 1) marked SN hyperechogenicity and 2) third-ventricle width < 10 mm indicated CBD with a sensitivity of 100%, a specificity of 83%, and a positive predictive value of 80%. Other BPS findings such as echogenicity of lentiform and caudate nuclei and widths of the frontal horns did not discriminate between CBD and PSP. One PSP patient could not be assessed because of insufficient acoustic temporal bone windows. CONCLUSIONS: Substantia nigra hyperechogenicity, reported earlier as characteristic brain parenchyma sonography finding in idiopathic Parkinson disease, is also typical for corticobasal degeneration.

Aged↗
PubMed2004

Escalating immunotherapy of multiple sclerosis--new aspects and practical application.

Recent clinical studies in multiple sclerosis (MS) provide new data on the treatment of clinically isolated syndromes, on secondary progression, on direct comparison of immunomodulatory treatments and on dosing issues. All these studies have important implications for the optimized care of MS patients. The multiple sclerosis therapy consensus group (MSTCG) critically evaluated the available data and provides recommendations for the application of immunoprophylactic therapies. Initiation of treatment after the first relapse may be indicated if there is clear evidence on MRI for subclinical dissemination of disease. Recent trials show that the efficacy of interferon beta treatment is more likely if patients in the secondary progressive phase of the disease still have superimposed bouts or other indicators of inflammatory disease activity than without having them. There are now data available, which suggest a possible dose-effect relation for recombinant beta-interferons. These studies have to be interpreted with caution, as some potentially important issues in the design of these studies (e. g. maintenance of blinding in the clinical part of the study) were not adequately addressed. A meta-analysis of selected interferon trials has been published challenging the value of recombinant IFN beta in MS. The pitfalls of that report are discussed in the present review as are other issues relevant to treatment including the new definition of MS, the problem of treatment failure and the impact of cost-effectiveness analyses. The MSTCG panel recommends that the new diagnostic criteria proposed by McDonald et al. should be applied if immunoprophylactic treatment is being considered. The use of standardized clinical documentation is now generally proposed to facilitate the systematic evaluation of individual patients over time and to allow retrospective evaluations in different patient cohorts. This in turn may help in formulating recommendations for the application of innovative products to patients and to health care providers. Moreover, in long-term treated patients, secondary treatment failure should be identified by pre-planned follow-up examinations, and other treatment options should then be considered.

Clinical Trials as Topic↗
PubMed2004

Probable multiple system atrophy in a German family.

Multiple system atrophy (MSA) is a neurodegenerative disorder of unknown aetiology. A possible underlying genetic component has not yet been identified. A family is reported with phenotypic MSA and probable autosomal dominant inheritance. The patients presented initially with either parkinsonian or cerebellar signs, and developed severe autonomic failure and typical atrophy of the brain stem and cerebellum in the course of the disease.

Adult↗
PubMed2003

Brain parenchyma sonography discriminates Parkinson's disease and atypical parkinsonian syndromes.

OBJECTIVE: To study the use of brain parenchyma sonography (BPS) in discriminating between patients with idiopathic PD (IPD) and atypical parkinsonian syndromes (APS). METHODS: Twenty-five patients with APS, 9 with progressive supranuclear palsy (PSP) and 16 with multiple-system atrophy (MSA), and 25 age-matched patients with IPD were prospectively studied with BPS according to a standardized protocol. RESULTS: Twenty-four of the 25 (96%) IPD patients exhibited hyperechogenicity of the substantia nigra (SN) but only 2 of 23 (9%) APS patients (Mann-Whitney U test, p < 0.001). In those two APS patients, SN hyperechogenicity was moderate only, whereas the remaining 21 APS patients had normal SN echogenicity. The specificity of SN hyperechogenicity in detection of clinically diagnosed IPD patients was 96%, and the sensitivity was 91%. If SN hyperechogenicity was marked, APS could be excluded because of a positive predictive value of 100% for IPD. Nucleus lentiformis hyperechogenicity was found in 17 of 22 (77%) APS patients but in only 5 of 22 (23%) IPD patients (Mann-Whitney U test, p < 0.001). Nucleus caudatus and thalamus echogenicity and widths of the third ventricle and of the frontal horns of the lateral ventricles did not discriminate between IPD and APS. Two patients with PSP could not be assessed because of an insufficient bone window. CONCLUSIONS: BPS is a novel and noninvasive method to differentiate highly specifically between IPD and APS. Therefore, BPS might become a standard investigation in parkinsonian disorders.

Aged↗
PubMed2003

[Use of botulinum toxin the the treatment of muscle pain].

The analgesic effects of botulinum toxin (BTX) have been discussed controversially due to substantial placebo effects and flaws in the study designs used. Additionally, pathophysiological concepts of pain and the specific analgesic mechanisms of BTX remain largely unclear. Apart from pain reduction through the well-documented effects of BTX at the neuromuscular endplate, additional analgesic mechanisms, including other synaptic and local effects, have been suggested. Currently, BTX can be recommended for pain treatment in dystonia and spasticity. In myofascial pain syndromes, pain relief by BTX injections has been reported, but definite proof according to evidence-based medicinal criteria is still lacking. In fibromyalgia, there seems to be no analgesic effect. The role of BTX in pain therapy is likely to increase in the future.

Botulinum Toxins, Type A↗
PubMed2002

[Initial experiences with clinical use of botulinum toxin type B].

Recently, botulinum toxin type B (BT-B) was introduced for treatment of cervical dystonia (CD). We wish to report on experience with BT-B derived from registration studies and from our own preliminary clinical experience. Botulinum toxin type B can be used successfully in CD patients with antibody-induced failure of botulinum toxin type A therapy (antibody patients). In our own 15 antibody patients, 11835 +/- 2,039 mouse units of BT-B (NeuroBloc) reduced the Toronto Western spasmodic torticollis rating scale (TWSTRS) score from 20.5 +/- 3.6 to 13.1 +/- 5.4. BT-B can also be used successfully in CD patients not previously exposed to BT-A or BT-B (de novo patients). In our own nine de novo patients, 10436 +/- 3,320 mouse units of BT-B reduced the TWSTRS score from 18.4 +/- 5.4 to 9.2 +/- 4.8. Altogether, dryness of the mouth occurred in 21 CD patients (severe 10, moderate 7, mild 4), accommodation difficulties in seven, conjunctival irritation in five, reduced sweating in four, swallowing difficulties in three, heartburn in three, constipation in three, bladder voiding difficulties in two, and dryness of nasal mucosa, head instability, and thrush in one each. BT-B produces substantially more systemic autonomic side effects than BT-A. It can be considered the therapy of choice in antibody patients. For de novo patients and for BT-A-treated CD patients, BT-B cannot be recommended currently.

Adult↗
PubMed2002

Substantia nigra echogenicity is normal in non-extrapyramidal cerebral disorders but increased in Parkinson's disease.

Transcranial sonography (TCS) revealed substantia nigra (SN) hyperechogenicity in idiopathic Parkinson's disease (IPD). To further evaluate specificity of this finding, we examined 30 IPD patients and 30 age-matched subjects with non-extrapyramidal cerebral disorders (NED). All IPD patients showed a SN hyperechogenicity, in 17 it was bilateral and in 13 unilateral. 7 NED patients had a SN hyperechogenicity, in all it was unilateral, confirming previous results in healthy subjects. Bilateral SN hyperechogenicity indicates IPD and normal SN echogenicity indicates NED. In 30% of patients TCS does not distinguish between IPD and NED. Data further support the assumption that bilateral SN hyperechogenicity is specific for IPD.

Aged↗
PubMed2001

The outcome in myasthenia gravis patients--an eight-year follow-up after finishing immunoabsorption therapy.

Eight years ago four patients suffering from myasthenia gravis (MG) type C and E according to Compston with failed drug therapy were treated three times (one patient 11 times) by protein A immunoabsorption (Immunosorba, Excorim, Fresenius Hemocare GmbH, StWendel, Germany). No further immunoabsorption treatments have been carried out. In addition, three patients were given a thymectomy. The present status of the patients was checked six and eight years thereafter. We could see a beneficial effect in all MG patients. The patients are fit for work; all have an improved Besinger index. The patients were used as their own controls. A higher anti-AChR-ab level six years after protein A immunoabsorption than at the beginning was seen in all patients combined with a less serious MG. In addition, their immunomodulation could be induced as seen in lymphocyte and inflammatory protein changes during the first 36 days after beginning immunoabsorption treatment. A larger population has to be investigated to verify these results.

Adult↗