Ultrasound guidance increases the success rate of peripheral i.v. cannulation, especially in patients with known or predicted difficult i.v. access.

Treatment of motor symptoms of degenerative cerebellar ataxia remains difficult. Yet there are recent developments that are likely to lead to significant improvements in the future. Most desirable would be a causative treatment of the underlying cerebellar disease. This is currently available only for a very small subset of cerebellar ataxias with known metabolic dysfunction. However, increasing knowledge of the pathophysiology of hereditary ataxia should lead to an increasing number of medically sensible drug trials. In this paper, data from recent drug trials in patients with recessive and dominant cerebellar ataxias will be summarized. There is consensus that up to date, no medication has been proven effective. Aminopyridines and acetazolamide are the only exception, which are beneficial in patients with episodic ataxia type 2. Aminopyridines are also effective in a subset of patients presenting with downbeat nystagmus. As such, all authors agreed that the mainstays of treatment of degenerative cerebellar ataxia are currently physiotherapy, occupational therapy, and speech therapy. For many years, well-controlled rehabilitation studies in patients with cerebellar ataxia were lacking. Data of recently published studies show that coordinative training improves motor function in both adult and juvenile patients with cerebellar degeneration. Given the well-known contribution of the cerebellum to motor learning, possible mechanisms underlying improvement will be outlined. There is consensus that evidence-based guidelines for the physiotherapy of degenerative cerebellar ataxia need to be developed. Future developments in physiotherapeutical interventions will be discussed including application of non-invasive brain stimulation.

Objective:The therapeutic effects of 4-aminopyridine (4AP) were investigated in a randomized, double-blind, crossover trial in 10 subjects with familial episodic ataxia with nystagmus.Methods: After randomization, placebo or 4AP (5 mg 3 times daily) was administered for 2 3-month-long treatment periods separated by a 1-month-long washout period. The primary outcome measure was the number of ataxia attacks per month; the secondary outcome measures were the attack duration and patient-reported quality of life (Vestibular Disorders Activities of Daily Living Scale [VDADL]). Nonparametric tests and a random-effects model were used for statistical analysis. Results:The diagnosis of episodic ataxia type 2 (EA2) was genetically confirmed in 7 subjects.Patients receiving placebo had a median monthly attack frequency of 6.50, whereas patients taking 4AP had a frequency of 1.65 (p ϭ 0.03). Median monthly attack duration decreased from 13.65 hours with placebo to 4.45 hours with 4AP (p ϭ 0.08). The VDADL score decreased from 6.00 to 1.50 (p ϭ 0.02). 4AP was well-tolerated.Conclusions: This controlled trial on EA2 and familial episodic ataxia with nystagmus demonstrated that 4AP decreases attack frequency and improves quality of life. Level of evidence:This crossover study provides Class II evidence that 4AP decreases attack frequency and improves the patient-reported quality of life in patients with episodic ataxia and related familial ataxias. Neurology Episodic ataxia type 2 (EA2) is a rare autosomal dominant hereditary disorder caused by heterozygous mutations of the gene CACNA1A on chromosome 19p13.1 The carbonic anhydrase inhibitor acetazolamide has been the drug of first choice for the preventive treatment of episodic ataxia (EA) and especially EA2 (doses of 250 -1,000 mg/day), 2,3 because of the serendipitous discovery of its dramatic impact. 4 Its efficacy, however, has never been proven in a randomized controlled trial. 5,6 Acetazolamide effectively prevents or attenuates the attacks in approximately 50%-75% of all patients with EA2. 7 Clinical experience, however, shows that many patients stop this treatment in the long run because they develop adverse effects or are no longer responsive. 5,6 Furthermore, the adverse effects of acetazolamide (such as nephrocalcinosis, hyperhidrosis, paresthesia, muscle stiffening with easy fatigability, and gastrointestinal disturbances) limit its usage. 6 In view of the need to identify an alternative treatment option to acetazolamide and on the basis of pilot studies in subjects with downbeat nystagmus 8 and EA 9 as well as findings from animal studies, 10,11 we conducted a prospective randomized, doubleblind, placebo-controlled crossover study of 4AP in familial EA with nystagmus (the majority

Deep brain stimulation (DBS) of the subthalamic nucleus (STN) has been shown to be an effective treatment for Parkinson's disease (PD). The intraoperative positioning of DBS electrodes and postoperative adjustment of the stimulation parameters, however, require continuous, precise evaluation. Moreover, ambulatory measurements of the symptoms would also help to evaluate changes in the progression of PD in these patients. To this aim, we objectified rigidity measurements via surface EMG recordings of the Mm. biceps (bic) and triceps brachii (tric) in patients treated with chronic stimulation of the STN. We show that cessation and initiation of DBS have effects on the EMG profile during standardized extension and flexion movements in the elbow joint. These data correlate significantly with clinical ratings. Thus, EMG recordings of the Mm. bic and tric during this standardized extension-flexion movement can be used to objectively measure rigidity and to monitor its course over time. In view of its low technical requirements, this technique lends itself to use during DBS implantation surgery and in the clinical environment.