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Berciano J. Microscopical anatomy of the peripheral nervous system: An essential notion for understanding the pathophysiology of very early classic Guillain-Barré syndrome. Neuropathology 2025; 45:85-99. [PMID: 39350534 DOI: 10.1111/neup.13006] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 07/30/2024] [Revised: 08/26/2024] [Accepted: 09/06/2024] [Indexed: 04/03/2025]
Abstract
The aim of this paper is to analyze the pathophysiological mechanisms acting in very early classic Guillain-Barré syndrome (GBS) (≤4 days of symptomatic onset). In this inaugural period, both in GBS and its animal model, experimental autoimmune neuritis, the outstanding pathological feature is inflammatory edema predominating in proximal nerve trunks, particularly spinal nerves, and possibly in preterminal nerve segments. Nerve trunks external to the subarachnoid angle possess epi- perineurium that is relatively inelastic and of low compliance. Here such edema can increase endoneurial fluid pressure that, when sufficiently critical, may stretch the perineurium and constrict transperineurial microcirculation, compromising blood flow and producing the potential for ischemic nerve injury, whose consequence is rapid partial or complete loss of nerve excitability. These histopathological features correlate well with electrophysiological and imaging findings reported in early GBS stages. Spinal nerve edema and ischemia help to understand the pattern of Wallerian-like degeneration observed in the axonal form of GBS, predominating in motor spinal roots at their exit from the dura matter (spinal nerves) with centrifugal distribution in more distant motor nerve trunks, and centripetal extension to the distal portion of intrathecal roots. The similarity of initial pathogenic mechanisms between demyelinating and axonal forms of GBS explains why an early increase of serum biomarkers of axonal damage is detected in both forms. In conclusion, knowledge of the microscopic anatomy of the peripheral nervous system is an essential step for a reliable understanding of pathophysiological mechanisms operating in the early phase of any classic GBS subtype.
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Affiliation(s)
- José Berciano
- University of Cantabria, Service of Neurology, University Hospital "Marqués de Valdecilla (IDIVAL)", and "Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)", Santander, Spain
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2
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Nasr-Eldin YK, Cartwright MS, Hamed A, Ali LH, Abdel-Nasser AM. Neuromuscular Ultrasound in Polyneuropathies. JOURNAL OF ULTRASOUND IN MEDICINE : OFFICIAL JOURNAL OF THE AMERICAN INSTITUTE OF ULTRASOUND IN MEDICINE 2024; 43:1181-1198. [PMID: 38504399 DOI: 10.1002/jum.16447] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Received: 09/29/2023] [Revised: 02/20/2024] [Accepted: 02/25/2024] [Indexed: 03/21/2024]
Abstract
Neuromuscular ultrasound is a painless, radiation-free, high-resolution imaging technique for assessing the peripheral nervous system. It can accurately depict changes in the nerves and muscles of individuals with neuromuscular conditions, and it is therefore a robust diagnostic tool for the assessment of individuals with polyneuropathies. This review will outline the typical ultrasonographic changes found in a wide variety of polyneuropathies. In general, demyelinating conditions result in greater nerve enlargement than axonal conditions, and acquired conditions result in more patchy nerve enlargement compared to diffuse nerve enlargement in hereditary conditions. This review is data-driven, but more nuanced anecdotal findings are also described. The overall goal of this paper is to provide clinicians with an accessible review of the ultrasonographic approaches and findings in a wide variety of polyneuropathies.
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Affiliation(s)
| | - Michael S Cartwright
- Neurology Department, Wake Forest School of Medicine, Winston-Salem, North Carolina, USA
| | - Ahmed Hamed
- Rheumatology and Rehabilitation Department, Minia University, Minia, Egypt
| | - Lamia Hamdy Ali
- Clinical Pathology Department, Minia University, Minia, Egypt
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3
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Berciano J. The pathophysiological role of endoneurial inflammatory edema in early classical Guillain-Barré syndrome. Clin Neurol Neurosurg 2024; 237:108131. [PMID: 38308937 DOI: 10.1016/j.clineuro.2024.108131] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/21/2023] [Revised: 12/22/2023] [Accepted: 01/21/2024] [Indexed: 02/05/2024]
Abstract
The objective of this review was to analyze the pathophysiological role of endoneurial inflammatory edema in initial stages of classic Guillain-Barré syndrome (GBS), arbitrarily divided into very early GBS (≤ 4 days after symptom onset) and early GBS (≤ 10 days). Classic GBS, with variable degree of flaccid and areflexic tetraparesis, encompasses demyelinating and axonal forms. Initial autopsy studies in early GBS have demonstrated that endoneurial inflammatory edema of proximal nerve trunks, particularly spinal nerves, is the outstanding lesion. Variable permeability of the blood-nerve barrier dictates such lesion topography. In proximal nerve trunks possessing epi-perineurium, edema may increase the endoneurial fluid pressure causing ischemic changes. Critical analysis the first pathological description of the axonal form GBS shows a combination of axonal degeneration and demyelination in spinal roots, and pure Wallerian-like degeneration in peripheral nerve trunks. This case might be reclassified as demyelinating GBS with secondary axonal degeneration. Both in acute motor axonal neuropathy and acute motor-sensory axonal neuropathy, Wallerian-like degeneration of motor fibers predominates in the distal part of ventral spinal roots abutting the dura mater, another feature re-emphasizing the pathogenic relevance of this area. Electrophysiological and imaging studies also point to a predominant alteration at the spinal nerve level, which is a hotspot in any early GBS subtype. Serum biomarkers of axonal damage, including neurofilament light chain and peripherin, are increased in the great majority of patients with any early GBS subtype; endoneurial ischemia of proximal nerve trunks could contribute to such axonal damage. It is concluded that inflammatory edema of proximal nerve trunks is an essential pathogenic event in early GBS, which has a tangible impact for accurate approach to the disease.
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Affiliation(s)
- José Berciano
- University of Cantabria, University Hospital "Marqués de Valdecilla (IDIVAL)", and "Centro de Investigación Biomédica en Red de Enfermedades Neurodegenerativas (CIBERNED)", Santander, Spain.
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Fodor D, Rodriguez-Garcia SC, Cantisani V, Hammer HB, Hartung W, Klauser A, Martinoli C, Terslev L, Alfageme F, Bong D, Bueno A, Collado P, D'Agostino MA, de la Fuente J, Iohom G, Kessler J, Lenghel M, Malattia C, Mandl P, Mendoza-Cembranos D, Micu M, Möller I, Najm A, Özçakar L, Picasso R, Plagou A, Sala-Blanch X, Sconfienza LM, Serban O, Simoni P, Sudoł-Szopińska I, Tesch C, Todorov P, Uson J, Vlad V, Zaottini F, Bilous D, Gutiu R, Pelea M, Marian A, Naredo E. The EFSUMB Guidelines and Recommendations for Musculoskeletal Ultrasound - Part I: Extraarticular Pathologies. ULTRASCHALL IN DER MEDIZIN (STUTTGART, GERMANY : 1980) 2022; 43:34-57. [PMID: 34479372 DOI: 10.1055/a-1562-1455] [Citation(s) in RCA: 20] [Impact Index Per Article: 6.7] [Reference Citation Analysis] [Abstract] [MESH Headings] [Track Full Text] [Subscribe] [Scholar Register] [Indexed: 06/13/2023]
Abstract
The first part of the guidelines and recommendations for musculoskeletal ultrasound, produced under the auspices of the European Federation of Societies for Ultrasound in Medicine and Biology (EFSUMB), provides information about the use of musculoskeletal ultrasound for assessing extraarticular structures (muscles, tendons, entheses, ligaments, bones, bursae, fasciae, nerves, skin, subcutaneous tissues, and nails) and their pathologies. Clinical applications, practical points, limitations, and artifacts are described and discussed for every structure. After an extensive literature review, the recommendations have been developed according to the Oxford Centre for Evidence-based Medicine and GRADE criteria and the consensus level was established through a Delphi process. The document is intended to guide clinical users in their daily practice.
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Affiliation(s)
- Daniela Fodor
- 2nd Internal Medicine Department, "Iuliu Hatieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | | | - Vito Cantisani
- Department of Radiological, Oncological and Anatomo-pathological Sciences, "Sapienza" University, Rome, Italy
| | - Hilde B Hammer
- Department of Rheumatology, Diakonhjemmet Hospital and Faculty of Medicine, University of Oslo, Oslo, Norway
| | - Wolfgang Hartung
- Clinic for Rheumatology and Clinical Immunology, Asklepios Clinic, Bad Abbach, Germany
| | - Andrea Klauser
- Department of Radiology, Medical University Innsbruck, Section Head Rheumatology and Sports Imaging, Innsbruck, Austria
| | - Carlo Martinoli
- Department of Health Science - DISSAL, University of Genova, Italy
- UO Radiologia, IRCCS Policlinico San Martino, Genova, Italy
| | - Lene Terslev
- Copenhagen Center for Arthritis Research, Center for Rheumatology and Spine Diseases, Rigshospitalet, Glostrup, Denmark; Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark
| | - Fernando Alfageme
- Dermatology Department, Hospital Universitario Puerta de Hierro Majadahonda, Madrid, Spain
| | - David Bong
- Instituto Poal de Reumatologia Barcelona, EULAR Working Group Anatomy for the Image, University of Barcelona, International University of Catalunya, Spain
| | - Angel Bueno
- Department of Musculoskeletal Radiology, Hospital Universitario Fundación Alcorcón, Madrid, Spain
| | - Paz Collado
- Rheumatology Department, Transitional Care Clinic, Hospital Universitario Severo Ochoa, Madrid, Spain
| | - Maria Antonietta D'Agostino
- Istituto di Reumatologia Università Cattolica del Sacro Cuore, UOC Reumatologia, Fondazione Policlinico Universitario Agostino Gemelli, IRCCS, Rome, Italy
| | | | - Gabriella Iohom
- Department of Anaesthesiology and Intensive Care Medicine, Cork University Hospital and University College Cork, Cork, Ireland
| | - Jens Kessler
- Department of Anaesthesiology, Division of Pain Medicine, University Hospital Heidelberg, Heidelberg, Germany
| | - Manuela Lenghel
- Radiology Department, "Iuliu Hatieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - Clara Malattia
- UOC Clinica Pediatrica e Reumatologia, IRCCS Istituto Giannina Gaslini, Department of Neurosciences, Rehabilitation, Ophthalmology, Genetic and Maternal Infantile Sciences (DINOGMI) University of Genoa, Genoa, Italy
| | - Peter Mandl
- Division of Rheumatology, Medical University of Vienna, Vienna, Austria
| | | | - Mihaela Micu
- Rheumatology Division, 2nd Rehabilitation Department, Rehabilitation Clinical Hospital Cluj-Napoca, Romania
| | - Ingrid Möller
- Instituto Poal de Reumatologia Barcelona, EULAR Working Group Anatomy for the Image, University of Barcelona, International University of Catalunya, Spain
| | - Aurelie Najm
- Institute of Infection, Immunity and Inflammation, College of Medical Veterinary and Life Sciences, University of Glasgow, Glasgow, United Kingdom
| | - Levent Özçakar
- Department of Physical and Rehabilitation Medicine, Hacettepe University Medical School, Ankara, Turkey
| | - Riccardo Picasso
- Department of Health Science - DISSAL, University of Genova, Italy
- UO Radiologia, IRCCS Policlinico San Martino, Genova, Italy
| | - Athena Plagou
- Ultrasound Unit, Private Radiological Institution, Athens, Greece
| | - Xavier Sala-Blanch
- Department of Anaesthesiology, Hospital Clinic, Department of Human Anatomy, Faculty of Medicine, University of Barcelona, Spain
| | - Luca Maria Sconfienza
- IRCCS Istituto Ortopedico Galeazzi, Milano Italy
- Department of Biomedical Sciences for Health, University of Milano, Milano, Italy
| | - Oana Serban
- 2nd Internal Medicine Department, "Iuliu Hatieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - Paolo Simoni
- Paediatric Imaging Department, "Reine Fabiola" Children's University Hospital, Université Libre de Bruxelles, Brussels, Belgium
| | - Iwona Sudoł-Szopińska
- Department of Radiology, National Institute of Geriatrics, Rheumatology and Rehabilitation, Warsaw, Poland
| | | | - Plamen Todorov
- Department of Internal Disease Propaedeutic and Clinical Rheumatology, Medical University of Plovdiv, Plovdiv, Bulgaria
| | - Jacqueline Uson
- Department of Rheumatology Hospital Universitario Móstoles, Universidad Rey Juan Carlos, Madrid, Spain
| | - Violeta Vlad
- Sf. Maria Hospital, Rheumatology Department, Bucharest, Romania
| | - Federico Zaottini
- Department of Health Science - DISSAL, University of Genova, Italy
- UO Radiologia, IRCCS Policlinico San Martino, Genova, Italy
| | - Diana Bilous
- 2nd Internal Medicine Department, "Iuliu Hatieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - Roxana Gutiu
- 2nd Internal Medicine Department, "Iuliu Hatieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - Michael Pelea
- 2nd Internal Medicine Department, "Iuliu Hatieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - Anamaria Marian
- 2nd Internal Medicine Department, "Iuliu Hatieganu" University of Medicine and Pharmacy, Cluj-Napoca, Romania
| | - Esperanza Naredo
- Department of Rheumatology, Bone and Joint Research Unit, Hospital Universitario Fundación Jiménez Díaz, IIS Fundación Jiménez Díaz, and Universidad Autónoma de Madrid, Madrid, Spain
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Hannaford A, Vucic S, Kiernan MC, Simon NG. Review Article "Spotlight on Ultrasonography in the Diagnosis of Peripheral Nerve Disease: The Evidence to Date". Int J Gen Med 2021; 14:4579-4604. [PMID: 34429642 PMCID: PMC8378935 DOI: 10.2147/ijgm.s295851] [Citation(s) in RCA: 8] [Impact Index Per Article: 2.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/22/2021] [Accepted: 07/27/2021] [Indexed: 11/23/2022] Open
Abstract
Neuromuscular ultrasound is rapidly becoming incorporated into clinical practice as a standard tool in the assessment of peripheral nerve diseases. Ultrasound complements clinical phenotyping and electrodiagnostic evaluation, providing critical structural anatomical information to enhance diagnosis and identify structural pathology. This review article examines the evidence supporting neuromuscular ultrasound in the diagnosis of compressive mononeuropathies, traumatic nerve injury, generalised peripheral neuropathy and motor neuron disease. Extending the sonographic evaluation of nerves beyond simple morphological measurements has the potential to improve diagnostics in peripheral neuropathy, as well as advancing the understanding of pathological mechanisms, which in turn will promote precise therapies and improve therapeutic outcomes.
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Affiliation(s)
- Andrew Hannaford
- Westmead Clinical School, Westmead Hospital, University of Sydney, Sydney, Australia
| | - Steve Vucic
- Westmead Clinical School, Westmead Hospital, University of Sydney, Sydney, Australia
| | - Matthew C Kiernan
- Brain and Mind Centre, University of Sydney, University of Sydney and Department of Neurology, Royal Prince Alfred Hospital, Sydney, Australia
| | - Neil G Simon
- Northern Beaches Clinical School, Macquarie University, Sydney, Australia
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Liu L, Ye Y, Wang L, Song X, Cao J, Qi Y, Xing Y. Nerve ultrasound evaluation of Guillain-Barré syndrome subtypes in northern China. Muscle Nerve 2021; 64:560-566. [PMID: 34355400 DOI: 10.1002/mus.27386] [Citation(s) in RCA: 9] [Impact Index Per Article: 2.3] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 06/13/2020] [Revised: 08/01/2021] [Accepted: 08/02/2021] [Indexed: 01/23/2023]
Abstract
INTRODUCTION/AIMS Ultrasound (US) studies have demonstrated patchy enlargement of spinal and peripheral nerves in Guillain-Barré syndrome (GBS). However, whether ultrasound yields useful information for early classification of GBS has not been established. We aimed to evaluate nerve ultrasound in patients with GBS in northern China and compare the sonographic characteristics between demyelinating and axonal subtypes. METHODS Between November 2018 and October 2019, 38 hospitalized GBS patients within 3 wk of disease onset and 40 healthy controls were enrolled. Ultrasonographic cross-sectional areas (CSA) of the peripheral nerves, vagus nerve, and cervical nerve roots were prospectively recorded in GBS subtypes and controls. RESULTS Ultrasonographic CSA exhibited significant enlargement in most patients' nerves compared with healthy controls, most prominent in cervical nerves. The CSA tended to be larger in acute inflammatory demyelinating polyneuropathy (AIDP) than in acute motor axonal neuropathy (AMAN)/acute motor and sensory axonal neuropathy (AMSAN), especially in cervical nerves (C5: 5.9 ± 1.6 mm2 vs. 7.0 ± 1.7 mm2 , p = .042; C6: 10.5 ± 1.8 mm2 vs. 12.0 ± 2.1 mm2 , p = .033). The chi-squared test revealed significant differences in nerve enlargement in C5 (p < .001), C6 (p < .001), the proximal median nerve (p < .001), and the vagus nerve (p = .003) between GBS and controls. The vagus nerve was larger in patients with autonomic dysfunction than in patients without it (2.3 ± 1.0 mm2 vs. 1.4 ± 0.5 mm2 , p = .003). DISCUSSION The demyelinating subtype presented with more significant cervical nerve enlargement in GBS. Vagus nerve enlargement may be a useful marker for autonomic dysfunction.
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Affiliation(s)
- Li Liu
- Department of Vascular Ultrasonography, Xuanwu Hospital, Capital Medical University, Beijing, China.,Center of Vascular Ultrasonography, Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, China.,Department of Neurology, Changchun City People's hospital, Changchun, China
| | - Yuqin Ye
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Lijuan Wang
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Xiaonan Song
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Jie Cao
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Yajie Qi
- Department of Neurology, The First Hospital of Jilin University, Changchun, China
| | - Yingqi Xing
- Department of Vascular Ultrasonography, Xuanwu Hospital, Capital Medical University, Beijing, China.,Center of Vascular Ultrasonography, Beijing Institute of Brain Disorders, Collaborative Innovation Center for Brain Disorders, Capital Medical University, Beijing, China.,Department of Neurology, The First Hospital of Jilin University, Changchun, China
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7
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Nerve Ultrasound as Helpful Tool in Polyneuropathies. Diagnostics (Basel) 2021; 11:diagnostics11020211. [PMID: 33572591 PMCID: PMC7910962 DOI: 10.3390/diagnostics11020211] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.5] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 12/30/2020] [Revised: 01/26/2021] [Accepted: 01/27/2021] [Indexed: 02/06/2023] Open
Abstract
Background: Polyneuropathies (PNP) are a broad field of diseases affecting millions of people. While the symptoms presented are mostly similar, underlying causes are abundant. Thus, early identification of treatable causes is often difficult. Besides clinical data and basic laboratory findings, nerve conduction studies are crucial for etiological classification, yet limited. Besides Magnetic Resonance Imaging (MRI), high-resolution nerve ultrasound (HRUS) has become a noninvasive, fast, economic and available tool to help distinguish different types of nerve alterations in neuropathies. Methods: We aim to describe typical ultrasound findings in PNP and patterns of morphological changes in hereditary, immune-mediated, diabetic, metabolic and neurodegenerative PNP. Literature research was performed in PubMed using the terms ‘nerve ultrasound’, neuromuscular ultrasound, high-resolution nerve ultrasound, peripheral nerves, nerve enlargement, demyelinating, hereditary, polyneuropathies, hypertrophy’. Results: Plenty of studies over the past 20 years investigated the value of nerve ultrasound in different neuropathies. Next to nerve enlargement, patterns of nerve enlargement, echointensity, vascularization and elastography have been evaluated for diagnostic terms. Furthermore, different scores have been developed to distinguish different etiologies of PNP. Conclusions: Where morphological alterations of the nerves reflect underlying pathologies, early nerve ultrasound might enable a timely start of available treatment and also facilitate follow up of therapy success.
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8
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Moss KR, Bopp TS, Johnson AE, Höke A. New evidence for secondary axonal degeneration in demyelinating neuropathies. Neurosci Lett 2021; 744:135595. [PMID: 33359733 PMCID: PMC7852893 DOI: 10.1016/j.neulet.2020.135595] [Citation(s) in RCA: 22] [Impact Index Per Article: 5.5] [Reference Citation Analysis] [Abstract] [Key Words] [MESH Headings] [Grants] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 08/02/2020] [Revised: 10/31/2020] [Accepted: 12/19/2020] [Indexed: 12/28/2022]
Abstract
Development of peripheral nervous system (PNS) myelin involves a coordinated series of events between growing axons and the Schwann cell (SC) progenitors that will eventually ensheath them. Myelin sheaths have evolved out of necessity to maintain rapid impulse propagation while accounting for body space constraints. However, myelinating SCs perform additional critical functions that are required to preserve axonal integrity including mitigating energy consumption by establishing the nodal architecture, regulating axon caliber by organizing axonal cytoskeleton networks, providing trophic and potentially metabolic support, possibly supplying genetic translation materials and protecting axons from toxic insults. The intermediate steps between the loss of these functions and the initiation of axon degeneration are unknown but the importance of these processes provides insightful clues. Prevalent demyelinating diseases of the PNS include the inherited neuropathies Charcot-Marie-Tooth Disease, Type 1 (CMT1) and Hereditary Neuropathy with Liability to Pressure Palsies (HNPP) and the inflammatory diseases Acute Inflammatory Demyelinating Polyneuropathy (AIDP) and Chronic Inflammatory Demyelinating Polyneuropathy (CIDP). Secondary axon degeneration is a common feature of demyelinating neuropathies and this process is often correlated with clinical deficits and long-lasting disability in patients. There is abundant electrophysiological and histological evidence for secondary axon degeneration in patients and rodent models of PNS demyelinating diseases. Fully understanding the involvement of secondary axon degeneration in these diseases is essential for expanding our knowledge of disease pathogenesis and prognosis, which will be essential for developing novel therapeutic strategies.
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Affiliation(s)
- Kathryn R Moss
- Department of Neurology, Neuromuscular Division, Johns Hopkins School of Medicine, Baltimore, MD, United States
| | - Taylor S Bopp
- Department of Neurology, Neuromuscular Division, Johns Hopkins School of Medicine, Baltimore, MD, United States
| | - Anna E Johnson
- Department of Neurology, Neuromuscular Division, Johns Hopkins School of Medicine, Baltimore, MD, United States
| | - Ahmet Höke
- Department of Neurology, Neuromuscular Division, Johns Hopkins School of Medicine, Baltimore, MD, United States.
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9
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Hsueh HW, Chang KC, Chao CC, Hsieh ST. A Pilot Study on Serial Nerve Ultrasound in Miller Fisher Syndrome. Front Neurol 2020; 11:865. [PMID: 32922359 PMCID: PMC7457056 DOI: 10.3389/fneur.2020.00865] [Citation(s) in RCA: 6] [Impact Index Per Article: 1.2] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Figures] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/13/2019] [Accepted: 07/07/2020] [Indexed: 12/28/2022] Open
Abstract
Objective: Miller Fisher syndrome (MFS) is predominantly a clinical diagnosis, with classic triad of ophthalmoplegia, ataxia, and generalized reduced reflexes. Previous studies in chronic and acute immune-mediated neuropathies indicated that ultrasound, may help to detect changes that could correspond with disease activity. We studied the feasibility of serial nerve ultrasound in MFS, using a healthy controls. Methods: All MFS patients (n = 5) and healthy controls (n = 18), underwent a standardized sonographic protocol that evaluated nerve sizes of facial, large arm and leg nerves, and spinal nerve roots. All MFS patients underwent routine ancillary investigations, including electrodiagnostic testing and for presence of anti-GQ1b antibodies. In addition, four MFS patients had 2nd, and 3rd clinical and sonographic evaluation at 14 and 90 days from onset. Results: The width of the facial nerve was significantly larger in the MFS group than in the control group (MFS: 1.19 ± 0.31 mm vs. normal: 0.67 ± 0.13 mm, P = 0.01). The size of the cervical roots and the nerves in the limbs were similar between the two groups. Two patients' facial nerve size subsided with time, but the decrease in other nerves' sizes were not obvious. Conclusion: Our study showed that serial nerve ultrasound studies are feasible in MFS, and can capture changes in facial nerve size that could complement routine diagnostic tests. Further studies are warranted to determine and compare its test characteristics in MFS.
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Affiliation(s)
- Hsueh-Wen Hsueh
- Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan
| | - Kai-Chieh Chang
- Department of Neurology, National Taiwan University Hospital Yunlin Branch, Yunlin, Taiwan
| | - Chi-Chao Chao
- Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan
| | - Sung-Tsang Hsieh
- Department of Neurology, National Taiwan University Hospital, Taipei, Taiwan.,Graduate Institute of Anatomy and Cell Biology, National Taiwan University Hospital College of Medicine, Taipei, Taiwan
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10
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Carroll AS, Simon NG. Current and future applications of ultrasound imaging in peripheral nerve disorders. World J Radiol 2020; 12:101-129. [PMID: 32742576 PMCID: PMC7364285 DOI: 10.4329/wjr.v12.i6.101] [Citation(s) in RCA: 25] [Impact Index Per Article: 5.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Download PDF] [Journal Information] [Submit a Manuscript] [Subscribe] [Scholar Register] [Received: 02/28/2020] [Revised: 05/10/2020] [Accepted: 05/28/2020] [Indexed: 02/06/2023] Open
Abstract
Neuromuscular ultrasound (NMUS) is a rapidly evolving technique used in neuromuscular medicine to provide complimentary information to standard electrodiagnostic studies. NMUS provides a dynamic, real time assessment of anatomy which can alter both diagnostic and management pathways in peripheral nerve disorders. This review describes the current and future techniques used in NMUS and details the applications and developments in the diagnosis and monitoring of compressive, hereditary, immune-mediated and axonal peripheral nerve disorders, and motor neuron diseases. Technological advances have allowed the increased utilisation of ultrasound for management of peripheral nerve disorders; however, several practical considerations need to be taken into account to facilitate the widespread uptake of this technique.
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Affiliation(s)
- Antonia S Carroll
- Brain and Mind Research Centre, University of Sydney, Camperdown 2050, NSW, Australia
- Department of Neurology, Westmead Hospital, University of Sydney, Westmead 2145, NSW, Australia
- Department of Neurology, St Vincent’s Hospital, Sydney, Darlinghurst 2010, NSW, Australia
| | - Neil G Simon
- Northern Clinical School, University of Sydney, Frenchs Forest 2086, NSW, Australia
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11
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Abstract
PURPOSE OF REVIEW The clinical presentation of Guillain-Barré syndrome (GBS) is highly variable, which can make the diagnosis challenging. Intravenous immunoglobulin (IVIg) and plasma exchange are the cornerstones of treatment since decades. But despite these treatments, 25% initially progress in muscle weakness, 25% require artificial ventilation, 20% is still not able to walk independently after 6 months, and 2-5% die, emphasizing the need for better treatment. We summarize new developments regarding the diagnosis, prognosis, and management of GBS. RECENT FINDINGS GBS is a clinical diagnosis that can be supported by cerebrospinal fluid examination and nerve conduction studies. Nerve ultrasound and MRI are potentially useful techniques to diagnose inflammatory neuropathies. Several novel infections have recently been associated to GBS. Evidence from experimental studies and recent phase 2 clinical trials suggests that complement inhibition combined with IVIg might improve outcome in GBS, but further studies are warranted. Prognostic models could guide the selection of patients with a relatively poor prognosis that might benefit most from additional IVIg or otherwise intensified treatment. SUMMARY New diagnostic tools may help to have early and accurate diagnosis in difficult GBS cases. Increased knowledge on the pathophysiology of GBS forms the basis for development of new, targeted, and personalized treatments that hopefully improve outcome.
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12
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Walker FO, Cartwright MS, Alter KE, Visser LH, Hobson-Webb LD, Padua L, Strakowski JA, Preston DC, Boon AJ, Axer H, van Alfen N, Tawfik EA, Wilder-Smith E, Yoon JS, Kim BJ, Breiner A, Bland JDP, Grimm A, Zaidman CM. Indications for neuromuscular ultrasound: Expert opinion and review of the literature. Clin Neurophysiol 2018; 129:2658-2679. [PMID: 30309740 DOI: 10.1016/j.clinph.2018.09.013] [Citation(s) in RCA: 49] [Impact Index Per Article: 7.0] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 04/26/2018] [Revised: 08/10/2018] [Accepted: 09/02/2018] [Indexed: 12/11/2022]
Abstract
Over the last two decades, dozens of applications have emerged for ultrasonography in neuromuscular disorders. We wanted to measure its impact on practice in laboratories where the technique is in frequent use. After identifying experts in neuromuscular ultrasound and electrodiagnosis, we assessed their use of ultrasonography for different indications and their expectations for its future evolution. We then identified the earliest papers to provide convincing evidence of the utility of ultrasound for particular indications and analyzed the relationship of their date of publication with expert usage. We found that experts use ultrasonography often for inflammatory, hereditary, traumatic, compressive and neoplastic neuropathies, and somewhat less often for neuronopathies and myopathies. Usage significantly correlated with the timing of key publications in the field. We review these findings and the extensive evidence supporting the value of neuromuscular ultrasound. Advancement of the field of clinical neurophysiology depends on widespread translation of these findings.
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Affiliation(s)
- Francis O Walker
- Department of Neurology at Wake Forest School of Medicine, Medical Center Blvd, Winston-Salem, NC, USA.
| | - Michael S Cartwright
- Department of Neurology at Wake Forest School of Medicine, Medical Center Blvd, Winston-Salem, NC, USA.
| | - Katharine E Alter
- Department of Rehabilitation Medicine, National INeurolnstitutes of Health, Bethesda, MD 20892, USA.
| | - Leo H Visser
- Departments of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands.
| | - Lisa D Hobson-Webb
- Department of Neurology, Neuromuscular Division, Duke University School of Medicine, Durham, NC, USA.
| | - Luca Padua
- Don Carlo Gnocchi ONLUS Foundation, Piazzale Rodolfo Morandi, 6, 20121 Milan, Italy; Department of Geriatrics, Neurosciences and Orthopaedics, Universita Cattolica del Sacro Cuore, Rome, Italy.
| | - Jeffery A Strakowski
- Department of Physical Medicine and Rehabilitation, The Ohio State University, Columbus, OH, USA; Department of Physical Medicine and Rehabilitation, OhioHealth Riverside Methodist Hospital, Columbus, OH, USA; OhioHealth McConnell Spine, Sport and Joint Center, Columbus, OH, USA.
| | - David C Preston
- Neurological Institute, University Hospitals, Cleveland Medical Center, Case Western Reserve University, Cleveland, OH, USA.
| | - Andrea J Boon
- Department of Physical Medicine and Rehabilitation, Mayo Clinic, Rochester, MN, USA.
| | - Hubertus Axer
- Hans Berger Department of Neurology, Jena University Hospital, Jena 07747, Germany.
| | - Nens van Alfen
- Department of Neurology and Clinical Neurophysiology, Donders Institute for Brain, Cognition, and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands.
| | - Eman A Tawfik
- Department of Physical Medicine & Rehabilitation, Faculty of Medicine, Ain Shams University, Cairo, Egypt.
| | - Einar Wilder-Smith
- Department of Neurology, Yong Loo Lin School of Medicine, National University Singapore, Singapore; Department of Neurology, Kantonsspital Lucerne, Switzerland; Department of Neurology, Inselspital Berne, Switzerland.
| | - Joon Shik Yoon
- Department of Physical Medicine and Rehabilitation, Korea University Guro Hospital, Seoul, Republic of Korea.
| | - Byung-Jo Kim
- Department of Neurology, Korea University Anam Hospital, Korea University College of Medicine, Seoul, Republic of Korea.
| | - Ari Breiner
- Division of Neurology, Department of Medicine, The Ottawa Hospital and University of Ottawa, Canada.
| | - Jeremy D P Bland
- Deparment of Clinical Neurophysiology, East Kent Hospitals University NHS Foundation Trust, Canterbury, Kent, UK.
| | - Alexander Grimm
- Department of Neurology, University Hospital Tuebingen, Tuebingen, Germany.
| | - Craig M Zaidman
- Division of Neuromuscular Medicine, Department of Neurology, Washington University in St. Louis, 660 S. Euclid Ave, Box 8111, St. Louis, MO 63110, USA.
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13
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Telleman JA, Grimm A, Goedee S, Visser LH, Zaidman CM. Nerve ultrasound in polyneuropathies. Muscle Nerve 2018; 57:716-728. [PMID: 29205398 DOI: 10.1002/mus.26029] [Citation(s) in RCA: 61] [Impact Index Per Article: 8.7] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 11/30/2017] [Revised: 11/30/2017] [Accepted: 11/30/2017] [Indexed: 12/13/2022]
Abstract
Ultrasound can be used to visualize pathology in the peripheral nerves of patients with polyneuropathy. Nerve enlargement is the most frequent pathology, but other abnormalities, including abnormal nerve echogenicity and vascularity, are also encountered. This monograph presents an overview of the role of nerve ultrasound in the evaluation and management of both inherited and acquired polyneuropathies. A description of the sonographic techniques and common abnormalities is provided, followed by a presentation of typical findings in different neuropathies. Scoring systems for characterizing the presence and pattern of nerve abnormalities as they relate to different polyneuropathies are presented. Muscle Nerve 57: 716-728, 2018.
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Affiliation(s)
- Johan A Telleman
- Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands.,Brain Center Rudolf Magnus, Department of Neurology and Neurosurgery, UMC Utrecht, Utrecht, The Netherlands
| | - Alexander Grimm
- Department Neurology, University Hospital Tuebingen, Germany
| | - Stephan Goedee
- Brain Center Rudolf Magnus, Department of Neurology and Neurosurgery, UMC Utrecht, Utrecht, The Netherlands
| | - Leo H Visser
- Department of Neurology and Clinical Neurophysiology, Elisabeth-Tweesteden Hospital, Tilburg, The Netherlands
| | - Craig M Zaidman
- Departments of Neurology and Pediatrics, Washington University St. Louis, Missouri, 660 South Euclid, Box 8111, St. Louis, Missouri, 63110-1093, USA
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14
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Coraci D, Santilli V, Padua L. Consideration about "sonographic evaluation of peripheral nerves in subtypes of Guillain-Barré syndrome". J Neurol Sci 2016; 366:246-247. [PMID: 27210049 DOI: 10.1016/j.jns.2016.05.030] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Key Words] [MESH Headings] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/09/2016] [Accepted: 05/13/2016] [Indexed: 11/17/2022]
Affiliation(s)
- Daniele Coraci
- Board of Physical Medicine and Rehabilitation, Department of Orthopaedic Science, "Sapienza" University, Rome, Italy; Don Carlo Gnocchi ONLUS Foundation, Milan, Italy
| | - Valter Santilli
- Board of Physical Medicine and Rehabilitation, Department of Orthopaedic Science, "Sapienza" University, Rome, Italy; Physical Medicine and Rehabilitation Unit, Azienda Policlinico Umberto I, Rome, Italy
| | - Luca Padua
- Don Carlo Gnocchi ONLUS Foundation, Milan, Italy; Department of Geriatrics, Neurosciences and Orthopaedics, Università Cattolica del Sacro Cuore, Rome, Italy.
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15
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Response to “Sonographic evaluation of peripheral nerves in subtypes of Guillain-Barré syndrome”. J Neurol Sci 2016; 366:234. [DOI: 10.1016/j.jns.2016.05.031] [Citation(s) in RCA: 0] [Impact Index Per Article: 0] [Reference Citation Analysis] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/13/2016] [Accepted: 05/13/2016] [Indexed: 11/21/2022]
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16
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Proximal nerve lesions in early Guillain-Barré syndrome: implications for pathogenesis and disease classification. J Neurol 2016; 264:221-236. [PMID: 27314967 DOI: 10.1007/s00415-016-8204-2] [Citation(s) in RCA: 53] [Impact Index Per Article: 5.9] [Reference Citation Analysis] [Abstract] [Key Words] [Track Full Text] [Journal Information] [Subscribe] [Scholar Register] [Received: 05/12/2016] [Revised: 06/10/2016] [Accepted: 06/10/2016] [Indexed: 12/13/2022]
Abstract
Guillain-Barré syndrome (GBS) is an acute-onset, immune-mediated disorder of the peripheral nervous system. In early GBS, arbitrarily established up to 10 days of disease onset, patients could exhibit selective manifestations due to involvement of the proximal nerves, including nerve roots, spinal nerves and plexuses. Such manifestations are proximal weakness, inaugural nerve trunk pain, and atypical electrophysiological patterns, which may lead to delayed diagnosis. The aim of this paper was to analyze the nosology of early GBS reviewing electrophysiological, autopsy and imaging studies, both in acute inflammatory demyelinating polyneuropathy (AIDP) and acute motor/motor-sensory axonal neuropathy (AMAN/AMSAN). Early electrophysiology showed either well-defined demyelinating or axonal patterns, or a non-diagnostic pattern with abnormal late responses; there may be attenuated M responses upon lumbar root stimulation as the only finding. Pathological changes predominated in proximal nerves, in some studies, most prominent at the sides where the spinal roots unite to form the spinal nerves; on very early GBS endoneurial inflammatory edema was the outstanding feature. In the far majority of cases, spinal magnetic resonance imaging showed contrast enhancement of cauda equina, selectively involving anterior roots in AMAN. Both in AIDP and AMAN/AMSAN, ultrasonography has demonstrated frequent enlargement of ventral rami of C5-C7 nerves with blurred boundaries, whereas sonograms of upper and lower extremity peripheral nerves exhibited variable and less frequent abnormalities. We provide new insights into the pathogenesis and classification of early GBS.
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