The clinical-radiological and biological overlap of the spectrum of pediatric demyelinating disorders makes the diagnostic process of a child with an acquired demyelinating syndrome truly ...challenging. We present a 9-year-old girl with subacute symptoms of severe decrease in bilateral visual acuity and gait ataxia. An urgent MRI showed inflammatory-demyelinating lesions affecting the periaqueductal gray matter, the cerebellar hemispheres, the area postrema as well as both optic nerves and chiasm. Likewise, multisegmental involvement of the cervical and dorsal spinal cord was found, with short and peripheral lesions. Anti myelin oligodendrocyte glycoprotein (MOG) antibodies (Abs) were positive in cerebrospinal fluid (CSF) and weakly in serum. Oligoclonal bands (OB) were positive in CSF. Based on all this, the diagnosis of MOG antibody disease (MOGAD) with a neuromyelitis optica spectrum disorder (NMOSD)-like picture was made. Given the good clinical and radiological recovery after the acute phase treatment, and that anti MOG Abs became negative, it was decided to keep the patient without specific treatment. However, during follow-up, while the patient was asymptomatic, a control brain MRI showed the appearance of new lesions with morphology and topography suggestive of multiple sclerosis (MS). This, added to the presence of OB, made the diagnosis of pediatric-onset MS (POMS) likely. Immunosuppressive treatment was restarted with a good response since then. Unlike adult-onset MS, children with POMS may usually not have entirely typical clinical and radiological features at presentation. In many cases, the time factor and close clinical and radiological monitoring could be critical to make an accurate diagnosis.
Despite advances in microsurgery, full functional recovery of severe peripheral nerve injuries is not commonly attained. The sheep appears as a good preclinical model since it presents nerves with ...similar characteristics to humans. In this study, we induced 5 or 7 cm resection in the peroneal nerve and repaired with an autograft. Functional evaluation was performed monthly. Electromyographic and ultrasound tests were performed at 6.5 and 9 months postoperation (mpo). No significant differences were found between groups with respect to functional tests, although slow improvements were seen from 5 mpo. Electrophysiological tests showed compound muscle action potentials (CMAP) of small amplitude at 6.5 mpo that increased at 9 mpo, although they were significantly lower than the contralateral side. Ultrasound tests showed significantly reduced size of tibialis anterior (TA) muscle at 6.5 mpo and partially recovered size at 9 mpo. Histological evaluation of the grafts showed good axonal regeneration in all except one sheep from autograft 7 cm (AG7) group, while distal to the graft there was a higher number of axons than in control nerves. The results indicate that sheep nerve repair is a useful model for investigating long-gap peripheral nerve injuries.
Background
Up to 20% of the cases of anorexia nervosa (AN) are chronic and treatment‐resistant. Recently, the efficacy of deep brain stimulation (DBS) for severe cases of AN has been explored, with ...studies showing an improvement in body mass index and other psychiatric outcomes. While the effects of DBS on cognitive domains have been studied in patients with other neurological and psychiatric conditions so far, no evidence has been gathered in AN.
Methods
Eight patients with severe, chronic, treatment‐resistant AN received DBS either to the nucleus accumbens (NAcc) or subcallosal cingulate (SCC; four subjects on each target). A comprehensive battery of neuropsychological and clinical outcomes was used before and 6‐month after surgery.
Findings
Although Body Mass Index (BMI) did not normalise, statistically significant improvements in BMI, quality of life, and performance on cognitive flexibility were observed after 6 months of DBS. Changes in BMI were related to a decrease in depressive symptoms and an improvement in memory functioning.
Interpretation
These findings, although preliminary, support the use of DBS in AN, pointing to its safety, even for cognitive functioning; improvements of cognitive flexibility are reported. DBS seems to exert changes on cognition and mood that accompany BMI increments. Further studies are needed better to determine the impact of DBS on cognitive functions.
Highlights
Although body mass index (BMI) did not normalise, statistically significant improvements in BMI, quality of life, and performance on cognitive flexibility were observed after 6 months of continuous DBS stimulation either to the nucleus accumbens or subcallosal cingulate
An increase in BMI was related to a decrease in depressive symptoms and an improvement in memory functioning
These preliminary findings suggest that DBS is not associated with any cognitive deterioration in people with AN
One of the most sought-after applications of neuroengineering is the communication between the arm and an artificial prosthetic device for the replacement of an amputated hand or the treatment of ...peripheral nerve injuries. For that, an electrode is placed around or inside the median nerve to serve as interface for recording and stimulation of nerve signals coming from the fascicles that innervate the muscles responsible for hand movements. Due to the lack of a standard procedure, the electrode implantation by the surgeon is strongly based on intuition, which may result in poor performance of the neuroprosthesis because of the suboptimal location of the neural interface. To provide morphological data that can aid the neuroprosthetic surgeon with this procedure, we investigated the fascicular topography of the human median nerve along the forearm and upper arm. We first performed a description of the fascicular content and branching patterns along the length of the arm. Next we built a 3D reconstruction of the median nerve so we could analyze the fascicle morphological features in relation to the arm level. Finally, we characterized the motor content of the median nerve fascicles in the upper arm. Collectively, these results indicate that fascicular organization occurs in a short segment distal to the epicondyles and remains unaltered until the muscular branches leave the main trunk. Based on our results, overall recommendations based on electrode type and implant location can be drawn to help and aid the neuroprosthetic procedure. Invasive interfaces would be more convenient for the upper arm and the most proximal third of the forearm. Epineural electrodes seem to be most suitable for the forearm segment after fascicles have been divided from the main trunk.
This paper demonstrates flexible epineural strip electrodes (FLESE) for recording from small nerves. Small strip-shaped FLESE enables us to easily and closely stick on various sized nerves for less ...damage in a nerve and optimal recording quality. In addition, in order to enhance the neural interface, the gold electrode contacts were coated with carbon nanotubes, which reduced the impedance of the electrodes. We used the FLESEs to record electrically elicited nerve signals (compound neural action potentials) from the sciatic nerve in rats. Bipolar and differential bipolar configurations for the recording were investigated to optimize the recording configuration of the FLESEs. The successful results from differential bipolar recordings showed that the total length of FLESEs could be further reduced, maintaining the maximum recording ability, which would be beneficial for recording in very fine nerves. Our results demonstrate that new concept of FLESEs could play an important role in electroceuticals in near future.
Benign external hydrocephalus (BEH) is considered a self-limiting pathology with a good prognosis. However, some children present a pathological intracranial pressure (ICP) characterized by ...quantitative and qualitative alterations (the so-called B-waves) that can lead to neurological sequelae.
Our purpose was to evaluate whether there were cerebral hemodynamic changes associated with ICP B-waves that could be evaluated with noninvasive neuromonitoring.
We recruited eleven patients (median age 16 months, range 7 to 55 months) with BEH and an unfavorable evolution requiring ICP monitoring. Bedside, nocturnal monitoring using near-infrared time-resolved and diffuse correlation spectroscopies synchronized to the clinical monitoring was performed.
By focusing on the timing of different ICP patterns that were identified manually by clinicians, we detected significant tissue oxygen saturation (
) changes (
) and blood flow index (BFI) variability (
) between regular and high-amplitude B-wave patterns. A blinded analysis looking for analogs of ICP patterns in BFI time traces achieved 90% sensitivity in identifying B-waves and 76% specificity in detecting the regular patterns.
We revealed the presence of
and BFI variations-detectable with optical techniques-during ICP B-waves in BEH children. Finally, the feasibility of detecting ICP B-waves in hemodynamic time traces obtained noninvasively was shown.
Dual-comb spectroscopy has become a topic of growing interest in recent years due to the advantages it offers in terms of frequency resolution, accuracy, acquisition speed, and signal-to-noise ratio, ...with respect to other existing spectroscopic techniques. In addition, its characteristic of mapping the optical frequencies into radio-frequency ranges opens up the possibility of using non-demanding digitizers. In this paper, we show that a low-cost software defined radio platform can be used as a receiver to obtain such signals accurately using a dual-comb spectrometer based on gain-switched semiconductor lasers. We compare its performance with that of a real-time digital oscilloscope, finding similar results for both digitizers. We measure an absorption line of a H 13 C 14 N cell and obtain that for an integration time of 1 s, the deviation obtained between the experimental data and the Voigt profile fitted to these data is around 0.97% using the low-cost digitizer while it is around 0.84% when using the high-end digitizer. The use of both technologies, semiconductor lasers and low-cost software defined radio platforms, can pave the way towards the development of cost-efficient dual-comb spectrometers.
Segregation of regenerating motor and sensory axons may be a good strategy to improve selective functionality of regenerative interfaces to provide closed‐loop commands. Provided that extracellular ...matrix components and neurotrophic factors exert guidance effects on different neuronal populations, we assessed in vivo the potential of separating sensory and motor axons regenerating in a bicompartmental Y‐type tube, with each branch prefilled with an adequate combination of extracellular matrix and neurotrophic factors.
The severed rat sciatic nerve was repaired using a bicompartmental tube filled with a collagen matrix enriched with fibronectin (FN) and brain‐derived neurotrophic factor (BDNF) encapsulated in poly‐lactic co‐glycolic acid microspheres (FN + MP.BDNF) in one compartment to preferentially attract motor axons and collagen enriched with laminin (LM) and nerve growth factor (NGF) and neurotrophin‐3 (NT‐3) in microspheres (LM + MP.NGF/NT‐3) in the other compartment for promoting sensory axons regeneration. Control animals were implanted with the same Y‐tube with a collagen matrix with microspheres (MP) containing PBS (Col + MP.PBS).
By using retrotracer labelling, we found that LM + MP.NGF/NT‐3 did not attract higher number of regenerated sensory axons compared with controls, and no differences were observed in sensory functional recovery. However, FN + MP.BDNF guided a higher number of regenerating motor axons compared with controls, improving also motor recovery. A small proportion of sensory axons with large soma size, likely proprioceptive neurons, was also attracted to the FN + MP.BDNF compartment. These results demonstrate that muscular axonal guidance can be modulated in vivo by the addition of fibronectin and BDNF.
The SNAP receptor (SNARE) complex, consisting of synaptosome-associated protein of 25 kDa (SNAP-25), synaptobrevin-2, and syntaxin-1, is involved in synaptic vesicles exocytosis. In addition, SNAP-25 ...has been implicated in constitutive exocytosis processes required for neurite outgrowth. However, at least three isoforms of SNAP-25 have been reported from neurons: SNAP-23, which is also present in non-neuronal cells, and the two alternative splice variants SNAP-25a and SNAP-25b. Here, we studied the differential ability of these isoforms to support the functions previously broadly ascribed to "SNAP-25." We studied the rescue of snap-25 null neurons in culture with different SNAP-25 homologs. We find that deletion of SNAP-25 leads to strongly reduced neuron survival, and, in the few surviving cells, impaired arborization, reduced spontaneous release, and complete arrest of evoked release. Lentiviral expression of SNAP-25a, SNAP-25b, or SNAP-23 rescued neuronal survival, arborization, amplitude, and frequency of spontaneous events. Also evoked release was rescued by all isoforms, but synchronous release required SNAP-25a/b in both glutamatergic and GABAergic neurons. SNAP-23 supported asynchronous release only, reminiscent of synaptotagmin-1 null neurons. SNAP-25b was superior to SNAP-25a in vesicle priming, resembling the shift to larger releasable vesicle pools that accompanies synaptic maturation. These data demonstrate a differential ability of SNAP-25b, SNAP-25a, and SNAP-23 to support neuronal function.
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