To better understand the effects of spasticity on peripheral nerves, we evaluated the electrodiagnostic and nerve ultrasonographic features of the median and ulnar nerves in adults with upper limb spasticity. Twenty chro...To better understand the effects of spasticity on peripheral nerves, we evaluated the electrodiagnostic and nerve ultrasonographic features of the median and ulnar nerves in adults with upper limb spasticity. Twenty chronic stroke patients with spastic hemiparesis underwent nerve conduction study and nerve ultrasonography of the median and ulnar nerves at both upper limbs. Affected versus unaffected upper limb comparisons showed significant differences in the median and ulnar nerve distal motor latencies, compound muscle action potentials and F-wave minimal latencies. Furthermore, we observed a significantly greater median nerve crosssectional area at the elbow of the affected upper limb compared with the unaffected one. Our findings confirmed electrodiagnostic asymmetries and nerve ultrasonographic abnormalities in the affected versus the unaffected upper limb after stroke. Slight changes in lower motor neuron activity and spasticity might contribute to these alterations.
Botulinum toxin is a well-established treatment for a number of conditions involving muscle hyperactivity, such as focal dystonia and spastic paresis. However, current injection practice is not standardized and there is...Botulinum toxin is a well-established treatment for a number of conditions involving muscle hyperactivity, such as focal dystonia and spastic paresis. However, current injection practice is not standardized and there is a clear need for structured training. An international group of experts in the management of patients with cervical dystonia (CD) and spastic paresis created a steering committee (SC). For each therapeutic area, the SC developed a core slide set on best practice, based on the literature. International sites of expertise were identified for training and courses were designed to include lectures and casebased learning. Where possible, courses received accreditation from the European Union of Medical Specialists (UEMS). Each course was peer reviewed by the SC, the UEMS accreditation board and the attendees themselves (through evaluation questionnaires). Attendees' feedback was shared with the SC and the trainers to tailor future training sessions. From the program launch in 2012 to December 2014, 328 physicians from 34 countries were trained in a total of 58 courses; 67% of the courses focused on spastic paresis and 33% on CD. Of the 225 (69%) physicians who completed feedback forms, 95% rated their course as 'above average/excellent' in meeting the preset learning objectives. Most (90%) physicians declared that attending a course would lead them to change their practice. The development of the 'Ixcellence Network' for continuous medical education in the fields of spastic paresis and CD has provided a novel and interactive way of training physicians with previous experience in botulinum toxin injection.
Diffusion tensor imaging (DTI) is sensitive to white matter (WM) damage in multiple sclerosis (MS), not only in focal lesions but also in the normal-appearing WM (NAWM). However, DTI indices can also be affected by natur...Diffusion tensor imaging (DTI) is sensitive to white matter (WM) damage in multiple sclerosis (MS), not only in focal lesions but also in the normal-appearing WM (NAWM). However, DTI indices can also be affected by natural spatial variation in WM, as seen in crossing and dispersing white matter fibers. Neurite orientation dispersion and density imaging (NODDI) is an advanced diffusion-weighted imaging technique that provides distinct indices of fiber density and dispersion. We performed NODDI of lesion tissue and NAWM in five MS patients and five controls, comparing the technique with traditional DTI. Both DTI and NODDI identified tissue damage in NAWM and in lesions. NODDI was able to detect additional changes and it provided better contrast in MS-NAWM microstructure, because it distinguished orientation dispersion and fiber density better than DTI. We showed that NODDI is viable in MS patients and that it offers, compared with DTI parameters, improved sensitivity and possibly greater specificity to microstructure features such as neurite orientation.
The present national survey seeking to identify unmet needs in the management of spasticity with botulinum toxin type A focused on the use of OnabotulinumoxinA, since this is the brand with the widest range of licensed i...The present national survey seeking to identify unmet needs in the management of spasticity with botulinum toxin type A focused on the use of OnabotulinumoxinA, since this is the brand with the widest range of licensed indications in Italy. Physicians from twenty-four Italian neurorehabilitation units compiled a questionnaire about "real-life" post-stroke spasticity management. OnabotulinumtoxinA was reported to be used in the following average doses: upper limb 316.7 ± 79.1 units; lower limb 327.8 ± 152.3; upper and lower limb 543.7 ± 123.7 units. Of the physicians surveyed, 37.5% felt that increasing the frequency of OnabotulinumtoxinA injection would improve its efficacy; 70.8% use electrical stimulation/electromyography guidance (one fourth of injections with no instrumental guidance). Instrumental evaluation was used by 41.7% of the physicians. The participants expressed the view that early identification of post-stroke spasticity would be facilitated by the availability of a post-stroke checklist, and that this should be used by physiotherapists (91.7%), physiatrists (58.3%), family doctors (50%), stroke unit physicians (25%), patients and caregivers (79.2%). According to our findings, the management of poststroke spasticity has several unmet needs that, were they addressed, might improve these patients' clinical outcomes and quality of life. These needs concern patient follow-up, where a clearly defined pathway is lacking; furthermore, there is a need to use maximum doses per treatment and to ensure early intervention on post-stroke spasticity.
Mosca L, Mantero V, Penco S
… +10 more, La Mantia L, De Benedetti S, Marazzi MR, Spreafico C, Erminio C, Grassi L, Lando G, Zagaria M, Agostoni E, Protti A
Environmental and genetic factors seem to play a pathogenetic role in multiple sclerosis (MS). The genetic component is partly suggested by familial aggregation of cases; however, MS families with affected subjects over...Environmental and genetic factors seem to play a pathogenetic role in multiple sclerosis (MS). The genetic component is partly suggested by familial aggregation of cases; however, MS families with affected subjects over different generations have rarely been described. The aim of this study was to report clinical and genetic features of a multigenerational MS family and to perform a review of the literature on this topic. We describe a multigenerational Italian family with six individuals affected by MS, showing different clinical and neuroradiological findings. HLA-DRB1* typing revealed the presence of the DRB1*15:01 allele in all the MS cases and in 4/5 non-affected subjects. Reports on six multigenerational MS families have previously been published, giving similar results. The HLA-DRB1*15:01 allele was confirmed to be linked to MS disease in this family; moreover, its presence in non-affected subjects suggests the involvement of other susceptibility factors in the development and expression of the disease, in accordance with the complex disease model now attributed to MS.
The aim of this study was to investigate whether transcranial static magnetic field stimulation (tSMS) delivered using a compact cylindrical NdFeB magnet over the cerebellum modulates the excitability of the cerebellum a...The aim of this study was to investigate whether transcranial static magnetic field stimulation (tSMS) delivered using a compact cylindrical NdFeB magnet over the cerebellum modulates the excitability of the cerebellum and contralateral primary motor cortex, as measured using cerebellar brain inhibition (CBI), motor evoked potentials (MEPs), and resting motor threshold (rMT). These parameters were measured before tSMS or sham stimulation and immediately, 5 minutes and 10 minutes after stimulation. There were no significant changes in CBI, MEPs or rMT over time in the sham stimulation condition, and no changes in MEPs or rMT in the tSMS condition. However, CBI was significantly decreased immediately after tSMS as compared to that before and 5 minutes after tSMS. Our results suggest that tSMS delivered to the cerebellar hemisphere transiently reduces cerebellar inhibitory output but does not affect the excitability of the contralateral motor cortex.
The aim of this research was to study cognitive dysfunctions in multiple sclerosis (MS) by exploring subtle cognitive tasks, usually not included in the standard neuropsychological assessment. We wished to investigate wh...The aim of this research was to study cognitive dysfunctions in multiple sclerosis (MS) by exploring subtle cognitive tasks, usually not included in the standard neuropsychological assessment. We wished to investigate whether it is possible to identify object decision deficits in MS patients without evident cognitive impairment; secondary objectives were to understand whether these deficits can be detected in the early stages of the disease and whether there are differences related to different phenotypes. Participants were divided into four groups: (a) 12 patients with early relapsing-remitting MS [ERR]; (b) 14 with late relapsing-remitting MS [LRR]; (c) 10 with secondary progressive MS [SP]; (d) 36 healthy controls [HCs]. All participants performed a series of experimental tasks: an object decision task (recognition of chimeric and real figures) and naming and visual discrimination tasks. Our results suggest that object decision disorders are detectable in patients without overt cognitive impairments and that performances on these tasks are related to phenotypes. On the other hand, the Chimeric Figures task is not appropriate for identifying cognitive dysfunctions in early MS.
Sandrini G, Binder H, Hömberg V
… +18 more, Saltuari L, Tarkka I, Smania N, Corradini C, Giustini A, Kätterer C, Picari L, Diserens K, Koenig E, Geurts A, Anghelescu A, Opara J, Tonin P, Kwakkel G, Golyk V, Onose G, Pérennou D, Picelli A
To date, medical education lacks Europe-wide standards on neurorehabilitation. To address this, the European Federation of NeuroRehabilitation Societies (EFNR) here proposes a postgraduate neurorehabilitation training sc...To date, medical education lacks Europe-wide standards on neurorehabilitation. To address this, the European Federation of NeuroRehabilitation Societies (EFNR) here proposes a postgraduate neurorehabilitation training scheme. In particular, the European medical core curriculum in neurorehabilitation should include a two-year residency in a neurorehabilitation setting where trainees can gain practical experience. Furthermore, it should comprise six modules of classroom training organized as weekend seminars or summer/winter schools. In conclusion, after defining the European medical core curriculum in neurorehabilitation, the next activities of the EFNR will be to try and reach the largest possible consensus on its content among all national societies across Europe in order to further validate it and try to extend it to the other, non-medical, professionals on the neurorehabilitation team in line with their core curricula defined by each professional association.
Seizures are a frequent acute neurological event in the neonatal period. Up to 12 to 18% of all seizures in newborns are due to perinatal stroke and up to 39% of affected children can then develop epilepsy in childhood....Seizures are a frequent acute neurological event in the neonatal period. Up to 12 to 18% of all seizures in newborns are due to perinatal stroke and up to 39% of affected children can then develop epilepsy in childhood. We report the case of a young patient who presented stroke-related seizures in the neonatal period and then developed focal symptomatic epilepsy at 15 years of age, and in whom the epileptic focus was found to co-localize with the site of his ischemic brain lesion. Such a prolonged silent period before onset of remote symptomatic epilepsy has not previously been reported. This case suggests that newborns with seizures due to a neonatal stroke are at higher risk of epilepsy and that the epileptogenic process in these subjects can last longer than a decade.
Lebanon is a developing country where the prevalence of stroke subtypes and their correlation with risk factors have not been studied, even though stroke management is an ongoing major national healthcare challenge. In a...Lebanon is a developing country where the prevalence of stroke subtypes and their correlation with risk factors have not been studied, even though stroke management is an ongoing major national healthcare challenge. In a retrospective study conducted in two university hospitals, data were collected on all stroke cases admitted in 2012 and 2013. Ischemic strokes were then classified according to a modified TOAST classification. A total of 254 inpatients (mean age 68.41 years ±13.34, 55.1% males) was included in the study; of these, 15% had had a hemorrhagic stroke and was therefore excluded. Conversely to findings from studies in other Arab and Asian countries, where small vessel disease is the most frequent subtype, our study showed a predominance of large artery atherosclerosis (53.5%) which, in comparison with other subtypes, was found to be associated with dyslipidemia (OR= 3.82, 95% CI= [1.76-8.28]; p=0.001). Cardioembolic stroke and small vessel disease were found to be positively associated with aging and living in Beirut, respectively. Larger studies are needed to explain these findings.
Vitamin D supplementation has been proposed as a potential treatment to delay amyotrophic lateral sclerosis (ALS) progression. The aims of this study were to compare retrospectively vitamin D blood levels in ALS patients...Vitamin D supplementation has been proposed as a potential treatment to delay amyotrophic lateral sclerosis (ALS) progression. The aims of this study were to compare retrospectively vitamin D blood levels in ALS patients with those in healthy subjects; to correlate vitamin D blood levels with clinical functions in patients; and to evaluate whether administration of vitamin D could modify the clinical progression of the disease. Vitamin D blood levels were evaluated in 57ALS patients and in 57 healthy subjects. In the ALS patients the following clinical variables were evaluated every 3 months: Medical Research Council scale (MRC) score; revised ALS functional rating scale (ALSFRS-R) score; forced vital capacity (FVC). Twentyfour patients were treated with high doses of cholecalciferol. No significant differences were found between the vitamin D blood levels in the ALS patients (18.8 ± 12.2) and the healthy subjects (20.7 ± 10.1). The vitamin D levels in the ALS patientsdid not correlate with recorded clinical parameters. No clinical differences in terms of ALSFRS-R, MRC or FVC were found between the treated and the untreated patients over time. In ALS, as in other chronic neurological diseases, levels of vitamin D in blood appeared reduced, but no difference was found between the levels in ALS patients and in healthy subjects. Oral vitamin D supplementation in ALS patients was not associated with better prognosis in comparison with untreated ALS patients. Further prospective controlled studies are needed to clarify the effect of vitamin D on the progression of ALS disease.
The aim of our study was to identify and quantify spatiotemporal and kinematic gait parameters obtained by 3D gait analysis (GA) in a group of Parkinson's disease (PD) patients compared with healthy subjects in order to...The aim of our study was to identify and quantify spatiotemporal and kinematic gait parameters obtained by 3D gait analysis (GA) in a group of Parkinson's disease (PD) patients compared with healthy subjects in order to investigate whether early PD patients could present an abnormal gait pattern. Forty-four patients affected by early-stage PD compared with a control group were analyzed. All participants were evaluated with 3D GA in the gait laboratory. The greatest significance in temporal parameters was found in cadence (102.46 ± 13.17 steps/min in parkinsonian patients vs 113.84 ± 4.30 steps/min in control subjects), followed by stride duration (1.19 ± 0.18 seconds right limb and 1.19 ± 0.19 seconds left limb in PD patients vs 0.426 ± 0.16 seconds right limb and 0.429 ± 0.23 seconds left limb in normal subjects) and stance duration. Marked differences were also found in the swing phase and in swing duration (p<0.05), while the stance phase was not significantly different in patients compared with healthy subjects. A statistically different velocity in PD patients (0.082 ± 0.29 m/s) vs healthy subjects (1.33 ± 0.06 m/s) was shown by spatial parameter analysis. Step width, stride length and swing velocity were highly significant parameters, as was average velocity. Our study highlighted some distinguishing characteristics of gait in early PD. Ambulation disorders may be present in the early stage of PD and their detection allows for early medical treatment and possible rehabilitation.
The F-wave/M-wave amplitude (F/M-amp) ratio has been shown to be increased in peripheral neuropathies, provided the maximum M-wave is relatively preserved. Reduced M-wave amplitudes and central facilitation of antidromic...The F-wave/M-wave amplitude (F/M-amp) ratio has been shown to be increased in peripheral neuropathies, provided the maximum M-wave is relatively preserved. Reduced M-wave amplitudes and central facilitation of antidromically-induced reactivation of the anterior horn cells' axon hillocks (F-wave) are believed to contribute to higher F/M-amp ratios. The present study was undertaken to re-evaluate mechanisms responsible for higher F/M-amp ratios in carpal tunnel syndrome (CTS). We enrolled 232 cases affected by CTS and 108 controls. Fand M-wave amplitudes and F-wave chronodispersion were analyzed for the median and ulnar nerves. The F/M-amp ratio of the median nerve in CTS subjects with moderate-severe nerve damage was significantly higher than that of mild CTS subjects and controls. Chronodispersion of the median nerve F-wave increased with increasing CTS severity. We conclude that the relative preservation of the median nerve F-wave is due to damage to the large diameter muscle afferent fibers responsible for the monosynaptic response. Absence of the monosynaptic response makes the small motoneurons, usually inaccessible to the antidromic volley because of its collision with the orthodromic reflex volley, able to fire in the F-wave.
This study aimed to characterize the coefficient of friction (COF) curves of patients with Parkinson's disease (PD) during barefoot gait and to evaluate the relationships between this variable and functional scales. Twen...This study aimed to characterize the coefficient of friction (COF) curves of patients with Parkinson's disease (PD) during barefoot gait and to evaluate the relationships between this variable and functional scales. Twenty-two subjects with PD (ON phase of levodopa) and 22 healthy subjects participated in this study. The participants walked barefoot along a pathway that went over two force plates embedded in the floor of the data collection room. The instantaneous COF was calculated as the ratio between the horizontal and vertical components of the ground reaction forces. Two-sample t-tests applied to every 1% of the support phase of the COF curve were used to compare the groups and to identify the phases in which the two groups were different. Specifically, three COF areas were computed: Area 1 (for the loading response phase), Area 2 (for the midstance phase) and Area 3 (for the terminal stance phase). Pearson's tests were applied to assess the associations between the COF curve areas and the clinical scales. The subjects with PD exhibited lower COF values during the loading response and terminal stance phases and higher COF values during the mid-stance phase compared with the control group. A strong positive correlation was observed between Area 1 and the Timed Up and Go Test (90.3%). In conclusion, the patients' COFs exhibited patterns that were different from those of the control group. Moreover, during the loading response phase, these differences were well-correlated with the Timed Up and Go Test scale data; Timed Up and Go Test data can be used to identify the risk of falls among PD patients.
The term frontotemporal lobar degeneration (FTLD) describes a spectrum of neurodegenerative disorders associated with deposition of misfolded proteins in the frontal and temporal lobes. Up to 40% of FTLD patients reports...The term frontotemporal lobar degeneration (FTLD) describes a spectrum of neurodegenerative disorders associated with deposition of misfolded proteins in the frontal and temporal lobes. Up to 40% of FTLD patients reports a family history of neurodegeneration, and approximately 1/3 of familial cases shows an autosomal dominant pattern of inheritance of the phenotype. Over the past two decades, several causative and susceptibility genes for FTLD have been discovered, supporting the notion that genetic factors are important contributors to the disease processes. Genetic variants in three genes, MAPT, GRN and C9orf72, account for about half of familial FTLD cases. In addition, rare defects in the CHMP2B, VCP, TARDBP, SQSTM1, FUS, UBQLN, OPTN, TREM2, CHCHD10 and TBK1 genes have been described. Additional genes are expected to be found in near future. The purpose of this review is to describe recent advances in the molecular genetics of the FTLD spectrum and to discuss implications for genetic counseling.
The aim of this study was to clarify the influence of anatomical (cerebral hemisphere) and demographic (age and gender) variables on the gray matter (GM) volumes and volumetric asymmetry indices (VAIs) of selected struct...The aim of this study was to clarify the influence of anatomical (cerebral hemisphere) and demographic (age and gender) variables on the gray matter (GM) volumes and volumetric asymmetry indices (VAIs) of selected structures involved in episodic memory. A cross-sectional study was performed in 47 healthy volunteers. Neuropsychological evaluation revealed similar IQs across the sample. Using SPM-based software, brain segmentation, labeling and volume measurements of the hippocampus, amygdala, middle temporal gyrus and parahippocampal gyrus were performed in each cerebral hemisphere. A two-way between-groups multivariate analysis of covariance (MANCOVA) was applied to GM volumes and VAIs. The main effects of gender and cerebral hemisphere on GM volumes were significant (p < .001), while there was no significant interaction effect between gender and cerebral hemisphere. VAI measurements showed a nonsignificant effect of gender, but a significant influence of age (p = .015). The linear model of interactions and main effects explained 33% of the variance influencing the GM volume quantification. While cerebral hemisphere and gender were found to affect the volumes of brain structures involved in episodic memory, the calculation of VAIs was affected only by age. A comprehensive understanding of the main effects and interaction effects of cerebral hemisphere, gender and age on the volumes and asymmetries of structures related to episodic memory might help neurologists, psychiatrists, geriatricians and other neuroscientists in the study of degenerative brain diseases.