Initiation of first disease-modifying treatment for multiple sclerosis patients in the Czech republic from 2013 to 2016: Data from the national registry ReMuS.

BACKGROUND: Proper management of multiple sclerosis (MS) requires feedback from clinical practice via registries. OBJECTIVE: To introduce the Czech national multiple sclerosis registry, ReMuS, and explore the availability and use of disease-modifying drugs (DMD). METHODS: The analysis focused on patients who started their first DMD, either with first-line or second-line medication and was based on reimbursement criteria set by Czech regulators. Baseline information was used to predict relapses after DMD initiation and to compare patients that started DMD in different years. RESULTS: A total of 3,328 patients started DMD treatment for MS between 2013 and 2016; 3,203 on first-line and 125 on second-line medication. The proportion of patients starting on second-line drugs increased from 1.8% in 2013 to 4.7% in 2016. The occurrence of a relapse within one year of DMD initiation was significantly related to (1) the Expanded Disability Status Scale (EDSS) score immediately prior to starting DMD and (2) the number of previous relapses. Both parameters were significantly lower in patients starting in later years of the explored interval. CONCLUSION: Data from the ReMuS registry highlights improvements made in the management of MS in the Czech Republic. However, a relatively low percentage of patients started treatment using second-line drugs, in contrast to trends in other countries.

Safety and efficacy of mechanical thrombectomy with stent-retrievers in anticoagulated patients with anterior circulation stroke.

AIM: To assess the safety and efficacy of mechanical thrombectomy (MT) with stent-retrievers in anterior circulation stroke (ACS) patients due to the occlusion of major cerebral arteries, and to compare the results achieved in patients on oral anticoagulation (OAC) and those not on OAC. MATERIALS AND METHODS: The present retrospective study comprised 285 consecutive patients (115 males; mean age 74±13 years). The following data were collected: baseline characteristics, occurrence of risk factors, pre-event treatment with OAC, neurological deficit at the time of treatment, time to therapy, recanalisation rate (successful recanalisation defined as Thrombolysis in Cerebral Infarction score ≥2b), post-treatment imaging findings. The 90-day clinical outcome was assessed using modified Rankin scale (good outcome defined as 0-2). RESULTS: The following statistically insignificant differences were found in 26 patients on OAC versus 259 patients without OAC: occurrence of symptomatic intracerebral haemorrhage 7.7% versus 8.1%, achievement of successful recanalisation 69.2% versus 82.6%, good 90-day clinical outcome 34.6% versus 56.8%, 90-day mortality 26.9% versus 20.8% (p>0.05 in all cases). Age and neurological deficit at the time of treatment were identified as independent negative predictors of good 90-day clinical outcome (odds ratio [OR]=0.90, 95% confidence interval [CI]: 0.88-0.94 and OR=0.83, 95% CI: 0.77-0.90, respectively) and as independent positive predictors of mortality (OR=1.12, 95% CI: 1.06-1.18 and, OR=1.17, 95% CI: 1.07-1.27, respectively; p<0.05 in all cases). CONCLUSIONS: MT with stent-retrievers is safe and effective in ACS patients on OAC.

Permutation Entropy and Signal Energy Increase the Accuracy of Neuropathic Change Detection in Needle EMG.

Background and Objective. Needle electromyography can be used to detect the number of changes and morphological changes in motor unit potentials of patients with axonal neuropathy. General mathematical methods of pattern recognition and signal analysis were applied to recognize neuropathic changes. This study validates the possibility of extending and refining turns-amplitude analysis using permutation entropy and signal energy. Methods. In this study, we examined needle electromyography in 40 neuropathic individuals and 40 controls. The number of turns, amplitude between turns, signal energy, and "permutation entropy" were used as features for support vector machine classification. Results. The obtained results proved the superior classification performance of the combinations of all of the above-mentioned features compared to the combinations of fewer features. The lowest accuracy from the tested combinations of features had peak-ratio analysis. Conclusion. Using the combination of permutation entropy with signal energy, number of turns and mean amplitude in SVM classification can be used to refine the diagnosis of polyneuropathies examined by needle electromyography.

Age delays the recovery of distal motor latency after carpal tunnel syndrome surgery.

BACKGROUND: Studies have shown slow healing of peripheral nerve injury in elderly patients. Carpal tunnel syndrome (CTS) is the most frequent compressive mononeuropathy, affecting mostly older people and females. Few studies have assessed electrophysiological differences between younger and older patients. We aimed to evaluate age-dependent differences in electrophysiological parameters preoperatively and postoperatively over a 100-day postoperative period. METHOD: This retrospective study included 258 hands of patients who underwent conventional open-technique carpal tunnel syndrome surgery. Patients with paresthesia in the median nerve distribution or with impaired sensation or abnormal findings in sensory and motor median nerve conduction studies were enrolled. The age dependence of the preoperative values of distal motor latency, amplitude of the compound motor action potential and sensory conduction velocity was estimated using regression analysis. RESULTS: Statistically significant age dependence was found for the preoperative distal motor latency, compound motor action potential, amplitude and sensory conduction velocity. The repair of segmental demyelination was nearly twice as slow in the older group, at a 5 % significance level, even when comparing groups with the same preoperative distal motor latency. CONCLUSIONS: Analysis of preoperative nerve conduction parameters indicates that surgery for carpal tunnel syndrome is performed later in older patients.

Effect of donepezil in Alzheimer disease can be measured by a computerized human analog of the Morris water maze.

BACKGROUND: Drug development for Alzheimer disease (AD) is challenged by the success in animal models tested in the Morris water maze (MWM) and the subsequent failures to meet primary outcome measures in phase II or III clinical trials in patients. The human variant of MWM (hMWM) enables us to examine allocentric and egocentric navigation as in the MWM. OBJECTIVE: It was the aim of this study to examine the utility of a computerized hMWM to assess the effects of donepezil in mild AD. METHODS: Donepezil 5 mg/day was started after initial hMWM testing in the treated group (n = 12), and after 28 days, the dose was increased to 10 mg/day. The performance after 3 months was compared to that of a non-treated group (n = 12). RESULTS: Donepezil stabilized or improved the spatial navigation performance after 3 months, especially in the allocentric delayed recall subtask (p = 0.014). CONCLUSIONS: The computerized hMWM has the potential to measure the effects of donepezil in mild AD. It is a sensitive cognitive outcome measure in AD clinical trials.

Malignant schwannoma of the obturator nerve.

Lesions of obturator nerve are rare. Tumours and mainly malignant schwannoma of this nerve are extremely rare. The authors describe an unusual case of a gigantic schwannoma of the obturator nerve in 69 year old woman. Due to tumour expansion in the proximal part of the thigh MRI was performed and demonstrated extensive tumour originating most probably from the obturator nerve. The patient had no neurological symptoms. Biopsy from the lesion was taken at the Department of Orthopaedics with the following conclusion: malignant schwannoma. The patient received neoadjuvant chemotherapy due to diffuse metastatic spread on the chest X ray, after which metastatic spread subsided. The main lesion reduced its size by 1 cm. In 4 months after biopsy the patient was referred for operation to neurosurgery. The tumour was removed along its borders and except of minimal weakness of adduction of the right thigh there was no neurological deterioration. She was subsequently referred for further care to oncology and radiotherapy.The goal of this work is to emphasize the extremely rare occurrence of tumours of this nerve and suggest therapeutic options (Fig. 4, Ref. 11).

Visual evoked potentials to pattern, motion and cognitive stimuli in Alzheimer's disease.

The aim of our study was to verify reported visual dysfunctions of patients with Alzheimer disease with the use of several variants of VEPs and visual ERPs and to learn whether these methods can be useful in diagnostics of AD. We tested 15 patients (6 women and 9 men, aged from 58 to 87) with mild to moderate Alzheimer disease (12-23 points of Mini Mental State Examination) and 15 age, gender and education level matched controls. The examination consisted of VEPs to pattern-reversal and motion-onset stimulation (to translational and radial movement) and of visual ERPs recorded during an odd-ball test. The subjects were instructed to signalize target stimuli by pressing of a button, which enabled to evaluate also the reaction time. While pattern-reversal VEPs were comparable in patients and controls, there were significantly smaller N2 peak amplitudes of motion-onset VEPs in patients with AD (in particular in radial moving stimuli outside the central 20 deg of the visual field), which suggests a dysfunction of the motion-processing (magnocellular) system or the dorsal cortical stream. ERPs, having significantly longer latencies in patients than in controls, distinguished well both groups. However, the individual AD diagnostics based on ERPs seems to be limited by rather high inter-individual variability of the ERP latencies. The ERPs might, however, be useful in disease progress and therapy effect estimation. Electrophysiological parameters did not correlate with neuropsychological ADAS cog test (Alzheimer Disease Assessment Scale--cognitive part).

[Total hip arthroplasty after femoral neck fracture in patients with acquired neurological deficit]. / Implantace totální endoprotézy kycelního kloubu pro zlomeninu krcku femuru u pacientu se získaným neurologickým deficitem.

PURPOSE OF THE STUDY: To evaluate the outcome of total hip arthroplasty (THA) performed for femoral neck fracture in patients with a paralytic hip. MATERIAL AND METHODS: Between 1997 and 2004, total hip arthroplasty was performed in 301 patients with a medio-cervical or sub-capital fracture of the femoral neck. In 17 of them there was also neurological deficit due to a stroke in 11, multiple sclerosis in four, and conditions following cranial injury in two. The degree of neurological deficit was assessed pre-operatively and its effect on the outcome was evaluated, in particular that on the overall results of THA, implant stability and post-operative complications. RESULTS: At an average follow-up of 61 months, 15 out of the 17 patients were examined, because one died and one failed to turn up. Four patients had a dislocation and two had a post-operative infection and para-articular ossification. Seven patients could walk with the use of walking aids, eight had serious difficulties or were in a wheelchair. DISCUSSION: The incidence of post-operative complications is much more frequent in patients with neurological deficit than in the other THA patients. Some possibilities to improve the prospects of neurologically affected patients are discussed, such as fall prevention, rational drug therapy, etc. Options for operative techniques and implant use are also dealt with as well as post-operative care to prevent complications. CONCLUSIONS: In patients with medio-cervical or sub-capital fracture of the femoral neck and co-existent neurological deficit, operative considerations should also involve further surgical techniques, such as osteosynthesis or Girdlestone arthroplasty. To achieve a good outcome it is necessary to choose a correct operative procedure with an optimal combination of acetabular and femoral components, to perform additional periarticular surgery and to indicate post-operative comprehensive rehabilitation.

[Proportion of tau protein to phosphorylated tau protein CSF levels in differential diagnosis of dementia]. / Bedeutung der Gesamt-tau- und Phospho-tau-Protein-Liquorspiegel in der Demenzdiagnostik.

BACKGROUND: The diagnosis of Creutzfeldt-Jakob disease (CJD) is based on typical clinical features and can be supported by detection of 14-3-3 protein in the CSF. The present study suggests the importance of investigating this ratio of total tau protein to phosphorylated tau protein in differentiating CJD from other dementias. Thirty-one patients with Alzheimer's disease (AD) or frontotemporal dementia and four with definitive diagnoses of CJD were included in the study. METHODS AND MATERIAL: Results from baseline investigations were compared with those from an age-matched cognitively controlled group with Bell's palsy. Tau protein, phosphorylated tau protein, and beta amyloid were analyzed using a commercially available enzyme-linked immunosorbent assay; 14-3-3 protein was assessed by Western blotting. RESULTS AND CONCLUSION: A distinctly high proportion of total tau protein to phosphorylated tau protein in CSF was found in all patients diagnosed with CJD, even in those with negative 14-3-3 protein blot results. In contrast, marker analysis in patients with Alzheimer's dementia revealed the highest CSF ratio of beta amyloid to phosphorylated tau protein levels. These proteins are important diagnostic biomarkers for CJD, especially in patients with negative 14-3-3 protein findings.

Genetic variants of transferrin in cystic fibrosis.

BACKGROUND: Genetic polymorphism of serum transferrin (Tf) was studied in order to differentiate between protein genetic variants and congenital disorders of glycosylation (CDG), further focusing on unusual findings. METHODS: Screening of Tf hypoglycosylation was carried out by isoelectric focusing with direct immunofixation and Coomassie blue staining in 100 healthy controls and a group of 1247 patients with various symptoms and diagnoses. RESULTS: Of the seven different genotypes detected, a significantly higher (p = 0.004) frequency of Tf C1C2 was found among 92 patients with cystic fibrosis; only the most severe DF508 mutation (in either homozygous or heterozygous form) was regularly present in the carriers of this Tf genotype, in contrast to those with the Tf C1C1 variant. CONCLUSIONS: Association of Tf C2 allele with various malfunctions has been noticed before, but is so far unresolved. This is the a report on increased frequency of Tf C1C2 genotype found in cystic fibrosis. Analysis of larger samples and independent confirmation of our results are needed.