Abstract Introduction High pain threshold is a supportive diagnosis criterion for Prader–Willi syndrome (PWS), but its pathogenesis is poorly understood. In this study we investigate sensory pathways ...in PWS, in order to evaluate peripheral or central involvement in altered sensory perception. Methods 14 adult PWS patients, 10 obese non-diabetic people and 10 age-matched controls underwent: (a) motor/sensory nerve conduction velocities at the upper and lower limbs; (b) palmar/plantar sympathetic skin response; (c) somatosensory evoked potentials from upper/lower limbs; (d) quantitative sensory testing to measure sensory threshold for vibration, warm and cold sensation, heat and cold-induced pain and (e) blood sample analysis to evaluate glucose and insulin levels and to calculate the quantitative insulin-sensitivity check index (QUICKI). Results Electroneurographic examination, sympathetic skin response and somatosensory evoked potentials were all within normal ranges. In the PWS group, thermal and pain thresholds but not vibratory were significantly higher than in healthy and obese people ( p < 0.05). Sensory threshold did not correlate with BMI nor with QUICKI. Conclusions Our data suggest that altered perception in PWS does not seem attributable to a peripheral nerve derangement due to metabolic factors or obesity. Impairment of the small nociceptive neurons of dorsal root ganglia or involvement of hypothalamic region may not be excluded.
Introduction
Up to one-third of ischemic strokes remained cryptogenic despite extensive investigations. Atrial fibrillation may be detected in a significant proportion of patients with embolic stroke ...of undetermined source, particularly after the introduction of implantable loop recorder in clinical practice.
Methods
We retrospectively included all the consecutive patients with embolic stroke of undetermined source referred to our units in the period November 2013 to December 2018 and in which an implantable loop recorder was positioned within 6 months from stroke event. Prevalence and predictors of atrial fibrillation were investigated.
Results
One hundred thirty-eight patients with embolic stroke of undetermined source fulfilling inclusion criteria were identified. The crude prevalence of atrial fibrillation at the end of observation period was of 45.7%. Incidence rates at 6, 12, 18, 24, and 36 months resulted, respectively, 31.8% (95% CI, 30.4–46.7), 38.0% (95% CI, 30.4–46.9), 42.6% (95% CI, 34.5–51.6), 46.6% (95% CI, 38.2–55.8), and 50.4% (95% CI, 41.6–59.9). On multivariate analysis, only excessive supraventricular electric activity and left atrial enlargement resulted to be significant predictors of atrial fibrillation (
p
= 0.037 and
p
< 0.0001, respectively).
Conclusions
Atrial fibrillation may be detected in a relevant proportion (up to 50%) of patients with embolic stroke of undetermined source if a careful and extensive diagnostic work-up is employed. Excessive supraventricular electric activity and left atrial enlargement are significant predictors of the occurrence of atrial fibrillation in these patients.
Abstract We investigated the gait pattern of 10 patients with myotonic dystrophy (Steinert disease; 4 females, 6 males; age: 41.5 + 7.6 years), compared to 20 healthy controls, through manual muscle ...test and gait analysis, in terms of kinematic, kinetic and EMG data. In most of patients (80%) distal muscle groups were weaker than proximal ones. Weakness at lower limbs was in general moderate to severe and MRC values evidenced a significant correlation between tibialis anterior and gastrocnemius medialis (R = 0.91). An overall observation of gait pattern in patients when compared to controls showed that most spatio-temporal parameters (velocity, step length and cadence) were significantly different. As concerns kinematics, patients' pelvic tilt was globally in a higher position than control group, with reduced hip extension ability in stance phase and limited range of motion; 60% of the limbs revealed knee hyperextension during midstance and ankle joints showed a quite physiological position at initial contact and higher dorsiflexion during stance phase if compared to healthy individuals. Kinetic plots evidenced higher hip power during loading response and lower ankle power generation in terminal stance. The main EMG abnormalities were seen in tibialis anterior and gastrocnemius medialis muscles. In this study gait analysis gives objective and quantitative information about the gait pattern and the deviations due to the muscular situation of these patients; these results are important from a clinical point of view and suggest that rehabilitation programs for them should take these findings into account.
Glucocorticoid (GC)-induced osteoporosis is the leading form of secondary osteoporosis. Bone loss can be rapid. However, longitudinal studies at the very beginning of treatment are scarce. Patients ...relapsing from multiple sclerosis are treated with high-dose, short-term iv GCs. A number of them are young, without concomitant disease affecting bone and with no substantial impairment of mobility. Such patients were selected for the present study. Thirteen patients suffering from multiple sclerosis 11 females, two males; age 32 ± 2 yr (mean ± se) and receiving iv methylprednisolone 15 mg/kg daily for 10 d completed the study. We measured serum osteocalcin (OC), aminoterminal propeptide of type I collagen (PINP), bone isoform of alkaline phosphatase (bALP), carboxyterminal telopeptide of type I collagen (CTX), and urinary calcium/creatinine ratio (uCa/Cr) during the 10-d cycle and 3 months later. Dual-energy x-ray absorptiometry and calcaneal quantitative ultrasonometry were performed before and 6 months after therapy. We found an immediate, impressive fall of OC and PINP (−80 ± 3 and −54 ± 5% at d 2, respectively), which persisted throughout the whole treatment period (P < 0.0001 for both markers). bALP levels showed only a modest decrease at d 6 (−19 ± 7%, P < 0.05), with subsequent return to baseline in d 7–10. After 3 months, OC, PINP, and bALP levels rose to +51 ± 22, +37 ± 16 (not significant), and +61 ± 17% (P < 0.01) with respect to baseline, respectively. uCa/Cr and CTX showed a progressive, marked increase during treatment, peaking at d 7–9 (+92 ± 44 and +149 ± 63%, respectively), with subsequent decrement at d 10 (P < 0.01 and P < 0.05, respectively) despite continuing GC administration. After 3 months, uCa/Cr and CTX levels were also higher than baseline. No change in quantitative ultrasonometry parameters and bone mineral density was observed 6 months after therapy. In conclusion, high-dose, short-term iv GC regimens cause an immediate and persistent decrease in bone formation and a rapid and transient increase of bone resorption. Our data also support the concept that discontinuation of such regimens is followed by a high bone turnover phase.
The physician and patient come into close contact during botulinum toxin treatments, increasing the chances of COVID-19 infection. Therefore, it is essential to use an effective injection method that ...can prevent infection with SARS-CoV-2 virus. In order to minimize the risk of SARS-CoV-2 transmission during botulinum toxin treatment in the COVID-19 era, the Italian Botulinum Toxin Network study group of the Italian Society of Neurology has prepared a video of best practice recommendations on how to organize the work of a clinic performing botulinum toxin treatments.
We studied efficacy, pharmacokinetic and tissue distribution of baclofen incorporated in solid lipid nanoparticles (SLN), after intraperitoneal administration in rats. SLN are able to give a ...sustained release and targeting the CNS. Our study demonstrated prolonged efficacy of this novel formulation of baclofen, even if high baclofen concentrations in brain tissue and sedation require optimization of dosages for clinical purposes.
Intrathecal baclofen administration is the reference treatment for spasticity of spinal or cerebral origin, but the risk of infection or catheter dysfunctions are important limits. To explore the possibility of alternative administration routes, we studied a new preparation comprising solid lipid nanoparticles (SLN) incorporating baclofen (baclofen-SLN). We used SLN because they are able to give a sustained release and to target the CNS. Wistar rats were injected intraperitoneally with baclofen-SLN or baclofen solution (baclofen-sol group) at increasing dosages. At different times up to 4
h, efficacy was tested by the H-reflex and two scales evaluating sedation and motor symptoms due to spinal lesions. Rats were killed and baclofen concentration determined in blood and tissues. Physiological solution or unloaded SLN was used as controls. After baclofen-SLN injection, the effect, consisting in a greater and earlier reduction of the H/M ratio than baclofen-sol group and controls, was statistically significant from a dose of 5
mg/kg and was inversely correlated with dose. Clinical effect of baclofen-SLN on both the behavioral scales was greater than that of baclofen-sol and lasted until 4th hour. Compared with baclofen-sol, baclofen-SLN produced significantly higher drug concentrations in plasma from 2nd hour until 4th hour with a linear decrement and in the brain at all times. In conclusion, our study demonstrated the efficacy of a novel formulation of baclofen, which exploits the advantages of SLN preparations. However, for clinical purposes, high baclofen concentrations in brain tissue and sedation may be unwanted effects, requiring further studies and optimization of dosages.
Acute mountain sickness is a common discomfort experienced by unacclimatized persons on ascent to high altitude. We tested the hypothesis that exposure to high altitude affects cortical excitability ...using transcranial magnetic stimulation. We specifically analyzed the motor cortex excitability in normal subjects at high altitude and in a control condition near sea level. Mean resting motor threshold (RMT) was significantly higher at high altitude than at sea level (69.3 ± 10.4 versus 56.3 ± 10.9%;
P
= 0.042). Mean short intracortical inhibition (SICI) was significantly lower at high altitude than at sea level (percentage of test motor-evoked potential = 79.3 ± 19.8 versus 28.7 ± 17.5%;
P
= 0.0004). Symptoms of acute mountain sickness correlated with resting motor threshold changes induced by high altitude (
R
2
= 0.53,
P
= 0.037). SaO
2
correlated with SICI changes induced by high altitude (
R
2
= 0.45,
P
= 0.036). We suggest that high altitude deeply changes cortical excitability by affecting both inhibitory and excitatory circuits and that this is reflected in acute mountain sickness symptoms.
Byline: Giacinta Miscio (1), Eva Milano (1), Juan Aguilar (3), Giulio Savia (2), Guglielmo Foffani (3), Alessandro Mauro (4), Laura Mordillo-Mateos (3), Javier Romero-Ganuza (3), Antonio Oliviero (3) ...Keywords: Transcranial magnetic stimulation; Motor cortex; Hypoxia; GABA; Acute mountain sickness Acute mountain sickness is a common discomfort experienced by unacclimatized persons on ascent to high altitude. We tested the hypothesis that exposure to high altitude affects cortical excitability using transcranial magnetic stimulation. We specifically analyzed the motor cortex excitability in normal subjects at high altitude and in a control condition near sea level. Mean resting motor threshold (RMT) was significantly higher at high altitude than at sea level (69.3 +- 10.4 versus 56.3 +- 10.9% P = 0.042). Mean short intracortical inhibition (SICI) was significantly lower at high altitude than at sea level (percentage of test motor-evoked potential = 79.3 +- 19.8 versus 28.7 +- 17.5% P = 0.0004). Symptoms of acute mountain sickness correlated with resting motor threshold changes induced by high altitude (R .sup.2 = 0.53, P = 0.037). SaO.sub.2 correlated with SICI changes induced by high altitude (R .sup.2 = 0.45, P = 0.036). We suggest that high altitude deeply changes cortical excitability by affecting both inhibitory and excitatory circuits and that this is reflected in acute mountain sickness symptoms. Author Affiliation: (1) Department of Neurology and Neurorehabilitation, Istituto Auxologico Italiano (IRCCS), San Giuseppe Hospital, Piancavallo, Oggebbio (VB), Italy (2) Department of General Medicine, Istituto Auxologico Italiano (IRCCS), San Giuseppe Hospital, Piancavallo, Oggebbio (VB), Italy (3) FENNSI Group and Servicio de Medicina Interna, Hospital Nacional deParaplejicos, SESCAM, Finca La Peraleda s/n, 45071, Toledo, Spain (4) Department of Neuroscience, University of Turin, Torino, Italy Article History: Registration Date: 29/01/2009 Received Date: 29/12/2008 Accepted Date: 27/01/2009 Online Date: 17/02/2009 Article note: G. Miscio sadly died in autumn 2007.
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