Does primary brachial plexus surgery alter palliative tendon transfer surgery outcomes in children with obstetric paralysis?

<p>Abstract</p> <p>Background</p> <p>The surgical management of obstetrical brachial plexus palsy can generally be divided into two groups; early reconstructions in which the plexus or affected nerves are addressed and late or palliative reconstructions in which the residual deformities are addressed. Tendon transfers are the mainstay of palliative surgery. Occasionally, surgeons are required to utilise already denervated and subsequently reinnervated muscles as motors. This study aimed to compare the outcomes of tendon transfers for residual shoulder dysfunction in patients who had undergone early nerve surgery to the outcomes in patients who had not.</p> <p>Methods</p> <p>A total of 91 patients with obstetric paralysis-related shoulder abduction and external rotation deficits who underwent a modified Hoffer transfer of the latissimus dorsi/teres major to the greater tubercle of the humerus tendon between 2002 and 2009 were retrospectively analysed. The patients who had undergone neural surgery during infancy were compared to those who had not in terms of their preoperative and postoperative shoulder abduction and external rotation active ranges of motion.</p> <p>Results</p> <p>In the early surgery groups, only the postoperative external rotation angles showed statistically significant differences (25 degrees and 75 degrees for total and upper type palsies, respectively). Within the palliative surgery-only groups, there were no significant differences between the preoperative and postoperative abduction and external rotation angles. The significant differences between the early surgery groups and the palliative surgery groups with total palsy during the preoperative period diminished postoperatively (p < 0.05 and p > 0.05, respectively) for abduction but not for external rotation. Within the upper type palsy groups, there were no significant differences between the preoperative and postoperative abduction and external rotation angles.</p> <p>Conclusions</p> <p>In this study, it was found that in patients with total paralysis, satisfactory shoulder abduction values can be achieved with tendon transfers regardless of a previous history of neural surgery even if the preoperative values differ.</p

Increasing cortical excitability: a possible explanation for the pro-convulsivant role of sleep deprivation

STUDY OBJECTIVE: Sleep deprivation (SD) is known to facilitate both seizures and interictal epileptiform abnormalities. For this reason, it is often used in the routine diagnostic workup of epileptic patients as an activating procedure for eliciting epileptiform and/or seizure patterns in their EEGs. In order to evaluate the effects of SD on cortical excitability, we studied the effects of sleep loss on healthy subjects by transcranial magnetic stimulation (TMS). DESIGN AND PARTICIPANTS: Seven normal subjects underwent TMS examination in baseline condition and after total sleep deprivation. The TMS investigation included two protocols: a) the evaluation of motor evoked potential and silent period parameters recorded in response to single-pulse magnetic stimulation; and b) the evaluation of the time course of intracortical motor activity tested with paired-pulse TMS applied at inter-stimulus intervals of 1-6 ms. SETTING: Clinical neurophysiology laboratory in a general hospital. INTERVENTIONS: None. RESULTS: After SD, the principal finding observed using single-pulse TMS was a decrease of the silent period duration, whereas a reduction of the intracortical inhibition, in particular at inter-stimulus intervals 1 and 2 ms, was found, using the paired-pulse TMS. CONCLUSION: Our findings suggest that SD may modify cortical excitability, seen as the balance between inhibitory and excitatory cortical phenomena, which could reduce the epileptic threshold

Increasing cortical excitability: A possible explanation for the proconvulsant role of sleep deprivation

Study Objective: Sleep deprivation (SD) is known to facilitate both seizures and interictal epileptiform abnormalities. For this reason, it is often used in the routine diagnostic workup of epileptic patients as an activating procedure for eliciting epileptiform and/or seizure patterns in their EEGs. In order to evaluate the effects of SD on cortical excitability, we studied the effects of sleep loss on healthy subjects by transcranial magnetic stimulation (TMS). Design and Participants: Seven normal subjects underwent TMS examination in baseline condition and after total sleep deprivation. The TMS investigation included two protocols: a) the evaluation of motor evoked potential and silent period parameters recorded in response to single-pulse magnetic stimulation; and b) the evaluation of the time course of intracortical motor activity tested with paired-pulse TMS applied at inter-stimulus intervals of 1-6 ms. Setting: Clinical neurophysiology laboratory in a general hospital. Interventions: None Results: After SD, the principal finding observed using single-pulse TMS was a decrease of the silent period duration, whereas a reduction of the intracortical inhibition, in particular at inter-stimulus intervals 1 and 2 ms, was found, using the paired-pulse TMS. Conclusion: Our findings suggest that SID may modify cortical excitability, seen as the balance between inhibitory and excitatory cortical phenomena, which could reduce the epileptic threshold

An exploratory case-control study on spinal and bulbar forms of amyotrophic lateral sclerosis in the province of Rome.

Several environmental and life-style factors reported as possibly associated with ALS have been analysed in the present study, focusing on the two clinical onsets of ALS. A case-control study (77 cases and 185 controls) has been performed in the province of Rome in the period 2005-2006. Increased risks were observed in bulbar cases for former smokers (OR: 4.55, 90% CI 1.72-12.08) and more than 24 pack-years, compared with spinal cases for employment in the construction sector and professional exposure to building materials (OR: 5.27, 90% CI 1.15-24.12) and metals (OR: 2.94, 90% CI 1.20-7.21). Overall and bulbar cases showed an increased risk for consumption of cold cuts and a decreased risk for vegetables intake. Regarding head injuries, differences were observed if the last injury occurred in the age range of 30-40 years, among all (OR: 14.2, 90% CI 1.04-194.42) and bulbar (OR: 17.4, 90% CI 1.70-178.5) cases, and less than 30 years among spinal cases (OR: 7.13, 90% CI 1.34-37.94). Moreover, a risk for a time period of 11-30 years since the last head injury suffered was found in bulbar cases (OR: 3.51, 90% CI 1.03-11.95). Some of the hypothesized risk factors for ALS have been found positively associated in this study, with different patterns between bulbar and spinal AL

Creutzfeldt Jakob Disease Associated with the R208H-129V Haplotype in the Protein Gene

Human Transmissible Spongiform Encephalopaties (TSEs) or prion diseases occur in sporadic, acquired and genetic forms. All the genetic forms of human TSEs are linked to point or insertion mutations of the PRNP and several of these genetic forms are transmissible. Among the rarer point mutations, R208H was reported in 3 patient (JA. Mastrianni; et al. 1996; S. Capellari et al. 2005; C. Basset-Leobon et al. 2006). Here we described the clinical and pathological features of the CJD phenotype associated with the R208H-129VV haplotype in an Italian patient. Case report A 64-year-old woman was referred to the Italian National Register of the Creutzfeldt-Jakob disease and related disorders, with a clinical presentation characterized by mood disorder and ataxia. Two months after the onset, she showed cognitive decay, cerebellar symptoms, pyramidal symptoms involving the left leg, and a progressive action myoclonus of left hand. Four months later the patient was bed ridden in a state of akinetic mutism. The family history was negative for dementia or neurological disorders. The protein 14-3-3 test gave a positive result. The EEG became typical 4 months after the onset. The MRI of the brain showed mild hyperintense signals in the basal ganglia on T2-weighted images. The sequence of the PRNP gene ORF revealed a mutation at the codon 208 with the substitution of histidine for arginine (R208H) and the polymorphism Val/Val at the level of the codon 129. Death occurred 28 months after onset. Neuropathological examination showed severe spongiform changes in the cerebral cortex, striatum, thalamus and cerebellum. Conclusions: this report is the first on the R208H mutation found in association with val/val polymorphism in Italy and it strengthens the linkage of the R208H mutation to CJD