Pathological Findings in Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Single-Center Experience

Luigetti, Marco; Romano, Ângela; Paolantonio, Andrea Di; Bisogni, Giulia; Rossi, Salvatore; Conte, Amelia; Madia, Francesca; Sabatelli, Mario

doi:10.3390/brainsci10060383

Cited by 10 publications

(11 citation statements)

References 34 publications

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“…Demyelination and mononuclear cell infiltration are the main findings. Secondary axonal loss may be seen, usually accompanied by clusters of regenerating fibers 116 . Onion bulbs can be seen in chronic cases.…”

Section: Methodsmentioning

confidence: 99%

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Nerve biopsy: Current indications and decision tools

et al. 2021

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After initial investigation of patients presenting with symptoms suggestive of neuropathy, a clinical decision is made for a minority of patients to undergo further assessment with nerve biopsy. Many nerve biopsies do not demonstrate a definitive pathological diagnosis and there is considerable cost and morbidity associated with the procedure. This highlights the need for appropriate selection of patients, nerves and neuropathology techniques. Additionally, concomitant muscle and skin biopsies may improve the diagnostic yield in some cases. Several advances have been made in diagnostics in recent years, particularly in genomics. The indications for nerve biopsy have consequently changed over time. This review explores the current indications for nerve biopsies and some of the issues surrounding its use. Also included are comments on alternative diagnostic modalities that may help to supplant or reduce the use of nerve biopsy as a diagnostic test. These primarily include extraneural biopsy and neuroimaging techniques such as magnetic resonance neurography and nerve ultrasound. Finally, we propose an algorithm to assist in deciding when to perform nerve biopsies.

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Section: Methodsmentioning

confidence: 99%

“…Secondary axonal loss may be seen, usually accompanied by clusters of regenerating fibers. 116 Onion bulbs can be seen in chronic cases. They are usually randomly distributed, lying adjacent to normally myelinated axons without onion bulbs.…”

Section: Methodsmentioning

confidence: 99%

Nerve biopsy: Current indications and decision tools

et al. 2021

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“…As a control group, we included ALS patients, in which we did not find any sensory abnormalities, and CIDP patients, in which we found sensory abnormalities involving large fibres, small fibres or both, according to a possible focal distribution of the inflammatory process [ 34 ].…”

Section: Discussionmentioning

confidence: 99%

Small Fibre Involvement in Multifocal Motor Neuropathy Explored with Sudoscan: A Single-Centre Experience

et al. 2020

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Objective: Multifocal motor neuropathy (MMN) is a rare inflammatory neuropathy, clinically characterized by exclusive motor involvement. We wished to evaluate the possible presence of sensory dysfunction, including the evaluation of small fibres, after a long-term disease course. Patients and methods: seven MMN patients, regularly followed in our Neurology Department, underwent clinical evaluation, neurophysiological examination by nerve conduction studies (NCSs), and Sudoscan. We compared neurophysiological data with a group of patients with other disorders of the peripheral nervous system. Results: NCSs showed a reduction of sensory nerve action potential amplitude in 2/7 MMN patients. Sudoscan showed borderline electrochemical skin conductance (ESC) values in 3/7 MMN patients (two of them with abnormal sensory NCSs). Conclusions: Our results confirm that sensory involvement may be found in some MMN after a long-term disease course, and it could also involve the small fibres.

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“…Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) is an immune-mediated neuropathy with a heterogeneous clinical presentation consisting of a roughly symmetric involvement of peripheral nerves, which affects both motor and sensory components [ 1 , 2 ]. The classic clinical presentation of CIDP consists of a mainly motor and symmetric neuropathy, affecting both peripheral nerves and nerve roots, manifesting in proximal and distal muscle weakness with either a relapsing–remitting or a progressive course [ 3 , 4 ].…”

Section: Introductionmentioning

confidence: 99%

Relevance of Nerve Biopsy in the Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy—A Systematic Review

et al. 2022

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Chronic Inflammatory Demyelinating Polyneuropathy is an immune-mediated pathology of the peripheral nerves and nerve roots that leads to weakness and sensory symptoms. Given its clinical heterogeneity, often times diagnosis is challenging. Even though nerve conduction studies and clinical features are the main criteria used for diagnosis, supplementary investigations, such as nerve biopsies, cerebral spinal fluid examination and magnetic resonance studies, may be used in order to confirm the diagnosis. Given the fact that the hallmark in CIDP physiopathology is the demyelination process, nerve biopsies are used to demonstrate and assess the magnitude of the phenomenon. The question and the main interest of this review is whether histopathological findings are relevant for the diagnosis and can be useful in disease assessment.

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Pathological Findings in Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Single-Center Experience

Cited by 10 publications

References 34 publications

Nerve biopsy: Current indications and decision tools

Nerve biopsy: Current indications and decision tools

Small Fibre Involvement in Multifocal Motor Neuropathy Explored with Sudoscan: A Single-Centre Experience

Relevance of Nerve Biopsy in the Diagnosis of Chronic Inflammatory Demyelinating Polyneuropathy—A Systematic Review

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