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Purpose: The purpose of this study was to evaluate retrospectively the efficacy and tolerability of retigabine (RTG) in residential patients of an epilepsy center., Method: We used an industry-independent noninterventional retrospective evaluation on the basis of paper and electronic records plus interrogation of the treating neurologists. All patients (N=20; 7 females; mean age: 31.8, range: 18-54years) started on RTG between May 2011 and March 2012 were included. Evaluation was carried out after 6, 12, and 24months. Changes in seizure frequency were measured as the number of seizures during three months on RTG compared with a three-month baseline period. The Clinical Global Impression scale was applied to include qualitative changes in seizure severity. All but one patient had symptomatic (structural; one patient: metabolic) or cryptogenic focal or multifocal epilepsy. All had grade III drug-resistant epilepsy and cognitive deficits of different degrees., Results: The retention rates were 60% after 6months, 35% after 12months, and 20% after 24months. At 12months, there were 2 responders (10%): one had a >90% seizure reduction and the other had a >50% seizure reduction. Another 5 patients were still on RTG because of minor improvements. The reasons for discontinuation in 13 patients were adverse effects (6), lack of effect (6), and both (1). Cognitive or emotional changes were the side effects that most frequently led to discontinuation. Beyond the 12-month evaluation, 3 patients were discontinued as a consequence of the FDA warning regarding retinal pigmentation and discoloration of skin and nails in patients exposed to RTG. One patient had a moderate blue-gray finger coloring. Ophthalmological changes were not discovered., Conclusion: Retigabine proved to be useful only for a small minority of patients in a sample of patients with particularly difficult-to-treat epilepsy., (Copyright © 2015 Elsevier Inc. All rights reserved.)

In a retrospective evaluation of 32 inpatients with therapy-resistant epilepsy and intellectual disability, the efficacy of pregabalin (PGB) treatment was assessed after 6 and 12 months. The combined efficacy measure included the percentage reduction in seizure frequency, as well as the Clinical Global Impression (CGI) scale. Tolerability was assessed using a list of the 10 adverse effects most frequently observed in the regulatory studies and also by the CGI scale. After 6 months, the retention rate was 75%. Six patients (18.75%) were responders (50% seizure reduction and/or "good" or "very good" effect on CGI). No patient was seizure free. Seven patients had adverse effects that were not impairing. Eight patients had side effects that were essentially impairing. Weight gain, somnolence, asthenia, and ataxia were the most frequent adverse effects. Rare adverse events were severe mental slowing and loss of daily life capacities on a low dose of PGB in one patient and increase in auto-aggression in another patient. After 12 months, the retention rate was 40.6%, the responder rate was 25%, and one patient was seizure free. Statistical analysis did not identify any predictor of outcome (seizure type, epilepsy syndrome, co-medication, degree of intellectual disability). In this highly selected population, the efficacy of PGB was only moderate.

Background/objective: Our purpose was to evaluate the utility of a brief screening cardiac ultrasonographic (SCU) examination. We prospectively compared the SCU with conventional clinical evaluation in 124 emergency department (ED) patients with suspected cardiac disease. Furthermore, we assessed the impact and quality of SCU examinations as obtained by briefly trained ED personnel (EP)., Methods: Patients underwent clinical evaluation by an ED physician and SCU examination by a sonographer or cardiologist. Patient disposition, hospital stay length, and the number of full echo examinations were compared with the presence of significant findings on SCU. In patients who received a full echocardiogram during hospitalization, results of the initial clinical examination were compared with results of the SCU examination in the diagnosis of significant findings. A similar analysis, but with quality assessment, was performed on only those SCU examinations acquired by 4 EP., Results: Of the 124 patients enrolled in the main study, 40 of 124 (32%) had significant findings on SCU. Of patients with abnormal SCUs versus normal SCUs, 16 of 40 (40%) versus 18 of 84 (21%) had hospital stay lengths >2 days (P < or =.05). Using the 36 inpatient full echo studies obtained for standard indications during hospitalization as a gold standard, initial clinical examination identified only 7 of 30 (23%) significant findings and had 16 false-positive diagnoses, whereas SCU identified 22 of 30 (73%) with 8 false positives. Although similar study results occurred with interpretation of 68 SCUs obtained by EP, quality was achieved in only 55% ED personnel versus 97% of sonographer-obtained SCUs (P <.05)., Conclusions: An SCU examination detects significant findings misdiagnosed on initial clinical evaluation in the ED and provides prognostic data regarding length of hospital stay.

The Kennedy-Syndrome is a X-linked recessive bulbospinal muscular atrophy, in some cases associated with endocrinological disturbances such as androgen resistance and diabetes mellitus. The age of onset is usually between 20 and 40. Presenting symptoms are proximal flaccid weakness, fasciculations, cramps or tremor. Disease progression is usually slow and live expectancy is normal. It is important to distinguish the Kennedy-Syndrome from amyotrophic lateral sclerosis, spinal muscular atrophy, muscular dystrophies and other types of motor neuron disease. Kennedy disease is caused by an expanded trinucleotide repeat in the androgen receptor gene. Genetic analysis allows a precise-diagnosis on an individual basis and reliable genetic counselling. An effective medical treatment does not yet exist.