Lejla Paracka

Botulinum toxin in the management of chronic migraine: clinical evidence and experience:

Chronic migraine (CM) is a severely disabling neurological condition characterized by episodes of pulsating unilateral or bilateral headache. The United States Food and Drug Administration (FDA) approved onabotulinumtoxinA (Botox®) for the prophylactic treatment of CM in 2010. It has been shown that onabotulinumtoxinA is effective in the reduction of headache frequency and severity in patients with CM. Treatment is well tolerated by the patients. This review reports on the history of botulinum neurotoxin (BoNT) in CM and presents the current clinical evidence for the use of onabotulinumtoxinA in the treatment of CM.

Impaired Quality of Life and Need for Palliative Care in a German Cohort of Advanced Parkinson’s Disease Patients

Background Parkinson’s disease (PD) is the second most frequent neurodegenerative disease of the elderly. Patients suffer from various motor and non-motor symptoms leading to reduced health-related quality of life (HRQOL) and an increased mortality. Their loss of autonomy due to dementia, psychosis, depression, motor impairments, falls, and swallowing deficits defines a phase when palliative care interventions might help to sustain or even improve quality of life. Objective The aim of this study was to investigate the current status of palliative care implementation and quality of life in a local cohort of advanced PD patients in order to frame and improve future care. Methods 76 geriatric patients with advanced idiopathic PD meeting the inclusion criteria for palliative care interventions were clinically evaluated by neurological examination using Movement Disorders Society Unified Parkinson’s Disease Rating Scale, Barthel Index, Montreal Cognitive Assessment Test, and a structured interview concerning palliative care implementation. Results HRQOL is severely reduced in our cohort of geriatric advanced PD patients. We found motor deficits, impairment of activities of daily living, depression, and cognitive decline as most relevant factors determining decreased HRQOL. Only 2.6% of our patients reported present implementation of palliative care. By contrast, 72% of the patients indicated an unmet need for palliative care. Conclusion Quality of life is dramatically affected in advanced PD patients. However, we found palliative care to be implemented extremely rare in their treatment concept. Therefore, geriatric patients suffering from advanced PD should be enrolled for palliative care to provide adequate and holistic treatment which may improve or sustain their quality of life.

Platform Session – Electromyography: Large inter-rater variation on revised El Escorial and Awaji diagnostic criteria for amyotrophic lateral sclerosis

Introduction The revised El Escorial Criteria (rEEC) from 2000 and their amendment, the Awaji Criteria (AC) from 2008, are widely used diagnostic criteria for amyotrophic lateral sclerosis (ALS) and their sensitivity and specificity have been evaluated in several studies, however, reproducibility among different raters has not been studied. This study was undertaken to assess inter-rater agreement on rEEC and AC. Methods Eight experienced physicians from seven different countries independently classified 399 patients referred for ALS according to the rEEC and the AC. Clinical and electrophysiological data were presented in standardised forms. Agreement was evaluated by Kappa coefficients. Diagnostic sensitivity and specificity were calculated from the majority diagnosis of the eight physicians for 349 cases with follow-up data. Results Kappa coefficients differed among the diagnostic categories for rEEC (“Definite”: 0.50, CI:0.39–0.60, “Probable”: 0.36, CI:0.31–0.41, “Probable Laboratory Supported”: 0.25,CI:0.18–0.34, “Possible”: 0.14, CI:0.09–0.20, and “Not-ALS”: 0.59, CI:0.51–0.65) as well as for AC (“Definite”: 0.44, CI:0.37–0.51, “Probable”: 0.34, CI:0.29–0.39, “Possible”: 0.33, CI:0.26–0.40, and “Not-ALS”: 0.65, CI:0.58–0.72). The only significant difference in agreement between rEEC and AC was for the category “Possible”. A large fraction of cases classified as “Probable” using the rEEC were upgraded to “Definite” using the AC, however, many cases classified as “Probable Laboratory Supported” by rEEC were downgraded to “Possible” by the AC. Sensitivity for “Definite/Probable” versus “Possible/Not-ALS” did not differ between rEEC (0.64, CI:0.58–0.69) and AC (0.63, CI:0.58–0.69). Similarly, there was no difference in specificity between rEEC (0.91, CI:0.74–0.98) and AC (0.85, CI:0.67–0.94). Conclusion There is a large inter-rater variation on rEEC and AC most pronounced for the “Probable Laboratory Supported” and “Possible” categories. The sensitivity was rather low compared to other studies and was not significantly increased using AC, probably due to omission of the “Probable Laboratory Supported” category. However, in trials including patients with possible disease AC may be advantageous. Inter-rater variation may be due to (1) a high complexity of the rEEC, which is also inherited in the AC, including definition of four body regions, interpretation of clinical findings (upper/lower motor neuron signs), significance of rostral/caudal signs, and significance of EMG findings (acute/chronic signs), and (2) the need to apply rEEC together with AC. However, pre-study training and quality control at individual laboratories may reduce variation and improve sensitivity and specificity. The results indicate that there is a need for initiatives to develop more simple and reproducible criteria.

Sensory Alterations in Patients with Isolated Idiopathic Dystonia: An Exploratory Quantitative Sensory Testing Analysis

Abnormalities in the somatosensory system are increasingly being recognized in patients with dystonia. The aim of this study was to investigate whether sensory abnormalities are confined to the dystonic body segments or whether there is a wider involvement in patients with idiopathic dystonia. For this purpose, we recruited 20 patients, 8 had generalized, 5 had segmental dystonia with upper extremity involvement, and 7 had cervical dystonia. In total, there were 13 patients with upper extremity involvement. We used Quantitative Sensory Testing (QST) at the back of the hand in all patients and at the shoulder in patients with cervical dystonia. The main finding on the hand QST was impaired cold detection threshold (CDT), dynamic mechanical allodynia (DMA), and thermal sensory limen (TSL). The alterations were present on both hands, but more pronounced on the side more affected with dystonia. Patients with cervical dystonia showed a reduced CDT and hot detection threshold (HDT), enhanced TSL and DMA at the back of the hand, whereas the shoulder QST only revealed increased cold pain threshold and DMA. In summary, QST clearly shows distinct sensory abnormalities in patients with idiopathic dystonia, which may also manifest in body regions without evident dystonia. Further studies with larger groups of dystonia patients are needed to prove the consistency of these findings.

O96 Large inter- and intra-rater variation on diagnostic criteria for amyotrophic lateral sclerosis

Objectives To study inter- and intra-rater agreement on diagnostic criteria for amyotrophic lateral sclerosis (ALS). Methods Four-hundred-and-two patients referred for ALS were classified according to the revised El Escorial criteria (EEC) and the Awaji criteria (AwC) independently by eight experienced physicians from seven different countries. Intra-rater agreement was further assessed from 352 of the cases, which were interpreted twice by the same physician. Clinical and electrophysiological data were presented in standardised forms. Results Two-by-two Kappa coefficients among the eight physicians varied from 0.15 to 0.75 (mean 0.40) for EEC and from 0.18 to 0.80 (mean 0.43) for AwC. Kappa coefficients for intra-rater agreement varied from 0.08 to 0.81 (mean 0.56) for EEC and from 0.18 to 0.85 (mean 0.67) for AwC. Disagreement was seen on all diagnostic categories, most pronounced for “probable lab-supported” and “possible”. There were no differences between EEC and AwC. Discussion The large variation on EEC and AwC may be due to the complexity of the criteria including the definition of four body regions, interpretation and significance of clinical findings of upper or lower motor neuron signs, significance of findings being rostral or caudal to each other, and interpretation of detailed electrophysiological findings as evidence of acute or chronic denervation. Conclusions The large variation in interpretation of both EEC and AwC may be considered unacceptable for criteria used to include patients in research studies. Significance Variability on interpretation of diagnostic criteria for ALS should be considered in the assessment of scientific studies. Initiatives to develop more reproducible diagnostic criteria for ALS are needed.

P 78 Involvement of the subthalamic nucleus in the maintenance of cognitive flexibility – Evidence from local field potential recordings

Functional imaging techniques have revealed that several cortical brain regions like the prefrontal cortex, the anterior cingulate cortex, and the posterior parietal cortex are involved in the maintenance of cognitive flexibility. However, the role of basal ganglia in the ability to switch between several concepts remains largely unknown. To address this issue further we recorded local field potentials via DBS electrodes from the nucleus subthalamicus (STN) while participants performed a task that required executive processes like planning and set shifting. Seven patients with Parkinson disease were included in the study. The patients performed a computerized version of the Wisconsin Card Sorting Test. The participants were required to match the cards according to three possible matching rules (color, shape and number). After the sorting choice was made, a feedback “switch” or “repeat” indicated that the rule had to be changed or repeated respectively. “Integration cue” as a measure of shifting rule with three levels of complexity was compared to “repeat cue” as a measure of keeping the same rule as before. The results show that the amplitudes of the integration cue are larger than those of the repeat cue. Integration condition induced oscillatory changes with the highest power in the theta band in comparison to the repeat condition. Moreover, there is a significant late higher activation of beta band for the integration trials. In conclusion, there are broadband oscillatory changes in the STN during executive processes. STN appears to be involved in shifting cognitive sets and modulating responses.