Self-biting behavior in patients with neonatal brachial plexus palsy.

PURPOSE: Self-biting behavior in patients with neonatal brachial plexus palsy (NBPP) has been associated with finger amputation. Our objective is to describe the incidence of this complication, risk factors, and clinical management. METHODS: We retrospectively analyzed 612 patients with NBPP. There were 303 males and 309 females. 51.8% of patients had C5-C6 lesions, 28.9% had C5-C7, 18.9% had C5-T1, and 0.3 had C7-T1 involvement. RESULTS: We identified 15 patients with self-biting behavior (2.5%). Ten patients had C5-T1 lesions, and five had C5-C7 lesions. Eight patents were submitted to brachial plexus surgery and seven were not. This behavior appeared between 8 and 46 months of life (mean 23.5), and it was always temporary. There was no difference between operated and non-operated patients (p > 0.05), and no correlation between age at surgery and age of appearance of self-biting behavior (p > 0.05). Physical restriction was effective in treating this complication and we had no case of finger amputation. CONCLUSION: Self-biting behavior is a rare complication of NBPP, and it is usually associated with severe motor involvement. The behavior duration is limited to a few months. This condition can be effectively treated with physical restriction to prevent hand biting.

Diagnostic accuracy of imaging studies for diagnosing root avulsions in post-traumatic upper brachial plexus traction injuries in adults.

BACKGROUND: There is no consensus about which type of imaging study, computed tomography myelography (CTM) or magnetic resonance imaging (MRI), provides better information concerning root avulsion in adult brachial plexus injuries. METHODS: Patients with upper brachial plexus traumatic injuries underwent both CTM and MRI and surgical exploration. The imaging studies were analyzed by two independent radiologists and the data were compared with the intraoperative findings. The statistical analysis was based on dichotomous classification of the nerve roots (normal or altered). The interobserver agreement was assessed using Cohen's Kappa. The accuracy of CTM and MRI in comparison with the intraoperative findings was evaluated using the same methodology. RESULTS: Fifty-two adult patients were included. CTM tended to yield slightly higher percentages of alterations than MRI The interobserver agreement was better on CTM than on MRI for all nerve roots: C5, 0.9960 (strong) vs. 0.145 (poor); C6, 0.970 (strong) vs. 0.788 (substantial); C7, 0.969 (strong) vs. 0.848 (strong). The accuracy regarding the intraoperative findings was also higher on CTM (moderate, kappa 0.40-0.59) than on MRI (minimal, kappa 0.20-0.39) for all nerve roots. Accordingly, the overall percentage concordance (both normal or both altered) was superior in the CTM evaluation (approx. 70-75% vs. 60-65%). CTM was superior for both sensitivity and specificity at all nerve roots. CONCLUSION: CTM had greater interobserver agreement and higher diagnostic accuracy than MRI in adult patients with root avulsions due to brachial plexus injury.

Surgical treatment of birth-related brachial plexus injuries: a historical review.

This historical review presents the relevant data about the evolution of the surgical treatment of neonatal brachial plexus palsy. Starting with the first clinical description by Smellie in 1754, we will present the initial enthusiasm for the surgery followed by a lack of interest that lasted many years, the resurgence of interest in operative management in the 1970s, and the consolidation in the 1980s of surgery as the standard indication in cases of neonatal brachial plexus palsy without a functional spontaneous recovery.

Long-term evaluation of hand function in children undergoing Oberlin and Oberlin-like procedures for reinnervation of the biceps muscle.

PURPOSE: Long-term evaluation of hand function in children who underwent transfer procedures to reinnervate the biceps muscle, using fascicles from the ulnar and median nerves as donors. METHODS: In the last follow-up evaluation, the children underwent a neurological examination, and their hand status was classified according to the Raimondi grading system for hand function. Two physical measurements, the child health assessment questionnaire (CHAQ) and the Sollerman hand function test, were applied to assess upper extremity function. RESULTS: Eight children were re-evaluated. In four the donor fascicle was from the ulnar nerve and in four from the median nerve. The average interval between surgery and the last evaluation was 8.3 years (range 6-10 years). Five patients scored 5 points in the Raimondi grading system, and 3 patients scored 4 points. The results from the CHAQ ranged from 0.03 to 0.41. The results from the Sollerman test were between 60 and 77 for the affected upper limb and between 65 and 79 for the dominant upper limb. CONCLUSION: Nerve transfer of a fascicle from the ulnar or median nerve to the biceps motor branch in children with neonatal brachial plexus palsy does not result in hand dysfunction.

Restoration of shoulder external rotation by means of the infraspinatus muscle reinnervation with a radial nerve branch transfer.

Background: Although reinnervation of the suprascapular nerve is frequently obtained through brachial plexus surgery, reestablishment of infraspinatus muscle function is rarely achieved.Methods: The viability of transfer of the radial nerve to the nerve branch to the infraspinatus muscle was determined anatomically, including histomorphometrical analysis on 30 adult cadavers. Eleven adult patients were then treated using the proposed nerve transfer.Results: The branch to the medial head was more suitable for the nerve transfer. In one cadaver, nerve transfer was impossible because there was no donor of sufficient length. According to axon counts, the branches to the lateral and medial heads had sufficient numbers of axons (means = 994.2 ± 447.6 and 1030.8 ± 258.5, respectively) for reinnervation of the branch to the infraspinatus (means = 830.2 ± 241.2 axons). In the surgical series, one patient was lost in the follow-up and only two patients achieved a good result from the transfer. Recovery of external shoulder rotation started 14 months after surgery in one patient and 8 months in the other. The first patient reached 90° of external rotation 6 months later and the second, achieved 120°of shoulder external rotation 6 months after surgery . Four other patients recovered small amounts of movement: 20, 35, 40 and 45°.Conclusions: Although anatomically feasible, the proposed nerve transfer resulted in a small number of good clinical outcomes.

Functional outcome of spinal accessory nerve transfer to the suprascapular nerve to restore shoulder function: Results in upper and complete traumatic brachial plexus palsy in adults.

BACKGROUND: Shoulder stability, abduction and external rotation are vital for the performance of usual daily tasks. AIMS: To compare the functional outcomes in the shoulder following spinal accessory to suprascapular nerve transfer (SASNT). PATIENTS AND METHODS: Comparison of the outcome of adult patients with upper traumatic brachial plexus palsy undergoing SASNT with patients with complete palsy submitted to the same procedure. STATISTICAL ANALYSIS: Ranges of motion were compared via the Mann-Whitney U test. The percentages of patients with a favorable outcome were compared by the chi-square test. All tests were two-tailed and P values <0.05 were considered statistically significant. RESULTS: SASNT was performed in 76 patients: 23 cases (30.2%) of upper-plexus injuries and 53 cases (69.7%) of complete brachial plexus palsy. Good shoulder abduction was achieved in 15 patients (65.2%) with upper plexus palsy and a good external rotation in 5 (21.7%). In those patients with a good recovery, the average range of motion (ROM) was 53° for shoulder abduction and 71.2° for external rotation. Thirty-six patients (67.9%) with complete palsy had a good shoulder abduction recovery with 30.7° of average ROM, but only 3 patients (5.6%) recovered a good shoulder external rotation with 68.3° of average ROM. There was no statistical difference for the abduction outcome, but the external rotation outcome was superior in the upper plexus palsy group. CONCLUSION: SASNT is a consistent procedure to achieve functional recovery of shoulder abduction after a partial or complete plexus injury, but the outcomes of external rotation were quite disappointing in both the groups.

Outcomes from primary surgical reconstruction of neonatal brachial plexus palsy in 104 children.

PURPOSE: The outcome from microsurgical reconstruction of neonatal brachial plexus palsy (NBPP) varies, and comparison between different series is difficult, given the differences in preoperative evaluation, surgical strategies, and outcome analysis. To evaluate our results, we reviewed a series of children who underwent surgical treatment in a period of 14 years. METHODS: We made a retrospective review of 104 cases in which microsurgical repair of the brachial plexus was performed. Strength was graded using the Active Movement Scale. Whenever possible, upper palsies underwent surgery 4 to 6 months after birth and total lesions around 3 months. The lesions were repaired, according to the type of injury: neurolysis, nerve grafting, nerve transfer, or a combination of techniques. The children were followed for at least 24 months. RESULTS: The majority of cases were complete lesions (56/53.8%). Erb's palsy was present in 10 cases (9.6%), and 39 infants (37.5%) presented an extended Erb's palsy. The surgical techniques applied were neurolysis (10.5%), nerve grafts (25.9%), nerve transfers (34.6%), and a combination of grafts and transfers (30.7%). The final outcome was considered poor in 41.3% of the cases, good in 34.3%, and excellent in 24%. A functional result (good plus excellent) was achieved in 58.3% of the cases. CONCLUSIONS: There is no consensus regarding strategies for treatment of NBPP. Our surgical outcomes indicated a good general result comparing with the literature. However, our results were lower than the best results reported. Maybe the explanation is our much higher number of total palsy cases (53.8% vs. 25% in the literature).

Restoration of Elbow Flexion in Traumatic Upper Brachial Plexus Palsy in Adults: Outcome with Intraplexus Distal Nerve Transfers in 78 Patients

Objective To present the functional outcomes of distal nerve transfer techniques for restoration of elbow flexion after upper brachial plexus injury. Method The files of 78 adult patients with C5, C6, C7 lesions were reviewed. The attempt to restore elbow flexion was made by intraplexus distal nerve transfers using a fascicle of the ulnar nerve (group A, n » 43), or a fascicle of themedian nerve (group B, n » 16) or a combination of both (group C, n » 19). The result of the treatment was defined based on the British Medical Research Council grading system: muscle strength < M3 was considered a poor result. Results The global incidence of good/excellent results with these nerve transfers was 80.7%, and for different surgical techniques (groups A, B, C), it was 86%, 56.2% and 100% respectively. Patients submitted to ulnar nerve transfer or double transfer (ulnar þ median fascicles transfer) had a better outcome than those submitted to median nerve transfer alone (p < 0.05). There was no significant difference between the outcome of ulnar transfer and double transfer. Conclusion In cases of traumatic injury of the upper brachial plexus, good and excelent results in the restoration of elbow flexion can be obtained using distal nerve transfers.

Distal nerve transfer versus supraclavicular nerve grafting: comparison of elbow flexion outcome in neonatal brachial plexus palsy with C5-C7 involvement.

PURPOSE: Ulnar and median nerve transfers to arm muscles have been used to recover elbow flexion in infants with neonatal brachial plexus palsy, but there is no direct outcome comparison with the classical supraclavicular nerve grafting approach. METHODS: We retrospectively analyzed patients with C5-C7 neonatal brachial plexus palsy submitted to nerve surgery and recorded elbow flexion recovery using the active movement scale (0-7) at 12 and 24 months after surgery. We compared 13 patients submitted to supraclavicular nerve grafting with 21 patients submitted to distal ulnar or median nerve transfer to biceps motor branch. We considered elbow flexion scores of 6 or 7 as good results. RESULTS: The mean elbow flexion score and the proportion of good results were better using distal nerve transfers than supraclavicular grafting at 12 months (p < 0.01), but not at 24 months. Two patients with failed supraclavicular nerve grafting at 12 months showed good elbow flexion recovery after ulnar nerve transfers. CONCLUSION: Distal nerve transfers provided faster elbow flexion recovery than supraclavicular nerve grafting, but there was no significant difference in the outcome after 24 months of surgery. Patients with failed supraclavicular grafting operated early can still benefit from late distal nerve transfers. Supraclavicular nerve grafting should remain as the first line surgical treatment for children with neonatal brachial plexus palsy.

Treatment of complete facial palsy in adults: comparative study between direct hemihypoglossal-facial neurorrhaphy, hemihipoglossal-facial neurorrhaphy with grafts, and masseter to facial nerve transfer.

BACKGROUND: The hypoglossal (with or without grafts) and masseter nerves are frequently used as axon donors for facial reinnervation when no proximal stump of the facial nerve is available. We report our experience treating facial nerve palsies via hemihypoglossal-to-facial nerve transfers either with (HFG) or without grafts (HFD), comparing these outcomes against those of masseteric-to-facial nerve transfers (MF). METHOD: A total of 77 patients were analyzed retrospectively, including 51 HFD, 11 HFG, and 15 MF nerve transfer patients. Both the House-Brackmann (HB) scale and our own, newly-designed scale to rate facial reanimation post nerve transfer (quantifying symmetry at rest and when smiling, eye occlusion, and eye and mouth synkinesis when speaking) were used to enumerate the extent of recovery. RESULTS: With both the HB and our own facial reanimation scale, the HFD and MF procedures yielded better outcome scores than HFG, though only the HGD was statistically superior. HGD produced slightly better scores than MF for everything but eye synkinesis, but these differences were generally not statistically significant. Delaying surgery beyond 2 years since injury was associated with appreciably worse outcomes when measured with our own but not the HB scale. The only predictors of outcome were the surgical technique employed and the duration of time between the initial injury and surgery. CONCLUSIONS: HFD appears to produce the most satisfactory facial reanimation results, with MF providing lesser but still satisfactory outcomes. Using interposed grafts while performing hemihypoglossal-to-facial nerve transfers should likely be avoided, whenever possible.

Gene expression profiling for human iPS-derived motor neurons from sporadic ALS patients reveals a strong association between mitochondrial functions and neurodegeneration.

Amyotrophic Lateral Sclerosis (ALS) is a fatal neurodegenerative disease that leads to widespread motor neuron death, general palsy and respiratory failure. The most prevalent sporadic ALS form is not genetically inherited. Attempts to translate therapeutic strategies have failed because the described mechanisms of disease are based on animal models carrying specific gene mutations and thus do not address sporadic ALS. In order to achieve a better approach to study the human disease, human induced pluripotent stem cell (hiPSC)-differentiated motor neurons were obtained from motor nerve fibroblasts of sporadic ALS and non-ALS subjects using the STEMCCA Cre-Excisable Constitutive Polycistronic Lentivirus system and submitted to microarray analyses using a whole human genome platform. DAVID analyses of differentially expressed genes identified molecular function and biological process-related genes through Gene Ontology. REVIGO highlighted the related functions mRNA and DNA binding, GTP binding, transcription (co)-repressor activity, lipoprotein receptor binding, synapse organization, intracellular transport, mitotic cell cycle and cell death. KEGG showed pathways associated with Parkinson's disease and oxidative phosphorylation, highlighting iron homeostasis, neurotrophic functions, endosomal trafficking and ERK signaling. The analysis of most dysregulated genes and those representative of the majority of categorized genes indicates a strong association between mitochondrial function and cellular processes possibly related to motor neuron degeneration. In conclusion, iPSC-derived motor neurons from motor nerve fibroblasts of sporadic ALS patients may recapitulate key mechanisms of neurodegeneration and may offer an opportunity for translational investigation of sporadic ALS. Large gene profiling of differentiated motor neurons from sporadic ALS patients highlights mitochondrial participation in the establishment of autonomous mechanisms associated with sporadic ALS.

Influence of body mass index on the outcome of brachial plexus surgery: are there any differences between elbow and shoulder results?

BACKGROUND: Body mass index (BMI) has recently been identified as a predictor of outcomes following reconstructive surgery of shoulder palsies. In this study, we sought to determine if the same holds true for the reconstruction of elbow flexion. METHODS: Forty patients who had undergone partial ulnar-to-biceps nerve transfer (Oberlin's procedure) for shoulder palsy were assessed and compared against 18 previously reported patients who had undergone reconstruction for elbow palsies. The British Medical Research Council (BMRC) scale and an index dividing shoulder abduction strength in the affected arm by healthy arm were recorded. All patients had undergone surgery within 12 months of injury and had ≥ 12 months of follow-up. RESULTS: M4 or M3 biceps strength was obtained in 90 % of patients. Final strength on the affected side averaged 5.8 kg, versus 20.2 kg on the normal side, for a mean recovery index score of 0.30. In this sample of 40 patients, BMI did not predict percentage strength or BMRC grade recovery. Neither did age, number of roots involved, the affected side, nor time to surgery. Comparing patients with elbow versus shoulder reconstruction, there were no differences, except that patients undergoing Oberlin's procedure had a statistically longer duration of time between injury and surgical repair (7.4 vs 5.1 months, p < 0.006). CONCLUSIONS: Our data suggest that proximal muscle re-innervation is functionally more dependent upon BMI than distal re-innervation, likely because proximal muscles must support the weight of the entire extremity, while more distal muscles do not. BMI should be taken into consideration when planning surgery.

Bilateral radial nerve compression neuropathy in the newborn.

PURPOSE: This study aims to discuss the diagnosis and management of radial nerve compression neuropathy in the newborn. METHODS: A personal case is presented, followed by a review and analysis of clinically similar cases identified via a PubMed search of published medical literature. RESULTS: We report a case of a term newborn with bilateral radial neuropathy at the humerus level. Despite severe axonal involvement in the electrophysiological evaluation, the patient showed complete bilateral recovery after 3 months of follow-up. CONCLUSIONS: Isolated radial nerve palsy is a rare event in the newborn. The condition does not require surgical treatment and usually proceeds to full and rapid spontaneous recovery.

The epidemiology of adult traumatic brachial plexus lesions in a large metropolis.

INTRODUCTION: Adult traumatic brachial plexus lesions are devastating injuries. Their real incidence is difficult to ascertain, but are certainly growing due to the increasing number of high-speed motor-vehicle accidents, especially in big cities. OBJECTIVES: Analysis of the epidemiological characteristics of patients with traumatic brachial plexus lesions in São Paulo, Brazil, the sixth largest city in the world. METHODS: This was a retrospective analysis of the epidemiological characteristics of patients submitted to surgical treatment of traumatic brachial plexus lesions in the Peripheral Nerve Surgery Unit of the Department of Neurosurgery of the University of São Paulo Medical School. RESULTS: In the period from 2004 to 2012, 406 patients underwent surgery. There were 384 (94.6 %) men and 22 (5.4 %) women. In 45.9 % the compromised plexus was the right and in 54.1 %, the left. The average age was 28.38 years. Among the causes, the most frequent was motorcycle accidents (79 %). Most of the lesions were supraclavicular. In 46.1 % of cases the lesions were complete, in 30.1 % the lesions compromised C5/C6 roots, in 20.9 % the C5/C6/C7 roots were lesioned and in 2.9 % the lesion was in the lower roots, C8/T1. Among the associated lesions the most prevalent were head trauma, observed in 34.2 % of the cases; lesions of long bones in 38.8 %; clavicle fractures in 25.9 %; and thoracic trauma in 12.9 %. CONCLUSION: In a population of adult patients with brachial plexus lesions with surgical indication, most of them comprise young male adults involved in high-energy motorcycle accidents.

Surgical treatment of typical peripheral schwannomas: the risk of new postoperative deficits.

BACKGROUND: Although peripheral schwannomas can be resected without postoperative neurological complications, surgeons must anticipate the possibility that new neurological deficits could develop. In order to evaluate the risk of neurological complications in the surgical treatment of these tumours, we performed a retrospective review of cases involving schwannomas in the extremities, as well as an analysis of the related literature. METHOD: We reviewed a combined series of 72 schwannomas from the extremities presenting for surgical excision. Meticulous analysis of the files was undertaken, searching for pre-operative findings that could be more frequent in patients with surgical complications. The incidence, severity, and transitory nature of post-operative complications in our series was observed and compared against the literature. RESULTS: Eleven patients (15.2 %) developed new neurological deficits after surgery: sensory disturbance in seven cases, motor weakness in three, and a single wound hematoma. Most of these complications were temporary. Statistical analysis demonstrated a positive relationship between the presence of complications and both patient age under 50 years (p = 0.02) and tumours greater than 3 cm in greatest diameter (p = 0.02). CONCLUSIONS: Although relatively infrequent, the potential for novel post-operative deficits after the surgical treatment of peripheral schwannomas does exist and should be included during pre-operative counseling.

Efficacy and safety of Oberlin's procedure in the treatment of brachial plexus birth palsy.

BACKGROUND: In brachial plexus injuries, when there are no available roots to use as a source for graft reconstruction, nerve transfers emerge as an elective technique. For this purpose, transfer of an ulnar nerve fascicle to the biceps motor branch (Oberlin's procedure) is often used. Despite the high rate of good to excellent results in adults, this technique is seldom used in children. OBJECTIVE: To evaluate the efficacy and safety of Oberlin's procedure in the surgical treatment of brachial plexus birth palsy. METHODS: Striving to restore elbow flexion, we performed Oberlin's procedure in 17 infants with brachial plexus birth palsy. After follow-up of at least 19 months, primary outcomes were the strength of elbow flexion (modified British Medical Research Council scale), hand function measured using Al-Qattan's scale, and comparative x-rays of both hands to detect altered growth. RESULTS: Good to excellent results related to biceps contraction were obtained in 14 patients (82.3%) (3/MRC3, 11/MRC4). The preoperative Al-Qattan scale score for the hand was maintained at final follow-up. Comparing the treated and normal limb, no difference was observed in hand development by x-ray. CONCLUSION: Oberlin's procedure is an effective and safe option for the surgical treatment of upper brachial plexus birth palsy.

Prognostic value of thumb pain sensation in birth brachial plexopathy.

OBJECTIVE: To evaluate the prognostic value of absent thumb pain sensation in newborns and young infants with birth brachial plexopathy. METHODS: We evaluated 131 patients with birth brachial plexopathy with less than two months of age. Pain sensation was evoked by thumb nail bed compression to evaluate sensory fibers of the upper trunk (C6). The patients were followed-up monthly. Patients with less than antigravity elbow flexion at six months of age were considered to have a poor outcome. RESULTS: Thirty patients had absent thumb pain sensation, from which 26 showed a poor outcome. Sensitivity of the test was 65% and specificity was 96%. CONCLUSION: Evaluation of thumb pain sensation should be included in the clinical assessment of infants with birth brachial plexopathy.

Prognostic value of thumb pain sensation in birth brachial plexopathy / Valor prognóstico da sensibilidade dolorosa do polegar na plexopatia braquial relacionada ao parto

OBJECTIVE: To evaluate the prognostic value of absent thumb pain sensation in newborns and young infants with birth brachial plexopathy. METHODS: We evaluated 131 patients with birth brachial plexopathy with less than two months of age. Pain sensation was evoked by thumb nail bed compression to evaluate sensory fibers of the upper trunk (C6). The patients were followed-up monthly. Patients with less than antigravity elbow flexion at six months of age were considered to have a poor outcome. RESULTS: Thirty patients had absent thumb pain sensation, from which 26 showed a poor outcome. Sensitivity of the test was 65% and specificity was 96%. CONCLUSION: Evaluation of thumb pain sensation should be included in the clinical assessment of infants with birth brachial plexopathy.

OBJETIVO: Avaliar o valor prognóstico da hipoestesia dolorosa no polegar em recém-nascidos e lactentes jovens com plexopatia braquial obstétrica. MÉTODOS: Avaliamos 131 pacientes com plexopatia braquial obstétrica com menos de dois meses de idade. A sensação dolorosa foi provocada pela compressão do leito ungueal do polegar para avaliar fibras sensitivas do tronco superior (C6). Os pacientes foram seguidos mensalmente. Prognóstico desfavorável foi atribuído aos pacientes sem força antigravitacional para flexão do cotovelo aos seis meses de idade. RESULTADOS: Trinta pacientes apresentaram hipoestesia dolorosa do polegar, dos quais 26 tiveram prognóstico desfavorável. A sensibilidade do teste foi de 65% e a especificidade 96%. CONCLUSÃO: A avaliação da sensibilidade dolorosa do polegar deve ser incluída na avaliação clínica de pacientes com plexopatia braquial obstétrica.

Management of desmoid-type fibromatosis involving peripheral nerves.

Desmoid-type fibromatosis is an uncommon and aggressive neoplasia, associated with a high rate of recurrence. It is characterized by an infiltrative but benign fibroblastic proliferation occurring within the deep soft tissues. There is no consensus about the treatment of those tumors. We present a surgical series of four cases, involving the brachial plexus (two cases), the median nerve and the medial brachial cutaneous nerve. Except for the last case, they were submitted to multiple surgical procedures and showed repeated recurrences. The diagnosis, the different ways of treatment and the prognosis of these tumoral lesions are discussed. Our results support the indication of radical surgery followed by radiotherapy as probably one of the best ways to treat those controversial lesions.