Health-related quality of life in segmental dystonia is improved by bilateral pallidal stimulation.

In contrast to generalized dystonia, reports on the effectiveness of pallidal stimulation on quality of life in patients with segmental dystonia are sparse to date. In ten patients with idiopathic segmental dystonia we prospectively evaluated the effect of pallidal stimulation on quality of life using the SF-36 questionnaire. Parallel to the improvement of motor scores, total SF-36 scores and physical and mental health subscores improved significantly at follow-up to a mean of 17 months postoperatively. Thus, pallidal stimulation should be recognized as a promising treatment option in patients with segmental dystonia.

Patterns of reoccurrence of segmental dystonia after discontinuation of deep brain stimulation.

The pattern of reoccurrence of symptoms after discontinuation of deep brain stimulation (DBS) has not been systematically studied in dystonia. Eight patients (mean age (SD) 53.8 (14.4) years) with segmental dystonia at a mean follow-up of 11.3 (4.2) months were studied after implantation of bilateral DBS electrodes in the internal globus pallidus using a standard video protocol and clinical rating scales, immediately and at 2 and 4 h after switching off DBS. Dystonic signs returned sequentially, with a rapid worsening of phasic and a slower worsening of tonic dystonic components. In all patients, phasic dystonic features appeared within a few minutes, whereas the tonic elements of dystonia reoccurred with a more variable delay. Differential clinical effects when withdrawing DBS might reflect its influence on different pathophysiological mechanisms in dystonia.

Chronic spinal cord stimulation in medically intractable orthostatic tremor.

BACKGROUND: Orthostatic tremor with its sense of unsteadiness when standing may have a devastating effect on affected persons. Currently, there are no other treatment options in those who do not respond or who do not tolerate medical treatment. OBJECTIVES: To report on a pilot study on spinal cord stimulation in medically intractable orthostatic tremor. METHODS: Chronic spinal cord stimulation (SCS) was performed in two patients with medically-intractable orthostatic tremor via quadripolar plate electrodes implanted at the lower thoracic spine. The electrodes were connected to implantable pulse generators. RESULTS: Subjective and objective improvement of unsteadiness was achieved within a frequency range of 50 to 150 Hz, and occurred in the presence of stimulation-induced paraesthesia. With optimized stimulation settings polygraphic electromyelogram (EMG) recordings continued to show the typical 14-16 Hz EMG activity. The beneficial effect of SCS was maintained at long-term follow-up. CONCLUSIONS: The results of this pilot study indicate that SCS may be an option in patients with otherwise intractable orthostatic tremor.

[Reversible pathological gambling under treatment with pramipexole]. / Reversible Glückspielsucht unter Pramipexol.

In the recent past, pathological gambling has been increasingly reported to be related with dopamine agonist application in patients with Parkinson's disease. We present the case of a female patient whose gambling behavior occurred shortly after reaching the final dose of a combined levodopa and pramipexole therapy and resolved completely after stopping pramipexole. Pathological gambling seems to be a rare and serious but reversible adverse reaction to treatment with dopamine agonists of which clinicians should be aware in patients with Parkinson's disease.

Amino acid metabolism in liver disease.

The impairment of transsulphuration during methionine degradation in hepatic failure can be counteracted by treatment with S-adenosylmethionine. Regarding the pathogenesis of hepatic encephalopathy, no convincing evidence exists for tryptophan, glutamine or glutamate being involved. Portal-systemic shunting-induced hyperammonaemia may reduce plasma branched-chain amino acids. The glucose effect on urea synthesis does not exist in cirrhosis.

[Heparin during acute ischemic stroke. Present data and clinical situation]. / Heparin in der Akutphase des ischämischen Schlaganfalls. Datenlage und klinische Realität.

Therapy with low- or high-dose heparin in acute stroke is changing. Despite several clinical studies (>20), some with quite large numbers of patients, no statistically significant benefit was found for the clinical endpoints of death and functional outcome. This negative result remains even when considering the preventive effect of high-dose heparin on secondary acute embolic events (e.g., cardiac emboli-arrhythmia) and low-dose heparin on venous thrombosis. Based on study results, most reviews and therapy recommendations for the treatment of acute stroke generally decline the use of high-dose heparins and heparinoids with full anticoagulation for improving outcome or preventing secondary embolic events as well as low-dose applications for venous thrombosis prophylaxis. This paper reviews the literature and presents the data of a standardised survey on coagulation therapy in acute stroke patients from all university and major stroke units in Germany (n=33). Contrary to the restrictive recommendations, therapy with heparin is firmly established in most stroke units. Full anticoagulation with heparin ("full dose") is performed on selected patients in 32/33 stroke units (97%). The selection criteria and thus the frequency of high-dose heparin use varies widely among the different centers. Almost all German stroke units (97%) routinely use low-dose heparin to prevent venous thrombosis and pulmonary embolism.The heparin agents and dosage, however, vary. These data correspond to those from the USA and Canada,where daily routine also departs from evidence-based treatment recommendations. That may be due to individual pathophysiological and aetiological considerations and of course the low acceptance of treatment recommendations based on classic, randomised trials. This underlines the need for new concepts (e.g.observational trials, continuous registers, etc.) addressing the adaptation of study-related conditions to the much more complex situation of daily routine (with risk/benefit, safety, and economic variables).

Risperidone-responsive segmental dystonia and pallidal deep brain stimulation.

A 67-year-old man with risperidone-responsive segmental dystonia underwent bilateral deep brain stimulation (DBS) of the globus pallidus internus. Prospectively, the authors assessed the Burke-Fahn-Marsden Dystonia Rating Scale in medication (M) and stimulation (S) "on"/"off" states. With DBS at 9 months, the score improved by 86% to 8.5 in M-"on"/S-"on" and 12.5 in M-"off"/S-"on." Studies of the effects of DBS and concomitant medication may be warranted in selected patients treated by DBS for dystonia.

Detection of delayed focal MR changes in the lateral hippocampus in transient global amnesia.

BACKGROUND: There is still limited knowledge on the location and etiology of transient global amnesia (TGA). MR studies including diffusion-weighted imaging (DWI) have been unable to demonstrate consistently the location and underlying pathology of TGA. OBJECTIVE: To investigate patients with TGA using serial DWI performed from the day of symptom onset through days 1 and 2. METHODS: After reporting negative DWI results in a previous study, the authors used a modified study design to investigate patients with TGA using serial DWI performed from the day of symptom onset through days 1 and 2. RESULTS: Of 31 consecutive patients studied, 26 developed a small, punctate DWI lesion in the lateral aspect of the hippocampal formation (pes and fimbria hippocampi) on either side (left, n = 15; right, n = 6) or bilaterally (n = 5). Lesions were rarely noted in the hyperacute phase (n = 2), but all became visible regularly at 48 hours. CONCLUSIONS: The study confirms the involvement of hippocampal parenchyma in the pathophysiology of TGA. The delayed detectability of the lesions may explain the incongruence of previous MR DWI studies in TGA patients.

[Glioblastoma multiforme as a manifestation of Turcot syndrome]. / Glioblastoma multiforme als Manifestation des Turcot-Syndroms.

Turcot's syndrome, clinically characterized by the coincident occurrence of primary tumors of the colon and the central nervous system, can genetically be divided into two syndromes: familial adenomatous polyposis (FAP) and hereditary nonpolyposis colon carcinoma (HNPCC). In the present case, a 60-year-old patient with glioblastoma multiforme and a history of hereditary malignomas is described as an example of a HNPCC-associated Turcot's syndrome. New molecular biological methods and results give deeper insight into clinical syndromes, and the better understanding improves diagnostics, therapy, and outcome estimations, even in rare diseases. In the present case, a new germinal mutation could be identified.