Accelerometry is a reliable tool for gauging the occurrence, amplitude and frequency of tremor. However, there is no consensus on criteria for accelerometric diagnosis of tremor syndromes. We enrolled 20 patients with es...Accelerometry is a reliable tool for gauging the occurrence, amplitude and frequency of tremor. However, there is no consensus on criteria for accelerometric diagnosis of tremor syndromes. We enrolled 20 patients with essential tremor (ET), 20 with dystonic tremor (DT), and 20 with classic parkinsonian tremor (PD-T), all meeting accepted clinical criteria. All the patients underwent dopamine transporter imaging (by means of single-photon emission computed tomography) and triaxial accelerometric tremor analysis. The latter revealed groupwise differences in tremor frequency, peak dispersion, spectral coherence, unilaterality and resting vs action tremor amplitude. From the above, five diagnostic criteria were extrapolated for each condition. Receiver operating characteristic curves, depicting criteriabased scoring of each tremor type, showed negligible declines in specificity for scores ≥4 in patients with ET or DT and scores ≥3 in patients with PD-T, thus providing a simple scoring method (accelerometrically derived) for differential diagnosis of the principal tremor syndromes.
The present paper provides practical guidance on the management of adult spasticity with OnabotulinumtoxinA. Advisory Board members reviewed the available evidence and discussed their personal experiences in order to add...The present paper provides practical guidance on the management of adult spasticity with OnabotulinumtoxinA. Advisory Board members reviewed the available evidence and discussed their personal experiences in order to address the unmet needs in the management of spasticity with botulinum toxin type A identified by the recent Italian Real-Life Post-Stroke Spasticity Survey. Stroke patients should be referred to spasticity services that have adequate facilities and multidisciplinary teams with the necessary training, competence and expertise. The current literature shows a strong correlation between the development of post-stroke spasticity and the degree of central sensorimotor system destruction/disorganization. Use of tools such as the Poststroke Checklist may help clinicians in the long-term follow-up of stroke patients. The maximum dose of onabotulinumtoxinA - according to the current literature this ranges from 300U to 400U for upper limb and from 500U to 600U for lower limb aggregate postures - should be re-considered. In addition, there is a need for future consensus (also based on pharmacoeconomic considerations) on consistent clinical care models for the management of patients with post-stroke spasticity.
Apathy is a state of diminished goal-directed speech, motor activity and emotions. The prevalence of apathy in Parkinson's disease (PD) ranges from 16 to 62%. Several studies have investigated the relationships between a...Apathy is a state of diminished goal-directed speech, motor activity and emotions. The prevalence of apathy in Parkinson's disease (PD) ranges from 16 to 62%. Several studies have investigated the relationships between apathy and other dimensions of PD, but little is known about possible discrepancies between self-evaluation (SE) and caregiver reporting (CR) of this symptom. The aim of this study is twofold: 1) to investigate the differences in apathy evaluations according to the point of view from which apathy is reported (SE vs CR); 2) to identify the possible relationships between each of the two evaluations (SE and CR) and cognitive and affective dimensions of PD. Forty-eight patients with PD were assessed using the Apathy Evaluation Scale (AES) in its SE and CR versions (AES-SE and AES-CR); cognitive, affective and behavioral symptoms were also assessed. AES-SE scores were significantly higher than AESCR ones. Neither AES version correlated with depression, whereas both correlated with motor impairment, disease stage and behavioral symptoms. Mini-Mental State Examination and Frontal Assessment Battery scores showed significant negative correlations only with AES-SE scores. Our findings suggest that the point of view from which apathy is seen can lead to significant discrepancies, even when using the same tool. This should be taken into account in order to obtain correct assessment of this disabling and distressing symptom.
Diagnostic accuracy and reliable estimation of clinical evolution are challenging issues in the management of patients with disorders of consciousness (DoC). Longitudinal systematic investigations conducted in large coho...Diagnostic accuracy and reliable estimation of clinical evolution are challenging issues in the management of patients with disorders of consciousness (DoC). Longitudinal systematic investigations conducted in large cohorts of patients with DoC could make it possible to identify reliable diagnostic and prognostic markers. On the basis of this consideration, we devised a multicentre prospective registry for patients with DoC admitted to ten intensive rehabilitation units. The registry collects homogeneous and detailed data on patients' demographic and clinical features, neurophysiological and neuroimaging findings, and medical and surgical complications. Here we present the rationale and the design of the registry and the preliminary results obtained in 53 patients with DoC (vegetative state or minimally conscious state) enrolled during the first seven months of the study. Data at 6-month post-injury follow-up were available for 46 of them. This registry could be an important tool for collecting high-quality data through the application of rigorous methods, and it could be used in the routine management of patients with DoC admitted to rehabilitation settings.
Three different type A botulinum neurotoxins (BoNTAs) - onabotulinumtoxinA, abobotulinumtoxinA and incobotulinumtoxinA) - are currently marketed in Europe to treat several conditions. Differences between BoNTA preparatio...Three different type A botulinum neurotoxins (BoNTAs) - onabotulinumtoxinA, abobotulinumtoxinA and incobotulinumtoxinA) - are currently marketed in Europe to treat several conditions. Differences between BoNTA preparations, which depend on their specific biotypes and manufacturing processes, lead to clinically relevant pharmacotherapeutic dissimilarities. All three available products are separately recognized and reviewed in American Academy of Neurology guidelines. The neurotoxin load/100U is likewise different among the different BoNTAs, with the result that the specific potency of the 150kD BoNTA neurotoxin is calculated as 137 units/ng for onabotulinumtoxinA, 154 units/ng for abobotulinumtoxinA, and 227 units/ng for incobotulinumtoxinA. It is important for clinicians to have all three BoNTAs available in order to choose the most suitable preparation for the specific indication in the single patient. Commercially available BoNTAs must be recognized as different from one another, and therefore as non-interchangeable. The essential experience of the clinician is of the utmost importance in choosing the most appropriate treatment.
This study aimed to investigate the incidence of aggressiveness in patients with severe drug-refractory focal epilepsy (DRE) who started perampanel (PER) as add-on treatment, and to identify possible predisposing factors...This study aimed to investigate the incidence of aggressiveness in patients with severe drug-refractory focal epilepsy (DRE) who started perampanel (PER) as add-on treatment, and to identify possible predisposing factors. Data on 49 consecutive patients with severe DRE who initiated PER were retrospectively collected. Twelve of the 49 patients experienced aggressiveness as adverse event related to PER treatment, one third of them on low (2-4 mg/day) PER dosages. PER was discontinued in 10/12 patients because of aggressive behaviors. Aggressiveness could appear after several months or even more than one year of PER treatment. One third of patients with PER-related aggressiveness had intellectual disabilities and 5/12 patients took levetiracetam as a concomitant antiepileptic drug. Our study suggests that the occurrence of aggressive behaviors in patients with severe DRE is not uncommon during PER treatment and that it may occur after months or even years of treatment with a stable dosage, requiring PER discontinuation in the great majority of patients.
The Authors of this letter look at consumption of opioids and a2- ligands, also known as "gabapentinoids", in Italy, and specifically in the Provincial Health District of Cosenza, as compared with USA trends of recent d...The Authors of this letter look at consumption of opioids and a2- ligands, also known as "gabapentinoids", in Italy, and specifically in the Provincial Health District of Cosenza, as compared with USA trends of recent decades. Access to analgesic drugs since the introduction of Italian law 38/2010 is also evaluated and possible future measures for better management of chronic pain are proposed.
The Barthel Index (BI) is widely used to determine eligibility criteria for inpatient rehabilitation and to monitor patients' recovery, irrespective of the illnesses that affect them. The culturally adapted Italian versi...The Barthel Index (BI) is widely used to determine eligibility criteria for inpatient rehabilitation and to monitor patients' recovery, irrespective of the illnesses that affect them. The culturally adapted Italian version of the Barthel Index (IcaBI) was recently validated. This paper reports the structural validity and inter-rater reliability of the IcaBI and its responsiveness to the results of inpatient rehabilitation. The IcaBI was administered to a cohort of 264 patients hospitalized in two rehabilitation centers in Rome, Italy. Factor analysis using principal component analysis revealed a monofactorial structure for neurological patients and, after removal of item 1 "feeding", also for orthopedic patients. Substantial to optimal inter-rater reliability was found (0.74 > intraclass correlation coefficient < 0.96). The IcaBI was found to be accurate (area under the curve= 0.72) with a minimal clinically important change score of 35 points. This work confirms that IcaBI is a useful tool for measuring disability in health and social care settings along the continuum of care. Further studies are needed to assess its criterion validity, interpretability and responsiveness in other specific disease conditions.
The first part of this paper reports an interview with the philosopher Peter Singer. In the second, we examine Singer's background: naturalism and neurophilosophy, discussing three of its theses, as presented by Patricia...The first part of this paper reports an interview with the philosopher Peter Singer. In the second, we examine Singer's background: naturalism and neurophilosophy, discussing three of its theses, as presented by Patricia Smith Churchland. Finally, we go back to Singer himself, to draw some conclusions.
Corticobasal degeneration is a rare disorder, which usually consists of a combination of complex movement disorders, apraxia and cortical changes. Its definition is still evolving and in 2013 an international consortium...Corticobasal degeneration is a rare disorder, which usually consists of a combination of complex movement disorders, apraxia and cortical changes. Its definition is still evolving and in 2013 an international consortium tried to develop new criteria, based on a systematic literature review. Over a long period of time, we carefully selected 23 patients who fulfilled the criteria for a diagnosis of corticobasal degeneration; all had the so-called corticobasal syndrome phenotype, in accordance with Armstrong et al. (2013). Through a dedicated study, we set out to study behavioral alterations, specifically apathy, and to compare the results obtained with those deriving from a well-defined Parkinson's disease population. On the basis of our limited but specific results, we argue for a possible role of the parietal neural networks as a determinant of apathy, and provide an overview of emerging data in the imaging and pathology literature.
Cytosine arabinoside (Ara-C) is one of the key drugs for treating acute myeloid leukemia (AML). High intravenous doses may produce a number of central nervous system (CNS) toxicities and contribute to modifications in br...Cytosine arabinoside (Ara-C) is one of the key drugs for treating acute myeloid leukemia (AML). High intravenous doses may produce a number of central nervous system (CNS) toxicities and contribute to modifications in brain functional connectivity. sLORETA is a software used for localizing brain electrical activity and functional connectivity. The aim of this study was to apply sLORETA in the evaluation of possible effects of Ara-C on brain connectivity in patients with AML without CNS involvement. We studied eight patients with AML; four were administered standard doses of Ara-C while the other four received high doses. sLORETA was computed from computerized EEG data before treatment and after six months of treatment. Three regions of interest, corresponding to specific combinations of Brodmann areas, were defined. In the patients receiving high-dose Ara-C, a statistically significant reduction in functional connectivity was observed in the fronto-parietal network, which literature data suggest is involved in attentional processes. Our data highlight the possibility of using novel techniques to study potential CNS toxicity of cancer therapy.
Bianchi F, Squintani GM, Osio M
… +18 more, Morini A, Bana C, Ardolino G, Barbieri S, Bertolasi L, Caramelli R, Cogiamanian F, Currà A, de Scisciolo G, Foresti C, Frasca V, Frasson E, Inghilleri M, Maderna L, Motti L, Onesti E, Romano MC, Del Carro U
Neurophysiological testing of the pelvic floor is recognized as an essential tool to identify pathophysiological mechanisms of pelvic floor disorders, support clinical diagnosis, and aid in therapeutic decisions. Neverth...Neurophysiological testing of the pelvic floor is recognized as an essential tool to identify pathophysiological mechanisms of pelvic floor disorders, support clinical diagnosis, and aid in therapeutic decisions. Nevertheless, the diagnostic value of these tests in specific neurological diseases of the pelvic floor is not completely clarified. Seeking to fill this gap, the members of the Neurophysiology of the Pelvic Floor Study Group of the Italian Clinical Neurophysiology Society performed a systematic review of the literature to gather available evidence for and against the utility of neurophysiological tests. Our findings confirm the utility of some tests in specific clinical conditions [e.g. concentric needle electromyography, evaluation of sacral reflexes and of pudendal somatosensory evoked potentials (pSEPs) in cauda equina and conus medullaris lesions, and evaluation of pSEPs and perineal sympathetic skin response in spinal cord lesions], and support their use in clinical practice. Other tests, particularly those not currently supported by high-level evidence, when employed in individual patients, should be evaluated in the overall clinical context, or otherwise used for research purposes.
Different rehabilitation models for persons diagnosed with disorders of consciousness have been proposed in Europe during the last decade. In Italy, the Ministry of Health has defined a national healthcare model, althoug...Different rehabilitation models for persons diagnosed with disorders of consciousness have been proposed in Europe during the last decade. In Italy, the Ministry of Health has defined a national healthcare model, although, to date, there is a lack of information on how this has been implemented at regional level. The INCARICO project collected information on different regional regulations, analysing ethical aspects and mapping care facilities (numbers of beds and medical units) in eleven regional territories. The researchers found a total of 106 laws; differences emerged both between regions and versus the national model, showing that patients with the same diagnosis may follow different pathways of care. An ongoing cultural shift from a treatment-oriented medical approach towards a care-oriented integrated biopsychosocial approach was found in all the welfare and healthcare systems analysed. Future studies are needed to explore the relationship between healthcare systems and the quality of services provided.
Gerstmann-Sträussler-Scheinker syndrome (GSS) is an inherited autosomal dominant prion disease, caused by a codon 102 proline to leucine substitution (P102L) in the prion protein gene (PRNP). We describe the case of a 40...Gerstmann-Sträussler-Scheinker syndrome (GSS) is an inherited autosomal dominant prion disease, caused by a codon 102 proline to leucine substitution (P102L) in the prion protein gene (PRNP). We describe the case of a 40-year-old male, affected by a slowly progressive gait disturbance, leg weakness and cognitive impairment. Genomic DNA revealed a point mutation of PRNP at codon 102, resulting in P102L, and the diagnosis of GSS was confirmed. Somatosensory evoked potentials showed alterations of principal parameters, particularly in the right upper and lower limbs. Laser-evoked potentials were indicative of nociceptive system impairment, especially in the right upper and lower limbs. Conventional magnetic resonance imaging (MRI) revealed marked atrophy of the vermis and cerebellar hemispheres and mild atrophy of the middle cerebellar peduncles and brainstem, as confirmed by a brain volume automatic analysis. Resting-state functional MRI showed increased functional connectivity in the bilateral visual cortex, and decreased functional connectivity in the bilateral frontal pole and supramarginal and precentral gyrus. Albeit limited to a single case, this is the first study to assess structural and functional connectivity in GSS using a multimodal approach.
Patients with Parkinson's disease (PD) and Pisa syndrome (PS) may present tonic dystonic or compensatory (i.e. acting against gravity) hyperactivity in the paraspinal and non-paraspinal muscles. Electromyographic (EMG) a...Patients with Parkinson's disease (PD) and Pisa syndrome (PS) may present tonic dystonic or compensatory (i.e. acting against gravity) hyperactivity in the paraspinal and non-paraspinal muscles. Electromyographic (EMG) activity was measured in nine patients with PD and PS, three with PD without PS, and five healthy controls. Fine-wire intramuscular electrodes were inserted bilaterally into the iliocostalis lumborum (ICL), iliocostalis thoracis (ICT), gluteus medius (GM), and external oblique (EO) muscles. The root mean square (RMS) of the EMG signal was calculated and normalized for each muscle. In stance condition, side-to-side muscle activity comparisons showed a higher RMS only for the contralateral ICL in PD patients with PS (p=0.028). Moreover, with increasing degrees of lateral flexion, the activity of the EO and the ICL muscles progressively increased and decreased, respectively. The present data suggest that contralateral paraspinal muscle activity plays a crucial compensatory role and can be dysfunctional in PD patients with PS.
An association of obstructive sleep apnea syndrome (OSAS) and other sleep-disordered breathing (SDB) with Parkinson's disease (PD) has been reported in some small studies. In the present study we investigated the occurre...An association of obstructive sleep apnea syndrome (OSAS) and other sleep-disordered breathing (SDB) with Parkinson's disease (PD) has been reported in some small studies. In the present study we investigated the occurrence of SDB in a large consecutive outpatient series. This is a case-control study in subjects attending a neurological clinic where all patients were screened for SDB by means of sleep-wake history, Epworth Sleepiness Scale, and full-night polysomnography, when indicated. 3194 patients were recruited. Of these, 194 were affected by PD and 77 by other parkinsonisms. Snoring, excessive daytime sleepiness and OSAS were more common in patients with PD or parkinsonisms (40.59, 5.9, and 4.06%) than in controls (35.58, 2.19, and 2.09%). Our study suggests an increased frequency of OSAS and other SDB in PD and parkinsonisms. Early detection and management of these disorders may have a substantial impact on quality of life and survival in these patients.
Patients who have suffered severe traumatic or nontraumatic brain injuries can show a progressive recovery, transitioning through a range of clinical conditions. They may progress from coma to a vegetative state (VS) and...Patients who have suffered severe traumatic or nontraumatic brain injuries can show a progressive recovery, transitioning through a range of clinical conditions. They may progress from coma to a vegetative state (VS) and/or a minimally conscious state (MCS). A longer duration of the VS is known to be related to a lower probability of emergence from it; furthermore, the literature seems to lack evidence of late improvements in these patients. This real-practice prospective cohort study was conducted in inpatients in a VS following a severe brain injury, consecutively admitted to a vegetative state unit (VSU). The aim of the study was to assess their recovery in order to identify variables that might increase the probability of a VS patient transitioning to MCS. Rehabilitation treatment included passive joint mobilisation and helping/placing patients into an upright sitting position on a tilt table. All the patients underwent a specific assessment protocol every month to identify any emergence, however late, from the VS. Over a 4-year period, 194 patients suffering sequelae of a severe brain injury, consecutively seen, had an initial Glasgow Coma Scale score ≤ 8. Of these, 63 (32.5%) were in a VS, 84 (43.3%) in a MCS, and 47 (24.2%) in a coma; of the 63 patients admitted in a VS, 49 (57.1% males and 42.9% females, mean age 25.34 ± 19.12 years) were transferred to a specialist VSU and put on a slow-to-recover brain injury programme. Ten of these 49 patients were still in a VS after 36 months; of these 10, 3 recovered consciousness, transitioning to a MCS, 2 died, and 5 remained in a VS during the last 12 months of the observation. Univariate analysis identified male sex, youth, a shorter time from onset of the VS, diffuse brain injury, and the presence of status epilepticus as variables increasing the likelihood of transition to a MCS. Long-term monitoring of patients with chronic disorders of consciousness should be adequately implemented in order to optimise their access to rehabilitation services.
Reduced habituation of the nociceptive blink reflex (NBR) is considered a trait marker for genetic predisposition to migraine. In this open-label randomized controlled study, we aimed to test the efficacy of a biofeedbac...Reduced habituation of the nociceptive blink reflex (NBR) is considered a trait marker for genetic predisposition to migraine. In this open-label randomized controlled study, we aimed to test the efficacy of a biofeedback training based on learning of habituation of the NBR (NBR biofeedback) compared with pharmacological (topiramate) treatment and NBR biofeedback plus topiramate treatment in a cohort of migraine without aura patients eligible for prophylaxis. Thirty-three migraine patients were randomly assigned to three months of treatment with: 1) NBR biofeedback, 2) NBR biofeedback plus topiramate 50 mg (b.i.d.), or 3) topiramate 50 mg (b.i.d.). Frequency of headache and disability changes were the main study outcomes. Anxiety, depression, sleep, fatigue, quality of life, allodynia and pericranial tenderness were also evaluated. NBR biofeedback reduced the R2 area, without improving R2 habituation. However, it reduced the frequency of headache and disability, similarly to the combined treatment and topiramate alone. Reduced habituation of the NBR is a stable neurophysiological pattern, scarcely modifiable by learning procedures. Training methods able to act on stress-related responses may modulate cortical mechanisms inducing migraine onset and trigeminal activation under stressful trigger factors.