Hand function outcomes following surgical treatment of complete neonatal brachial plexus palsy.

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.

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.

True neurogenic thoracic outlet syndrome: late outcomes from a surgical series.

BACKGROUND: True neurogenic thoracic outlet syndrome (TNTOS) is rare, and evaluation of surgical treatment is limited to a few studies in the literature. The purpose of this study is to present the results from a surgical series of 21 patients with TNTOS. METHODS: Retrospective analysis on 21 patients diagnosed with TNTOS who underwent surgery. Demographic data and neurological status were characterized, and patients were classified in accordance with a pre-established scale for assessing the severity of hand impairment before and after surgery. Neuropathic pain was assessed using a visual analogue scale (VAS) and functional disability was quantified using the QuickDASH questionnaire. The results from before and after surgery were compared using the Wilcoxon test, and the significance level was taken to be 5%. RESULTS: There was a significant difference in VAS values from before to after the operation (Wilcoxon test: p = 0.0001; r = 0.86). Most patients (90%) improved after surgery, and in 85% of these patients, the VAS improvement was greater than 50%. Improvement in hand function occurred in seven patients (33.3%), and in most of these cases (28.6%), this improvement was classified as mild. Most patients (93.3%) showed moderate to very severe functional disability at the end of the follow-up. CONCLUSION: After surgery, only one-third of the cases showed improvement in motor function and most patients had significant functional disability. However, the improvement regarding pain was significant. Surgery to control this symptom should be recommended, even in cases of late presentation and severe motor impairment.

One-year prospective nerve conduction study of thalidomide neuropathy in lupus erythematosus: Incidence, coasting effect and drug plasma levels.

BACKGROUND: Few prospective studies in cutaneous and systemic lupus erythematosus (CLE/SLE) assessed thalidomide-induced peripheral neuropathy (TiPN) incidence/reversibility, and most have not excluded confounding causes neither monitored thalidomide plasma levels. OBJECTIVES: To evaluate TiPN incidence/reversibility, coasting effect and its association with thalidomide plasma levels in CLE/SLE. METHODS: One-year prospective study of thalidomide in 20 CLE/SLE patients without pregnancy potential, with normal nerve conduction study (NCS), and excluded other PN causes. Thalidomide levels were determined by high-performance liquid chromatography/tandem mass spectrometry. RESULTS: Twelve patients (60%) developed TiPN: 33.3% were symptomatic and 66.6% asymptomatic. Half of this latter group developed coasting effect (TiPN symptoms 1-3 months after drug withdrawal). The main predictive factors for TiPN were treatment duration ≥6 months (p = 0.025) and cumulative dose (p = 0.023). No difference in plasma thalidomide levels between patients with/without TiPN was observed (p = 0.464). After drug withdrawal, 75% symptomatic TiPN patients improved their symptoms. Seven TiPN patients underwent an additional NCS after drug withdrawal: 42.8% worsened NCS, 14.2% was stable, and 42.8% had improved NCS. CONCLUSION: Our data provides novel evidence of coasting effect in half of asymptomatic patients with TiPN. The irreversible nature of this lesion in 25% of TiPN patients reinforces the relevance of early NCS monitoring, and suggests thalidomide use solely as a bridge for other effective therapy for refractory cutaneous lupus patients.

Thalidomide and Lenalidomide for Refractory Systemic/Cutaneous Lupus Erythematosus Treatment: A Narrative Review of Literature for Clinical Practice.

BACKGROUND: Thalidomide has shown exceptional results in systemic/cutaneous lupus erythematosus(SLE/CLE). Recently, lenalidomide has been also prescribed for SLE/CLE treatment. Literature regarding efficacy/adverse events for these drugs is scarce with a single systematic review and meta-analysis focused solely on thalidomide for refractory cutaneous lupus subtypes. OBJECTIVE: We, therefore, addressed in this narrative review the efficacy/adverse effects of thalidomide and lenalidomide for SLE and CLE. In addition, we provide a specialist approach for clinical practice based on the available evidence. RESULTS: Efficacy of thalidomide for refractory cutaneous lupus treatment was demonstrated by several studies, mostly retrospective with small sample size(≤20). The frequency of peripheral polyneuropathy is controversial varying from 15-80% with no consistent data regarding cumulative dose and length of use. Drug withdrawn results in clinical partial/complete reversibility for most cases (70%). For lenalidomide, seven studies (small sample sizes) reported its efficacy for SLE/CLE with complete/partial response in all patients with a mean time to response of 3 months. Flare rate varied from 25-75% occurring 0.5-10 months after drug withdrawn. There were no reports of polyneuropathy/worsening of previous thalidomide-induced neuropathy, but most of them did not perform nerve conduction studies. Teratogenicity risk exist for both drugs and strict precautions are required. CONCLUSIONS: Thalidomide is very efficacious as an induction therapy for patients with severe/refractory cutaneous lupus with high risk of scarring, but its longstanding use should be avoided due to neurotoxicity. Lenalidomide is a promising drug for skin lupus treatment, particularly regarding the apparent lower frequency of nerve side effects.

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.

Facial myokymia in inherited peripheral nerve hyperexcitability syndrome.

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.

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).

Interdigital direct neurorrhaphy for treatment of painful neuroma due to finger amputation.

BACKGROUND: Painful neuroma following amputation is a disabling condition for which treatments are not usually satisfactory. The aim of this study is to retrospectively evaluate the results of interdigital neurorrhaphy after neuroma resection as an option for the surgical treatment of painful digital neuroma. METHOD: We retrospectively analyzed data from seven patients submitted to interdigital neurorrhaphy for treatment of digital neuroma. For evaluation of the results, the visual analog scale (VAS) for pain assessment, measurements with Simmens-Weinstein filaments and a handgrip dynamometer, and quantification of the Disabilities of the Arm, Shoulder and Hand (DASH) score were all performed. The mean improvements on the VAS and DASH scores were calculated. RESULTS: The mean follow-up was 28.3 months. All patients presented some degree of improvement in upper limb functionality and pain, and handling the affected finger was possible in all cases. The mean improvement on the VAS and DASH scores was 29.8 and 55.5 %, respectively. No patients presented long-term complications. CONCLUSION: Although this study was based on a small number of patients, it indicates that interdigital neurorrhaphy seems to be a feasible surgical treatment for painful digital neuroma.

Nerve conduction studies in spastic paraplegia, optic atrophy, and neuropathy (SPOAN) syndrome.

INTRODUCTION: SPOAN (spastic paraplegia, optic atrophy, and neuropathy) syndrome is an autosomal recessive neurodegenerative disorder identified in a large consanguineous Brazilian family. METHODS: Twenty-seven patients with SPOAN syndrome (20 women), aged 4-58 years, underwent nerve conduction studies (NCS) of the median, ulnar, tibial, and fibular nerves, and sensory NCS of the median, ulnar, radial, sural, and superficial fibular nerves. RESULTS: Sensory nerve action potentials were absent in the lower limbs and absent in >80% of upper limbs. Motor NCS had reduced amplitudes and borderline velocities in the upper limbs and absent compound muscle action potentials (CMAPs) in the lower limbs. CONCLUSIONS: The neuropathy in SPOAN syndrome is a severe, early-onset sensory-motor axonal polyneuropathy. Normal NCS seem to rule-out this condition.

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.

Fibrolipomatous hamartoma of the median nerve: An unusual cause of carpal tunnel syndrome.

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.

The nerve to the levator scapulae muscle as donor in brachial plexus surgery: an anatomical study and case series.

OBJECTIVE: Nerve transfers are commonly used in treating complete injuries of the brachial plexus, but donor nerves are limited and preferentially directed toward the recovery of elbow flexion and shoulder abduction. The aims of this study were to characterize the anatomical parameters for identifying the nerve to the levator scapulae muscle (LSN) in brachial plexus surgery, to evaluate the feasibility of transferring this branch to the suprascapular nerve (SSN) or lateral pectoral nerve (LPN), and to present the results from a surgical series. METHODS: Supra- and infraclavicular exposure of the brachial plexus was performed on 20 fresh human cadavers in order to measure different anatomical parameters for identification of the LSN. Next, an anatomical and histomorphometric evaluation of the feasibility of transferring this branch to the SSN and LPN was made. Lastly, the effectiveness of the LSN-LPN transfer was evaluated among 10 patients by quantifying their arm adduction strength. RESULTS: The LSN was identified in 95% of the cadaveric specimens. A direct coaptation of the LSN and SSN was possible in 45% of the specimens (n = 9) but not between the LSN and LPN in any of the specimens. Comparison of axonal counts among the three nerves did not show any significant difference. Good results from reinnervation of the major pectoral muscle (Medical Research Council grade ≥ 3) were observed in 70% (n = 7) of the patients who had undergone LSN to LPN transfer. CONCLUSIONS: The LSN is consistently identified through a supraclavicular approach to the brachial plexus, and its transfer to supply the functions of the SSN and LPN is anatomically viable. Good results from an LSN-LPN transfer are observed in most patients, even if long nerve grafts need to be used.

Motor unit number index (MUNIX) in children and adults with 5q-spinal muscular atrophy: Variability and clinical correlations.

Spinal muscular atrophy (SMA) is a motor neuron disease associated with progressive muscle weakness and motor disability. The motor unit number index (MUNIX) is a biomarker used to assess loss of motor units in later-onset SMA patients. Twenty SMA patients (SMA types 3 and 4), aged between 7 and 41 years, were clinically evaluated through the Hammersmith Motor Functional Scale Expanded and the Spinal Muscular Atrophy-Functional Rating Scale. The patients underwent compound motor action potential (CMAP) and MUNIX studies of the right abductor pollicis brevis, abductor digiti minimi and tibialis anterior (TA) muscles. Age-matched healthy controls (n = 20) were enrolled to obtain normative CMAP and MUNIX values from the same muscles. Compared to healthy controls, SMA patients showed significant reductions in MUNIX values among all muscles studied, whereas CMAP showed reductions only in the weaker muscles (abductor digiti minimi and TA). MUNIX variability was significantly higher in the SMA group than in the control group. MUNIX variability in TA correlated with CMAP variability. Motor functional scores correlated with TA MUNIX. The MUNIX study is feasible in later-onset SMA patients, and TA MUNIX values correlate with disease severity in patients with mild motor impairment.

Electrophysiological study of neuromuscular junction in congenital myasthenic syndromes, congenital myopathies, and chronic progressive external ophthalmoplegia.

This study was designed to analyze the sensitivity, specificity, and accuracy of jitter parameters combined with repetitive nerve stimulation (RNS) in congenital myasthenic syndrome (CMS), chronic progressive external ophthalmoplegia (CPEO), and congenital myopathies (CM). Jitter was obtained with a concentric needle electrode during voluntary activation of the Orbicularis Oculi muscle in CMS (n = 21), CPEO (n = 20), and CM (n = 18) patients and in controls (n = 14). RNS (3 Hz) was performed in six different muscles for all patients (Abductor Digiti Minimi, Tibialis Anterior, upper Trapezius, Deltoideus, Orbicularis Oculi, and Nasalis). RNS was abnormal in 90.5% of CMS patients and in only one CM patient. Jitter was abnormal in 95.2% of CMS, 20% of CPEO, and 11.1% of CM patients. No patient with CPEO or CM presented a mean jitter higher than 53.6 µs or more than 30% abnormal individual jitter (> 45 µs). No patient with CPEO or CM and mild abnormal jitter values presented an abnormal decrement. Jitter and RNS assessment are valuable tools for diagnosing neuromuscular transmission abnormalities in CMS patients. A mean jitter value above 53.6 µs or the presence of more than 30% abnormal individual jitter (> 45 µs) strongly suggests CMS compared with CPEO and CM.

Neurological consultations and diagnoses in a large, dedicated COVID-19 university hospital.

BACKGROUND: More than one-third of COVID-19 patients present neurological symptoms ranging from anosmia to stroke and encephalopathy. Furthermore, pre-existing neurological conditions may require special treatment and may be associated with worse outcomes. Notwithstanding, the role of neurologists in COVID-19 is probably underrecognized. OBJECTIVE: The aim of this study was to report the reasons for requesting neurological consultations by internists and intensivists in a COVID-19-dedicated hospital. METHODS: This retrospective study was carried out at Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Brazil, a 900-bed COVID-19 dedicated center (including 300 intensive care unit beds). COVID-19 diagnosis was confirmed by SARS-CoV-2-RT-PCR in nasal swabs. All inpatient neurology consultations between March 23rd and May 23rd, 2020 were analyzed. Neurologists performed the neurological exam, assessed all available data to diagnose the neurological condition, and requested additional tests deemed necessary. Difficult diagnoses were established in consensus meetings. After diagnosis, neurologists were involved in the treatment. RESULTS: Neurological consultations were requested for 89 out of 1,208 (7.4%) inpatient COVID admissions during that period. Main neurological diagnoses included: encephalopathy (44.4%), stroke (16.7%), previous neurological diseases (9.0%), seizures (9.0%), neuromuscular disorders (5.6%), other acute brain lesions (3.4%), and other mild nonspecific symptoms (11.2%). CONCLUSIONS: Most neurological consultations in a COVID-19-dedicated hospital were requested for severe conditions that could have an impact on the outcome. First-line doctors should be able to recognize neurological symptoms; neurologists are important members of the medical team in COVID-19 hospital care.