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1.
Orphanet J Rare Dis ; 17(1): 301, 2022 07 29.
Article in English | MEDLINE | ID: mdl-35906604

ABSTRACT

BACKGROUND: SPG11-linked hereditary spastic paraplegia is characterized by multisystem neurodegeneration leading to a complex clinical and yet incurable phenotype of progressive spasticity and weakness. Severe cognitive symptoms are present in the majority of SPG11 patients, but a systematic and multidimensional analysis of the neuropsychological phenotype in a larger cohort is lacking. While thinning of the corpus callosum is a well-known structural hallmark observed in SPG11 patients, the neuroanatomical pattern of cortical degeneration is less understood. We here aimed to integrate neuropsychological and brain morphometric measures in SPG11. METHODS: We examined the neuropsychological profile in 16 SPG11 patients using a defined neuropsychological testing battery. Long-term follow up testing was performed in 7 patients. Cortical and subcortical degeneration was analyzed using an approved, artificial intelligence based magnetic resonance imaging brain morphometry, comparing patients to established reference values and to matched controls. RESULTS: In SPG11 patients, verbal fluency and memory as well as frontal-executive functions were severely impaired. Later disease stages were associated with a global pattern of impairments. Interestingly, reaction times correlated significantly with disease progression. Brain morphometry showed a significant reduction of cortical and subcortical parenchymal volume following a rostro-caudal gradient in SPG11. Whereas performance in memory tasks correlated with white matter damage, verbal fluency measures showed strong associations with frontal and parietal cortical volumes. CONCLUSIONS: The present data will help define neuropsychological and imaging read out parameters in early as well as in advanced clinical stages for future interventional trials in SPG11.


Subject(s)
Spastic Paraplegia, Hereditary , Artificial Intelligence , Humans , Magnetic Resonance Imaging/methods , Mutation , Neuropsychology , Proteins/genetics , Spastic Paraplegia, Hereditary/diagnostic imaging , Spastic Paraplegia, Hereditary/genetics , Spastic Paraplegia, Hereditary/pathology
2.
J Parkinsons Dis ; 11(4): 2035-2045, 2021.
Article in English | MEDLINE | ID: mdl-34366379

ABSTRACT

BACKGROUND: There is growing interest in non-motor symptoms in Parkinson's disease (PD), due to the impact on quality of life. Anhedonia, the inability to experience joy and lust, has a prevalence of up to 46% in PD. The perception of pleasantness of an odor is reduced in anhedonia without PD. We previously showed a reduced hedonic olfactory perception in PD, i.e., patients evaluated odors as less pleasant or unpleasant compared to controls. This deficit correlated with anhedonia. OBJECTIVE: We aimed to confirm these findings. Moreover, we hypothesized that the perception of pleasantness in PD is affected on a multisensory level and correlates with anhedonia. Therefore, we assessed olfactory, visual and acoustic evaluation of pleasantness in PD and healthy individuals. METHODS: Participants had to rate the pleasantness of 22 odors, pictures, and sounds on a nine-point Likert scale. Depression, anhedonia, and apathy were assessed by means of questionnaires. Results of the pleasantness-rating were compared between groups and correlated to scores of the questionnaires. RESULTS: In particular pleasant and unpleasant stimuli across all three modalities are perceived less intense in PD, suggesting that a reduced range of perception of pleasantness is a multisensory phenomenon. However, only a reduction of visual hedonic perception correlated with anhedonia in PD. A correlation of reduced perception of pleasantness with apathy or depression was not present. CONCLUSION: We provide evidence for a multisensory deficit in the perception of pleasantness. Further studies should delineate the underlying neural circuity and the diagnostic value to detect neuropsychiatric symptoms in PD.


Subject(s)
Anhedonia , Olfactory Perception , Parkinson Disease , Humans , Parkinson Disease/complications , Quality of Life , Smell
3.
J Neurol Sci ; 419: 117169, 2020 Dec 15.
Article in English | MEDLINE | ID: mdl-33035867

ABSTRACT

Intravenous immunoglobulins (IVIg) represent an established cornerstone for the immunotherapy of chronic inflammatory demyelinating polyneuropathy (CIDP). Efficacy of IVIg for CIDP was proven in a large phase III trial. Yet, data on longer-term efficacy and effects in distinct subgroups are scarce. Our trial investigates the long-term efficacy of IVIg treatment in CIDP patients. In this observational real-world study, we retrospectively analyzed 49 CIDP patients receiving continuous IVIg treatment with a mean initial dosage of 87 g (1 g/kg body weight) every 4 weeks over a mean time of 45 months between 2010 and 2018. INCAT-Scores before the start of treatment and at the end of the observation period were compared. Over the observation period, IVIg treatment led to a median improvement of one INCAT score point. Subgroup analyses revealed a more pronounced improvement of INCAT scores in female CIDP patients, individuals with relapsing disease courses, patients with more pronounced motor impairment (higher initial INCAT scores) and in the cohort without need for concomitant other immunotherapies. These data argue for sustained beneficial effects of longer-term immunotherapy with IVIg in CIDP, particularly in females and relapsing disease forms with higher disease activity.


Subject(s)
Polyradiculoneuropathy, Chronic Inflammatory Demyelinating , Cohort Studies , Female , Humans , Immunoglobulins, Intravenous/therapeutic use , Immunologic Factors/therapeutic use , Polyradiculoneuropathy, Chronic Inflammatory Demyelinating/drug therapy , Retrospective Studies , Treatment Outcome
4.
J Neurol Sci ; 415: 116973, 2020 08 15.
Article in English | MEDLINE | ID: mdl-32563101

ABSTRACT

BACKGROUND: Pivotal trials showed good clinical efficiency of the monoclonal antibody ocrelizumab while being well tolerated and manageable in multiple sclerosis (MS). However, data on adverse events in everyday practice are scarce. Hence, our study aims at investigating short-term tolerability of ocrelizumab in a "real-world" setting. METHODS: In this retrospective cohort study, data of 128 (86 relapsing-remitting, 42 progressive) MS patients at initiation of ocrelizumab were analyzed at the MS center of the University of Erlangen, Germany. Additionally, follow-up data of 68 patients at 6-months retreatment were analyzed. Structured phone interviews were applied after ocrelizumab initiation to report undocumented side effects. RESULTS: Patients predominantly switched from monoclonal antibodies (46%), orals (20%), injectables (10%), steroids or immunosuppressants (each 8%), with a mean interval of 9.0 months after the last application of the previous immunotherapy. Applying a combined premedication with steroids, antihistamines and antipyretics for >90% of patients, ocrelizumab treatment was well tolerated and mainly comprised mild (n = 59/128 at initiation, n = 5/68 at 6 months retreatment) and rarely moderate (n = 7/128 at initiation, n = 2/68 at 6 months) side effects. Predominantly mild infusion related reactions (IRR) were reported with a declining percentage over the follow-up applications. Infections occurred rarely. No severe side effects were observed. Secondary, treatment appeared efficient when looking at clinical surrogates of stable disease. DISCUSSION: Our study delineates good short-term tolerability of ocrelizumab in a miscellaneous "real-world" MS cohort. Additional studies are warranted to confirm these beneficial findings and to reveal safety concerns in the longer-term follow-up.


Subject(s)
Multiple Sclerosis, Relapsing-Remitting , Multiple Sclerosis , Antibodies, Monoclonal, Humanized , Germany , Humans , Multiple Sclerosis/drug therapy , Multiple Sclerosis, Relapsing-Remitting/drug therapy , Retrospective Studies
5.
Neurobiol Aging ; 84: 241.e5-241.e11, 2019 12.
Article in English | MEDLINE | ID: mdl-31627977

ABSTRACT

We report the novel presenilin 1 (PSEN1) single amino acid deletion mutation F175del. Comprehensive clinical work-up, including cerebral MRI, FDG-PET, and CSF analysis, was performed in a male who had developed forgetfulness at the age of 39. Alzheimer's disease dementia was diagnosed according to established criteria. The index patient manifested rapid progressive dementia, seizures, and myoclonus, and a Pisa syndrome as a side effect of donepezil treatment. The PSEN1 mutation F175del was found on genetic testing. It was rendered very likely pathogenic as amyloid-ß (Aß) peptide 42 was elevated in a cell culture model compared to presenilin 1 wild-type controls. An additional, unusual increase in Aß39 indicates a rarely observed product line deviation in the generation of the shorter Aß species. Our observations extend the range of PSEN1 mutations to be considered in familial dementia. We demonstrate that deletion of a single conserved amino acid, which is very rare compared to missense mutations as the common cause for PSEN1-associated Alzheimer's disease, can lead to an unusual profile of Aß species.


Subject(s)
Alzheimer Disease/metabolism , Amyloid beta-Peptides/metabolism , Mutation , Presenilin-1/genetics , Alzheimer Disease/diagnostic imaging , Humans
6.
Neurocase ; 25(6): 243-250, 2019 12.
Article in English | MEDLINE | ID: mdl-31532322

ABSTRACT

We describe a patient with acute herpes simplex encephalitis with left-hemispheric hippocampal, parahippocampal and insular lesions. Although prototypic language areas were unaffected, the patient suffered from an inability to name objects or animals displayed on pictures. This deficit was transient and gradually disappeared 8 weeks after the initial diagnosis. Our findings are in line with a previous report showing similar deficits in a patient with a comparable lesion pattern and support the hypothesis that left insular lesions can produce severe naming deficits. Using FDG-PET we ruled out that functional deactivation in classical language areas account for the observed naming deficits.


Subject(s)
Cerebral Cortex/pathology , Encephalitis, Herpes Simplex/pathology , Encephalitis, Herpes Simplex/psychology , Mental Recall , Pattern Recognition, Visual , Adult , Aphasia/etiology , Attention , Encephalitis, Herpes Simplex/complications , Female , Hippocampus/pathology , Humans , Language , Neuropsychological Tests
7.
J Psychosom Res ; 122: 36-38, 2019 07.
Article in English | MEDLINE | ID: mdl-31126409

ABSTRACT

OBJECTIVE: We aimed to determine the prevalence of posttraumatic stress disorder (PTSD) 12 months after transient ischemic attack (TIA). METHOD: TIA patients of our previous investigation (examined 3 months after the event) were again examined 12 months after the diagnosis. PTSD and associated variables were assessed via self-rating instruments. RESULTS: Eighty-four patients were included in the analyses. Twelve months after TIA the prevalence of probable PTSD was reduced (8.3%) compared to that found 3 months after TIA (29.6%). Coping assessed 3 months after TIA predicted long-term PTSD severity. CONCLUSION: Although the prevalence of probable PTSD decreased 12 months after experiencing a TIA compared to 3 months after TIA, prevalence of probable PTSD is still increased relative to that in the general population.


Subject(s)
Ischemic Attack, Transient/complications , Stress Disorders, Post-Traumatic/etiology , Aged , Female , Follow-Up Studies , Humans , Male
8.
Mult Scler Relat Disord ; 27: 133-138, 2019 Jan.
Article in English | MEDLINE | ID: mdl-30384198

ABSTRACT

BACKGROUND: Over the last decade, therapy of relapsing remitting multiple sclerosis (RRMS) has evolved with the approval of several new treatment concepts. Thus, treatment goals have become more ambitious aiming at "no evidence of disease activity" (NEDA). As NEDA-3, this concept comprises freedom of clinical disease progression and relapses as well as inflammatory MRI activity. So far, data on NEDA status mainly stem from post-hoc analyses of drug approval studies. Yet, less is known about the significance of NEDA in "real-world" clinical settings. Hence, our study aims at investigation of NEDA in a heterogeneous cohort of relapsing MS patients. METHODS: This is a retrospective single-center study at the Department of Neurology of the University Hospital Erlangen, Germany, including data of 306 patients with relapsing forms of MS (RMS) or clinical isolated syndrome (CIS) from 2009 to 2016. Inclusion required sufficient clinical information and in house cranial MRI follow-up data sets at baseline and at follow-up after one year with a potential extension to two and three year follow-up, if possible. NEDA-3 status, its correlation to clinical features, associated medication and NEDA failure (EDA) were analyzed. RESULTS: In a cohort of RMS patients at the early stages of the disease (median EDSS 1.5, mean disease duration 30 months) at baseline, 45% retained NEDA-3 status after one year. This percentage decreased in year two (29%) and three (21%) of follow-up. MRI criteria were responsible for loss of NEDA status in 64% of cases and CIS patients were more likely to sustain NEDA status. Therapy with monoclonal antibodies appeared superior in sustaining NEDA status as compared to injectables or oral treatment options. DISCUSSION: In our real-world analysis, we confirm the potential of NEDA for the evaluation and surveillance of MS disease activity, progression and therapy efficacy. Despite highly efficient immunomodulatory treatment, NEDA-3 was only preserved in a minority of patients. Monoclonal antibodies may yield best NEDA rates. Further studies are warranted to evaluate the value of the NEDA concept in real-world settings beyond standardized clinical studies.


Subject(s)
Antibodies, Monoclonal/pharmacology , Disease Progression , Immunologic Factors/pharmacology , Multiple Sclerosis, Relapsing-Remitting , Adult , Female , Follow-Up Studies , Germany , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Multiple Sclerosis, Relapsing-Remitting/diagnostic imaging , Multiple Sclerosis, Relapsing-Remitting/drug therapy , Multiple Sclerosis, Relapsing-Remitting/physiopathology , Retrospective Studies , Severity of Illness Index
9.
Cortex ; 98: 114-127, 2018 01.
Article in English | MEDLINE | ID: mdl-28648411

ABSTRACT

Rossit et al. (2011) showed that neglect patients perform normally in a propointing task but not in an antipointing task which requires pointing towards the mirrored position of a target. It is assumed that antipointing relies on information from the perceptual pathway of our visual brain. Therefore, this finding supports the notion that neglect is a disorder that primarily affects perceptual spatial representations within the brain leaving spatial maps used for visuomotor guidance intact. Alternatively, performance of patients might be compromised in both tasks, but only obviously so in tasks in which online corrections are made more difficult. It can be argued that online-corrections via visual feedback are less effective in antipointing because a direct comparison between hand and target is not possible in this condition. Secondly, it is also known that neglect patients have a pronounced egocentric bias which is assumed to be associated with a deviation of the perceived body midline. Since the midline is used to compute the end-position in the antipointing task this could also explain why patients are worse in antipointing. We investigated the influence of visual feedback on pro- and antipointing and the effect of providing a visual reference line for the antipointing task in right-brain damaged patients with neglect (n = 20), right-brain damaged patients without neglect (n = 23) and in a group of healthy participants (n = 22). The withdrawal of visual feedback had a stronger effect on propointing compared to antipointing. This effect was stronger in neglect patients than in patients without neglect or healthy controls. The introduction of a reference line reduced errors in antipointing performance, particularly in neglect patients with a strong egocentric bias. The results support our alternative account and challenge the hypothesis that the spatial disorder in neglect affects primarily perceptual maps within the visual system.


Subject(s)
Feedback, Sensory/physiology , Perceptual Disorders/physiopathology , Psychomotor Performance/physiology , Visual Fields/physiology , Visual Perception/physiology , Aged , Female , Functional Laterality/physiology , Humans , Male , Middle Aged , Reaction Time/physiology
10.
Vision Res ; 140: 140-143, 2017 11.
Article in English | MEDLINE | ID: mdl-28965858

ABSTRACT

The violation of Weber's law in grasping has been presented as evidence for the claim that grasping is guided by visual information which is distinct from the information used in perceptual tasks. Previously, we contested this claim and argued that biomechanical constraints of the hand might explain why Weber's law cannot be reliably uncovered in grasping movements. In a recent article Manzone and colleagues (2017) show that pantomime grasping follows Weber's law even with objects whose width is close to the hand's biomechanical limit. In this commentary we explain why the biomechanical account does not necessarily predict the violation of Weber's law in a pantomime grasping task and why it seems problematic to use adherence or violation of Weber's law as a criterion to assign tasks to different anatomical pathways.


Subject(s)
Feedback, Sensory , Movement/physiology , Psychomotor Performance/physiology , Sensory Receptor Cells/physiology , Visual Perception/physiology , Hand/physiology , Humans , Psychophysics
11.
J Neurol Sci ; 370: 1-6, 2016 Nov 15.
Article in English | MEDLINE | ID: mdl-27772735

ABSTRACT

BACKGROUND: Multiple Sclerosis (MS) is a chronic inflammatory disease of the CNS typically affecting younger adults and resulting in neuro-axonal degeneration already at early stages of the disease. Less is known about the effects of a later disease onset (LOMS, onset >50years of age). Analysis of retinal layers by optical coherence tomography (OCT) is a non-invasive method to investigate retinal and neuro-axonal degeneration. We applied OCT to detect differences in retinal damage depending on a later disease manifestation. METHODS: 14 LOMS patients, 14 age- and 14 disease duration-matched normal onset (NOMS) patients with a relapsing remitting disease course and 15 healthy controls (HC) were included. OCT measurement of peripapillary retinal nerve fiber layer (RNFL), total macular volume (TMV), combined ganglion cell/inner plexiform layer (GCIPL), inner nuclear layer (INL) and outer retinal layers (ORL) was conducted. Furthermore, analysis of clinical features and of effects of previous optic neuritis (ON) was performed RESULTS: In a GEE based analysis of age- and disease duration matched NOMS, LOMS patients show no significant differences in retinal layer thickness whereas ON significantly reduced thickness of retinal layers. All MS groups display lower retinal layer thickness as compared to HC independently of type of onset. DISCUSSION: Our LOMS findings are well in line with published OCT data of normal onset MS. As the degree of retinal layer thinning was similar in MS subgroups, retinal neurodegeneration in MS may occur independently of time of disease onset.


Subject(s)
Multiple Sclerosis, Relapsing-Remitting/diagnostic imaging , Retina/diagnostic imaging , Retinal Degeneration/diagnostic imaging , Adult , Age of Onset , Axons , Biomarkers/cerebrospinal fluid , Cross-Sectional Studies , Female , Humans , Magnetic Resonance Imaging , Male , Middle Aged , Multiple Sclerosis, Relapsing-Remitting/complications , Multiple Sclerosis, Relapsing-Remitting/physiopathology , Retinal Degeneration/complications , Retinal Degeneration/physiopathology , Retrospective Studies , Tomography, Optical Coherence , Visual Acuity
12.
Ther Adv Neurol Disord ; 9(4): 269-80, 2016 Jul.
Article in English | MEDLINE | ID: mdl-27366233

ABSTRACT

OBJECTIVES: Disease-modifying therapies (DMTs) are applied to delay or prevent disease progression in multiple sclerosis (MS). While this has mostly been proven for physical symptoms, available studies regarding long-term effects of DMTs on cognitive functions are rare and sometimes inconsistent due to methodological shortcomings. Particularly in the case of fingolimod, comprehensive data on cognitive functions are not yet available. Therefore, we set out to reliably assess cognitive functions in patients with relapsing-remitting MS (RRMS) treated with DMTs over 1 year. METHODS: Cognitive functions were assessed with eight tests at three timepoints: baseline, 6-month follow up and 12-month follow up. First, we investigated whether the stability of cognitive functions (i.e. not falling below the 5% cut-off in more than one test) over 1 year in RRMS patients (n = 41) corresponds to the stability in healthy individuals (n = 40) of a previous study. Second, we compared the percentage of declined and improved patients in the different tests. Third, we compared patients treated with fingolimod (n = 22) with patients treated with natalizumab (n = 11) with regard to cognitive stability. Fourth, based on the patient data, the Reliable Change Index was applied to compute cut-offs for reliable cognitive change. RESULTS: Approximately 75% of RRMS patients treated with DMTs remained stable over the course of 1 year. The Paced Auditory Serial Addition Test (PASAT) and the Spatial Recall Test (SPART), produced improvements in 12.5% and 30.6%, respectively, probably due to practice effects. Patients treated with fingolimod did not differ from patients treated with natalizumab with regard to cognitive stability. CONCLUSIONS: Cognitive functions remain relatively stable under DMT treatment over 1 year, irrespective of the type of medication. Furthermore, the tests PASAT and SPART should be interpreted cautiously in studies examining performance changes over time. The provided RCI norms may help clinicians to determine whether a difference in two measurements observed in a RRMS patient is reliable.

14.
Vision Res ; 111(Pt A): 22-30, 2015 Jun.
Article in English | MEDLINE | ID: mdl-25872174

ABSTRACT

For grasping, Ganel, Chajut, and Algom (2008) demonstrated that the variability of the maximum grip aperture (MGA) does not increase with the size of the target object. This seems to violate Weber's law, a fundamental law of psychophysics. They concluded that the visual representations guiding grasping are distinct from representations used for perceptual judgments. Weber's law is however only relevant for one component of the measurable variability of MGA, namely the variability in the sensory system. We argue that when looking at the relationship between object size and grasping, the gain (often called slope) governing the relationship between target size and MGA can be used as an approximation to estimate the contribution of sensory noise to MGA variability. To test the idea that differences in gain modulate the relationship between target size and MGA variability, we examined grasping under a variety of conditions. We found that gain varied quite significantly across different tasks, but irrespective of gain Weber's law could not be found in any of the grasping tasks. Instead we repeatedly found an inverse relationship between variability and object size, i.e. variability decreased for bigger objects. This trend may reflect the reduced biomechanical freedom found for movements at the end an effector's effective range of motion. MGA variability may thus be dominated by non-sensory factors and therefore may constitute a poor choice to estimate the variability of the visual signals used by the brain to guide our grasping actions.


Subject(s)
Feedback, Sensory , Movement/physiology , Psychomotor Performance/physiology , Touch Perception/physiology , Visual Perception/physiology , Adult , Analysis of Variance , Differential Threshold , Female , Hand , Humans , Male , Young Adult
15.
Neuropsychologia ; 74: 178-83, 2015 Jul.
Article in English | MEDLINE | ID: mdl-25744870

ABSTRACT

Stroke of the right cerebral hemisphere often causes deficits in the judgement of the subjective visual vertical (SVV) and subjective tactile vertical (STV) which are related to central vestibular functioning. Clinically, deficits in the SVV/STV are linked to balance problems and poor functional outcome. Galvanic Vestibular Stimulation (GVS) is a non-invasive, save stimulation technique that induces polarity-specific changes in the cortical vestibular systems. Subliminal GVS induces imperceptible vestibular stimulation without unpleasant side effects. Here, we applied bipolar subliminal GVS over the mastoids (mean intensity: 0.7 mA, 20 min duration per session) to investigate its online-influence on constant errors, difference thresholds and range values in the SVV and STV. 24 patients with subacute, single, unilateral right hemisphere stroke were studied and assigned to two patient groups (impaired vs. normal in the SVV and STV) on the basis of cut-off scores from healthy controls. Both groups performed these tasks under three experimental conditions on three different days: a) sham GVS where electric current was applied only for 30s and then turned off, b) left-cathodal GVS and c) right-cathodal GVS, for a period of 20 min per session. Left-cathodal GVS, but not right-cathodal GVS significantly reduced all parameters in the SVV. Concerning STV GVS also reduced constant error and range numerically, though not significantly. These effects occurred selectively in the impaired patient group. In conclusion, we found that GVS rapidly influences poststroke verticality deficits in the visual and tactile modality, thus highlighting the importance of the vestibular system in the multimodal elaboration of the subjective vertical.


Subject(s)
Electric Stimulation/methods , Functional Laterality/physiology , Perceptual Disorders/rehabilitation , Touch/physiology , Vestibule, Labyrinth/physiology , Visual Perception/physiology , Adult , Aged , Aged, 80 and over , Analysis of Variance , Female , Humans , Male , Middle Aged , Perceptual Disorders/etiology , Photic Stimulation , Stroke/complications
16.
PLoS One ; 10(1): e0113961, 2015.
Article in English | MEDLINE | ID: mdl-25635863

ABSTRACT

BACKGROUND: In acute optic neuritis, magnetic resonance imaging (MRI) may help to confirm the diagnosis as well as to exclude alternative diagnoses. Yet, little is known on the value of optic nerve imaging for predicting clinical symptoms or therapeutic outcome. PURPOSE: To evaluate the benefit of optic nerve MRI for predicting response to appropriate therapy and recovery of visual acuity. METHODS: Clinical data as well as visual evoked potentials (VEP) and MRI results of 104 patients, who were treated at the Department of Neurology with clinically definite optic neuritis between December 2010 and September 2012 were retrospectively reviewed including a follow up within 14 days. RESULTS: Both length of the Gd enhancing lesion (r = -0.38; p = 0.001) and the T2 lesion (r = -0.25; p = 0.03) of the optic nerve in acute optic neuritis showed a medium correlation with visual acuity after treatment. Although visual acuity pre-treatment was little but nonsignificantly lower if Gd enhancement of the optic nerve was detected via orbital MRI, improvement of visual acuity after adequate therapy was significantly better (0.40 vs. 0.24; p = 0.04). Intraorbitally located Gd enhancing lesions were associated with worse visual improvement compared to canalicular, intracranial and chiasmal lesions (0.35 vs. 0.54; p = 0.02). CONCLUSION: Orbital MRI is a broadly available, valuable tool for predicting the improvement of visual function. While the accurate individual prediction of long-term outcomes after appropriate therapy still remains difficult, lesion length of Gd enhancement and T2 lesion contribute to its prediction and a better short-term visual outcome may be associated with detection and localization of Gd enhancement along the optic nerve.


Subject(s)
Magnetic Resonance Imaging , Optic Nerve/diagnostic imaging , Optic Neuritis/diagnostic imaging , Vision Disorders/diagnostic imaging , Adult , Aged , Aged, 80 and over , Eye/diagnostic imaging , Eye/physiopathology , Female , Humans , Male , Middle Aged , Multiple Sclerosis/physiopathology , Optic Nerve/physiopathology , Optic Neuritis/physiopathology , Radiography , Vision Disorders/physiopathology , Vision, Ocular/physiology
17.
Ther Adv Neurol Disord ; 7(6): 263-75, 2014 Nov.
Article in English | MEDLINE | ID: mdl-25371708

ABSTRACT

OBJECTIVES: With an increasing number of disease-modifying treatments (DMTs) for multiple sclerosis (MS), patient preferences will gain importance in the decision-making process. We assessed patients' implicit preferences for oral versus parenteral DMTs and identified factors influencing patients' treatment preference. METHODS: Patients with relapsing-remitting MS (n = 156) completed a questionnaire assessing treatment preferences, whereby they had to decide between pairs of hypothetical treatment scenarios. Based on this questionnaire a choice-based conjoint analysis was conducted. RESULTS: Treatment frequency and route of administration showed a stronger influence on patient preference compared with frequency of mild side effects. The latter attribute was more important for treatment-naïve patients compared with DMT-experienced patients. The higher the Extended Disability Status Scale score, the more likely pills, and the less likely fewer side effects were preferred. Pills were preferred over injections by 93% of patients, when treatment frequency and frequency of side effects were held constant. However, preference switched to injections when pills had to be taken three times daily and injections only once per week. Injections were also preferred when pills were associated with frequent side effects. CONCLUSIONS: Our results suggest that route of administration and treatment frequency play an important role in the patients' preference for a given DMT.

18.
Stroke ; 45(11): 3360-6, 2014 Nov.
Article in English | MEDLINE | ID: mdl-25278556

ABSTRACT

BACKGROUND AND PURPOSE: A transient ischemic attack (TIA) involves temporary neurological symptoms but leaves a patient symptom-free. Patients are faced with an increased risk for future stroke, and the manifestation of the TIA itself might be experienced as traumatizing. We aimed to investigate the prevalence of posttraumatic stress disorder (PTSD) after TIA and its relation to patients' psychosocial outcome. METHODS: Patients with TIA were prospectively studied, and 3 months after the diagnosis, PTSD, anxiety, depression, quality of life, coping strategies, and medical knowledge were assessed via self-rating instruments. RESULTS: Of 211 patients with TIA, data of 108 patients were complete and only those are reported. Thirty-two (29.6%) patients were classified as having PTSD. This rate is 10× as high as in the general German population. Patients with TIA with PTSD were more likely to show signs of anxiety and depression. PTSD was associated with the use of maladaptive coping strategies, subjectively rated high stroke risk, as well as with younger age. Finally, PTSD and anxiety were associated with decreased mental quality of life. CONCLUSIONS: The experience of TIA increases the risk for PTSD and associated anxiety, depression, and reduced mental quality of life. Because a maladaptive coping style and a subjectively overestimated stroke risk seem to play a crucial role in this adverse progression, the training of adaptive coping strategies and cautious briefing about the realistic stroke risk associated with TIA might be a promising approach. Despite the great loss of patients to follow-up, the results indicate that PTSD after TIA requires increased attention.


Subject(s)
Adaptation, Psychological , Ischemic Attack, Transient/epidemiology , Ischemic Attack, Transient/psychology , Quality of Life/psychology , Stress Disorders, Post-Traumatic/epidemiology , Stress Disorders, Post-Traumatic/psychology , Adaptation, Psychological/physiology , Adult , Aged , Aged, 80 and over , Female , Humans , Ischemic Attack, Transient/diagnosis , Male , Middle Aged , Prevalence , Prospective Studies , Stress Disorders, Post-Traumatic/diagnosis
19.
PLoS One ; 8(11): e81531, 2013.
Article in English | MEDLINE | ID: mdl-24282604

ABSTRACT

BACKGROUND: Despite the high frequency of cognitive impairment in multiple sclerosis, its assessment has not gained entrance into clinical routine yet, due to lack of time-saving and suitable tests for patients with multiple sclerosis. OBJECTIVE: The aim of the study was to compare the paradigm of visual search with neuropsychological standard tests, in order to identify the test that discriminates best between patients with multiple sclerosis and healthy individuals concerning cognitive functions, without being susceptible to practice effects. METHODS: Patients with relapsing remitting multiple sclerosis (n = 38) and age-and gender-matched healthy individuals (n = 40) were tested with common neuropsychological tests and a computer-based visual search task, whereby a target stimulus has to be detected amongst distracting stimuli on a touch screen. Twenty-eight of the healthy individuals were re-tested in order to determine potential practice effects. RESULTS: Mean reaction time reflecting visual attention and movement time indicating motor execution in the visual search task discriminated best between healthy individuals and patients with multiple sclerosis, without practice effects. CONCLUSIONS: Visual search is a promising instrument for the assessment of cognitive functions and potentially cognitive changes in patients with multiple sclerosis thanks to its good discriminatory power and insusceptibility to practice effects.


Subject(s)
Cognition , Multiple Sclerosis, Relapsing-Remitting/psychology , Adult , Case-Control Studies , Female , Humans , Male , Middle Aged , Neuropsychological Tests , ROC Curve , Young Adult
20.
Front Hum Neurosci ; 7: 90, 2013.
Article in English | MEDLINE | ID: mdl-23519604

ABSTRACT

Tactile extinction is frequent, debilitating, and often persistent after brain damage. Currently, there is no treatment available for this disorder. In two previous case studies we showed an influence of galvanic vestibular stimulation (GVS) on tactile extinction. Here, we evaluated in further patients the immediate and lasting effects of GVS on tactile extinction. GVS is known to induce polarity-specific changes in cerebral excitability in the vestibular cortices and adjacent cortical areas. Tactile extinction was examined with the Quality Extinction Test (QET) where subjects have to discriminate six different tactile fabrics in bilateral, double simultaneous stimulations on their dorsum of hands with identical or different tactile fabrics. Twelve patients with stable left-sided tactile extinction after unilateral right-hemisphere lesions were divided into two groups. The GVS group (N = 6) performed the QET under six different experimental conditions (two Baselines, Sham-GVS, left-cathodal/right-anodal GVS, right-cathodal/left-anodal GVS, and a Follow-up test). The second group of patients with left-sided extinction (N = 6) performed the QET six times repetitively, but without receiving GVS (control group). Both right-cathodal/left-anodal as well as left-cathodal/right-anodal GVS (mean: 0.7 mA) improved tactile identification of identical and different stimuli in the experimental group. These results show a generic effect of GVS on tactile extinction, but not in a polarity-specific way. These observed effects persisted at follow-up. Sham-GVS had no significant effect on extinction. In the control group, no significant improvements were seen in the QET after the six measurements of the QET, thus ruling out test repetition effects. In conclusion, GVS improved bodily awareness permanently for the contralesional body side in patients with tactile extinction and thus offers a novel treatment option for these patients.

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