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Contrast-enhanced magnetic resonance neurography (CE-MRN) holds promise for diagnosing brachial plexopathy by enhancing nerve visualization and revealing additional imaging features in various lesions. This study aims to validate CE-MRN's efficacy in improving brachial plexus (BP) imaging across different patient cohorts. Seventy-one subjects, including 19 volunteers and 52 patients with BP compression/entrapment, injury, and neoplasms, underwent both CE-MRN and plain MRN. Two radiologists assessed nerve visibility, with inter-reader agreement evaluated. Quantitative parameters such as signal intensity (SI), contrast-to-noise ratio (CNR), and contrast ratio (CR) of the C7 nerve were measured. Both qualitative scoring and quantitative metrics were compared between CE-MRN and plain MRN within each patient group. Patient classification followed the Neuropathy Score Reporting and Data System (NS-RADS), summarizing additional imaging features for each brachial plexopathy type. Inter-reader agreement for qualitative assessment was strong. CE-MRN significantly enhanced BP visualization and nerve-tissue contrast across all cohorts, particularly in volunteers and patients with injuries. It also uncovered additional imaging features such as hypointense signals in ganglia, compressed nerve sites, and neoplastic enhancements. CE-MRN effectively mitigated muscle edema and vascular contamination, enabling precise classification of BP injuries. Overall, CE-MRN consistently enhances BP visualization and provides valuable imaging features for accurate diagnosis.

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Takayasu arteritis is an inflammatory disease of unknown aetiology affecting large vessels. Medium vessel involvement is also well documented; however, neuropathy as a presenting manifestation is rare. In this case report, a young woman in her 20s presented with an 8-month history of intermittent claudication in the right upper limb progressing to rest pain with allodynia in C5-C8 distribution and painless right axillary mass. On examination, she had absent pulses in the right radial, brachial and subclavian artery with audible bruit in the right subclavian and abdominal aorta. CT angiogram showed features suggestive of Takayasu arteritis with a partially thrombosed aneurysm arising from the right axillary artery leading to compression of the right brachial plexus. This patient received treatment with methotrexate and oral corticosteroids. At 3 months follow-up, there was a reduction in the size of the aneurysm, resolution of compressive symptoms and normalisation of inflammatory markers.

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BACKGROUND: Brachial amyotrophic diplegia (BAD) is typically linked to a neurodegenerative etiology such as amyotrophic lateral sclerosis (ALS). Clinical and serological characterizations of paraneoplastic neurologic syndromes resembling BAD are limited. METHODS: A retrospective chart review of patients with BAD-like presentations was conducted. Clinical/paraclinical features of paraneoplastic BAD and neurodegenerative BAD cases were compared. RESULTS: Between 2017 and 2023, 13 cases of BAD were identified, of these 10 were neurodegenerative BAD (ALS variant), and 3 cases associated with paraneoplastic autoimmunity. An additional paraneoplastic BAD case diagnosed in 2005 was included. LUZP4-IgG was detected in all four paraneoplastic cases, with coexisting KLHL11-IgG in three cases and ANNA1 (anti-Hu)-IgG in one case. Out of the four paraneoplastic cases, two patients had seminoma, while the remaining two had limited cancer investigation. Three patients exhibited bi-brachial weakness as the initial symptom before the onset of brainstem symptoms or seizures. Compared to BAD patients with a neurodegenerative etiology, a higher proportion of paraneoplastic cases had ataxia (75% vs 0%, p = 0.011). Other clinical features only detected in the paraneoplastic BAD group were vertigo (n = 2), hearing loss (n = 2) and ophthalmoplegia (n = 2). Electrodiagnostic studies in these patients revealed cervical myotome involvement, supportive of motor neuronopathy. All paraneoplastic cases but none of the neurodegenerative BAD cases exhibited inflammatory cerebrospinal fluid (CSF) findings (lymphocytic pleocytosis and/or supernumerary oligoclonal bands; p = 0.067). Despite the administration of immunotherapy and/or cancer treatment, none of the paraneoplastic patients reported clinical improvement. DISCUSSION: BAD or bi-brachial neurogenic weakness is a rare phenotypic presentation associated with paraneoplastic autoimmunity. Co-existing features of brainstem dysfunction or cerebellar ataxia should prompt further paraneoplastic evaluation. Common serological and cancer associations among these cases include LUZP4-IgG and KLHL11-IgG, along with testicular germ cell tumors, respectively.

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BACKGROUND: The Sup-ER protocol involves a repositioning program for infants with brachial plexus birth injury to position the shoulder in external rotation (ER) to address progressive loss in passive range of motion (PROM). The British Columbia Children's Hospital (BCCH) eligibility criteria for this protocol are infants aged 4-8 weeks with decreased shoulder ER PROM and/or Active Movement Scale (AMS) shoulder ER and/or supination scores ≤2. The resources needed to implement this protocol in large clinics have not been studied. PURPOSE: This study aims to evaluate the BCCH criteria that are used to identify appropriate candidates for the Sup-ER protocol. STUDY DESIGN: A retrospective cohort study was conducted to identify the percentage of infants who would have been recommended the Sup-ER protocol based on their PROM and AMS scores between 4 and 8 weeks of age. METHODS: A sensitivity and specificity evaluation was used to describe the BCCH criteria's ability to identify infants in this historical cohort who went on to have incomplete shoulder function (ie, true positive) vs infants who had functional shoulder outcome at 9 months of age (ie, false positive). RESULTS: At a mean of 5.8 weeks (95% confidence interval [CI] 5.3, 6.3), 46 of the 87 (53%) infants satisfied the BCCH Sup-ER protocol criteria. Forty-four (51%) were female, half (n = 45) were left side affected, and 88% had upper plexus injury. The BCCH Sup-ER protocol criteria had sensitivity of 100% and specificity of 71% to identify infants with incomplete shoulder function. Removing the AMS supination ≤2 score criterion from the criteria improves the specificity to 84%, while sensitivity remains high (97%). CONCLUSIONS: Modifying the BCCH criteria to all infants aged 4-8 weeks with AMS shoulder ER ≤2 and/or decreased shoulder ER PROM improves the precision of identifying infants who would benefit from the Sup-ER protocol.

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Radiation-induced brachial plexopathy (RIBP) is an iatrogenic, progressively disabling, and often very late effect of adjuvant radiotherapy most commonly seen in breast cancer survivors but also in those treated for lymphoma, lung, and head and neck cancers. In late-onset RIBP following breast cancer, the nerve injury is chronic and irreversible, occurring more commonly when axillary and/or supraclavicular nodes have been irradiated, as well as the breast/chest wall. RIBP is manifested initially by paresthesia, hypoesthesia, dysesthesia, and later by weakness in the ipsilateral hand with those symptoms progressing distally to proximally up through the shoulder. Depressed/absent deep tendon reflexes in the upper extremity and muscle fasciculations occur also. Neither patients nor their health care providers tend to associate these unusual neurological symptoms with cancer treatments received ≥20 years prior, often failing to link these sensory-motor symptoms with radiotherapy decades before. Because long-term follow-up of these patients now typically falls to general practitioners, many cases may be missed or misdiagnosed because of the rarity of this disorder. Physiatrists and allied rehabilitation professionals must be aware of this progressively disabling, incurable condition to provide appropriate diagnoses and compensatory rehabilitation therapies. Additionally, professional oncology organizations should include RIBP in their long-term, survivorship guidelines for breast cancer. Researchers examining the iatrogenic late effects of radiotherapy should extend their follow-up periods well beyond the current 5-6 years to ascertain the true incidence of RIBP today. Rehabilitation providers must continue to advocate for awareness, diagnosis, and management of iatrogenic outcomes experienced by long-term cancer survivors.

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OBJECTIVE: To determine if children who present with an elbow flexion contracture (EFC) from brachial plexus birth injury (BPBI) are more likely to develop shoulder contracture and undergo surgical treatment. STUDY DESIGN: Retrospective review of children <2 years of age with BPBI who presented to a single children's hospital from 1993 to 2020. Age, elbow and shoulder range of motion (ROM), imaging measurements, and surgical treatment and outcome were analyzed. Patients with an EFC of ≥10° were included in the study sample. Data from 2445 clinical evaluations (1190 patients) were assessed. The final study cohort included 72 EFC cases matched with 230 non-EFC controls. Three patients lacked sufficient follow-up data. RESULTS: There were 299 included patients who showed no differences between study and control groups with respect to age, sex, race, ethnicity, or functional score. Patients with EFC had 12° less shoulder range of motion (95% CI, 5°-20°; P < .001) and had 2.5 times the odds of shoulder contracture (OR, 2.5; 95% CI, 1.3-4.7; P = .006). For each additional 5° of EFC, the odds of shoulder contracture increased by 50% (OR, 1.5; 95% CI, 1.2-1.8; P < .001) and odds of shoulder procedure increased by 62% (OR, 1.62; 95% CI, 1.04-2.53; P = .03). Sensitivity of EFC for predicting shoulder contracture was 49% and specificity was 82%. CONCLUSIONS: In patients with BPBI <2 years of age, presence of EFC can be used as a screening tool in identifying shoulder contractures that may otherwise be difficult to assess. Prompt referral should be arranged for evaluation at a BPBI specialty clinic, because delayed presentation risks worsening shoulder contracture and potentially more complicated surgery.

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Forearm deformities are often seen in children with severe birth brachial plexus palsy (BBPP). They may be either a supination or a pronation deformity and both hinder normal use of the hand and parents often request for corrective surgery. However, the correction of these deformities can be challenging due to a paucity of options. Also, there is less information in literature on the management of forearm deformities in BBPP compared to the information with regard to nerve surgery or correction of shoulder deformities. This article presents a synopsis of incidence, pathogenesis, clinical presentation and parental concerns related to these deformities. The decision-making considerations, management strategies and outcome expectations are also discussed. Patient selection is very crucial, and the treatment plan must be individualised depending on the disability, parental expectations and existing motor power in the involved limb. Correction of both the deformities have different considerations; however, effective correction of these deformities is immensely satisfactory to the patient/parents in terms of improved function and appearance of the limb. Level of Evidence: Level V (Therapeutic).

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BACKGROUND: Neuralgic amyotrophy (NA) is a clinically acute or subacute disease. To study the characteristics of brachial plexus magnetic resonance neurography (MRN) in patients with NA, and to explore the clinical application value of MRN combined with electromyography (EMG) in the diagnosis of NA. METHODS: Brachial plexus MRN images of 32 patients with NA were retrospectively analyzed, and their characteristics were investigated. The accuracy, sensitivity and specificity of MRN, EMG, and the combination of the 2 methods for NA diagnosis were compared. RESULTS: Among the 32 patients with NA, 28 (87.5%) cases of unilateral brachial plexus involvement, 18 (56.3%) cases of multiple nerve roots involvement. In 10 cases, C5 nerve roots were involved alone, and in 9 cases, C5 to C6 nerve roots were involved together. The T2 signal intensity of the affected nerve increased, and 19 cases showed thickened and smooth nerve root edges. Twelve cases showed uneven thickening and segmental stenosis of the involved nerve roots. The diagnostic accuracy, sensitivity, and specificity of MRN for NA were higher than those of EMG. Combining MRN and EMG could improve the sensitivity and specificity of diagnosis. CONCLUSION: The main feature of MRN in patients with NA was that it was unilateral brachial plexus asymmetric involvement. The diagnostic effect of MRN was better than that of EMG. The combined diagnosis of MRN and EMG can help clinicians diagnose NA accurately.

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LEARNING OBJECTIVES: After studying this article, the participant should be able to: 1. Describe methods of clinical evaluation for neurologic recovery in brachial plexus birth injury. 2. Understand the role of different diagnostic imaging modalities to evaluate the upper limb. 3. List nonsurgical strategies and surgical procedures to manage shoulder abnormality. 4. Explain the advantages and disadvantages of microsurgical nerve reconstruction and distal nerve transfers in brachial plexus birth injury. 5. Recognize the prevalence of pain in this population and the need for greater sensory outcomes evaluation. SUMMARY: Brachial plexus birth injury (BPBI) results from closed traction injury to the brachial plexus in the neck during an infant's vertex passage through the birth canal. Although spontaneous upper limb recovery occurs in most instances of BPBI, some infants do not demonstrate adequate motor recovery within an acceptable timeline and require surgical intervention to restore upper limb function. This article reviews major advances in the management of BPBI in the past decade that include improved understanding of shoulder pathology and its impact on observed motor recovery, novel surgical techniques, new insights in sensory function and pain, and global efforts to develop standardized outcomes assessment scales.

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OBJECTIVES: The main objective of this study was to determine whether infrared thermography could be used as an efficient technique to evaluate the impact of a birth-related brachial plexus injury on the temperature of the injured arm and whether it could be used as a complementary method when diagnosing this injury in clinical praxis. BACKGROUND: Clinically, the brachial plexus injury is a peripheral paresis, which occurs when nerves that send signals from the spinal cord to the shoulder, arm, and hand are stretched or compressed. In principle, the brachial plexus injury, as a long-lasting injury, should be causing hypothermia of the injured arm. METHODS: The usage of contactless infrared thermography could offer a "new view" of the diagnostic process in this case. The present study, therefore, describes a process of clinical infrared thermography examination of three patients of different age and presents results from those examinations. RESULTS AND CONCLUSION: From our results, it can be confirmed that the birth-related brachial plexus injury affects the temperature of the affected arm, especially in the area of the cubital fossa, to an extent that the thermal camera is capable of detecting significant temperature differences between the healthy and injured arms (Tab. 3, Fig. 7, Ref. 13). Text in PDF www.elis.sk Keywords: birth brachial plexus injury, upper type palsy, peripheral palsy, infrared thermography.

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BACKGROUND: The utilization of International Classification of Diseases, Ninth or Tenth Revision, (ICD-9/10) coding to identify the incidence of disease is frequently performed in medical research. This study attempts to assess the validity of using ICD-9/10 codes to identify patients with shoulder dystocia (SD) with concurrent neonatal brachial plexus palsy (NBPP). METHODS: This retrospective cohort study examined patients evaluated at the University of Michigan Brachial Plexus and Peripheral Nerve Program (UM-BP/PN) from 2004 to 2018. We reported the percentage of patients with reported NBPP ICD-9/10 and SD ICD-9/10 discharged at birth who were later diagnosed with NBPP by a specialty clinic by interdisciplinary faculty and staff utilizing physical evaluations and ancillary testing such as such as electrodiagnostics and imaging. The relationship of reported NBPP ICD-9/10, SD ICD-9/10, extent of NBPP nerve involvement, and NBPP persistence at age two years were examined via chi-square or Fischer exact test. RESULTS: Of the 51 mother-infant dyads with complete birth discharge records evaluated at the UM-BP/PN, 26 (51%) were discharged without an ICD-9/10 code documenting NBPP; of these 26 patients, only four had ICD-9/10 documentation of SD at discharge, which left 22 patients with no ICD-9/10 code documentation of either SD or NBPP (43%). Patients with pan-plexopathy were more likely to be discharged with an NBBP ICD-9/10 code than those infants with upper nerve involvement (77% vs 39%, P < 0.02). CONCLUSION: Use of ICD-9/10 codes for the identification of NBPP appears to undercount the true incidence. This underestimation is more pronounced for milder forms of NBPP.

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Neonatal brachial plexus palsies (NBPP) occur in 1.74 per 1000 live births with 20% to 30% having persistent deficits. Dysfunction can range from mild to severe and is correlated with the number of nerves involved and the degree of injury. In addition, there are several comorbidities and musculoskeletal sequelae that directly impact the overall functional development. This review addresses the nonsurgical and surgical management options and provides guidance for pediatricians on monitoring and when to refer for specialty care.

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BACKGROUND: Brachial and lumbosacral plexopathies can result from numerous non-traumatic etiologies, including those of inflammatory, autoimmune, or neoplastic origin, that often require nerve biopsy for diagnosis. The purpose of this study was to evaluate the diagnostic efficacy of medial antebrachial cutaneous nerve (MABC) and posterior femoral cutaneous nerve (PFCN) nerve biopsies in proximal brachial and lumbosacral plexus pathology. METHOD: Patients undergoing MABC or PFCN nerve biopsies at a single institution were reviewed. Patient demographics, clinical diagnosis, symptom duration, intraoperative findings, post-operative complications, and pathology results were recorded. Biopsy results were classified as diagnostic, inconclusive, or negative based on the final pathology. RESULTS: Thirty patients undergoing MABC biopsies in the proximal arm or axilla and five patients with PFCN biopsies in the thigh or buttock were included. MABC biopsies were diagnostic in 70% of cases overall and 85% diagnostic in cases where pre-operative MRI also demonstrated abnormalities in the MABC. PFCN biopsies were diagnostic in 60% of cases overall and in 100% of patients with abnormal pre-operative MRIs. There were no biopsy-related post-operative complications in either group. CONCLUSIONS: In diagnosing non-traumatic etiologies of brachial and lumbosacral plexopathies, proximal biopsies of the MABC and PFCN provide high diagnostic value with low donor morbidity.

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Infants can sustain traction injury to brachial plexus nerves during birth, called brachial plexus birth injury (BPBI). While spontaneous recovery is possible, upper extremity weakness can linger. We report our experience at a brachial plexus clinic from a retrospective chart review of infants with BPBI from September 2017 to September 2019. We determined Narakas Classification (NC) and Active Movement Scale (AMS) at predetermined follow-up points. Of 15 patients, 8 presented with NC-I, 5 with NC-II, and 2 with NC-III without Horner's syndrome. By 7 months, 3 had spontaneous recovery, and 4 achieved all and another 4 achieved most AMS5-7 scores. Eleven patients undergoing surgery had little-to-no improvement of shoulder abduction and shoulder external rotation AMS categories by 6 months. Our small sample size prevents us from making definitive conclusions but gave beneficial insight into our clinic barriers to follow-up, data collection, and collaboration with physical and occupational therapy.

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BACKGROUND: Manual muscle testing is a mainstay of strength assessment despite not having been compared with intraoperative electrical stimulation of peripheral nerves. METHODS: Intraoperative electrical stimulation served as the reference standard in evaluating predictive accuracy of the Active Movement Scale (AMS) and the Medical Research Council (MRC) scale. Retrospective consecutive sampling of all patients with AFM who underwent exploration or nerve transfer at a pediatric multidisciplinary brachial plexus and peripheral nerve center from March of 2016 to July of 2020 were included. The nonparametric area under the curve (AUC) was calculated. Optimal cutoff score (Youden J ) and diagnostic accuracy values were reported. The AMS and MRC scale were directly compared for predictive superiority. RESULTS: A total of 181 upper extremity nerves (73 donor nerve candidates and 108 recipient nerve candidates) were tested intraoperatively from 40 children (mean age ± SD, 7.9 ± 4.9 years). The scales performed similarly ( P = 0.953) in classifying suitable donor nerves with satisfactory accuracy (AUC AMS , 71.5%; AUC MRC , 70.7%; optimal cutoff, AMS >5 and MRC >2). The scales performed similarly ( P = 0.688) in classifying suitable recipient nerves with good accuracy (AUC AMS , 92.1%; AUC MRC :, 94.9%; optimal cutoff, AMS ≤3 and MRC ≤1). CONCLUSIONS: Manual muscle testing is an accurate, noninvasive means of identifying donor and recipient nerves for transfer in children with acute flaccid myelitis. The utility of these results is in minimizing unexpected findings in the operating room and aiding in the development of contingency plans. Further research may extend these findings to test the validity of manual muscle testing as an outcome measure of the success of nerve transfer. CLINICAL QUESTION/LEVEL OF EVIDENCE: Diagnostic, I.

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BACKGROUND: Nivolumab is an immune checkpoint inhibitor that targets the programmed cell death-1 protein and is effective in treating advanced cancer. However, it is also associated with various immune-related neurological complications, including myasthenia gravis, Guillain-Barré syndrome, and demyelinating polyneuropathy. These complications can easily mimic other neurological diseases and have greatly varying therapeutic approaches depending on the underlying pathophysiology. CASE PRESENTATION: Here, we report a case of nivolumab-induced demyelinating peripheral polyneuropathy involving the brachial plexus in a patient with Hodgkin lymphoma. Approximately 7 months after nivolumab treatment, the patient experienced muscle weakness with a tightness and tingling sensation in the right forearm. Electrodiagnostic studies showed features of demyelinating peripheral neuropathy with right brachial plexopathy. Magnetic resonance imaging revealed thickening with a diffuse enhancement of both brachial plexuses. The patient was eventually diagnosed with nivolumab-induced demyelinating polyneuropathy involving the brachial plexus. Oral steroid therapy improved motor weakness and sensory abnormalities without aggravation. CONCLUSION: Our study indicates the possibility of nivolumab-induced neuropathies in cases involving muscle weakness with sensory abnormalities of the upper extremity following nivolumab administration in patients with advanced cancer. Comprehensive electrodiagnostic studies and magnetic resonance imaging are helpful in the differential diagnosis of other neurological diseases. Appropriate diagnostic and therapeutic approaches may prevent further neurological deterioration.

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BACKGROUND: Neonatal brachial plexus palsy (NBPP) results in muscle weakness and impaired somatosensory function of the arm. Current functional assessment is primarily based on clinician-elicited measurements including muscle strength and range of motion. To what extent these measures are representative of real-world arm movement is unclear. OBJECTIVE: To determine the feasibility of using body-worn accelerometers to remotely assess arm movements in children with NBPP. DESIGN: Prospective criterion validity study of accelerometry versus clinician assessment. SETTING: Academic medical center. PARTICIPANTS: Nine adolescents with NBPP and nine age- and gender-matched control adolescents participated in the study. All were enrolled in school and participated in community activities. INTERVENTIONS: Not applicable. METHODS: Standard clinician-elicited measurements were collected. For assessing spontaneous arm movements, participants wore activity monitors during all waking hours for 7 days. Results were expressed as ratios of affected to unaffected arm motion for duration and magnitude and correlated with traditional clinic-based assessments. Spearman correlations were used to determine relationships between accelerometry results and traditional assessments. A p value <.05 was considered statistically significant. MAIN OUTCOME MEASUREMENTS: Accelerometry measurements of arm motion and traditional clinical assessments. RESULTS: Compared to control ratios, duration of arm movement and magnitude ratios were reduced in the NBPP group, particularly for arm magnitude due to reduced affected arm movement and an increase in unaffected arm movement. Ratios were highly correlated with shoulder function and, to a lesser extent, with elbow function. CONCLUSION: Real-world arm use is an appropriate outcome measure that reflects functional recovery. This study demonstrates the feasibility of wearable technology to quantify duration and intensity of spontaneous arm movement in children with NBPP. Accelerometry also allows for the association between traditional clinician-elicited assessment measures and spontaneous arm movements, demonstrating the importance of the shoulder as a focus of treatment in NBPP.

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