Intrathecal baclofen for dystonia of complex regional pain syndrome.

Dystonia in complex regional pain syndrome (CRPS) responds poorly to treatment. Intrathecal baclofen (ITB) may improve this type of dystonia, but information on its efficacy and safety is limited. A single-blind, placebo-run-in, dose-escalation study was carried out in 42 CRPS patients to evaluate whether dystonia responds to ITB. Thirty-six of the 38 patients, who met the responder criteria received a pump for continuous ITB administration, and were followed up for 12 months to assess long-term efficacy and safety (open-label study). Primary outcome measures were global dystonia severity (both studies) and dystonia-related functional limitations (open-label study). The dose-escalation study showed a dose-effect of baclofen on dystonia severity in 31 patients in doses up to 450 microg/day. One patient did not respond to treatment in the dose-escalation study and three patients dropped out. Thirty-six patients entered the open-label study. Intention-to-treat analysis revealed a substantial improvement in patient and assessor-rated dystonia scores, pain, disability and quality-of-life (Qol) at 12 months. The response in the dose-escalation study did not predict the response to ITB in the open-label study. Eighty-nine adverse events occurred in 26 patients and were related to baclofen (n=19), pump/catheter system defects (n=52), or could not be specified (n=18). The pump was explanted in six patients during the follow-up phase. Dystonia, pain, disability and Qol all improved on ITB and remained efficacious over a period of one year. However, ITB is associated with a high complication rate in this patient group, and methods to improve patient selection and catheter-pump integrity are warranted.

Feasibility and reliability of on-line automated microemboli detection after carotid endarterectomy. A transcranial Doppler study.

OBJECTIVES: recently, a new algorithm for transcranial Doppler (TCD) ultrasound detection of microembolic signals (MES) was developed. In the present study, we investigated its on-line performance in TCD monitoring after carotid endarterectomy (CEA) and assessed off-line its accuracy in detecting MES. MATERIALS AND METHODS: first, the feasibility of MES detection in TCD monitoring after CEA in a routine clinical setting was evaluated in 50 patients. Second, to test the reliability of the software a 2-h digital audio study tape was made and analysed by the algorithm and five human experts. The "gold standard" was defined as the agreement between human experts: a MES was considered to be present if at least three human observers agreed. RESULTS: TCD monitoring for emboli detection after CEA was well tolerated by the patients and could be performed reliably. In the study tape, the human gold standard detected 107 MES, with 93 MES having an intensity of > or =7 dB. The software detected 81 and 77 MES, respectively. Using the 7 dB intensity threshold, the software had no false positives and 16 false negatives. The kappa value between the human gold standard and the software was 0.91, the proportion of specific agreement was 0.83. CONCLUSIONS: the tested algorithm provides a reliable method for automated on-line microemboli detection after CEA. This makes monitoring of the effectiveness of antiplatelet agents in the prevention of stroke after CEA more practicable.

[A child with peculiar movements: Sydenham chorea]. / Een kind met merkwaardige bewegingen: chorea van Sydenham.

An 11-year-old girl with Sydenham chorea presented with a rapid onset of serious restlessness of mainly the right side of the body. Additional laboratory investigations revealed no abnormalities, yet this is not unusual for such cases. Valproic acid and pimozide were then successively prescribed because of the chorea. For secondary prevention she received long-term oral penicillin. Sydenham chorea is a manifestation of rheumatic fever and occurs after a throat infection by group A streptococci. The disease is characteristic and consists of a combination of choreic movements, hypotonia and emotional lability. The clinical course is diverse. Improvement usually occurs over a period of several months, although a significant proportion of patients exhibit little recovery.

Coagulation disorders in young adults with acute cerebral ischaemia.

We analysed the results of coagulation studies in an unselected series of young adults with acute cerebral ischaemia. Our aims were (a) to determine the prevalence of coagulation disorders among these patients, (b) to investigate the relation between the presence of coagulation abnormalities and large vessel disease or potential sources of cardiac embolism and (c) to evaluate the occurrence of thrombotic events in patients with or without coagulation disorders. One hundred and twenty consecutively admitted patients (53 men, 67 women, median age 38 years, range 15-45) who presented with acute cerebral infarction (n = 89) or a transient ischaemic attack (n = 31) were evaluated. Diagnostic studies consisted of electrocardiography, echocardiography, duplex scanning, and/or angiography. Coagulation studies included activity tests of protein S, protein C, antithrombin, plasminogen, measurement of immunoglobulin G (IgG) anticardiolipin antibodies (ACLA), and a dilute prothrombin assay. Initially, 30 patients had increased ACLA titres and 28 had an abnormal dilute prothrombin assay, suggesting lupus anticoagulant. Decreased protein S, protein C and antithrombin activity were detected in 20, 3 and 3 patients, respectively, excluding patients in whom the abnormalities could be explained by the use of medication, by pregnancy or puerperium. We detected a decreased activity of plasminogen in 5 patients. The disorders could be confirmed by a second assessment in only 2 patients with a protein S deficiency, in none of the patients with a protein C or antithrombin deficiency and in 1 patient with plasminogen deficiency. However, the abnormalities persisted in 19 of 21 patients with increased anticardiolipin IgG titres and in 9 of 20 patients with lupus anticoagulant. A confirmed coagulation disorder was not associated with stroke type or vascular risk factors, but it was more common among patients with large vessel disease (odds ratio: 3.8, 95% confidence interval (CI): 1.1-12.8). Sixteen patients had a recurrent thromboembolic event, but the risk of recurrence was not increased among patients with a confirmed coagulation disorder. Our results suggest that idiopathic coagulation disorders are found in about a quarter of young stroke patients. They are difficult to predict and probably interact with other risk factors.