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Peripheral neuropathy
Published in Ibrahim Natalwala, Ammar Natalwala, E Glucksman, MCQs in Neurology and Neurosurgery for Medical Students, 2022
Ibrahim Natalwala, Ammar Natalwala, E Glucksman
viii – GBS. The history suggests a distal peripheral neuropathy that ascends rapidly in keeping with GBS. GBS is an autoimmune disease caused by immune ‘mimicry’ where autoantibodies are produced against an antigen from a pathogen but these same autoantibodies attack the myelin on peripheral nerves. It affects motor, sensory and autonomic nerves. The paralysis ascends and can cause respiratory depression and subsequent death. Treatment is using plasma exchange or intravenous immunoglobulin, with the later usually the preferred treatment of choice. Triggers include Campylobacter jejuni and Epstein-Barr virus, although the flu vaccine has been linked to GBS. If progression of symptoms lasts longer than 4 weeks, the patient is then diagnosed as having chronic inflammatory demyelinating polyneuropathy.3
Demyelinating Neuropathy
Published in Maher Kurdi, Neuromuscular Pathology Made Easy, 2021
The characteristic features of demyelination in nerve biopsy are categorized based on the process activity. In early stage of active demyelination. Schwann cell vacuolation, macrophage accumulation, and myeloid bodies are seen. Small myelin ovoids are scattered within the section, which indicate ongoing degeneration. These ovoids cannot be distinguished from the ones seen in the acute axonal degeneration process in which myelination is damaged. Partial or complete loss of myelination surrounding the axons with myelin debris-filled macrophages is the hallmark feature of demyelination (Figure 27.1a) (Table 27.2). In some cases, perivascular or endoneurial inflammation is seen. The inflammation mainly consists of macrophages and scattered lymphocytes. The most common cause of demyelinating neuropathy associated with inflammation is acute and chronic inflammatory demyelinating polyneuropathy (ACDP/CIDP).
Epstein—Barr Virus and CNS Infections
Published in Sunit K. Singh, Daniel Růžek, Neuroviral Infections, 2013
EBV-associated lymphoma in immunocompetent patients is very rare. One case reports a patient who presented with rapidly progressive weakness consistent with chronic inflammatory demyelinating polyneuropathy (Kasamon et al. 2002). This indicates that, albeit very rarely, EBV-associated lymphoproliferative disease can develop in the absence of overt immunodeficiency and may trigger a demyelinating polyneuropathy.
Therapeutic issues in Guillain–Barré syndrome
Published in Expert Review of Neurotherapeutics, 2023
The effectiveness of corticosteroids in chronic inflammatory demyelinating polyneuropathy (CIDP) has long been reported [65]. Although no high-powered randomized control trial is available [66], clinicians’ experience [67,68] and non-randomized studies suggest that corticosteroids are beneficial in CIDP. Despite promising results of high-dose methylprednisolone in a rat model of acute neuritis [69], corticosteroids did not show the anticipated efficacy in human GBS. A Cochrane review of six trials including 587 participants showed no significant effect [70]. However, it is possible that the benefits of suppressing the inflammatory response in GBS may be offset by unintended effects on the repair process, such as the loss of skeletal muscle electrical excitability [71]. If this hypothesis is correct, a very early course of corticosteroids may be beneficial in a subset of patients with conduction block but not denervation [70]. Thus, it cannot be completely excluded that a very early corticosteroid treatment could be beneficial before the destructive impact of endoneurial edema in the proximal nerve trunks. Indeed, some authors tend to demonstrate some favorable outcomes of high doses glucocorticoids in AIDP patients [72].
Value of nerve biopsy in the management of peripheral neuropathies
Published in Expert Review of Neurotherapeutics, 2018
Stéphane Mathis, Laurent Magy, Gwendal Le Masson, Laurence Richard, Antoine Soulages, Guilhem Solé, Fanny Duval, Karima Ghorab, Jean-Michel Vallat, Mathilde Duchesne
Peripheral neuropathy is a common disorder in the general population.Nerve biopsy is rarely mandatory, but it is crucial in the management of some peripheral neuropathies.Vasculitis, amyloidosis, granulomatosis, atypical chronic inflammatory demyelinating polyneuropathy and any neuropathy with rapid worsening (without a determined cause) may be an indication for nerve biopsy.Various pathological hallmarks are observed in many types of acquired and inherited peripheral neuropathy.
Corneal confocal microscopy: ready for prime time
Published in Clinical and Experimental Optometry, 2020
Ioannis N Petropoulos, Georgios Ponirakis, Adnan Khan, Hoda Gad, Hamad Almuhannadi, Michael Brines, Anthony Cerami, Rayaz A Malik
Bechet's disease is a chronic relapsing vascular inflammatory disease with a number of neurological manifestations, including peripheral neuropathy. We have recently shown corneal nerve alterations and a significant increase in dendritic cell density,2011 which have been associated with nerve regeneration.2017 Chronic inflammatory demyelinating polyneuropathy has been labelled as a condition which primarily affects large myelinated fibres; however, recent studies have revealed significant small sensory nerve pathology.2002 Schneider et al.2014 showed a significant reduction in sub‐basal nerve fibre density and length with an increase in dendritic cell density in patients with chronic inflammatory demyelinating polyneuropathy.