| 1. |
- Bontioti, Eleana, et al.
(författare)
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End-to-side nerve repair in the upper extremity of rat.
- 2005
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489 .- 1529-8027. ; 10:1, s. 58-68
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Tidskriftsartikel (refereegranskat)abstract
- The end-to-side nerve-repair technique, i.e., when the distal end of an injured nerve is attached end-to-side to an intact nerve trunk in an attempt to attract nerve fibers by collateral sprouting, has been used clinically. The technique has, however, been questioned. The aim of the present study was to investigate end-to-side repair in the upper extremity of rats with emphasis on functional recovery, source, type, and extent of regenerating fibers. End-to-side repair was used in the upper limb, and the radial or both median/ulnar nerves were attached end-to-side to the musculocutaneous nerve. Pawprints and tetanic muscle force were used to evaluate functional recovery during a 6-month recovery period, and double retrograde labeling was used to detect the source of the regenerated nerve fibers. The pawprints showed that, in end-to-side repair of either one or two recipient nerves, there was a recovery of toe spreading to 60-72% of the preoperative value (lowest value around 47%). Electrical stimulation of the end-to-side attached radial or median/ulnar nerves 6 months after repair resulted in contraction of muscles in the forearm innervated by these nerves (median tetanic muscle force up to 70% of the contralateral side). Retrograde labeling showed that both myelinated (morphometry) sensory and motor axons were recruited to the end-to-side attached nerve and that these axons emerged from the motor and sensory neuronal pool of the brachial plexus. Double retrograde labeling indicated that collateral sprouting was one mechanism by which regeneration occurred. We also found that two recipient nerves could be supported from a single donor nerve. Our results suggest that end-to-side repair may be one alternative to reconstruct a brachial plexus injury when no proximal nerve end is available.
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| 2. |
- Bontioti, Eleana N, et al.
(författare)
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Regeneration and functional recovery in the upper extremity of rats after various types of nerve injuries.
- 2003
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489 .- 1529-8027. ; 8:3, s. 159-168
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Tidskriftsartikel (refereegranskat)abstract
- The aim was to establish an accurate, reproducible, and simple method to evaluate functional recovery after different types of nerve injuries to the brachial plexus of rats. To that end, pawprints, measured as distance between the first and fourth and second and third digits, were used for evaluation of injuries including crush injury, transection/repair, or graft repair of the median, ulnar, and radial nerves. Immunocytochemistry of the C-terminal flanking peptide of neuropeptide Y (CPON) and neurofilaments was used to investigate the cell body response and axonal outgrowth, respectively. Functional recovery was dependent on the severity as well as on the level of the lesion. Neither a single injury to the median nerve nor an injury to the ulnar nerve affected the pawprint, while an injury to both these nerves or a single injury to the radial nerve caused impairment of pawprints. There was a rapid recovery after crush injury to these nerves compared to previous reports of a similar injury to the sciatic nerve. The pattern of axonal outgrowth was related to the severity of the lesion. A conditioning lesion, i.e., an initial lesion of the same nerve preceding a test injury by a few days, of both motor/sensory fibers led to a quicker functional recovery. Surprisingly, conditioning of only sensory fibers had nearly the same effect. The cell body response was dependent on the level of the nerve lesion. The upper extremity of rats might be useful to evaluate the effects of new repair methods after nerve injuries using functional evaluation with pawprints as a simple and accurate method
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| 3. |
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| 4. |
- Haapaniemi, T, et al.
(författare)
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Functional evaluation after rat sciatic nerve injury followed by hyperbaric oxygen treatment
- 2002
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489 .- 1529-8027. ; 7:3, s. 149-154
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Tidskriftsartikel (refereegranskat)abstract
- Previous experimental studies have shown positive effects of hyperbaric oxygen treatment in the early regeneration phase in the first few days following a nerve injury. In this study, functional effects of hyperbaric oxygen treatment were studied in 2 series of rats after an injury to the sciatic nerve - a standardized crush injury and nerve transection and repair, respectively. Postoperatively the animals were treated with 100% oxygen at 2.5 atmospheres absolute pressure for 90 minutes and the treatment was employed twice daily for 7 days. The animals were evaluated with walking track analysis up to twice weekly. The experiments were terminated after 90 days when the tetanic force was measured in the tibial anterior and gastrocnemius muscles. No statistically significant differences were found in either of these tests. It is concluded that hyperbaric oxygen treatment, given in accordance with clinical protocols used in limb crush injuries and other peripheral conditions, was not effective in the restoration of gait or the muscular strength after 90 days in rats after these nerve injuries. This study does not support nerve crush injury or nerve transection and repair as indications for hyperbaric oxygen treatment.
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| 5. |
- Kvist, Martin, et al.
(författare)
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Costimulation blockade in transplantation of nerve allografts: long-term effects.
- 2008
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1529-8027 .- 1085-9489. ; 13:3, s. 200-207
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Tidskriftsartikel (refereegranskat)abstract
- Costimulation blockade can prevent rejection of nerve allografts in short-term studies. We tested if costimulation blockade also prevented rejection of nerve allografts in long-term experiments, thereby improving functional recovery. A 7-mm sciatic nerve defect in C57/BL6 mice was bridged either by nerve allografts from Balb/C mice or by isogenic nerve grafts (isografts) from C57/BL6 mice. Costimulation blockade in the form of a triple treatment with anti-LFA-1, anti-CD40L, and CTLA4Ig was given at post-operative days 0, 2, 4, and 6 (intraperitoneal). Control mice (placebo; allografts) with nerve grafts were treated with isotype antibodies during the same time period. After 49 days, tetanic muscle force, wet weight of gastrocnemius muscle, histology, and morphometry in the tibial nerve were evaluated. Costimulation blockade diminished rejection of the nerve allografts. Axons bridged the graft. Treatment increased wet weight of the gastrocnemius muscle and resulted in a higher mean myelin area/nerve fiber in the tibial nerve distal to the nerve grafts. Tetanic muscle force and number of axons in tibial nerve showed no differences between groups. We conclude that rejection is suppressed by costimulation blockade. Treatment improves recovery of target muscle and myelination after nerve allografting.
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| 6. |
- Kvist, Martin, et al.
(författare)
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Effects of FK506 on regeneration and macrophages in injured rat sciatic nerve.
- 2003
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489 .- 1529-8027. ; 8:4, s. 251-259
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Tidskriftsartikel (refereegranskat)abstract
- Effects of FK506 [5.0 mg/kg body weight (BW), subcutaneous, daily] on nerve regeneration and presence of macrophages in lesioned rat sciatic nerves were studied. Models of autologous nerve graft or a nerve crush lesion were used and regeneration was evaluated by immunocytochemistry (also used to detect ED1/ED2 macrophages) and sensory pinch reflex test, respectively. Treatment with FK506 did not increase regeneration distance or regeneration rate in the autologous nerve grafts. However, regeneration distances after nerve crush were significantly longer following treatment with FK506. The number of macrophages (ED1/ED2) in nerve grafts increased over time, but treatment with FK506 had limited effects only in the presence of ED2 macrophages. Present and previously published studies may imply that there is a time-related and type-of-injury-related profile of FK506's pro-regenerative effect.
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| 7. |
- Kvist, Martin, et al.
(författare)
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Immunomodulation by costimulation blockade inhibits rejection of nerve allografts
- 2007
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489 .- 1529-8027. ; 12:2, s. 83-90
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Tidskriftsartikel (refereegranskat)abstract
- The aim of this study was to investigate if costimulation blockade could be used to modulate the immune response, to prevent rejection, and to stimulate regeneration into nerve allografts. Nerve allografts from Balb/C mice, and isogenic nerve grafts (isografts) from C57/BL6 mice, were used to bridge a 7-mm gap of the sciatic nerve in C57/BL6 mice. Allograft recipients were treated with either a triple treatment with anti-lymphocyte function antigen-1 (anti-LFA), anti-CD40 ligand (anti-CD40L), and cytotoxic T-lymphocyte antigen 4 immunoglobulin (anti-CTLA4Ig) or isotype antibodies (placebo) at postoperative days 0, 2, 4, and 6 (intraperitoneal). After 5 or 9 days, the nerve grafts, together with the proximal and the distal nerve segments, were evaluated by histology and immunocytochemistry for inflammatory cells [CD4-positive (CD4+) and CD8-positive (CD8+) staining cells] and axonal outgrowth (neurofilaments). The immune response was inhibited by costimulation blockade with less extensive inflammation and a lower number of CD4+ staining cells in triple-treated allografts at 9 days. The regeneration rate was significantly faster in isografts (0.75 mm/day) compared with allografts with placebo treatment (0.39 mm/day), but not when compared with triple-treated allografts (0.49 mm/day). At 9 days, the axons were significantly longer in nerve isografts than in nerve allografts, irrespective of treatment. Hence, costimulation blockade neither increased the regeneration rate nor the outgrowth length in triple-treated allografts. We conclude that costimulation blockade inhibits the immune response in nerve allografts without deterring early axonal outgrowth.
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| 8. |
- Lundborg, Göran
(författare)
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Richard P. Bunge memorial lecture. Nerve injury and repair--a challenge to the plastic brain.
- 2003
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489 .- 1529-8027. ; 8:4, s. 209-226
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Tidskriftsartikel (refereegranskat)abstract
- Repair and reconstruction of major nerve trunks in the upper extremity is a very challenging surgical problem. Today, there is no surgical repair technique that can assure recovery of tactile discrimination in the hand of an adult patient following nerve repair. In contrast, young individuals usually attain a complete recovery of functional sensibility. The outcome from nerve repair depends mainly on central nervous system factors including functional cortical reorganizational processes caused by misdirection in axonal outgrowth. Deafferentation due to local anesthetic block, amputation or nerve transection in the upper extremity leads to very rapid cortical synaptic remodeling, resulting in a distorted cortical hand representation as well as in enlarged and overlapping cortical receptive fields. Sensory relearning programs are aimed at refinement of these receptive fields to normalize the distorted hand map and improve processing at a high-order cortical level in the context of the 'new language spoken by the hand'. As peripheral nerve repair techniques cannot be further refined, there is a need for new and improved strategies for sensory relearning following nerve repair. We propose the utilization of multimodal capacity of the brain, using another sense (hearing) to substitute for lost hand sensation and to provide an alternate sensory input from the hand early after transection. The purpose was to modulate cortical reorganizations due to deafferentation to preserve cortical hand representation. Preliminary results from a prospective clinical randomized study indicate that the use of a Sensor Glove System, which stereophonically transposes the friction sound elicited by active touch, results in improved recovery of tactile discrimination in the nerve-injured hand. Future strategies for treatment of nerve injuries should promote cellular methods to minimize post-traumatic nerve cell death and to improve axonal outgrowth rate and orientation, but high on the agenda are new strategies for refined sensory relearning following nerve repair.
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| 9. |
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| 10. |
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| 11. |
- Lindh, Jonas, et al.
(författare)
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Cryptogenic polyneuropathy : Clinical and neurophysiological findings
- 2005
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Ingår i: Journal of the peripheral nervous system. - : Wiley. - 1085-9489 .- 1529-8027. ; 10:1, s. 31-37
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Tidskriftsartikel (refereegranskat)abstract
- The purpose of this study was to describe the clinical and neurophysiological features of cryptogenic polyneuropathy in 168 patients in the neurological departments at three Swedish hospitals. The medical records of all patients aged 40-79 years with the diagnosis of cryptogenic polyneuropathy from 1993 to 2000 were analysed. One hundred and fourteen patients (68%) were men. The mean age at first symptom was 61 years and at diagnosis it was 64 years. Distal numbness (n=115, 68%) was the most common symptom, but some patients complained of pain, pedal paresthesiae, and impairment of balance. The most common clinical findings were decreased or lost proprioception or sense of vibration (n=135, 80%) and loss of ankle jerks (n=131, 78%). Neurography in 139 patients showed mixed sensorimotor polyneuropathy of axonal or mixed axonal and demyelinating type in 97 (70%). Cryptogenic polyneuropathy is a slowly progressive sensorimotor nerve lesion of mainly axonal type. Men are more often affected than women. Most patients have a minor or moderate severe polyneuropathy.
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| 12. |
- Nishiura, Y, et al.
(författare)
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Hyperbaric oxygen treatment has different effects on nerve regeneration in acellular nerve and muscle grafts
- 2001
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Ingår i: Journal of the peripheral nervous system. - : Wiley. - 1085-9489 .- 1529-8027. ; 6:2, s. 73-78
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Tidskriftsartikel (refereegranskat)abstract
- Effects of hyperbaric oxygen treatment (HBO) on nerve regeneration in acellular nerve and muscle grafts were investigated in rats. Nerve and muscle grafts were made acellular by freeze-thawing and the obtained grafts were used to bridge a 10-mm gap in the sciatic nerve on the left and right sides, respectively. Rats were treated with HBO (100% oxygen for 90 minutes at 2.5 atmospheres absolute pressure ATA) twice a day for 7 days. Axonal outgrowth, Schwann cell migration and invasion of macrophages were examined 10 days after the graft procedure by staining neurofilaments, S-100 proteins and the macrophage antibodies ED1 and ED2, respectively. Axonal outgrowth and Schwann cell migration in acellular nerve grafts were superior to that found in the acellular muscle grafts. However, there was no difference between HBO-treated and non-treated rats in acellular nerve grafts. Such a difference was found in acellular muscle grafts concerning both axonal outgrowth and Schwann cell migration from the proximal nerve end. No differences in the content of macrophages or neovascularization (alkaline phosphatase staining) in either of the grafts and treatments were seen. It is concluded that there is a differential effect of HBO-treatment in acellular nerve and muscle grafts and that HBO-treatment has no effect on the regeneration process in acellular nerve grafts, in contrast to fresh cellular nerve grafts where a beneficial effect has previously been reported.
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| 13. |
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| 14. |
- Schmidt, Roland, et al.
(författare)
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Time course of acetylcholine-induced activation of sympathetic efferents matches axon reflex sweating in humans
- 2011
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Ingår i: Journal of the peripheral nervous system. - : Wiley. - 1085-9489 .- 1529-8027. ; 16:1, s. 30-36
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Tidskriftsartikel (refereegranskat)abstract
- Action potentials from postganglionic C-fibres were recorded in healthy volunteers by microneurography in the peroneal nerve. Their responsiveness to mechanical or heat stimuli or to sympathetic reflex provocation tests was determined by transient slowing of conduction velocity following activation. Twenty units were classified as sympathetic efferent units. Acetylcholine (ACh) iontophoresis (10%, 1 mA, 1 min) inside their innervation territory activated 8 of 20 sympathetic fibres with a mean delay of 61 +/- 12 s, peak response at 175 +/- 38 s, and a duration of 240 +/- 42 s, whereas iontophoresis of saline did not activate any of them. The time course of neuronal activation correlated with the axon reflex sweating measured by an evaporimeter in a separate session (delay 76 +/- 9 s, peak at 195 +/- 12 s, decline to 50% of peak 312 +/- 25 s). No ACh-induced vasoconstriction was observed by laser Doppler scanning (n = 11) even after depletion of neuropeptides by chronic topical capsaicin treatment (n = 8). We conclude that ACh iontophoresis activates about half of the sympathetic fibres in human skin and provokes a corresponding axon reflex sweating. The absence of ACh-induced vasoconstriction even after the depletion of neuropeptides by capsaicin suggests that only sudomotor fibres, but not sympathetic vasoconstrictor fibres are activated by this stimulus.
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| 15. |
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| 16. |
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| 17. |
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| 18. |
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| 19. |
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| 20. |
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| 21. |
- de Carvalho, Mamede, et al.
(författare)
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Peripheral neuropathy in ALS : Phenotype association
- 2018
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Ingår i: Journal of the peripheral nervous system. - : John Wiley & Sons. - 1085-9489 .- 1529-8027. ; 23:4, s. 391-392
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)
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| 22. |
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| 23. |
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| 24. |
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| 25. |
- Jergovic, Davor, et al.
(författare)
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Repair of the mandibular branch of the rat facial nerve through transmedian grafting in one or two stages : Functional evaluation
- 1998
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Ingår i: Journal of the peripheral nervous system. - 1085-9489 .- 1529-8027. ; 3:1, s. 54-62
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Tidskriftsartikel (refereegranskat)abstract
- A previous study examined the morphological outcome of axonal regeneration in the mandibular branch (ramus marginalis mandibulae) of the rat facial nerve after transmedian nerve grafting in one or two stages. The present study supplements the morphological data with a functional evaluation. Recordings of the force of tetanic muscle contractions elicited through stimulation of the mandibular branch showed that upper and lower lip data obtained from animals grafted in one stage did not differ significantly from control data. However, animals grafted in two stages exhibited significantly lower muscle forces compared to one-stage data and to control data. Electromyographic recordings of the M-response showed multiple prolonged potential fluctuations with subnormal amplitudes in grafted cases. In both groups of grafted rats, the mean voltage amplitudes recorded from the upper lip were weaker than the amplitudes seen at the angle of the mouth or the lower lip. The two-stage cases exhibited the most obvious deficit. In conclusion, the present results show that, with respect to the functional restoration achieved three months after nerve injury, repair through transmedian grafting in one stage gives better results than repair in two stages. This finding, which conforms with previous morphological data, suggests that the one-stage procedure should be considered for clinical use.
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| 26. |
- Jergovic, Davor, et al.
(författare)
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Repair of the mandibular branch of the rat facial nerve through transmedian grafting in one or two stages : Morphological evaluation
- 1997
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Ingår i: Journal of the peripheral nervous system. - 1085-9489 .- 1529-8027. ; 2:2, s. 181-188
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Tidskriftsartikel (refereegranskat)abstract
- This study examined by electron microscopy the normal fibre composition of the mandibular branch (MB) of the rat facial nerve and the outcome of axon regeneration in the MB after transmedian grafting in one or two stages. The average normal MB contained 2,185 axons, 17 % of which were unmyelinated. The myelinated axons had a unimodal diameter distribution (range 1.5-9.5 μm, mode 4.5 μm). After superior cervical ganglionectomy, the MB lost 1/3 of the C-fibres and 10% of the myelinated axons. In neonatally capsaicin-treated rats the occurrence of unmyelinated axons was reduced by about 50%. After repair in one or two stages the MB contained more myelinated and unmyelinated axons than normal. The myelinated axons showed a unimodal size distribution with a subnormal diameter range. Statistical comparisons showed that MBs from both experimental groups were significantly abnormal with respect to total axon number as well as numbers of unmyelinated and myelinated axons. In these respects the grafted MBs did not differ significantly from each other. However, the myelinated axons in MBs from one-stage cases showed larger mean and maximum diameters compared to MBs from two-stage cases. These data suggest that the normal MB of the rat contains myelinated and unmyelinated sympathetic axons and that about half the C-fibres in the normal MB come from capsaicin-sensitive sensory neurons. The comparison of the two reparative procedures used provides evidence in favor of the one-stage alternative.
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| 27. |
- Kapoor, M., et al.
(författare)
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Plasma neurofilament light chain concentration is increased and correlates with the severity of neuropathy in hereditary transthyretin amyloidosis
- 2019
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489 .- 1529-8027. ; 24:4, s. 314-319
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Tidskriftsartikel (refereegranskat)abstract
- Hereditary transthyretin amyloidosis (ATTRm) causes a disabling peripheral neuropathy as part of a multisystem disorder. The recent development of highly effective gene silencing therapies has highlighted the need for effective biomarkers of disease activity to guide the decision of when to start and stop treatment. In this study, we measured plasma neurofilament light chain (pNfL) concentration in 73 patients with ATTR and found that pNfL was significantly raised in ATTRm patients with peripheral neuropathy compared to healthy controls. Furthermore, pNFL correlated with disease severity as defined by established clinical outcome measures in patients for whom this information was available. These findings suggest a potential role of pNfL in monitoring disease activity and progression in ATTRm patients.
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| 28. |
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| 29. |
- Liu, Li, et al.
(författare)
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Hereditary absence of complement C5 in adult mice influences Wallerian degeneration, but not retrograde responses, following injury to peripheral nerve
- 1999
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Ingår i: Journal of the peripheral nervous system. - 1085-9489 .- 1529-8027. ; 4:2, s. 123-133
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Tidskriftsartikel (refereegranskat)abstract
- We have examined the role of complement component 5 (C5) in peripheral nerve fiber degeneration and regeneration, as well as in glial and neuronal cell responses in the central nervous system (CNS). Adult congenic mice lacking C5 (C5(-)) and the corresponding normal strain (C5(+)) were used. Macrophage recruitment as well as axonal and myelin sheath elimination were delayed from 1 to 21 days postinjury in C5(-) mice compared to the C5(+) group after sciatic nerve crush. Despite this, recovery of motor function was not delayed. In the CNS, microglial cells and astrocytes responded in the same way from 3 to 21 days after sciatic nerve injury in C5(-) and C5(+) mice, and the extent of neuron death following hypoglossal nerve avulsion was the same in both groups. These findings suggest that C5 and/or its derivatives play an important role in initiating the recruitment of macrophages to the injured nerve and, probably indirectly, in early remyelination of regenerating axons, but does not influence the longterm functional restoration or axotomy-induced nerve cell death. C5-derived molecules do not appear to participate in central glial cell responses to peripheral nerve injury. These findings elucidate new aspects on the functional role of the complement system in the peripheral nervous system following peripheral nerve injury.
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| 30. |
- Løseth, Sissel, et al.
(författare)
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Small and large fiber neuropathy in those with type 1 and type 2 diabetes : a 5-year follow-up study
- 2016
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Ingår i: Journal of the peripheral nervous system. - : Wiley. - 1085-9489 .- 1529-8027. ; 21:1, s. 15-21
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Tidskriftsartikel (refereegranskat)abstract
- The purpose of this study was to evaluate progression of diabetic polyneuropathy and differences in the spectrum and evolution of large- and small-fiber involvement in patients with diabetes type 1 and 2 over 5 years. Fifty-nine patients (35 type 1 and 24 type 2) were included. Nerve conduction studies (NCS), quantitative sensory testing, skin biopsy for quantification of intraepidermal nerve fiber density (IENFD), symptom scoring and clinical evaluations were performed. Z-scores were calculated to adjust for the physiologic effects of age and height/gender. Neuropathic symptoms were not significantly more frequent in type 2 than in type 1 diabetic patients at follow-up (54% vs. 37%). The overall mean NCS Z-score remained within the normal range, but there was a small significant decline after 5 years in both groups: type 1 (p = 0.004) and type 2 (p = 0.02). Mean IENFD Z-scores changed from normal to abnormal in both groups, but only significantly in those with type 2 diabetes (reduction from 7.9 +/- 4.8 to 4.3 +/- 2.8 fibers/mm, p = 0.006). Cold perception threshold became more abnormal only in those with type 2 diabetes (p = 0.049). There was a minimal progression of large fiber neuropathy in both groups. Reduction of small fibers predominated and progressed more rapidly in those with type 2 diabetes.
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| 31. |
- Magistroni, Ernesta, et al.
(författare)
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Cold intolerance and neuropathic pain after peripheral nerve injury in upper extremity
- 2020
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489 .- 1529-8027. ; 25:2, s. 184-190
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Tidskriftsartikel (refereegranskat)abstract
- Cold intolerance and pain can be a substantial problem in patients with peripheral nerve injury. We aimed at investigating the relationships among sensory recovery, cold intolerance, and neuropathic pain in patients affected by upper limb peripheral nerve injury (Sunderland type V) treated with microsurgical repair, followed by early sensory re-education. In a cross-sectional clinical study, 100 patients (male/female 81/19; age 40.5 ± 14.8 years and follow-up 17 ± 5 months, mean ± SD), with microsurgical nerve repair and reconstruction in the upper extremity and subsequent early sensory re-education, were evaluated, using Cold Intolerance Symptoms Severity questionnaire-Italian version (CISS-it, cut-off pathology >30/100 points), CISS questionnaire-12 item version (CISS-12, 0-46 points-grouping: healthy that means no cold intolerance [0-14], mild [15-24], moderate [25-34], severe [35-42], very severe [43-46] cold intolerance), probability of neuropathic pain (DouleurNeuropathique-4; [DN4] 4/10), deep and superficial sensibility, tactile threshold (monofilaments), and two-point discrimination (cutoff S2; Medical Research Council scale for sensory function; [MRC-scale]). A high CISS score is associated with possible neuropathic pain (DN4 ≥ 4). Both a low CISS-it score (ie, < 30) and DN4 < 4 is associated with good sensory recovery (MRC ≥ 2). In conclusion patients affected by upper limb peripheral nerve injuries with higher CISS scores more often suffer from cold intolerance and neuropathic pain, and the better their sensory recovery is, the less likely they are to suffer from cold intolerance and neuropathic pain.
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| 32. |
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| 33. |
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| 34. |
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| 35. |
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| 36. |
- Rossor, Alexander Martin, et al.
(författare)
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A longitudinal and cross-sectional study of plasma neurofilament light chain concentration in Charcot-Marie-Tooth disease.
- 2022
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Ingår i: Journal of the peripheral nervous system : JPNS. - : Wiley. - 1529-8027 .- 1085-9489. ; 27:1, s. 50-57
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Tidskriftsartikel (refereegranskat)abstract
- Advances in genetic technology and small molecule drug development have paved the way for clinical trials in Charcot-Marie-Tooth disease (CMT); however, the current FDA-approved clinical trial outcome measures are insensitive to detect a meaningful clinical response. There is, therefore, a need to identify sensitive outcome measures or clinically relevant biomarkers. The aim of this study was to further evaluate plasma neurofilament light chain (NFL) as a disease biomarker in CMT. Plasma NFL was measured using SIMOA technology in both a cross-sectional study of a US cohort of CMT patients and longitudinally over 6years in a UK CMT cohort. In addition, plasma NFL was measured longitudinally in two mouse models of CMT2D. Plasma concentrations of NFL were increased in a US cohort of patients with CMT1B, CMT1X and CMT2A but not CMT2E compared with controls. In a separate UK cohort, over a 6-year interval, there was no significant change in plasma NFL concentration in CMT1A or HSN1, but a small but significant reduction in patients with CMT1X. Plasma NFL was increased in wild type compared to GARSC201R mice. There was no significant difference in plasma NFL in GARSP278KY compared to wild type mice. In patients with CMT1A, the small difference in cross-sectional NFL concentration vs healthy controls and the lack of change over time suggests that plasma NFL may lack sufficient sensitivity to detect a clinically meaningful treatment response in adulthood.
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| 37. |
- Sekijima, Yoshiki, et al.
(författare)
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Pathological, biochemical, and biophysical characteristics of the transthyretin variant Y114H (p.Y134H) explain its very mild clinical phenotype
- 2015
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Ingår i: Journal of the peripheral nervous system. - : WILEY-BLACKWELL. - 1085-9489 .- 1529-8027. ; 20:4, s. 372-379
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Tidskriftsartikel (refereegranskat)abstract
- Transthyretin (TTR) is a homotetrameric protein that must misfold in order to form amyloid fibrils. Misfolding includes rate limiting tetramer dissociation, followed by fast tertiary structural changes of the monomer that enable aggregation. Hereditary ATTR amyloidosis is an autosomal dominant genetic disorder with systemic deposition of amyloid fibrils induced by TTR gene mutation. We identified a rare Y114H (p.Y134H) TTR variant in a Japanese patient presenting with late-onset, very mild clinical course. The patient had an extremely low serum variant TTR concentration (18% of total TTR), whereas the composition of variant TTR was 55% in amyloid fibrils in tenosynovial tissues obtained at carpal tunnel release surgery. The amyloid fibril deposits in the ATTR Y114H patient had an altered structure compared with that in wild-type ATTR patients, as determined by luminescent conjugated poly/oligo-thiophene fluorescence spectroscopy. Biophysical studies using recombinant protein showed that Y114H TTR was markedly destabilized both thermodynamically and kinetically and was highly amyloidogenic in vitro. These data suggest that extremely low serum variant Y114H TTR concentration, probably due to endoplasmic reticulum-associated degradation of unstable variant TTR protein, protected this patient from severe amyloidosis, as self-assembly of the amyloidogenic intermediate is a concentration-dependent process.
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| 38. |
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| 39. |
- Zimmerman, Jordan J, et al.
(författare)
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Riveting hammer vibration damages mechanosensory nerve endings
- 2020
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Ingår i: Journal of the peripheral nervous system. - : John Wiley & Sons, Ltd. - 1085-9489 .- 1529-8027. ; 25:3, s. 279-287
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Tidskriftsartikel (refereegranskat)abstract
- Background and Aims Hand-arm vibration syndrome (HAVS) is an irreversible neurodegenerative, vasospastic and musculoskeletal occupational disease of workers using powered hand tools. The etiology is poorly understood. Neurological symptoms include numbness, tingling and pain. This study examines impact hammer vibration-induced injury and recoverability of hair mechanosensory innervation. Methods Rat tails were vibrated 12?min/d for 5 wk followed by 5 wk recovery with synchronous non-vibrated controls. Nerve fibers were PGP9.5 immunostained. Lanceolate complex innervation was compared quantitatively in vibrated vs sham. Vibration peak acceleration magnitudes were characterized by frequency power spectral analysis. Results Average magnitude (2515?m/s2, rms) in kHz frequencies was 109 times that (23?m/s2) in low Hz. Percentage of hairs innervated by lanceolate complexes was 69.1% in 5wk sham and 53.4% in 5wk vib generating a denervation difference of 15.7% higher in vibration. Hair innervation was 76.9% in 5wk recovery sham and 62.0% in 5wk recovery vibration producing a denervation difference 14.9% higher in recovery vibration. Lanceolate number per complex (18.4?±?0.2) after vibration remained near sham (19.3?±?0.3), but 44.9% of lanceolate complexes were abnormal in 5 wk vibrated compared to 18.8% in sham. Interpretation The largest vibration energies are peak kHz accelerations (~?100?000?m/s2) from shock waves. The existing ISO 5349-1 standard excludes kHz vibrations, seriously underestimating vibration injury risk. The present study validates the rat-tail, impact hammer vibration as a model for investigating irreversible nerve damage. Persistence of higher denervation difference after 5-week recovery suggests repeated vibration injury destroys the capability of lanceolate nerve endings to regenerate.
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- Haapaniemi, T, et al.
(författare)
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Untitled - Response
- 2003
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Ingår i: Journal of the Peripheral Nervous System. - : Wiley. - 1085-9489. ; 8:2, s. 63-64
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Tidskriftsartikel (övrigt vetenskapligt/konstnärligt)
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