scholarly journals Inflammatory polyradiculoneuropathies: Clinical and immunological aspects, current therapies, and future perspectives

2020 ◽  
Vol 18 ◽  
pp. 205873922094234
Author(s):  
Vincenzo Di Stefano ◽  
Filomena Barbone ◽  
Camilla Ferrante ◽  
Roberta Telese ◽  
Michela Vitale ◽  
...  
Keyword(s):  
Peripheral Nerves ◽  
Axonal Degeneration ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Leukocyte Infiltration ◽  
Potential Treatment ◽  
Future Perspectives ◽  
Nerve Roots ◽  
Immune Mediated ◽  
Current Therapies ◽  
Made In

Inflammatory polyradiculoneuropathies are heterogeneous disorders characterized by immune-mediated leukocyte infiltration of peripheral nerves and nerve roots leading to demyelination or axonal degeneration or both. Inflammatory polyradiculoneuropathies can be divided into acute and chronic: Guillain–Barré syndrome and chronic inflammatory demyelinating polyneuropathy and their variants. Despite major advances in immunology and molecular biology have been made in the last years, the pathogenesis of these disorders is not completely understood. This review summarizes the current literature of the clinical features and pathogenic mechanisms of inflammatory polyradiculoneuropathies and focuses on current therapies and new potential treatment for the future.

scholarly journals Translational strategies in peripheral neuroinflammation and neurovascular repair

2012 ◽  
Vol 3 (4) ◽  
Author(s):  
Eroboghene Ubogu
Keyword(s):  
Peripheral Nerve ◽  
Nerve Regeneration ◽  
Axonal Degeneration ◽  
Peripheral Nerve Regeneration ◽  
Treatment Strategies ◽  
Normal Function ◽  
New Drugs ◽  
Immune Mediated ◽  
Current Therapies

AbstractCurrent therapies for immune-mediated inflammatory disorders in peripheral nerves are non-specific, and partly efficacious. Peripheral nerve regeneration following axonal degeneration or injury is suboptimal, with current therapies focused on modulating the underlying etiology and treating the consequences, such as neuropathic pain and weakness. Despite significant advances in understanding mechanisms of peripheral nerve inflammation, as well as axonal degeneration and regeneration, there has been limited translation into effective new drugs for these disorders. A major limitation in the field has been the unavailability of reliable disease models or research tools that mimic some key essential features of these human conditions. A relatively overlooked aspect of peripheral nerve regeneration has been neurovascular repair required to restore the homeostatic microenvironment necessary for normal function. Using Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) as examples of human acute and chronic immune-mediated peripheral neuroinflammatory disorders respectively, we have performed detailed studies in representative mouse models to demonstrate essential features of the human disorders. These models are important tools to develop and test treatment strategies using realistic outcomes measures applicable to affected patients. In vitro models of the human blood-nerve barrier using endothelial cells derived by endoneurial microvessels provide insights into pro-inflammatory leukocyte-endothelial cell interactions relevant to peripheral neuroinflammation, as well as potential mediators and signaling pathways required for vascular proliferation, angiogenesis, remodeling and tight junction specialization necessary to restore peripheral nerve function following injury. This review discusses some of the progress being made in translational peripheral neurobiology and some future

scholarly journals Ultrastrutitural Pathology of an Inherited Lower Motor Neuron Disease of Pigs

1994 ◽  
Vol 6 (2) ◽  
pp. 230-237
Author(s):  
Donal O'Toole ◽  
Gerald Wells ◽  
James Ingram ◽  
William Cooley ◽  
Stephan Hawkins
Keyword(s):  
Spinal Cord ◽  
Motor Neuron ◽  
Motor Neuron Disease ◽  
Peripheral Nerves ◽  
Axonal Degeneration ◽  
Clinical Signs ◽  
Spinal Nerve ◽  
Spinal Nerve Roots ◽  
Nerve Roots ◽  
Lumbar Spinal

The ultrastructural features of a recently described inherited lower motor neuron disease were studied in 5 affected pigs. Clinical signs comprised progressive ataxia and paresis of variable severity. Affected pigs, 6, 7, 15, 15, and 19 weeks of age, and 2 unrelated healthy pigs, 9 and 15 weeks of age, were anesthetized and their tissues were fixed by whole body perfusion with mixed aldehydes. From 1 or more affected pigs, samples of cervical and lumbar spinal ventral horn, lateral and ventral spinal columns, dorsal and ventral lumbar spinal nerve roots, 2 peripheral nerves (Nn. phrenicus and fibularis communis), and 2 skeletal muscles (Mm. diaphragma and tibialis cranialis) were examined ultrastructurally. There was widespread degeneration of myelinated axons in peripheral nerves and in lateral and ventral columns of lumbar and cervical segments of spinal cord. Axonal degeneration was present in ventral spinal nerve roots and was absent in dorsal spinal nerve roots sampled at the same lumbar levels. Unmyelinated axons in peripheral nerves and spinal nerve roots were unaffected. In 4 of 5 affected pigs, there were atrophic alpha motor neurons in cervical spinal cord that contained dense, round osmiophilic perikaryal inclusions up to 4 μm in diameter and round swollen mitochondria. Axonal regeneration was present in N. phrenicus of the 19-week-old affected pig that had clinical signs of longest duration (10 weeks). There was no morphologic evidence of axonal degeneration or spinal neuronal atrophy in either control pig. The ultrastructural features of this motor neuron disease distinguish it from other reported progressive spinal neuropathies of pigs.

scholarly journals Clinical case: criteria for differential diagnosis and treatment of chronic inflammatory demyelinating polyneuropathy

2015 ◽  
pp. 48-52
Author(s):  
N.O. Kravchuk
Keyword(s):  
Peripheral Nerves ◽  
Progressive Disease ◽  
Age Of Onset ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Polyneuropathy ◽  
Age Group ◽  
Nerve Roots ◽  
Acute Inflammatory Demyelinating Polyneuropathy ◽  
Sensory Disorders ◽  
Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare neurological disease that is characterized by inflammation of nerve roots and peripheral nerves, nerve destruction of the myelin sheath, the appearance of slowly-progressive symmetric symptoms, motor and sensory disorders. CIDPsometimes considered a chronic form of acute inflammatory demyelinating polyneuropathy (АIDP) - Guillain Barré syndrome (GBS). In contrast to GBS, most patients do not mark HDPNP previous viral or infectious disease. CIDP is subacute-progressive disease that clinically takes 3-4 weeks, then usually comes plateau phase, which changes the phase of gradual regression of symptoms. CIDP can affect any age group. CIDP registered twice as often in men. The average age of onset of the disease is 50 years. CIDP prevalence is estimated at about 5-7 cases per 100 thousand people.    

scholarly journals Chronic inflammatory demyelinating polyneuropathy: update on diagnosis, immunopathogenesis and treatment

2019 ◽  
Vol 90 (9) ◽  
pp. 981-987 ◽  
Author(s):  
Helmar Christoph Lehmann ◽  
David Burke ◽  
Satoshi Kuwabara
Keyword(s):  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Demyelinating Polyneuropathy ◽  
Motor Neuropathy ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Costly Treatment ◽  
Relative Rarity ◽  
Treatment Responses ◽  
Immunomodulating Drugs ◽  
Made In

Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune-mediated neuropathy typically characterised by symmetrical involvement, and proximal as well as distal muscle weakness (typical CIDP). However, there are several ‘atypical’ subtypes, such as multifocal acquired demyelinating sensory and motor neuropathy (Lewis-Sumner syndrome) and ‘distal acquired demyelinating symmetric neuropathy’, possibly having different immunopathogenesis and treatment responses. In the absence of diagnostic and pathogenetic biomarkers, diagnosis and treatment may be difficult, but recent progress has been made in the application of neuroimaging tools demonstrating nerve hypertrophy and in identifying subgroups of patients who harbour antibodies against nodal proteins such as neurofascin and contactin-1. Despite its relative rarity, CIDP represents a significant economic burden, mostly due to costly treatment with immunoglobulin. Recent studies have demonstrated the efficacy of subcutaneous as well as intravenous immunoglobulin as maintenance therapy, and newer immunomodulating drugs can be used in refractory cases. This review provides an overview focusing on advances over the past several years.

The Role of Neuromuscular Ultrasound in the Diagnostic of the Chronic Inflammatory Demyelinating Polyneuropathy

2012 ◽  
Vol 8 (1) ◽  
pp. 62
Author(s):  
Antonios Kerasnoudis ◽  
Keyword(s):  
Peripheral Nerves ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Holistic Approach ◽  
Nerve Biopsy ◽  
Dynamic Imaging ◽  
Demyelinating Polyneuropathy ◽  
Inflammatory Disorder ◽  
Immune Mediated ◽  
Ultrasound Findings ◽  
Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common acquired immune-mediated inflammatory disorder of the peripheral nervous system. The diagnosis is based in classic cases, on the distribution pattern of the neurological semiology and pathological changes of nerve conduction studies (NCS). However, in cases with subtle clinical presentation, an extended diagnostic workup may be needed (cerebrospinal fluid examination, laboratory tests, nerve biopsy). NCS remain fundamental for the diagnosis, follow-up and measurement of response to immunetreatment in CIDP. However, new challenges arose on how best to acquire a static and dynamic imaging of the peripheral nerves, with the aim of providing a holistic approach to the nerve impairment. According to the literature, neuromuscular ultrasound is able to detect in cases of CIDP thickened or swollen roots, peripheral nerves or brachial plexus, findings that are consistent with ongoing inflammation. This review provides a timely update on the nerve ultrasound findings of CIDP and future possibilities of neuromuscular ultrasound are also discussed.

Nodal and paranodal antibody-associated neuropathies

2021 ◽  
pp. practneurol-2021-002960
Author(s):  
Janev Fehmi ◽  
Tom Vale ◽  
Stephen Keddie ◽  
Simon Rinaldi
Keyword(s):  
Monoclonal Antibody ◽  
B Cell ◽  
Peripheral Nerves ◽  
Functional Outcomes ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Current Evidence ◽  
Antibody Testing ◽  
Immune Mediated ◽  
Inflammatory Demyelinating Polyneuropathy ◽  
Immunomodulatory Therapies

Within the last decade, antibodies targeting the node and paranode of myelinated peripheral nerves have been increasingly identified in patients with acquired immune-mediated neuropathies, commonly termed ‘nodo-paranodopathies’. Crucially, these patients often present with additional clinical features not usually seen with the most common immune-mediated neuropathies, Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy, and respond poorly to conventionally used immunomodulatory therapies. Emerging evidence that these are pathologically distinct diseases has further prompted the use of more targeted treatment, such as the B cell depleting monoclonal antibody rituximab, which has been reported to significantly improve functional outcomes in this subset of patients. We provide an overview of the emerging clinical and serological phenotypes in patients with specific nodal/paranodal antibodies, the practicalities of antibody testing and current evidence supporting the use of non-standard therapies.

scholarly journals The Role of Neuromuscular Ultrasound when Diagnosing Chronic Inflammatory Demyelinating Polyneuropathy

US Neurology ◽  
2013 ◽  
Vol 09 (01) ◽  
pp. 52
Author(s):  
Antonios Kerasnoudis ◽  
Keyword(s):  
Peripheral Nerves ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Holistic Approach ◽  
Nerve Biopsy ◽  
Dynamic Imaging ◽  
Demyelinating Polyneuropathy ◽  
Inflammatory Disorder ◽  
Immune Mediated ◽  
Ultrasound Findings ◽  
Inflammatory Demyelinating Polyneuropathy

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common acquired immune-mediated inflammatory disorder of the peripheral nervous system. The diagnosis is based in classic cases, on the distribution pattern of the neurologic semiology, and pathologic changes of nerve conduction studies (NCS). However, in cases with subtle clinical presentation, an extended diagnostic workup may be needed (cerebrospinal fluid examination, laboratory tests, nerve biopsy). NCS remain fundamental for the diagnosis, follow up, and measurement of response to immune-treatment in CIDP. However, new challenges arose on how best to acquire a static and dynamic imaging of the peripheral nerves, with the aim of providing a holistic approach to the nerve impairment. According to the literature, neuromuscular ultrasound is able to detect in cases of CIDP thickened or swollen roots, peripheral nerves, or brachial plexus, findings that are consistent with ongoing inflammation. This review provides a timely update on the nerve ultrasound findings of CIDP and future possibilities of neuromuscular ultrasound are also discussed.

scholarly journals IgG1 pan-neurofascin antibodies identify a severe yet treatable neuropathy with a high mortality

2021 ◽  
Author(s):  
Janev Fehmi ◽  
Alexander J. Davies ◽  
R. Jon Walters ◽  
Tim Lavin ◽  
Ryan Keh ◽  
...  
Keyword(s):  
Peripheral Nerves ◽  
Chronic Inflammatory Demyelinating Polyneuropathy ◽  
Infectious Complications ◽  
Protein Antigens ◽  
Respiratory Involvement ◽  
Immune Mediated ◽  
Improved Outcomes ◽  
Autoimmune Neuropathy ◽  
Igg1 Subclass ◽  
Care Support

AbstractGuillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy are umbrella terms for a number of pathologically distinct diseases involving disabling, immune-mediated injury to peripheral nerves. Current treatments involve non-specific immunomodulation. Prospective identification of patients with specific disease mechanisms should increasingly inform the use of more targeted disease modifying therapies and may lead to improved outcomes. In this cohort study, we tested serum for antibodies directed against nodal/paranodal protein antigens. The clinical characteristics of antibody positive and seronegative patients were then compared. Eight patients with IgG1-subclass antibodies directed against both isoforms of the nodal/paranodal cell-adhesion molecule neurofascin were identified. All developed rapidly progressive tetraplegia. Cranial nerve deficits (100% versus 26%), autonomic dysfunction (75% versus 13%) and respiratory involvement (88% versus 14%) were more common than in seronegative patients. The response to intravenous immunoglobulin, steroids and/or plasmapheresis was poor. Four patients received the B-cell-depleting therapy rituximab. After several weeks, these patients began to show progressive functional improvements, became seronegative, and were ultimately discharged home. Four patients who did not receive rituximab did not improve and died following the development of infectious complications and/or withdrawal of intensive care support. IgG1 panneurofascin antibodies define a very severe autoimmune neuropathy. We urgently recommend trials of targeted immunotherapy for this serologically-classified patient group.

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