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OBJECTIVE: To explore the clinical utility of ultrasound in evaluating and grading neuromuscular diseases in the lower extremities of patients with diabetes mellitus. METHODS: A total of 126 inpatients from the Department of Diabetes at Zhangzhou Affiliated Hospital of Fujian Medical University, China, were recruited from June 2020 to December 2022. The cohort included 69 patients with type 2 diabetes mellitus (T2DM) and diabetic peripheral neuropathy (DPN group) and 57 patients with T2DM but without DPN (non-DPN group). Additionally, 80 healthy controls were included. High-frequency ultrasound was used to scan the common peroneal, sural, and tibial nerves, measuring their transverse (D1) and anteroposterior (D2) diameters, and calculating the cross-sectional area (CSA). Changes in the internal echo of the extensor digitorum brevis (EDB) muscle, including maximum thickness and CSA, were also recorded. The DPN group was further subdivided based on disease duration to assess ultrasonic changes over time and the statistical significance of these variations. RESULTS: Ultrasonic changes such as uneven internal echo reduction, ill-defined epineurial boundaries, and obscured cribriform structures were most prevalent in the DPN group. Significant differences in ultrasound parameters (D1, D2, CSA) were observed among the groups (all P<0.05), with the most pronounced changes in the DPN group. In patients with a disease duration of over 15 years, a significant increase in CSA of lower extremity nerves and a decrease in CSA of the EDB were noted compared to those in the 5-10 years subgroup (19.89±0.98 vs 19.00±0.94; 5.25±0.74 vs 5.93±0.94; all P<0.05). CONCLUSIONS: High-frequency ultrasound provides a valuable imaging basis for diagnosing and monitoring DPN, demonstrating significant changes in nerve and muscle parameters among diabetic patients.
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Hypertension is usually accompanied by elevated sympathetic tonicity, but how sympathetic hyperactivity is triggered is not clear. Recent advances revealed that microglia-centered neuroinflammation contributes to sympathetic excitation in hypertension. In this study, we performed a temporospatial analysis of microglia at both morphological and transcriptomic levels and found that microglia in the hypothalamic paraventricular nucleus (PVN), a sympathetic center, were early responders to hypertensive challenges. Vasculature analyses revealed that the PVN was characterized by high capillary density, thin vessel diameter, and complex vascular topology relative to other brain regions. As such, the PVN was susceptible to the penetration of ATP released from the vasculature in response to hemodynamic disturbance after blood pressure increase. Mechanistically, ATP ligation to microglial P2Y12 receptor was responsible for microglial inflammatory activation and the eventual sympathetic overflow. Together, these findings identified a distinct vasculature pattern rendering vulnerability of PVN pre-sympathetic neurons to hypertension-associated microglia-mediated inflammatory insults.
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We developed a rat dorsal root ganglion (DRG)-derived sensory nerve organotypic model by culturing DRG explants on an organoid culture device. With this method, a large number of organotypic cultures can be produced simultaneously with high reproducibility simply by seeding DRG explants derived from rat embryos. Unlike previous DRG explant models, this organotypic model consists of a ganglion and an axon bundle with myelinated A fibers, unmyelinated C fibers, and stereo-myelin-forming nodes of Ranvier. The model also exhibits Ca2+ signaling in cell bodies in response to application of chemical stimuli to nerve terminals. Further, axonal transection increases the activating transcription factor 3 mRNA level in ganglia. Axons and myelin are shown to regenerate 14 days following transection. Our sensory organotypic model enables analysis of neuronal excitability in response to pain stimuli and tracking of morphological changes in the axon bundle over weeks.
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BACKGROUND: Elderly patients with vestibular schwannoma (VS) are commonly observed. OBJECT: Retrospective analysis of 25 patients aging ≥ 70 operated on in our neurosurgical department for unilateral VS. The purpose of our study is to propose an algorithm for the treatment of VS in elderly patients. METHODS: American Society of Anesthesiology (ASA) Grade I-II patients and Grade III with life-threatening tumors were enrolled. Karnofsky Performance Status Scale (KPS) was used for evalutation of the quality of life. The House-Brackmann (HB) scale for facial nerve (FN) outcome was used. Tumor size was categorized according to Koos' classification. A retrosigmoid approach was used in all cases, except one in which a translabyrinthine approach was performed. Surgical removal graduation: total (GTR), near total (NTR > 95%), subtotal (STR > 90%). The clinical and radiological follow-up period was set first at six months and then at one year after surgery. FN results evaluation was performed at one year, categorized according to House-Brackmann grades I-VI. RESULTS: Mean age: 74,4 years (70-83); 28% ASA I, 56% ASA II, 16% ASA III. Mean tumor size: 2,7 cm (1,5-4,2 cm). GTR/NTR: 68%, STR 32%. Mortality was zero. At last follow-up (one year after surgery) FN results were: HBI 81%, HBII 9.5%, HBIII 9.5%; HB IV 0%. Only 4 patients had preoperative HB IV, of whom one improved from HB IV to HB III. Transient complications occurred only in large VS. Re-growth of residue after STR was observed in 3 cases, treated with SRS in 2 cases and observed in 1. CONCLUSIONS: An algorithm of treatment of vestibular schwannoma in the elderly is proposed. In particular, in patients in general good conditions, age does not appear to be a major contraindication for microsurgery of VS. FN results at last follow-up are satisfactory and the complication rates are acceptable.
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Algoritmos , Microcirugia , Neuroma Acústico , Procedimientos Neuroquirúrgicos , Humanos , Neuroma Acústico/cirugía , Neuroma Acústico/patología , Anciano , Masculino , Femenino , Anciano de 80 o más Años , Estudios Retrospectivos , Microcirugia/métodos , Procedimientos Neuroquirúrgicos/métodos , Resultado del Tratamiento , Calidad de VidaRESUMEN
Increased sympathetic and reduced parasympathetic nerve activity is associated with disease progression and poor outcomes in patients with chronic heart failure. The demonstration that markers of autonomic imbalance and vagal dysfunction, such as reduced heart rate variability and baroreflex sensitivity, hold prognostic value in patients with chronic heart failure despite modern therapies encourages the research for neuromodulation strategies targeting the vagus nerve. However, the approaches tested so far have yielded inconclusive results. This review aims to summarize the current knowledge about the role of the parasympathetic nervous system in chronic heart failure, describing the pathophysiological background, the methods of assessment, and the rationale, limits, and future perspectives of parasympathetic stimulation either by drugs or bioelectronic devices.
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The measurement of skeletal muscle fatigue in response to cycling exercise is commonly done in isometric conditions, potentially limiting its ecological validity, and creating challenges in monitoring the time course of muscle fatigue across an exercise bout. This study aimed to determine if muscle fatigue could be reliably assessed by measuring quadriceps twitch force evoked while pedaling, using instrumented pedals. Nine participants completed three laboratory visits: a step incremental test to determine power output at lactate threshold, and on separate occasions, two constant-intensity bouts at a power output 10% above lactate threshold. Femoral nerve electrical stimulation was applied to elicit quadriceps twitch force both while pedaling (dynamic) and at rest (isometric). The test-retest reliability of the dynamic twitch forces and the agreement between the dynamic and isometric twitch forces were evaluated. Dynamic twitch force was found to have excellent reliability in an unfatigued state (intraclass correlation coefficient (ICC) = 0.920 and mean coefficient of variation (CV) = 7.5%), and maintained good reliability at task failure (ICC = 0.846 and mean CV = 11.5%). When comparing dynamic to isometric twitch forces across the task, there was a greater relative decline in the dynamic condition (P = 0.001). However, when data were normalized to the 5 min timepoint when potentiation between conditions was presumed to be more similar, this difference disappeared (P = 0.207). The reliability of this method was shown to be commensurate with the gold standard method utilizing seated isometric dynamometers and offers a new avenue to monitor the kinetics of muscle fatigue during cycling in real time.
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The lingual nerve (LN) is a branch of the mandibular division of the fifth cranial nerve, the trigeminal. It primarily carries sensory fibers from the lingual gingiva, mucous membranes of the floor of the mouth, sublingual gland, and the anterior two-thirds of the tongue. Recent studies have explored and reclassified the five branches of the LN as branches to the isthmus of the fauces, lingual branches, sublingual nerves, posterior branch to the submandibular ganglion, and branches to the sublingual ganglion. The knowledge of the LN anatomy and its variants is clinically relevant to avoid its injury during oral procedures. The objective of this paper is to review the literature on the LN and to describe the anatomy, its course, and its functions.
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Developing long bones alter their shape while maintaining uniform cortical thickness via coordinated activity of bone-forming osteoblasts and bone-resorbing osteoclasts at periosteal and endosteal surfaces, a process we designate trans-pairing. Two types of trans-pairing shift cortical bone in opposite orientations: peri-forming trans-pairing (peri-t-p) increases bone marrow space and endo-forming trans-pairing (endo-t-p) decreases it, via paired activity of bone resorption and formation across the cortex. Here, we focused on endo-t-p in growing bones. Analysis of endo-t-p activity in the cortex of mouse fibulae revealed osteoclasts under the periosteum compressed by muscles and expression of RANKL in periosteal cells of the cambium layer. Furthermore, mature osteoblasts were localized on the endosteum, while preosteoblasts were at the periosteum and within cortical canals. X-ray tomographic microscopy revealed the presence of cortical canals more closely associated with endo- than with peri-t-p. Sciatic nerve transection followed by muscle atrophy and unloading induced circumferential endo-t-p with concomitant spread of cortical canals. Such canals likely supply the endosteum with preosteoblasts from the periosteum under endo-t-p, allowing bone shape to change in response to mechanical stress or nerve injury.
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A bronchogenic cyst (BC) is a rare congenital cystic lesion, especially when located in the suprahyoid region of the anterior cervical front. We report a case of a male child who was misdiagnosed with a thyroglossal duct cyst due to the clinical presentation of a mass located near the anterior midline of the neck, in the region above the hyoid bone, which moved up and down with swallowing, characteristics consistent with a thyroglossal duct cyst. We excised the lesion under general anesthesia. Histopathological analysis revealed cartilage tissue, pseudostratified columnar epithelium of the cyst wall with cilia and mucous glands, finally diagnosing it as BC. BC should be considered in the differential diagnosis of neck masses.
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BACKGROUND: Fifteen-and-a-Half Syndrome is an uncommon clinical presentation characterized by the coexistence of one-and-a-half syndrome and bilateral facial palsy. In this study, we provide a comprehensive description of symptom evolution and imaging changes in a patient with Fifteen-and-a-Half Syndrome. CASE PRESENTATION: A 54-year-old male presented with sudden onset of one-and-a-half syndrome, which gradually progressed to fifteen-and-a-half syndrome. The final diagnosis was confirmed to be pontine infarction which occurred at the midline of the pontine tegmentum. CONCLUSION: This case highlights the diverse and progressive early clinical manifestations associated with Fifteen-and-a-half Syndrome. Currently, all reported cases of this syndrome are linked to brainstem infarction; however, early differential diagnosis is crucial to ensure prompt initiation of appropriate treatment for affected patients.
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Infartos del Tronco Encefálico , Parálisis Facial , Humanos , Masculino , Persona de Mediana Edad , Parálisis Facial/diagnóstico , Parálisis Facial/diagnóstico por imagen , Parálisis Facial/etiología , Infartos del Tronco Encefálico/diagnóstico por imagen , Infartos del Tronco Encefálico/complicaciones , Infartos del Tronco Encefálico/diagnóstico , Síndrome , Imagen por Resonancia Magnética/métodos , Tegmento Pontino/diagnóstico por imagenRESUMEN
Jansen metaphyseal chondrodysplasia (JMC) is an ultra-rare disorder caused by germline heterozygous PTHR1 variants resulting in constitutive activation of parathyroid hormone type 1 receptor. A description of ocular manifestations of the disease is lacking. Six patients with JMC underwent a detailed ophthalmic evaluation, spectral-domain optical coherence tomography (OCT), visual field testing, and craniofacial CT scans. Five of 6 patients had good visual acuity. All patients had widely spaced eyes; 5/6 had downslanted palpebral fissures. One patient had proptosis, and another had bilateral ptosis. Two patients had incomplete closure of the eyelids (lagophthalmos), one had a history of progressive right facial nerve palsy with profuse epiphora, while the second had advanced optic nerve atrophy with corresponding retinal nerve fiber layer (RNFL) thinning on OCT and significant bilateral optic canal narrowing on CT scan. Additionally, this patient also had central visual field defects and abnormal color vision. A third patient had normal visual acuity, subtle temporal pallor of the optic nerve head, normal average RNFL, but decreased temporal RNFL and retinal ganglion cell layer analysis (GCA) on OCT. GCA was decreased in 4/6 patients indicating a subclinical optic nerve atrophic process. None of the patients had glaucoma or high myopia. These data represent the first comprehensive report of ophthalmic findings in JMC. Patients with JMC have significant eye findings associated with optic canal narrowing due to extensive skull base dysplastic bone overgrowth that appear to be more prevalent and pronounced with age. Progressive optic neuropathy from optic canal narrowing may be a feature of JMC, and OCT GCA can serve as a useful biomarker for progression in the setting of optic canal narrowing. We suggest that patients with JMC should undergo regular ophthalmic examination including color vision, OCT, visual field testing, orbital, and craniofacial imaging.
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Background: Several anatomical variations of the ilioinguinal nerve branches have been recorded in older studies. Knowledge of these variations is useful for the improvement of peripheral nerve blocks and avoidance of iatrogenic nerve injuries during abdominal surgeries. The purpose of this study is to perform a systematic review of the literature about the anatomical topography and variations of the ilioinguinal nerve. Methods: An extensive search in PubMed, Scopus, and Web of Science electronic databases was conducted by the first author in November 2021, with the use of the PRISMA guidelines. Anatomical or cadaveric studies about the origin, the course, and the distribution of the ilioinguinal nerve were included in this review. Thirty-one cadaveric studies were included for qualitative analysis. Results: Several anatomical variations of the ilioinguinal nerve were depicted including its general properties, its origin, its branching patterns, its course, its relation to anatomical landmarks, and its termination. Among them, the absence of ilioinguinal nerve ranged from 0% to 35%, its origin from L1 ranged from 65% to 100%, and its isolated emergence from psoas major ranged from 47% to 94.5%. Numerous anatomical variations of the ilioinguinal nerve exist, not commonly cited in classic anatomical textbooks. The branches of the ilioinguinal nerve may be damaged during spinal anesthesia and surgical procedures in the lower abdominal region. Conclusion: Therefore, a better understanding of the regional anatomy and its variations is of vital importance for the prevention of ilioinguinal nerve injuries.
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Background: Malignant melanotic nerve sheath tumors (MMNSTs) are relatively rare, comprising <1% of all neoplastic peripheral nerve lesions. Here, we describe a 79-year-old male who presented with atypical magnetic resonance imaging (MRI) findings of an MMNST. Case Description: A 79-year-old male presented with lower back pain, paraparesis, and bladder/bowel dysfunction. The MRI showed an intradural extramedullary (IE) lesion at the T9-T10 level with low-signal intensity on T1-weighted images (WI) and high intensity on T2-WI, which markedly enhanced with contrast. The IE nerve root involved with the tumor was completely removed surgically. The lesion was confirmed to be an MMNST. In the absence of metastases, adjuvant therapy was deemed unnecessary. One year later, the lesion has not recurred. Conclusion: A 79-year-old male patient presented with a T9-T10 MR intradural lesion that was pathologically proved to be an MMNST, which was treated with gross total surgical resection (i.e., removal of the involved nerve root alone).
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Background: Patients with congenital absence of a lumbar pedicle and nerve root anomaly presenting with ipsilateral foraminal stenosis are extremely rare. Case Presentation: An 80-year-old man had low back and right thigh pain. Radiographs and computed tomography (CT) showed L3 vertebral body fracture and the absence of the right L3 lumbar pedicle. He was diagnosed with L2-L3 right foraminal stenosis caused by an L3 vertebral fracture and underwent lumbar fusion at L2-L3 and L3-L4. Intraoperatively, we confirmed that an anomalous nerve root was divided from the right L2 nerve root near the dorsal root ganglion (DRG). Conclusions: Patients with congenital absence of a lumbar pedicle are less prone to ipsilateral foraminal stenosis because they theoretically have a large space in the foramen. This rare case was caused because of additional instability due to vertebral fracture under the condition of a nerve root anomaly and lumbar degeneration.
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Purpose: This study aimed to compare the risk of vagal reflex during microsurgical subinguinal varicocelectomy (MSV) under general anesthesia (GA) with or without additional local anesthetic (LA) spermatic cord block (SCB). Method: A single-center randomized controlled trial was conducted between January 2022 and June 2023.300 patients with left-sided grade â ¢ varicocele were randomly divided into two groups: SCB group (n = 153) and control group (n = 147)ï¼computer-generated random numbers listï¼. During MSV under GA, the SCB group was given of ropivacaine for SCB before pulling the spermatic cord, while the control group was directly lifted. The primary outcome was the reduction in the lowest heart rate in the SCB group as compared with the control group during spermatic cord traction (SCT). Secondary outcomes included the reduction in the lowest blood pressure in the SCB group as compared with the control group; and the reductions in the lowest heart rate and lowest blood pressure as compared with baseline during SCT. The number of times that surgery and medications were suspended because of symptomatic reflex bradycardia was also recorded. Adverse events were also recorded as secondary outcomes. Result: Five patients in the SCB group and 10 patients in the CG were excluded. The lowest heart rate and systolic blood pressure during SCT in the SCB group and the control group were significantly lower than the baseline values (P < 0.05). However, the decrease in the SCB group (70-73bpm VS 108-115 mmHg) was milder than that of the control groupï¼66-72 bpm VS 105-114 mmHgï¼(P < 0.05). The number of surgeries and medication pauses due to symptomatic reflex bradycardia during surgery was significantly lower in the SCB group (2 VS 1) than in the control group (9 VS 7) (P < 0.05). Conclusion: SCB can effectively reduce the vagal reflex caused by pulling the spermatic cord during MSV, and reduce the risk of anesthesia and surgery.
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Linear morphea is the most disabling subtype of morphea, which may cause a series of excutaneous manifestations and sequelae. To futher explore the clinical characteristics of linear morphea, we conducted a retrospective study of 22 patients diagnosed with linear morphea in our department during the past 2 years. Their baseline clinical information, skin manifestations, complications and therapeutic effect were analyzed. Here, we report six cases of a special linear morphea, usually occurring on the unilateral upper limbs of young women, spreading along the distribution of the radial nerve and frequently progressing across the joint, which increases the incidence of neuromusculoskeletal disorders. Instead of traditional topical drugs, a combination of systemic prednisone and methotrexate improved their skin lesions and complications. Recognition of this special type of linear morphea enables earlier diagnosis and active treatment plan, which contributes to ameliorate the symptoms and avoid functional sequelae.
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Dental implantology has been considered the mainstay in the rehabilitation of partial or complete edentulism. Nevertheless, complications and failures are occasionally encountered, and the most significant is the neurosensory disturbance. It not only causes persistent discomfort to the patient but frequently degrades the patient's oral health-related quality of life, even leading to a negative psychological impact. This paper presents a case report of a 65-year-old male patient who underwent the replacement of his missing tooth in the right mandibular region (46) with an implant-supported prosthesis two years ago. Since then, he has been experiencing numbness in the right side of the lip and occasional drooling of saliva from the right corner of the mouth. Clinical examination revealed the presence of a prosthetic crown supported by an implant in relation to 46 with inflamed and enlarged gingiva in the region with paresthesia of the right lower lip region. Radiological examination with orthopantomogram (OPG) and cone beam computed tomography (CBCT) revealed that the implant apex rested on the inferior alveolar nerve canal. Careful surgical retrieval of the well-osseointegrated implant was performed under local anesthesia in about seven days. The patient gradually experienced neurosensory improvement, and the paraesthesia was completely resolved in a six-week period. After complete recovery, as evaluated with an objective and subjective assessment, the edentulous site was successfully restored with a provisional fixed partial denture.