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1.
PLoS One ; 19(7): e0305808, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-39024363

RESUMO

INTRODUCTION/AIMS: Leprosy is the most common treatable peripheral neuropathy worldwide. The detection of peripheral nerve impairment is essential for its diagnosis and treatment, in order to prevent stigmatizing deformities and disabilities. This study was performed to identify neural thickening through multisegmental ultrasound (US). METHODS: We assessed US measurements of cross-sectional areas (CSAs) of ulnar, median and tibial nerves at two points (in the osteofibrous tunnel and proximal to the tunnel), and also of the common fibular nerve at the fibular head level in 53 leprosy patients (LP), and compared with those of 53 healthy volunteers (HV), as well as among different clinical forms of leprosy. RESULTS: US evaluation detected neural thickening in 71.1% (38/53) of LP and a mean number of 3.6 enlarged nerves per patient. The ulnar and tibial were the most frequently affected nerves. All nerves showed significantly higher measurements in LP compared with HV, and also greater asymmetry, with significantly higher values for ulnar and tibial nerves. We found significant CSAs differences between tunnel and pre-tunnel points for ulnar and tibial nerves, with maximum values proximal to the tunnel. All clinical forms of leprosy evaluated showed neural enlargement through US. DISCUSSION: Our findings support the role of multisegmental US as a useful method for diagnosing leprosy neuropathy, revealing that asymmetry, regional and non-uniform thickening are characteristics of the disease. Furthermore, we observed that neural involvement is common in different clinical forms of leprosy, reinforcing the importance of including US evaluation of peripheral nerves in the investigation of all leprosy patients.


Assuntos
Hanseníase , Doenças do Sistema Nervoso Periférico , Ultrassonografia , Humanos , Hanseníase/diagnóstico por imagem , Hanseníase/diagnóstico , Masculino , Feminino , Ultrassonografia/métodos , Adulto , Pessoa de Meia-Idade , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/diagnóstico , Idoso , Nervo Tibial/diagnóstico por imagem , Adulto Jovem , Nervo Ulnar/diagnóstico por imagem , Nervo Ulnar/patologia , Estudos de Casos e Controles , Nervo Mediano/diagnóstico por imagem
2.
J Peripher Nerv Syst ; 29(2): 173-184, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38887985

RESUMO

Corneal confocal microscopy (CCM) is an ophthalmic imaging technique that enables the identification of corneal nerve fibre degeneration and regeneration. To undertake a systematic review and meta-analysis of studies utilizing CCM to assess for corneal nerve regeneration after pharmacological and surgical interventions in patients with peripheral neuropathy. Databases (EMBASE [Ovid], PubMed, CENTRAL and Web of Science) were searched to summarize the evidence from randomized and non-randomized studies using CCM to detect corneal nerve regeneration after pharmacological and surgical interventions. Data synthesis was undertaken using RevMan web. Eighteen studies including 958 patients were included. CCM identified an early (1-8 months) and longer term (1-5 years) increase in corneal nerve measures in patients with peripheral neuropathy after pharmacological and surgical interventions. This meta-analysis confirms the utility of CCM to identify nerve regeneration following pharmacological and surgical interventions. It could be utilized to show a benefit in clinical trials of disease modifying therapies for peripheral neuropathy.


Assuntos
Córnea , Microscopia Confocal , Regeneração Nervosa , Humanos , Córnea/inervação , Córnea/cirurgia , Córnea/diagnóstico por imagem , Regeneração Nervosa/efeitos dos fármacos , Regeneração Nervosa/fisiologia , Doenças do Sistema Nervoso Periférico/tratamento farmacológico , Doenças do Sistema Nervoso Periférico/fisiopatologia , Doenças do Sistema Nervoso Periférico/cirurgia , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem
4.
Handb Clin Neurol ; 201: 19-42, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38697740

RESUMO

Electrodiagnostic testing (EDX) has been the diagnostic tool of choice in peripheral nerve disease for many years, but in recent years, peripheral nerve imaging has been used ever more frequently in daily clinical practice. Nerve ultrasound and magnetic resonance (MR) neurography are able to visualize nerve structures reliably. These techniques can aid in localizing nerve pathology and can reveal significant anatomical abnormalities underlying nerve pathology that may have been otherwise undetected by EDX. As such, nerve ultrasound and MR neurography can significantly improve diagnostic accuracy and can have a significant effect on treatment strategy. In this chapter, the basic principles and recent developments of these techniques will be discussed, as well as their potential application in several types of peripheral nerve disease, such as carpal tunnel syndrome (CTS), ulnar neuropathy at the elbow (UNE), radial neuropathy, brachial and lumbosacral plexopathy, neuralgic amyotrophy (NA), fibular, tibial, sciatic, femoral neuropathy, meralgia paresthetica, peripheral nerve trauma, tumors, and inflammatory neuropathies.


Assuntos
Doenças do Sistema Nervoso Periférico , Humanos , Eletrodiagnóstico/métodos , Imageamento por Ressonância Magnética , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Ultrassonografia
5.
J Neuroimaging ; 34(4): 486-492, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38764165

RESUMO

BACKGROUND AND PURPOSE: Uremic neuropathy (UN) is a disabling neuropathy in end-stage kidney disease (ESKD) affecting the majority of patients receiving long-term hemodialysis (HD). One previous nerve ultrasound study reported an increased cross-sectional area (CSA) of the median nerve in moderate UN, while another study found enlarged sural nerves in small-fiber polyneuropathy associated with ESKD. The present cohort study aims to analyze bilateral CSA of multiple nerves in UN. METHODS: Ten nondiabetic ESKD patients with UN on HD for at least 2 years and 10 healthy age-matched controls underwent bilateral ultrasound examinations with CSA measurements in 13 arm and leg nerve sites. Nerve conduction studies (NCS) and the total neuropathy score (TNS) were recorded. Pearson's coefficient and the Mann-Whitney U-test were used to analyze correlations and compare groups. RESULTS: ESKD patients presented advanced neuropathic symptoms (mean TNS 15.9). NCS showed significantly reduced motor and sensory amplitudes in the UN group compared to the control group, and a slightly reduced nerve CSA was observed in 5 of 13 nerve sites (p < .05); the other nerve sites were not enlarged. Sural nerve CSA (p < .05) and sensory amplitude (p < .01) were negatively correlated with the TNS. CONCLUSIONS: Nerve enlargement was not observed in the present study in advanced UN. A reduced nerve CSA observed in the sural nerve suggests an axonal loss associated with long-term HD in ESKD. During clinical workup of an acute disease of the peripheral nervous system in ESKD patients, nerve enlargement might be attributable to other causes than chronic UN.


Assuntos
Falência Renal Crônica , Ultrassonografia , Uremia , Humanos , Masculino , Feminino , Projetos Piloto , Pessoa de Meia-Idade , Uremia/complicações , Uremia/diagnóstico por imagem , Falência Renal Crônica/complicações , Falência Renal Crônica/diagnóstico por imagem , Falência Renal Crônica/terapia , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Idoso , Reprodutibilidade dos Testes , Sensibilidade e Especificidade , Nervo Sural/diagnóstico por imagem , Condução Nervosa
7.
Semin Musculoskelet Radiol ; 28(1): 92-102, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38330973

RESUMO

Peripheral neuropathy is a prevalent and debilitating condition affecting millions of individuals globally. Magnetic resonance neurography (MRN) and ultrasonography (US) are noninvasive methods offering comprehensive visualization of peripheral nerves, using anatomical and functional imaging biomarkers to ensure accurate evaluation. For optimized MRN, superior and high-resolution two-dimensional and three-dimensional imaging protocols are essential. The anatomical MRN and US imaging markers include quantitative measures of nerve and fascicular size and signal, and qualitative markers of course and morphology. Among them, quantitative markers of T2-signal intensity ratio are sensitive to nerve edema-like signal changes, and the T1-mapping technique reveals nerve and muscle tissue fatty and fibrous compositional alterations.The functional markers are derived from physiologic properties of nerves, such as diffusion characteristics or blood flow. They include apparent diffusion coefficient from diffusion-weighted imaging and fractional anisotropy and tractography from diffusion tensor imaging to delve into peripheral nerve microstructure and integrity. Peripheral nerve perfusion using dynamic contrast-enhanced magnetic resonance imaging estimates perfusion parameters, offering insights into nerve health and neuropathies involving edema, inflammation, demyelination, and microvascular alterations in conditions like type 2 diabetes, linking nerve conduction pathophysiology to vascular permeability alterations.Imaging biomarkers thus play a pivotal role in the diagnosis, prognosis, and monitoring of nerve pathologies, thereby ensuring comprehensive assessment and elevating patient care. These biomarkers provide valuable insights into nerve structure, function, and pathophysiology, contributing to the accurate diagnosis and management planning for peripheral neuropathy.


Assuntos
Diabetes Mellitus Tipo 2 , Doenças do Sistema Nervoso Periférico , Humanos , Imagem de Tensor de Difusão/métodos , Diabetes Mellitus Tipo 2/patologia , Nervos Periféricos/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Ultrassonografia/métodos , Biomarcadores , Espectroscopia de Ressonância Magnética , Edema
8.
Radiographics ; 44(1): e230106, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38170677

RESUMO

Endometriosis is a common condition that mostly affects people assigned as female at birth. The most common clinical symptom of endometriosis is pain. Although the mechanism for this pain is poorly understood, in some cases, the nerves are directly involved in endometriosis. Endometriosis is a multifocal disease, and the pelvis is the most common location involved. Nerves in the pelvis can become entrapped and involved in endometriosis. Pelvic nerves are visible at pelvic MRI, especially when imaging planes and sequences are tailored for neural evaluation. In particular, high-spatial-resolution anatomic imaging including three-dimensional isotropic imaging and contrast-enhanced three-dimensional short inversion time inversion-recovery (STIR) fast spin-echo sequences are useful for nerve imaging. The most commonly involved nerves are the sciatic, obturator, femoral, pudendal, and inferior hypogastric nerves and the inferior hypogastric and lumbosacral plexuses. Although it is thought to be rare, the true incidence of nerve involvement in endometriosis is not known. Symptoms of neural involvement include pain, weakness, numbness, incontinence, and paraplegia and may be constant or cyclic (catamenial). Early diagnosis of neural involvement in endometriosis is important to prevent irreversible nerve damage and chronic sensorimotor neuropathy. Evidence of irreversible damage can also be seen at MRI, and radiologists should evaluate pelvic nerves that are commonly involved in endometriosis in their search pattern and report template to ensure that this information is incorporated into treatment planning.


Assuntos
Endometriose , Doenças do Sistema Nervoso Periférico , Recém-Nascido , Humanos , Feminino , Endometriose/diagnóstico por imagem , Pelve/diagnóstico por imagem , Dor , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos
9.
Eur Radiol ; 34(8): 5228-5238, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38244046

RESUMO

OBJECTIVE: To determine the inter-reader reliability and diagnostic performance of classification and severity scales of Neuropathy Score Reporting And Data System (NS-RADS) among readers of differing experience levels after limited teaching of the scoring system. METHODS: This is a multi-institutional, cross-sectional, retrospective study of MRI cases of proven peripheral neuropathy (PN) conditions. Thirty-two radiology readers with varying experience levels were recruited from different institutions. Each reader attended and received a structured presentation that described the NS-RADS classification system containing examples and reviewed published articles on this subject. The readers were then asked to perform NS-RADS scoring with recording of category, subcategory, and most likely diagnosis. Inter-reader agreements were evaluated by Conger's kappa and diagnostic accuracy was calculated for each reader as percent correct diagnosis. A linear mixed model was used to estimate and compare accuracy between trainees and attendings. RESULTS: Across all readers, agreement was good for NS-RADS category and moderate for subcategory. Inter-reader agreement of trainees was comparable to attendings (0.65 vs 0.65). Reader accuracy for attendings was 75% (95% CI 73%, 77%), slightly higher than for trainees (71% (69%, 72%), p = 0.0006) for nerves and comparable for muscles (attendings, 87.5% (95% CI 86.1-88.8%) and trainees, 86.6% (95% CI 85.2-87.9%), p = 0.4). NS-RADS accuracy was also higher than average accuracy for the most plausible diagnosis for attending radiologists at 67% (95% CI 63%, 71%) and for trainees at 65% (95% CI 60%, 69%) (p = 0.036). CONCLUSION: Non-expert radiologists interpreted PN conditions with good accuracy and moderate-to-good inter-reader reliability using the NS-RADS scoring system. CLINICAL RELEVANCE STATEMENT: The Neuropathy Score Reporting And Data System (NS-RADS) is an accurate and reliable MRI-based image scoring system for practical use for the diagnosis and grading of severity of peripheral neuromuscular disorders by both experienced and general radiologists. KEY POINTS: • The Neuropathy Score Reporting And Data System (NS-RADS) can be used effectively by non-expert radiologists to categorize peripheral neuropathy. • Across 32 different experience-level readers, the agreement was good for NS-RADS category and moderate for NS-RADS subcategory. • NS-RADS accuracy was higher than the average accuracy for the most plausible diagnosis for both attending radiologists and trainees (at 75%, 71% and 65%, 65%, respectively).


Assuntos
Imageamento por Ressonância Magnética , Variações Dependentes do Observador , Doenças do Sistema Nervoso Periférico , Humanos , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Estudos Transversais , Estudos Retrospectivos , Reprodutibilidade dos Testes , Feminino , Masculino , Pessoa de Meia-Idade , Adulto , Idoso , Índice de Gravidade de Doença , Radiologistas , Competência Clínica , Radiologia/educação
10.
J Neurol ; 271(5): 2494-2502, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38261029

RESUMO

BACKGROUND: To specify peripheral nerve affection in autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) by correlating high-resolution nerve ultrasound and nerve conduction studies. METHODS: We assessed a cohort of 11 ARSACS patients with standardized nerve conduction studies and high-resolution ultrasound of peripheral nerves and compared nerve ultrasound findings to a healthy control group matched for age, sex, size and weight. RESULTS: Mean age of patients was 39.0 (± 14.1) years and disease duration at assessment 30.6 (± 12.5) years. All patients presented with a spasticity, ataxia and peripheral neuropathy. Neuropathy appeared to be primarily demyelinating in 9/11 cases and was not classifiable in 2/11 cases due to not evocable potentials. Nerve ultrasound revealed a normal ultrasound pattern sum score (UPSS) in each ARSACS patient and no significant nerve enlargement compared to the control group. CONCLUSIONS: Peripheral neuropathy in ARSACS showed primarily demyelinating rather than axonal characteristics and presented without nerve enlargement. As demyelinating neuropathies do commonly present enlarged nerves we recommend further genetic testing of the SACS gene in patients who present with this combination of demyelinating neuropathy without nerve enlargement. ARSACS cases that initially presented only with neuropathy without spasticity or ataxia and therefore were misdiagnosed as Charcot-Marie-Tooth disease are supporting this suggestion.


Assuntos
Doenças Desmielinizantes , Espasticidade Muscular , Condução Nervosa , Ataxias Espinocerebelares , Ataxias Espinocerebelares/congênito , Ultrassonografia , Humanos , Masculino , Feminino , Adulto , Pessoa de Meia-Idade , Condução Nervosa/fisiologia , Doenças Desmielinizantes/diagnóstico por imagem , Espasticidade Muscular/diagnóstico por imagem , Espasticidade Muscular/etiologia , Espasticidade Muscular/fisiopatologia , Ataxias Espinocerebelares/diagnóstico por imagem , Ataxias Espinocerebelares/complicações , Adulto Jovem , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/patologia , Doenças do Sistema Nervoso Periférico/fisiopatologia , Nervos Periféricos/diagnóstico por imagem , Nervos Periféricos/patologia , Estudos de Coortes
11.
Eur J Neurol ; 31(2): e16141, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-37969021

RESUMO

BACKGROUND AND PURPOSE: The role of high-resolution nerve ultrasound (HRUS) and corneal confocal microscopy (CCM) in the early detection of taxane-induced polyneuropathy (TIPN) is unclear. The present prospective longitudinal controlled observational pilot study estimates the role of HRUS and CCM in the early diagnosis of TIPN in breast cancer patients. METHODS: Fifteen breast cancer patients receiving paclitaxel and 15 healthy age matched controls were included. Visits before and 3 weeks, 8 weeks and 6 months after treatment included clinical examination, the total neuropathy score, nerve conduction studies (NCS), monocular CCM including corneal nerve fibre length, density and branching and HRUS of bilateral median, ulnar, radial, tibial, peroneal and sural nerves. Patients were compared between different visits and to healthy controls. RESULTS: Total neuropathy score increased from 2.2 at baseline to 5.8 (p < 0.001) at week 8. NCS showed a decreased sensory amplitude in the sural, radial, ulnar and median nerve after 6 months (p < 0.001). HRUS revealed a significant increase of cross-sectional area in the sural nerve (p = 0.004), the median nerve (p = 0.003) at the carpal tunnel and the ulnar nerve in the forearm (p = 0.006) after 6 months. CCM showed no changes at different visits. CONCLUSIONS: Corneal confocal microscopy and HRUS do not detect early signs of TIPN during the paclitaxel treatment period. HRUS and NCS might detect congruent signs of an axonal, predominantly sensory polyneuropathy after 6 months. The clinical examination remains the most sensitive tool in the early detection of TIPN in breast cancer patients.


Assuntos
Neoplasias da Mama , Neuropatias Diabéticas , Doenças do Sistema Nervoso Periférico , Polineuropatias , Feminino , Humanos , Neoplasias da Mama/tratamento farmacológico , Neuropatias Diabéticas/diagnóstico , Microscopia Confocal , Condução Nervosa/fisiologia , Paclitaxel , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Polineuropatias/induzido quimicamente , Polineuropatias/diagnóstico por imagem , Estudos Prospectivos , Taxoides/efeitos adversos , Projetos Piloto
12.
Adv Mater ; 36(11): e2310605, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38040414

RESUMO

Chemotherapy-induced peripheral neuropathy (CIPN) has a high prevalence but is poorly managed for cancer patients due to the lack of reliable and sensitive diagnostic techniques. Molecular optical imaging can provide a noninvasive way for real-time monitoring of CIPN; However, this is not reported, likely due to the absence of optical probes capable of imaging deep into the spinal canal and possessing sufficient sensitivity for minimal dosage through local injection into the dorsal root ganglia. Herein, a near-infrared (NIR) chemiluminophore (MPBD) with a chemiluminescence quantum yield higher than other reported probes is synthesized and a NIR activatable chemiluminescent probe (CalCL) is developed for in vivo imaging of CIPN. CalCL is constructed by caging MPBD with calpain-cleavable peptide moiety while conjugating polyethylene glycol chain to endow water solubility. Due to the deep-tissue penetration of chemiluminescence and specific turn-on response of CalCL toward calpain (a hallmark of CIPN), it allows for sensitive detection of paclitaxel-mediated CIPN in living mice, which is unattainable by fluorescence imaging. This study thus not only develops a highly efficient chemiluminescent probe, but also presents the first optical imaging approach toward high-throughput screening of neurotoxic drugs.


Assuntos
Antineoplásicos , Doenças do Sistema Nervoso Periférico , Humanos , Camundongos , Animais , Luminescência , Calpaína/efeitos adversos , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Paclitaxel , Antineoplásicos/efeitos adversos , Imagem Óptica
13.
Acta Neuropathol Commun ; 11(1): 187, 2023 11 27.
Artigo em Inglês | MEDLINE | ID: mdl-38012771

RESUMO

A relevant number of cancer patients who receive potentially neurotoxic cytostatic agents develop a chemotherapy-induced peripheral neuropathy over time. Moreover, the increasing use of immunotherapies and targeted agents leads to a raising awareness of treatment-associated peripheral neurotoxicity, e.g., axonal and demyelinating neuropathies such as Guillain-Barré-like syndromes. To date, the differentiation of these phenomena from concurrent neurological co-morbidities or (para-)neoplastic nerve affection as well as their longitudinal monitoring remain challenging. Neuromuscular ultrasound (NMUS) is an established diagnostic tool for peripheral neuropathies. Performed by specialized neurologists, it completes clinical and neurophysiological diagnostics especially in differentiation of axonal and demyelinating neuropathies. No generally approved biomarkers of treatment-induced peripheral neurotoxicity have been established so far. NMUS might significantly extend the repertoire of diagnostic and neuromonitoring methods in this growing patient group in short term. In this article, we present enlargements of the dorsal roots both in cytostatic and in immunotherapy-induced neurotoxicity for the first time. We discuss related literature regarding new integrative applications of NMUS for cancer patients by reference to two representative case studies. Moreover, we demonstrate the integration of NMUS in a diagnostic algorithm for suspected peripheral neurotoxicity independently of a certain cancer treatment regimen emphasizing the emerging potential of NMUS for clinical routine in this interdisciplinary field and prospective clinical trials.


Assuntos
Antineoplásicos , Citostáticos , Síndromes Neurotóxicas , Doenças do Sistema Nervoso Periférico , Humanos , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Citostáticos/efeitos adversos , Estudos Prospectivos , Antineoplásicos/toxicidade , Imunoterapia/efeitos adversos
14.
J Ultrasound Med ; 42(12): 2803-2813, 2023 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-37661825

RESUMO

OBJECTIVES: To explore neurofilament light chain (NfL) levels in leprotic neuropathy compared to controls, and to determine if the changes correlate with ultrasonographic nerve findings. METHODS: Individuals with leprosy with signs or symptoms suggestive of peripheral nerve involvement were recruited. They were evaluated by clinical examination, functional scores, laboratory assessments (including NfL), nerve conduction studies (NCS), and ultrasound. Ultrasound was conducted in bilateral median, ulnar, tibial, fibular, sural, and vagus nerves as well as cervical roots 5 and 6. Results were compared to age, sex, and body mass index matched healthy controls. RESULTS: A total of 320 nerves from 20 patients and 480 nerves from 30 controls were evaluated. NfL was significantly elevated in those with leprosy with a mean and standard deviation of 7.50 + 2.83 compared with 3.42 + 1.18 in controls (P < .001). Ultrasound showed focal enlargement of the nerves, particularly at entrapment sites. Additionally, there were noticeable changes in neural Doppler signal, echogenicity, and epineural thickness among the measured nerve sites. NfL levels in those with leprosy correlated closely with nerve cross-sectional area at all sites (P < .05). Functional and clinical assessment scores correlated with NfL and sonographic cross-sectional area as well (P ≤ .05). CONCLUSIONS: NfL is elevated in leprotic neuropathy. Ultrasound showed specific morphological changes in individuals with leprosy, and nerve enlargement correlated with NfL levels. Thus, both modalities may be useful for the diagnosis, prognosis, and disease monitoring in those with leprotic neuropathy, and further investigations are warranted.


Assuntos
Hanseníase , Doenças do Sistema Nervoso Periférico , Humanos , Condução Nervosa/fisiologia , Filamentos Intermediários , Nervos Periféricos/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Ultrassonografia/métodos , Hanseníase/complicações , Hanseníase/diagnóstico por imagem
15.
J Peripher Nerv Syst ; 28(4): 664-676, 2023 12.
Artigo em Inglês | MEDLINE | ID: mdl-37651181

RESUMO

BACKGROUND AND AIMS: The diagnosis of small fiber neuropathy (SFN) is supported by reduced intraepidermal nerve fiber density (IENFD). The noninvasive method corneal confocal microscopy (CCM) has the potential to be a practical alternative. We aimed to estimate the diagnostic accuracy of CCM compared with IENFD and cold detection thresholds (CDT) in SFN and mixed fiber neuropathy (MFN). METHODS: CCM was performed in an unselected prospective cohort of patients with a clinical suspicion of polyneuropathy. Predefined criteria were used to classify SFN and MFN. Neuropathy scores, including the Utah early neuropathy scale (UENS), were used to describe severity. Patients with established other diagnoses were used for diagnostic specificity calculations. RESULTS: Data were taken from 680 patients, of which 244 had SFN or MFN. There was no significant difference in sensitivities [95%CI] of CCM (0.44 [0.38-0.51]), IEFND (0.43 [0.36-0.49]), and CDT (0.34 [0.29-0.41]). CCM specificity (0.75 [0.69-0.81]) was lower (p = .044) than for IENFD (0.99 [0.96-1.00]) but not than for CDT (0.81 [0.75-0.86]). The AUCs of the ROC curves of 0.63, 0.63 and 0.74 respectively, was lower for corneal nerve fiber density (p = .0012) and corneal nerve fiber length (p = .0015) compared with IENFD. While UENS correlated significantly with IENFD (p = .0016; R2 = .041) and CDT (p = .0002; R2 = .056), it did not correlate with CCM measures. INTERPRETATION: The diagnostic utility of CCM in SNF and MFN is limited by the low specificity compared with skin biopsy. Further, CCM is less suitable than skin biopsy and CDT as a marker for neuropathy severity.


Assuntos
Doenças do Sistema Nervoso Periférico , Neuropatia de Pequenas Fibras , Humanos , Estudos Prospectivos , Pele/patologia , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/patologia , Biópsia , Neuropatia de Pequenas Fibras/diagnóstico , Neuropatia de Pequenas Fibras/patologia , Microscopia Confocal/métodos , Córnea/diagnóstico por imagem , Córnea/inervação
16.
Semin Ultrasound CT MR ; 44(4): 386-397, 2023 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-37437974

RESUMO

The Neuropathy Score Reporting and Data System (NS-RADS) is a newly developed MR imaging-based classification that standardizes reporting and multidisciplinary communication for MR imaging diagnosis and follow-up of peripheral neuropathies. NS-RADS classification has shown to be accurate and reliable across different centers, readers' experience levels, and degrees of peripheral neuropathies, which include nerve injury, entrapment, neoplasm, diffuse neuropathy, post-interventional status, and temporal changes in muscle denervation. This article brings a practical review of NS-RADS classification, representative MR cases, and a step-by-step tutorial on how to approach this staging system. Readers can gain knowledge and apply it in their practice, aiming to standardize the communications between specialties and improve patient management.


Assuntos
Doenças do Sistema Nervoso Periférico , Humanos , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Imageamento por Ressonância Magnética
17.
Eur J Neurol ; 30(8): 2442-2452, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-37154411

RESUMO

BACKGROUND AND OBJECTIVES: Hereditary spastic paraplegias (HSPs) are heterogenous genetic disorders. While peripheral nerve involvement is frequent in spastic paraplegia 7 (SPG7), the evidence of peripheral nerve involvement in SPG4 is more controversial. We aimed to characterize lower extremity peripheral nerve involvement in SPG4 and SPG7 by quantitative magnetic resonance neurography (MRN). METHODS: Twenty-six HSP patients carrying either the SPG4 or SPG7 mutation and 26 age-/sex-matched healthy controls prospectively underwent high-resolution MRN with large coverage of the sciatic and tibial nerve. Dual-echo turbo-spin-echo sequences with spectral fat-saturation were utilized for T2-relaxometry and morphometric quantification, while two gradient-echo sequences with and without an off-resonance saturation rapid frequency pulse were applied for magnetization transfer contrast (MTC) imaging. HSP patients additionally underwent detailed neurologic and electroneurographic assessments. RESULTS: All microstructural (proton spin density [ρ], T2-relaxation time, magnetization transfer ratio) and morphometric (cross-sectional area) quantitative MRN markers were decreased in SPG4 and SPG7 indicating chronic axonopathy. ρ was superior in differentiating subgroups and identifying subclinical nerve damage in SPG4 and SPG7 without neurophysiologic signs of polyneuropathy. MRN markers correlated well with clinical scores and electroneurographic results. CONCLUSIONS: MRN characterizes peripheral nerve involvement in SPG4 and SPG7 as a neuropathy with predominant axonal loss. Evidence of peripheral nerve involvement in SPG4 and SPG7, even without electroneurographically manifest polyneuropathy, and the good correlation of MRN markers with clinical measures of disease progression, challenge the traditional view of the existence of HSPs with isolated pyramidal signs and suggest MRN markers as potential progression biomarkers in HSP.


Assuntos
Doenças do Sistema Nervoso Periférico , Polineuropatias , Paraplegia Espástica Hereditária , Humanos , Paraplegia Espástica Hereditária/diagnóstico por imagem , Paraplegia Espástica Hereditária/genética , Nervos Periféricos/diagnóstico por imagem , Nervos Periféricos/patologia , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/patologia , Polineuropatias/patologia , Espectroscopia de Ressonância Magnética , Imageamento por Ressonância Magnética/métodos
18.
Neurol Sci ; 44(10): 3691-3696, 2023 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-37140833

RESUMO

BACKGROUND: Gonadal dysgenesis with minifascicular neuropathy (GDMN) is a rare autosomal recessive condition associated with biallelic DHH pathogenic variants. In 46, XY individuals, this disorder is characterized by an association of minifascicular neuropathy (MFN) and gonadal dysgenesis, while in 46, XX subjects only the neuropathic phenotype is present. Very few patients with GDMN have been reported so far. We describe four patients with MFN due to a novel DHH likely pathogenic homozygous variant and the results of nerve ultrasound assessment. METHODS: This retrospective observational study included 4 individuals from 2 unrelated Brazilian families evaluated for severe peripheral neuropathy. Genetic diagnosis was performed with a peripheral neuropathy next-generation sequencing (NGS) panel based on whole exome sequencing focused analysis that included a control SRY probe to confirm genetic sex. Clinical characterization, nerve conduction velocity studies, and high-resolution ultrasound nerve evaluation were performed in all subjects. RESULTS: Molecular analysis disclosed in all subjects the homozygous DHH variant p.(Leu335Pro). Patients had a striking phenotype, with marked trophic changes of extremities, sensory ataxia, and distal anesthesia due to a sensory-motor demyelinating polyneuropathy. One 46, XY phenotypically female individual had gonadal dysgenesis. High-resolution nerve ultrasound showed typical minifascicular formation and increased nerve area in at least one of the nerves assessed in all patients. CONCLUSION: Gonadal dysgenesis with minifascicular neuropathy is a severe autosomal recessive neuropathy characterized by trophic alterations in limbs, sensory ataxia, and distal anesthesia. Nerve ultrasound studies are very suggestive of this condition and may help to avoid invasive nerve biopsies.


Assuntos
Disgenesia Gonadal 46 XY , Disgenesia Gonadal , Doenças do Sistema Nervoso Periférico , Síndrome de Turner , Humanos , Feminino , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Doenças do Sistema Nervoso Periférico/genética , Doenças do Sistema Nervoso Periférico/complicações , Disgenesia Gonadal/complicações , Disgenesia Gonadal 46 XY/complicações , Disgenesia Gonadal 46 XY/diagnóstico , Disgenesia Gonadal 46 XY/genética , Síndrome de Turner/complicações
19.
Arch Phys Med Rehabil ; 104(8): 1236-1242, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-36854349

RESUMO

OBJECTIVES: Using ultrasound (US) scanning to examine the correlation between increase of common fibular nerve's (CFN) cross sectional area (CSA) and functional impairment of foot dorsiflexor muscles as an early sign of peripheral neuropathy. DESIGN: Retrospective observational study. SETTING: In-patient rehabilitation unit between November 2020 and July 2021. PARTICIPANTS: Twenty-six inpatients who underwent prolonged hospitalization in intensive care units (ICUs) and were diagnosed with critical illness myopathy and polyneuropathy after SARS-COV-2 infection (N=26). Physical examination and US scanning of the CFN and EMG/ENG were carried out on each patient. INTERVENTIONS: Not applicable. MAIN OUTCOME MEASURE(S): CFN's CSA at the peroneal head. RESULTS: We verified a significant increase in the CSA of the CFN measured at the peroneal head in more than 90% of the nerves tested. A cut off value of CFN's CSA of 0.20 cm was used to identify pathologic nerves. No correlations with other variables (body mass index, ICU days) were found. CONCLUSION: US scanning of the CFN appears to be an early and specific test in the evaluation of CPN's abnormalities in post COVID-19 patients. US scanning is a reproducible, cost effective, safe, and easily administered bedside tool to diagnose a loss of motor function when abnormalities in peripheral nerves are present.


Assuntos
COVID-19 , Doenças do Sistema Nervoso Periférico , Humanos , SARS-CoV-2 , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem , Nervos Periféricos , Cuidados Críticos , Unidades de Terapia Intensiva , Teste para COVID-19
20.
Radiol Clin North Am ; 61(2): 381-392, 2023 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-36739152

RESUMO

Continued advancements in magnetic resonance (MR) neurography and ultrasound have made both indispensable tools for the workup of peripheral neuropathy. Ultrasound provides high spatial resolution of superficial nerves, and techniques such as "sonopalpation" and dynamic maneuvers can improve accuracy. Superior soft tissue contrast, ability to evaluate both superficial and deep nerves with similar high resolution, and reliable characterization of denervation are strengths of MR neurography. Nevertheless, familiarity with normal anatomy, anatomic variants, and common sites of nerve entrapment is essential for radiologists to use both MR neurography and ultrasound effectively.


Assuntos
Síndromes de Compressão Nervosa , Doenças do Sistema Nervoso Periférico , Humanos , Imageamento por Ressonância Magnética/métodos , Nervos Periféricos/diagnóstico por imagem , Extremidade Inferior/diagnóstico por imagem , Extremidade Inferior/inervação , Doenças do Sistema Nervoso Periférico/diagnóstico por imagem
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