ABSTRACT
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.
Subject(s)
Brachial Plexus Neuropathies , Brachial Plexus , Neonatal Brachial Plexus Palsy , Infant, Newborn , Infant , Humans , Neonatal Brachial Plexus Palsy/surgery , Retrospective Studies , Brachial Plexus Neuropathies/surgery , Neurosurgical ProceduresABSTRACT
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.
ABSTRACT
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.
Subject(s)
Thoracic Outlet Syndrome , Decompression, Surgical/methods , Hand/surgery , Humans , Retrospective Studies , Thoracic Outlet Syndrome/diagnosis , Thoracic Outlet Syndrome/surgery , Treatment Outcome , Upper Extremity/surgeryABSTRACT
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.
Subject(s)
Brachial Plexus Neuropathies , Brachial Plexus , Neonatal Brachial Plexus Palsy , Brachial Plexus/surgery , Brachial Plexus Neuropathies/etiology , Brachial Plexus Neuropathies/surgery , Female , Humans , Infant, Newborn , Male , Retrospective StudiesABSTRACT
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 (nâ¯=â¯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.
Subject(s)
Motor Disorders/physiopathology , Muscular Atrophy, Spinal/physiopathology , Action Potentials , Adolescent , Adult , Biomarkers , Child , Electromyography , Humans , Male , Motor Neurons/physiology , Muscle Weakness , Muscle, Skeletal/physiopathology , Severity of Illness Index , Young AdultABSTRACT
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.
Subject(s)
Lupus Erythematosus, Cutaneous/drug therapy , Lupus Erythematosus, Systemic/drug therapy , Peripheral Nervous System Diseases/chemically induced , Thalidomide/adverse effects , Adult , Female , Humans , Incidence , Male , Middle Aged , Prospective Studies , Thalidomide/blood , Thalidomide/therapeutic use , Treatment Outcome , Withholding TreatmentABSTRACT
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.
ABSTRACT
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.
Subject(s)
Lupus Erythematosus, Cutaneous , Lupus Erythematosus, Systemic , Humans , Lenalidomide , Lupus Erythematosus, Cutaneous/chemically induced , Lupus Erythematosus, Cutaneous/diagnosis , Lupus Erythematosus, Cutaneous/drug therapy , Lupus Erythematosus, Systemic/diagnosis , Lupus Erythematosus, Systemic/drug therapy , Retrospective Studies , Thalidomide/adverse effectsABSTRACT
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 (nâ¯=â¯21), CPEO (nâ¯=â¯20), and CM (nâ¯=â¯18) patients and in controls (nâ¯=â¯14). RNS (3â¯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.
Subject(s)
Muscular Diseases/physiopathology , Myasthenic Syndromes, Congenital/physiopathology , Neuromuscular Junction/physiopathology , Ophthalmoplegia, Chronic Progressive External/physiopathology , Adolescent , Adult , Case-Control Studies , Electric Stimulation , Electrodes , Electromyography , Female , Humans , Male , Middle Aged , Muscle, Skeletal/physiopathology , Sensitivity and Specificity , Young AdultABSTRACT
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.
Subject(s)
Coronavirus Infections/diagnosis , Nervous System Diseases/etiology , Pandemics , Pneumonia, Viral/diagnosis , Referral and Consultation/statistics & numerical data , Betacoronavirus , Brazil/epidemiology , COVID-19 , Coronavirus Infections/complications , Coronavirus Infections/epidemiology , Hospital Bed Capacity , Hospitals, University , Humans , Nervous System Diseases/diagnosis , Nervous System Diseases/therapy , Neurology , Pneumonia, Viral/complications , Pneumonia, Viral/epidemiology , Retrospective Studies , SARS-CoV-2ABSTRACT
ABSTRACT 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.
RESUMO Introdução: Mais de um terço dos pacientes com COVID-19 apresentam sintomas neurológicos que variam de anosmia a AVC e encefalopatia. Além disso, doenças neurológicas prévias podem exigir tratamento especial e estar associadas a piores desfechos. Não obstante, o papel dos neurologistas na COVID-19 é provavelmente pouco reconhecido. Objetivo: O objetivo deste estudo foi relatar os motivos para solicitar consultas neurológicas por clínicos e intensivistas em um hospital dedicado à COVID-19. Métodos: Estudo retrospectivo realizado no Hospital das Clínicas da Faculdade de Medicina da Universidade de São Paulo, Brasil, um centro dedicado à COVID-19 com 900 leitos (incluindo 300 leitos para unidades de terapia intensiva). O diagnóstico de COVID-19 foi confirmado por SARS-CoV-2-RT-PCR em swabs nasais. Todas as interconsultas de neurologia hospitalar entre 23 de março e 23 de maio de 2020 foram analisadas. Os neurologistas realizaram o exame neurológico, avaliaram todos os dados disponíveis para diagnosticar a patologia neurológica e solicitaram exames adicionais conforme necessidade. Diagnósticos difíceis foram estabelecidos em reuniões de consenso. Após o diagnóstico, os neurologistas participaram da condução dos casos. Resultados: Foram solicitadas consultas neurológicas para 89 de 1.208 (7,4%) em pacientes internados por COVID-19 durante o período. Os principais diagnósticos neurológicos incluíram: encefalopatia (44,4%), acidente vascular cerebral (16,7%), doenças neurológicas prévias (9,0%), crises epilépticas (9,0%), transtornos neuromusculares (5,6%), outras lesões encefálicas agudas (3,4%) e outros sintomas leves inespecíficos (11,2%). Conclusões: A maioria das consultas neurológicas em um hospital dedicado à COVID-19 foi solicitada para condições graves que poderiam afetar o desfecho clínico. Os médicos na linha de frente devem ser capazes de reconhecer sintomas neurológicos. Os neurologistas são membros importantes da equipe médica no atendimento hospitalar à COVID-19.
Subject(s)
Humans , Pneumonia, Viral/diagnosis , Referral and Consultation/statistics & numerical data , Coronavirus Infections/diagnosis , Pandemics , Nervous System Diseases/etiology , Pneumonia, Viral/complications , Pneumonia, Viral/epidemiology , Brazil/epidemiology , Retrospective Studies , Coronavirus Infections , Coronavirus Infections/complications , Coronavirus Infections/epidemiology , Betacoronavirus , Hospital Bed Capacity , Hospitals, University , Nervous System Diseases/diagnosis , Nervous System Diseases/therapy , NeurologyABSTRACT
Background: More than one-third of COVID-19 patients present neurological symptomsranging from anosmia to stroke and encephalopathy. Furthermore, pre-existingneurological conditions may require special treatment and may be associated with worseoutcomes. Notwithstanding, the role of neurologists in COVID-19 is probablyunderrecognized. Objective: The aim of this study was to report the reasons forrequesting neurological consultations by internists and intensivists in a COVID-19-dedicated hospital. Methods: This retrospective study was carried out at Hospital dasClínicas da Faculdade de Medicina da Universidade de São Paulo, Brazil, a 900-bedCOVID-19 dedicated center (including 300 intensive care unit beds). COVID-19 diagnosiswas confirmed by SARS-CoV-2-RT-PCR in nasal swabs. All inpatient neurologyconsultations between March 23rd and May 23rd, 2020 were analyzed. Neurologistsperformed the neurological exam, assessed all available data to diagnose theneurological condition, and requested additional tests deemed necessary. Difficultdiagnoses were established in consensus meetings. After diagnosis, neurologists wereinvolved in the treatment. Results: Neurological consultations were requested for 89 outof 1,208 (7.4%) inpatient COVID admissions during that period. Main neurologicaldiagnoses included: encephalopathy (44.4%), stroke (16.7%), previous neurologicaldiseases (9.0%), seizures (9.0%), neuromuscular disorders (5.6%), other acute brainlesions (3.4%), and other mild nonspecific symptoms (11.2%). Conclusions: Mostneurological consultations in a COVID-19-dedicated hospital were requested for severeconditions that could have an impact on the outcome. First-line doctors should be able torecognize neurological symptoms; neurologists are important members of the medicalteam in COVID-19 hospital care.
Introdução: Mais de um terço dos pacientes com COVID-19 apresentam sintomasneurológicos que variam de anosmia a AVC e encefalopatia. Além disso, doençasneurológicas prévias podem exigir tratamento especial e estar associadas a pioresdesfechos. Não obstante, o papel dos neurologistas na COVID-19 é provavelmentepouco reconhecido. Objetivo: O objetivo deste estudo foi relatar os motivos para solicitarconsultas neurológicas por clínicos e intensivistas em um hospital dedicado à COVID-19. Métodos: Estudo retrospectivo realizado no Hospital das Clínicas da Faculdade deMedicina da Universidade de São Paulo, Brasil, um centro dedicado à COVID-19 com900 leitos (incluindo 300 leitos para unidades de terapia intensiva). O diagnóstico deCOVID-19 foi confirmado por SARS-CoV-2-RT-PCR em swabs nasais. Todas asinterconsultas de neurologia hospitalar entre 23 de março e 23 de maio de 2020 foramanalisadas. Os neurologistas realizaram o exame neurológico, avaliaram todos os dadosdisponíveis para diagnosticar a patologia neurológica e solicitaram exames adicionaisconforme necessidade. Diagnósticos difíceis foram estabelecidos em reuniões deconsenso. Após o diagnóstico, os neurologistas participaram da condução dos casos.Resultados: Foram solicitadas consultas neurológicas para 89 de 1.208 (7,4%) empacientes internados por COVID-19 durante o período. Os principais diagnósticosneurológicos incluíram: encefalopatia (44,4%), acidente vascular cerebral (16,7%),doenças neurológicas prévias (9,0%), crises epilépticas (9,0%), transtornosneuromusculares (5,6%), outras lesões encefálicas agudas (3,4%) e outros sintomasleves inespecíficos (11,2%). Conclusões: A maioria das consultas neurológicas em umhospital dedicado à COVID-19 foi solicitada para condições graves que poderiam afetaro desfecho clínico. Os médicos na linha de frente devem ser capazes de reconhecersintomas neurológicos. Os neurologistas são membros importantes da equipe médica noatendimento hospitalar à COVID-19.
ABSTRACT
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.
Subject(s)
Birth Injuries , Brachial Plexus Neuropathies , Brachial Plexus , Neonatal Brachial Plexus Palsy , Birth Injuries/surgery , Brachial Plexus/injuries , Brachial Plexus/surgery , Brachial Plexus Neuropathies/etiology , Brachial Plexus Neuropathies/surgery , Humans , Infant, Newborn , Recovery of FunctionABSTRACT
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.
Subject(s)
Brachial Plexus Neuropathies , Nerve Transfer , Arm , Brachial Plexus Neuropathies/surgery , Child , Humans , Infant, Newborn , Muscle, Skeletal , Treatment Outcome , Ulnar Nerve/surgeryABSTRACT
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.
Subject(s)
Facial Nerve Diseases/diagnosis , Facial Nerve Diseases/genetics , Peripheral Nervous System Diseases/diagnosis , Peripheral Nervous System Diseases/genetics , Adolescent , Facial Nerve Diseases/complications , Female , Humans , Peripheral Nervous System Diseases/complications , Video Recording/methodsSubject(s)
Humans , Female , Adult , Sjogren's Syndrome/pathology , Sjogren's Syndrome/diagnostic imaging , Peripheral Nervous System Diseases/pathology , Peripheral Nervous System Diseases/diagnostic imaging , Salivary Glands/pathology , Biopsy , Magnetic Resonance Imaging , Sensation Disorders/pathology , Sensation Disorders/diagnostic imagingABSTRACT
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).
Subject(s)
Neonatal Brachial Plexus Palsy/surgery , Neurosurgical Procedures/methods , Plastic Surgery Procedures/methods , Female , Humans , Infant, Newborn , Male , Retrospective Studies , Treatment OutcomeSubject(s)
Peripheral Nervous System Diseases/diagnostic imaging , Peripheral Nervous System Diseases/pathology , Sjogren's Syndrome/diagnostic imaging , Sjogren's Syndrome/pathology , Adult , Biopsy , Female , Humans , Magnetic Resonance Imaging , Salivary Glands/pathology , Sensation Disorders/diagnostic imaging , Sensation Disorders/pathologyABSTRACT
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.