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ABSTRACT 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.

Abstract BACKGROUND: The recovery of elbow flexion in upper brachial plexus injury can be achieved by the reinnervation of the biceps muscle (single reinnervation), but concomitant restoration of brachialis and biceps function (double reinnervation) has been recommended to improve elbow flexion strength. OBJECTIVE: To prospectively compare morbidity and outcomes of single or double muscle reinnervation in restoring elbow flexion following incomplete injury to the brachial plexus. METHODS: Forty consecutive patients were prospectively submitted to single or double muscle reinnervation. Elbow flexion strength was evaluated with a push-and-pull dynamometer 12 months after surgery. Hand morbidity related to the procedures was evaluated by the Semmes-Weinstein monofilaments test, quantification of static 2-point discrimination, and measurements of handgrip and lateral pinch strength in serial evaluations up to the final follow-up. RESULTS: Similar results for the strength of elbow flexion were observed in both groups. A worsening of sensibility, measured by using Semmes-Weinstein monofilaments, was identified in 8 patients, all of whom showed recovery during follow-up. No worsening of 2-point discrimination was observed. A decrease in handgrip and lateral pinch strength was identified in 8 and 9 patients, respectively, which improved during follow-up. There was no difference between the groups in the incidence of hand motor morbidity parameters. CONCLUSION: The strength of elbow flexion did not differ significantly between the groups treated with single or double muscle reinnervation. Deterioration of handgrip, lateral pinch strength, and sensibility measured by using Semmes-Weinstein monofilaments, was temporary, resulting in low morbidity for both techniques.

The most common causes of congenital myasthenic syndromes (CMS) are CHRNE mutations, and some pathogenic allelic variants in this gene are especially frequent in certain ethnic groups. In the southern region of Brazil, a study found the c.130dupG CHRNE mutation in up to 33% of families with CMS. Here, we aimed to verify the frequency of this mutation among individuals with CMS in a larger cohort of CMS patients from different areas of Brazil and to characterize clinical features of these patients. Eighty-four patients with CMS, from 72 families, were clinically evaluated and submitted to direct sequencing of the exon 2 of CHRNE. The c.130dupG mutation was found in 32 patients (23 families), with 26 patients (19 families, 26.3%) in homozygosis, confirming its high prevalence in different regions of Brazil. Among the homozygous patients, the following characteristics were frequent: onset of symptoms before 2 years of age (92.3%), little functional restriction (92.3%), fluctuating symptoms (100%), ocular muscle impairment (96.1%), ptosis (100%), limb weakness (88.4%), response to pyridostigmine (100%), facial involvement (77%), and bulbar symptoms (70.8%). The pretest probability of finding at least one allele harbouring the c.130dupG mutation was 38.1%. Selecting only patients with impaired eye movement together with limb weakness and improvement with pyridostigmine, the probability increases to 72.2%. This clinical pre-selection of patients is likely a useful tool for regions where CHRNE mutations have a founder effect. In conclusion, the CHRNE mutation c.130dupG leads to fairly benign natural course of the disease with relative homogeneity.

ABSTRACT BACKGROUND Though anatomy of the brachial plexus is well known, its fascicular anatomy is still a challenge. In case of the musculocutaneous nerve (MCN), the position of the motor branches at its terminations is well known; however, their position in the lateral cord has been rarely investigated. OBJECTIVE To describe the position of motor branches of the MCN at its origin in the lateral cord. METHODS The MCN of 26 adult nonfixed cadavers was dissected from its terminal branches in the medial aspect of the arm to its origin from the lateral cord of the brachial plexus (from distal to proximal) on the right side, removed, and fixed. Intraneural longitudinal fascicular dissection was performed using microsurgical techniques, with meticulous removal of connective tissue and subsequent identification and tracking of fibers of each MCN branch. RESULTS At the origin of the nerve, biceps and brachialis muscle fibers were concentrated in the lateral portion of the nerve (100%), and the sensory fibers were preferentially located in the medial portion (78%). CONCLUSION At its origin, the lateral portion of the MCN is the best location to coapt. donor nerves in order to improve motor results.

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.

Fibrolipomatous hamartoma is a rare benign tumor‐like condition that affects most commonly the median nerve. The diagnosis is usually confirmed through its typical appearance on magnetic resonance imaging (MRI) without the need for a nerve biopsy. There are divergent views regarding treatment of this entity, but open carpal tunnel release for nerve decompression currently constitutes the standard care for alleviation of compressive neuropathy of the median nerve. In this report, we describe a case of fibrolipomatous hamartoma that was diagnosed via MRI and underwent open carpal tunnel release, with alleviation of the patient's symptoms.

Peripheral nerve hyperexcitability syndrome comprises a heterogeneous group of diseases, clinically characterised by myokymia, fasciculation, muscle cramps and stiffness. The causes are either immune mediated or non-immune mediated. Non-immune-mediated forms are mostly genetic, relating to two main genes: KCNQ2 and KCNA1. Patients with KCNQ2 gene mutations typically present with epileptic encephalopathy, benign familial neonatal seizures and myokymia, though occasionally with purely peripheral nerve hyperexcitability. We report a woman with marked facial myokymia and distal upper limb contractures whose mother also had subtle facial myokymia; both had the c.G620A (p.R207Q) variant in the KCNQ2 gene. Patients with familial myokymia and peripheral nerve hyperexcitability syndrome should be investigated for KCNQ2 variants. This autosomal dominant condition may respond to antiepileptic medications acting at potassium channels.

To evaluate donor site morbidity following sural nerve harvesting, with special attention to the recovery of sensory loss. We prospectively followed 38 subjects who underwent sural nerve harvest, including two with bilateral nerve excision. Symptoms related to sural nerve excision were evaluated and demarcation of the area with reduced touch sensation was quantified. Assessments were performed periodically up to 1 year after surgery and the results of different sensory evaluations were compared. A significant reduction of sensory deficit was identified between consecutive evaluations (p<0.05). Decreases of 26.85%, 20.69% and 24.29% were observed 3, 6 and 12 months after surgery, respectively. Shock-like pain (7.5%), stabbing pain (7.5%), and numbness (5%) were the most frequently reported symptoms. All symptoms were brief and resolved spontaneously 3–6 months after surgery. Sural nerve harvest can be performed with acceptable morbidity. When present, symptoms resolve between the third and sixth month after surgery and a significant reduction of sensory loss in the area innervated by the sural nerve was observed during the first year of follow-up.

Purpose Although re-innervation of the hand is considered a priority in the treatment of infants with complete brachial plexus injury, there is currently a paucity of publications investigating hand function outcomes following primary nerve reconstruction in infants with neonatal brachial plexus palsy (NBPP). This study therefore aimed to evaluate hand function outcomes in a series of patients with complete NBPP. Methods This retrospective case series included all patients who underwent primary nerve surgery for complete neonatal brachial plexus palsy over an 8-year period. Outcomes were assessed using the Raimond Hand Scale. Classification of grade 3 or higher indicates a functional hand (assistance in bimanual activity). Results Nineteen patients with a complete NBPP underwent primary nerve reconstruction at a mean age of 3.7 months. Periodic clinical evaluations were performed until at least 4 years of age. According to the Raimondi hand scale, one patient did not recover (grade 0), three patients attained grade 1, four grade 2, ten grade 3, and in one grade 4. Overall hand functional recovery was achieved in 57.8% (11/19) of patients. Conclusion Sufficient recovery of hand function to perform bimanual activity tasks in patients with complete NBPP lesions is possible and should be a priority in the surgical treatment of these infants.

Summary 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.