RESUMEN
Little is known about the electrophysiologic activity of the intact human spinal cord during volitional movement. We analyzed epidural spinal recordings from a total of five human subjects of both sexes during a variety of upper extremity movements and found that these spinal epidural electrograms contain spectral information distinguishing periods of movement, rest, and sensation. Cervical epidural electrograms also contained spectral changes time-locked with movement. We found that these changes were primarily associated with increased power in the theta (4-8â Hz) band and feature increased theta phase to gamma amplitude coupling, and this increase in theta power can be used to topographically map distinct upper extremity movements onto the cervical spinal cord in accordance with established myotome maps of the upper extremity. Our findings have implications for the development of neurostimulation protocols and devices focused on motor rehabilitation for the upper extremity, and the approach presented here may facilitate spatiotemporal mapping of naturalistic movements.
Asunto(s)
Movimiento , Humanos , Masculino , Femenino , Movimiento/fisiología , Adulto , Volición/fisiología , Médula Cervical/fisiología , Espacio Epidural/fisiología , Vértebras Cervicales/fisiología , Electromiografía/métodos , Persona de Mediana EdadRESUMEN
INTRODUCTION: Traumatic spinal cord injuries (tSCI) are common, often leaving patients irreparably debilitated. Therefore, novel strategies such as nerve transfers (NT) are needed for mitigating secondary SCI damage and improving function. Although different tSCI NT options exist, little is known about the epidemiological and injury-related aspects of this patient population. Here, we report such characteristics to better identify and understand the number and types of tSCI individuals who may benefit from NTs. MATERIALS AND METHODS: Two peripheral nerve experts independently evaluated all adult tSCI individuals < 80 years old admitted with cervical tSCI (C1-T1) between 2005 and 2019 with documented tSCI severity using the ASIA Impairment Scale for suitability for NT (nerve donor with MRC strength ≥ 4/5 and recipient ≤ 2/5). Demographic, traumatic injury, and neurological injury variables were collected and analyzed. RESULTS: A total of 709 tSCI individuals were identified with 224 (32%) who met the selection criteria for participation based on their tSCI level (C1-T1). Of these, 108 (15% of all tSCIs and 48% of all cervical tSCIs) were deemed to be appropriate NT candidates. Due to recovery, 6 NT candidates initially deem appropriate no longer qualified by their last follow-up. Conversely, 19 individuals not initially considered appropriate then become eligible by their last follow-up. CONCLUSION: We found that a large proportion of individuals with cervical tSCI could potentially benefit from NTs. To our knowledge, this is the first study to detail the number of tSCI individuals that may qualify for NT from a large prospective database.
RESUMEN
OBJECTIVE. This systematic review and meta-analysis evaluates the diagnostic accuracy of MRI for differentiating malignant (MPNSTs) from benign peripheral nerve sheath tumors (BPNSTs). MATERIALS AND METHODS. A systematic review of MEDLINE, Embase, Scopus, the Cochrane Library, and the gray literature from inception to December 2019 was performed. Original articles that involved at least 10 patients and that evaluated the accuracy of MRI for detecting MPNSTs were included. Two reviewers independently extracted clinical and radiologic data from included articles to calculate sensitivity, specificity, PPV, NPV, and accuracy. A meta-analysis was performed using a bivariate mixed-effects regression model. Risk of bias was evaluated using QUADAS-2. RESULTS. Fifteen studies involving 798 lesions (252 MPNSTs and 546 BPNSTs) were included in the analysis. Pooled and weighted sensitivity, specificity, and AUC values for MRI in detecting MPNSTs were 68% (95% CI, 52-80%), 93% (95% CI, 85-97%), and 0.89 (95% CI, 0.86-0.92) when using feature combination and 88% (95% CI, 74-95%), 94% (95% CI, 89-96%), and 0.97 (95% CI, 0.95-0.98) using diffusion restriction with or without feature combination. Subgroup analysis, such as patients with neurofibromatosis type 1 (NF1) versus those without NF1, could not be performed because of insufficient data. Risk of bias was predominantly high or unclear for patient selection, mixed for index test, low for reference standard, and unclear for flow and timing. CONCLUSION. Combining features such as diffusion restriction optimizes the diagnostic accuracy of MRI for detecting MPNSTs. However, limitations in the literature, including variability and risk of bias, necessitate additional methodologically rigorous studies to allow subgroup analysis and further evaluate the combination of clinical and MRI features for MPNST diagnosis.
Asunto(s)
Imagen por Resonancia Magnética/métodos , Neoplasias de la Vaina del Nervio/diagnóstico por imagen , Neoplasias de la Vaina del Nervio/patología , Diagnóstico Diferencial , Humanos , Reproducibilidad de los Resultados , Sensibilidad y EspecificidadRESUMEN
AIMS: Lumbar to sacral rerouting surgery can potentially allow voiding via a skin-central nervous system-bladder reflex pathway. Here, we assessed if this surgery was effective in treating neurogenic bladder dysfunction/sphincter in felines. METHODS: Eight cats underwent spinal cord transection (SCT) at thoracic level 10/11. Unilateral L7 to S1 ventral root anastomosis was performed 1 month later in six cats. Two cats served as transection-only controls. Electrical and manual stimulation of L6-S1 dermatomes, and urodynamics were performed at 3, 5, 7, and 9/10 months post transection. At 9/10 months, cats were also evaluated by direct electrophysiological testing of anastomosed roots with urodynamics, then tissue collection and examination of the root anastomosis site and lumbosacral cord ventral horns for cells retrogradely labeled from tracer dye injected 2 weeks earlier into the bladder wall. RESULTS: At 9/10 months, four of six rerouted cats exhibited increased detrusor pressure provoked by cutaneous stimulation, one cat bilaterally. Two cats presented with a voiding stream after ipsilateral cutaneous stimulation at 7 and 9 months. All six rerouted animals showed regrowth of axons from the L7 ventral horn to the bladder, although some aberrant axonal regrowth was also observed. CONCLUSION: L7 to S1 ventral root rerouting below the level of SCT showed successful axonal regrowth to the bladder from the L7 spinal cord segment in all rerouted animals, and induced increased detrusor pressure response to cutaneous stimulation in a subset. This feasibility study paves the way for future animal studies for bladder reinnervation.
Asunto(s)
Anastomosis Quirúrgica/métodos , Sacro/cirugía , Traumatismos de la Médula Espinal/cirugía , Raíces Nerviosas Espinales/cirugía , Vejiga Urinaria Neurogénica/cirugía , Urodinámica/fisiología , Animales , Gatos , Estudios de Factibilidad , Proyectos Piloto , Sacro/fisiopatología , Traumatismos de la Médula Espinal/complicaciones , Traumatismos de la Médula Espinal/fisiopatología , Raíces Nerviosas Espinales/fisiopatología , Vejiga Urinaria Neurogénica/etiología , Vejiga Urinaria Neurogénica/fisiopatología , Micción/fisiologíaRESUMEN
Neurogenic thoracic outlet syndrome (nTOS) is caused by compression of the brachial plexus as it traverses from the thoracic outlet to the axilla. Diagnosing nTOS can be difficult because of overlap with other complex pain and entrapment syndromes. An nTOS diagnosis is made based on patient history, physical exam, electrodiagnostic studies, and, more recently, interpretation of MR neurograms with tractography. Advances in high-resolution MRI and tractography can confirm an nTOS diagnosis and identify the location of nerve compression, allowing tailored surgical decompression. In this report, the authors review the current diagnostic criteria, present an update on advances in MRI, and provide case examples demonstrating how MR neurography (MRN) can aid in diagnosing nTOS. The authors conclude that improved high-resolution MRN and tractography are valuable tools for identifying the source of nerve compression in patients with nTOS and can augment current diagnostic modalities for this syndrome.
Asunto(s)
Imagen por Resonancia Magnética , Síndrome del Desfiladero Torácico/diagnóstico , Descompresión Quirúrgica , Femenino , Humanos , Persona de Mediana Edad , Síndrome del Desfiladero Torácico/cirugíaRESUMEN
INTRODUCTION: Many studies have demonstrated the efficacy and the medical/economic value of epidural spinal cord stimulation for the treatment of "failed back surgery syndrome" (FBSS). However, the back pain component of FBSS has been recalcitrant. Recent clinical trials have suggested that multicolumn surgically implanted leads combined with enhanced programming capabilities in the newer implantable pulse generators demonstrate the ability to treat the back pain component of FBSS. The objective of our present international multicentre study is to prospectively evaluate these findings in a larger population. METHODS: We conducted a prospective, nonrandomized, observational study on 76 patients with refractory FBSS, consecutively implanted with multicolumn spinal cord stimulation (SCS) between 2008 and 2011 in three neurosurgical pain management centers (Poitiers, France; Montréal, Canada; and Regina, Canada). The primary objective of this study was to prospectively analyze the effect of multicolumn lead programming on paresthesia coverage for the back pain region in these patients. The secondary objective was to assess the analgesic efficacy of this technique on the global and back pain components. RESULTS: Paresthesia could be induced in the lower extremities in the majority of patients with at least one of the configurations tested. Bilateral low back paresthesia was induced in 53.5% of patients, while unilateral low back paresthesia was induced in 78.9% of patients. Multicolumn configurations were statistically more effective than monocolumn configurations for all anatomic regions studied. At 6 months, 75.4% of patients receiving multicolumn stimulation (n = 57) obtained at least a 30% improvement of the back pain VAS score, while 42.1% of patients obtained at least a 50% improvement of the back pain VAS score. CONCLUSION: This study confirms the hypothesis that multicolumn SCS should be considered as an important tool in the treatment of radicular and axial pain in FBSS patients. The efficacy of this modality is based on a rigorous patient selection process, access to new generation lead technologies, but most importantly an algorithmic programming approach for optimal stimulation and electrical field shaping. With over 40 million potential programming combinations associated with 16 contact leads to achieve paresthesia coverage, optimal stimulation is often missed as either the patient or the clinician become exhausted or overwhelmed during the course of therapy programming and optimization session.
Asunto(s)
Electrodos Implantados , Síndrome de Fracaso de la Cirugía Espinal Lumbar/terapia , Estimulación de la Médula Espinal/métodos , Adulto , Anciano , Algoritmos , Analgésicos , Canadá , Femenino , Francia , Humanos , Masculino , Persona de Mediana Edad , Manejo del Dolor , Estudios Prospectivos , Resultado del TratamientoAsunto(s)
Granuloma de Células Plasmáticas/etiología , Neuroglía/trasplante , Enfermedades del Sistema Nervioso Periférico/etiología , Enfermedades de la Médula Espinal/etiología , Trasplante de Células Madre , Trasplante Homólogo , Granuloma de Células Plasmáticas/diagnóstico por imagen , Granuloma de Células Plasmáticas/patología , Granuloma de Células Plasmáticas/cirugía , Humanos , Inyecciones Espinales , Región Lumbosacra , Masculino , Persona de Mediana Edad , Enfermedades del Sistema Nervioso Periférico/diagnóstico por imagen , Enfermedades del Sistema Nervioso Periférico/patología , Enfermedades del Sistema Nervioso Periférico/cirugía , Enfermedades de la Médula Espinal/diagnóstico por imagen , Enfermedades de la Médula Espinal/patología , Enfermedades de la Médula Espinal/cirugía , Raíces Nerviosas Espinales/diagnóstico por imagen , Raíces Nerviosas Espinales/patología , Raíces Nerviosas Espinales/cirugíaRESUMEN
OBJECTIVE: Ulnar neuropathy at the elbow (UNE) is common, affecting 1%-6% of the population. Despite this, there remains a lack of consensus regarding optimal treatment. This is primarily due to the difficulty one encounters when trying to assess the literature. Outcomes are inconsistently reported, which makes comparing studies or developing meta-analyses difficult or even impossible. Thus, there is a need for a core outcome set (COS) for UNE (COS-UNE) to help address this problem. The objective of this study was to utilize a modified Delphi method to develop COS-UNE. METHODS: A 5-stage approach was utilized to develop COS-UNE: stage 1, consortium development; 2, literature review to identify potential outcome measures; 3, Delphi survey to develop consensus on outcomes for inclusion; 4, Delphi survey to develop definitions; and 5, consensus meeting to finalize the COS and definitions. The study followed the Core Outcome Set-STAndards for Development (COS-STAD) recommendations. RESULTS: The Core Outcomes in Nerve Surgery (COINS) Consortium comprised 21 participants, all neurological surgeons representing 11 countries. The final COS-UNE consisted of 22 data points/outcomes covering the domains of demographic characteristics, diagnostics, patient-reported outcomes, motor/sensory outcomes, and complications. Appropriate instruments, methods of testing, and definitions were set. The consensus minimum duration of follow-up was 6 months, with the consensus optimal timepoints for assessment identified as preoperatively and 3, 6, and 12 months postoperatively. CONCLUSIONS: The authors identified consensus data points/outcomes and also provided definitions and specific scales to be utilized to help ensure that clinicians are consistent in their reporting across studies on UNE. This COS should serve as a minimum set of data to be collected in all future neurosurgical studies on UNE. The authors hope that clinicians evaluating ulnar neuropathy will incorporate this COS into routine practice and that future studies will consider this COS in the design phase.
Asunto(s)
Articulación del Codo , Neuropatías Cubitales , Humanos , Codo/cirugía , Neuropatías Cubitales/cirugía , Articulación del Codo/cirugía , Evaluación de Resultado en la Atención de Salud/métodos , Proyectos de Investigación , Resultado del TratamientoRESUMEN
OBJECTIVE: When considering traumatic brachial plexus and upper extremity nerve injuries, iatrogenic nerve injuries, and nontraumatic nerve injuries, brachial plexus and upper extremity nerve injuries are commonly encountered in clinical practice. Despite this, data synthesis and comparison of available studies are difficult. This is at least in part due to the lack of standardization in reporting and a lack of a core outcome set (COS). Thus, there is a need for a COS for adult brachial plexus and upper extremity nerve injuries (COS-BPUE). The objective of this study was to develop a COS-BPUE using a modified Delphi approach. METHODS: A 5-stage approach was used to develop the COS-BPUE: 1) consortium development, 2) literature review to identify potential outcome measures, 3) Delphi survey to develop consensus on outcomes for inclusion, 4) Delphi survey to develop definitions, and 5) consensus meeting to finalize the COS and definitions. The study followed the Core Outcome Set-STAndards for Development (COS-STAD) recommendations. RESULTS: The Core Outcomes in Nerve Surgery (COINS) Consortium comprised 23 participants, all neurological surgeons, representing 13 countries. The final COS-BPUE consisted of 36 data points/outcomes covering demographic, diagnostic, patient-reported outcome, motor/sensory outcome, and complication domains. Appropriate instruments, methods of testing, and definitions were set. The consensus minimum duration of follow-up was 24 months, with the consensus optimal time points for assessment being preoperatively and 3, 6, 12, and 24 months postoperatively. CONCLUSIONS: The COINS Consortium developed a consensus COS and provided definitions, methods of implementation, and time points for assessment. The COS-BPUE should serve as a minimum set of data that should be collected in all future neurosurgical studies on adult brachial plexus and upper extremity nerve injuries. Incorporation of this COS should help improve consistency in reporting, data synthesis, and comparability, and should minimize outcome reporting bias.
Asunto(s)
Plexo Braquial , Técnica Delphi , Procedimientos Neuroquirúrgicos , Traumatismos de los Nervios Periféricos , Extremidad Superior , Humanos , Plexo Braquial/lesiones , Plexo Braquial/cirugía , Extremidad Superior/inervación , Extremidad Superior/cirugía , Extremidad Superior/lesiones , Traumatismos de los Nervios Periféricos/cirugía , Procedimientos Neuroquirúrgicos/métodos , Evaluación de Resultado en la Atención de Salud , Resultado del Tratamiento , Consenso , AdultoRESUMEN
Schwann cell tumors are the most common cancers of the peripheral nervous system and can arise in patients with neurofibromatosis type-1 (NF-1) or neurofibromatosis type-2 (NF-2). Functional interactions between NF1 and NF2 and broader mechanisms underlying malignant transformation of the Schwann lineage are unclear. Here we integrate bulk and single-cell genomics, biochemistry, and pharmacology across human samples, cell lines, and mouse allografts to identify cellular de-differentiation mechanisms driving malignant transformation and treatment resistance. We find DNA methylation groups of Schwann cell tumors can be distinguished by differentiation programs that correlate with response to the MEK inhibitor selumetinib. Functional genomic screening in NF1-mutant tumor cells reveals NF2 loss and PAK activation underlie selumetinib resistance, and we find that concurrent MEK and PAK inhibition is effective in vivo. These data support a de-differentiation paradigm underlying malignant transformation and treatment resistance of Schwann cell tumors and elucidate a functional link between NF1 and NF2.
Asunto(s)
Neurilemoma , Neurofibromatosis , Neurofibromatosis 1 , Neurofibromatosis 2 , Animales , Humanos , Ratones , Quinasas de Proteína Quinasa Activadas por Mitógenos/metabolismo , Neurilemoma/genética , Neurilemoma/patología , Neurofibromatosis/metabolismo , Neurofibromatosis/patología , Neurofibromatosis 1/genética , Neurofibromatosis 1/metabolismo , Neurofibromatosis 2/genética , Neurofibromatosis 2/patología , Células de Schwann/metabolismo , Resistencia a Antineoplásicos/genéticaRESUMEN
OBJECTIVE: Common peroneal (fibular) neuropathy is the most common mononeuropathy of the lower extremity. Despite this, there are surprisingly few studies on the topic, and a knowledge gap remains in the literature. As one attempts to address this knowledge gap, a core outcome set (COS) is needed to guide the planning phases of future studies to allow synthesis and comparability of these studies. The objective of this study was to develop the COS-common peroneal neuropathy (CoPe) using a modified Delphi approach. METHODS: A 5-stage approach was used to develop the COS-CoPe: 1) stage 1, consortium development; 2) stage 2, a literature review to identify potential outcome measures; 3) stage 3, a Delphi survey to develop consensus on outcomes for inclusion; 4) stage 4, a Delphi survey to develop definitions; and 5) stage 5, a consensus meeting to finalize COS and definitions. The study followed the COS-STAndards for Development (COS-STAD) recommendations. RESULTS: The Core Outcomes in Nerve Surgery (COINS) Consortium comprised 23 participants, all neurological surgeons, representing 13 countries. The final COS-CoPe consisted of 31 data points/outcomes covering domains of demographics, diagnostics, patient-reported outcomes, motor/sensory outcomes, and complications. Appropriate instruments, methods of testing, and definitions were set. The consensus minimum duration of follow-up was 12 months. The consensus optimal time points for assessment were preoperatively and 3, 6, 12, and 24 months postoperatively. CONCLUSIONS: The COINS Consortium developed a consensus COS and provided definitions, methods of implementation, and time points for assessment. The COS-CoPe should serve as a minimum set of data that should be collected in all future neurosurgical studies on common peroneal neuropathy. Incorporation of this COS should help improve consistency in reporting, data synthesis, and comparability, and should minimize outcome reporting bias.
RESUMEN
BACKGROUND: Schwannomas are common peripheral nerve sheath tumors that can cause severe morbidity given their stereotypic intracranial and paraspinal locations. Similar to many solid tumors, schwannomas and other nerve sheath tumors are primarily thought to arise due to aberrant hyperactivation of the RAS growth factor signaling pathway. Here, we sought to further define the molecular pathogenesis of schwannomas. METHODS: We performed comprehensive genomic profiling on a cohort of 96 human schwannomas, as well as DNA methylation profiling on a subset. Functional studies including RNA sequencing, chromatin immunoprecipitation-DNA sequencing, electrophoretic mobility shift assay, and luciferase reporter assays were performed in a fetal glial cell model following transduction with wildtype and tumor-derived mutant isoforms of SOX10. RESULTS: We identified that nearly one-third of sporadic schwannomas lack alterations in known nerve sheath tumor genes and instead harbor novel recurrent in-frame insertion/deletion mutations in SOX10, which encodes a transcription factor responsible for controlling Schwann cell differentiation and myelination. SOX10 indel mutations were highly enriched in schwannomas arising from nonvestibular cranial nerves (eg facial, trigeminal, vagus) and were absent from vestibular nerve schwannomas driven by NF2 mutation. Functional studies revealed these SOX10 indel mutations have retained DNA binding capacity but impaired transactivation of glial differentiation and myelination gene programs. CONCLUSIONS: We thus speculate that SOX10 indel mutations drive a unique subtype of schwannomas by impeding proper differentiation of immature Schwann cells.
Asunto(s)
Neoplasias de la Vaina del Nervio , Neurilemoma , Neuroma Acústico , Humanos , Mutación INDEL , Activación Transcripcional , Neurilemoma/genética , Neurilemoma/patología , Neuroma Acústico/patología , Mutación , Factores de Transcripción SOXE/genética , Factores de Transcripción SOXE/metabolismoRESUMEN
BACKGROUND: Identifying peripheral nerve surgery procedure (PNSP) competencies is crucial to ensure adequate resident training. We examine PNSP training at neurosurgical centers in the US and Canada to compare resident-reported competence, PNSP exposure, and resident technical abilities in performing 3 peripheral nerve coaptations (PNC). METHODS: Resident-reported PNSP competence and PNSP exposure data were collected using questionnaires from neurosurgical residents at North American neurosurgical training centers. Exposure and self-reported competency were correlated with technical skills. Technical PNC variables collected included: time-to-completion, nerve-handling from video-analysis, independent and blinded visual-analog-scale (VAS) PNC quality grading by 3 judges, and training level. RESULTS: A total of 40 neurosurgical residents participated in the study. Although self-reported competency scores correlated with procedural exposure (P < 0.01, rs = 0.88), a discrepancy was found between the degree of self-reported competency and amount of exposure. The discrepancy was greater in senior residents. A significant VAS difference was found between PNC types with the direct-suture and connector-assister groups scoring higher than connector-only (P = 0.02, P < 0.01, respectively). No difference was observed between training level and VAS grading, nor time-to completion (P = 0.33 and 0.25, respectively). No correlation was found between self-reported competency performing PNSPs and PNC VAS scores, nor nerve handling. CONCLUSIONS: Despite more exposure and a higher self-reported PNSP competency in senior residents, no difference was seen between senior/junior residents in PNC quality. A discrepancy in PNSP exposure and self-reported competency exists. This information will provide guidance for the direction of resident PNS training.
Asunto(s)
Internado y Residencia , Competencia Clínica , Humanos , Procedimientos Neuroquirúrgicos , Nervios Periféricos/cirugía , AutoinformeRESUMEN
Appendiceal neurofibromas are exceedingly rare, with neither experimental nor observational data to support evidence-based diagnosis or treatment. We describe the case of a 52-year-old woman with neurofibromatosis 1 (NF1) complicated by aqueductal stenosis and resultant hydrocephalus needing a ventriculoperitoneal shunt (VPS). She presented to the emergency department with abdominal pain and was found to have abnormalities in the right hemiabdomen on cross-section imaging, also a Staphylococcus epidermidis growth at the distal portion of the VPS. She was initially treated with two rounds of intravenous antibiotics and VPS removal without improvement. She ultimately underwent an appendectomy, which revealed pathologic evidence of NF. The appendectomy was key to ruling out malignancy, addressing further symptoms and preventing future malignant transformation. This case highlights the importance of including appendiceal neurofibromas in the differential diagnoses of abdominal pain in patients with NF1.
RESUMEN
BACKGROUND: Although distal dorsal scapular nerve (DSN) anatomy has been well characterized, a paucity of literature exists detailing its proximal origin. To our knowledge, this is the first study examining DSN origin and its anatomy relative to the C5 nerve root, which may help localize pathology and provide insight into timing of DSN or C5 nerve root clinical and electrophysiological recovery. METHODS: Eighteen cadaveric dissections were performed using a posterior-midline approach. Calipers were used for DSN branching and course characterization with statistical analysis completed for the following measurements: DSN diameter, C5 nerve root diameter, distance of DSN branch-point from the C5 ganglion, dural edge, and posterior foraminal tubercle (intra-vs. extraforaminal origin), as well as C5 root-SC branch-point distance. RESULTS: Average/mean measurements (standard error) were as follows: DSN diameter: 3.7 mm (0.3 mm), C5 nerve root diameter: 6.2 mm (0.5 mm), DSN origin to C5 DRG: 12.4 mm (1.9 mm) distal, DSN origin to dural edge: 19. 6mm (1.8 mm), DSN origin to C5 root origin: 23.3 mm (2.2 mm), DSN origin to the posterior foraminal tubercle: 2.3 mm (2.5 mm) proximal/intraforaminal (first branch from C5 in all cases, and the majority [12 of 18, 67%] of DSNs originating from the C5 spinal nerve root within the foramen). CONCLUSIONS: The C5 nerve root contributed to the DSN in all specimens that originated from the proximal, intraforaminal, C5 nerve root in the majority of specimens. As the first C5 nerve branch, surgeon knowledge of this proximal DSN pattern will help localize lesional pathology, as well as may help monitor clinical and electrophysiological recovery.
Asunto(s)
Plexo Braquial/anatomía & histología , Escápula/inervación , Raíces Nerviosas Espinales/anatomía & histología , Cadáver , Vértebras Cervicales , HumanosRESUMEN
We enjoyed reading Povleson et al.'s review entitled "Diagnostic thoracic outlet syndrome: current approaches and future directions" [...].
RESUMEN
Spinal cord herniation (SCH) is a rare cause of myelopathy. When reported, SCH has most commonly been described as occurring spontaneously in the thoracic spine, and being idiopathic in nature (anterior thoracic spinal cord herniation, ATSCH) [1-3]. Several theories have been proposed to explain its occurrence, including congenital, inflammatory, and traumatic etiologies alike [1-4]. Even more rarely, SCH has been described to occur in the cervical spine in association with brachial plexus avulsion injuries (BPAI-SCH). In our accompanying article, "Late Cervical Spinal Cord Herniation Resulting from Post-Traumatic Brachial Plexus Avulsion Injury," two cases of BPAI-SCH are presented and discussed in the context of the reviewed literature [5]. Here, pertinent accompanying follow-up data was collected and is presented for the cases, including postoperative radiographic outcome imaging. Furthermore, a table is presented comparing and contrasting ATSCH to BPAI-SCH. Although the two phenomena have been previously grouped together, this table highlights ATSCH and BPAI-SCH as distinct entities; more specifically, BPAI-SCH is a separate, long-term complicating feature of BPAI. This supplementary data helps treating physicians by increasing awareness and knowledge of BPAI-SCH as a distinct entity from ATSCH and cause of delayed neurological deterioration.
RESUMEN
BACKGROUND: Spinal cord herniation (SCH) is often described as occurring spontaneously in the thoracic spine, with few cases of cervical SCH reported as a late complication of traumatic brachial plexus avulsion. We present 2 cases of nerve root avulsion and pseudomeningocele formation, resulting in delayed cervical SCH and neurologic deterioration. CASE DESCRIPTION: Case 1: A 37-year old man presented with progressive leg weakness 2 years after experiencing traumatic C8 and T1 root avulsions. Magnetic resonance imaging (MRI) showed previously documented C8-T1 nerve avulsions with new SCH in a T1 pseudomeningocele. A C7-T1 costotransversectomy and C4-T4 instrumented fusion were completed, allowing SCH reduction and patch graft repair of the dural defects without the need for adhesiolysis. At last follow-up, the patient's leg weakness had resolved. Case 2: A 32-year old man presented with progressive right arm numbness, weakness, and signs of myelopathy 9 years after experiencing C8 and T1 root avulsions. MRI showed previously documented root avulsions and new SCH with extensive and compressive pseudomeningocele formation. A C7 transpedicular approach with C5-T1 instrumented fusion was completed for dural repair. A large pseudomeningocele was found and drained on drilling the C7 pedicle, and adhesiolysis was required at the spinal cord avulsion site to reduce the SCH and allow patch graft repair. At last follow-up, the patient's right arm weakness was improving, although numbness persisted. CONCLUSIONS: SCH is a rare cause of delayed neurologic deterioration after brachial plexus avulsion, with few case reports describing its occurrence. We present 2 cases of this complication and describe its successful surgical treatment through dural repair after instrumented fusion.
Asunto(s)
Plexo Braquial/lesiones , Médula Cervical , Vértebras Cervicales/cirugía , Desplazamiento del Disco Intervertebral/etiología , Enfermedades de la Médula Espinal/etiología , Adulto , Herniorrafia/métodos , Humanos , Desplazamiento del Disco Intervertebral/cirugía , Masculino , Enfermedades de la Médula Espinal/cirugía , Fusión Vertebral/métodos , Resultado del TratamientoRESUMEN
BACKGROUND: Malignant peripheral nerve sheath tumor (MPNST) is an aggressive and poorly understood malignant neoplasm. Even in the setting of multimodal therapy, the clinical course of MPNST is frequently marked by metastatic conversion and poor overall prognosis, with optimal treatment paradigms for this rare tumor unknown. METHODS: We reviewed the medical records and histopathology of 54 consecutive patients who were treated at University of California San Francisco between 1990 and 2018. RESULTS: Our cohort consisted of 24 male and 30 female patients (median age 38 years). Fédération Nationale des Centres de Lutte Contre Le Cancer (FNCLCC) sarcoma grading criteria segregated patients into groups with differences in overall survival (OS) (P = .02). Increasing Ki-67 labeling index was associated with poor OS (hazard ratio [HR] 1.36 per 10%, P = .0002). Unsupervised hierarchical clustering-based immunohistochemical staining patterns identified 2 subgroups of tumors with differences in H3K27me3, Neurofibromin, S100, SOX10, p16, and EGFR immunoreactivity. In our cohort, cluster status was associated with improved locoregional failure-free rate (P = .004) in response to radiation. CONCLUSIONS: Our results lend support to the FNCLCC sarcoma grading criteria as a prognostic scheme for MPNST, although few cases of grade 1 were included. Further, we identify increased Ki-67 labeling as a strong predictor of poor OS from MPNST. Finally, we identify a subset of MPNSTs with a predictive immunohistochemical profile that has improved local control with adjuvant radiotherapy. These data provide insights into the grading and therapy for patients with MPNST, although further studies are needed for independent validation.