Normal structure and pathological features in peripheral neuropathies

Höke, Ahmet; Cerri, Federica; Fisgin, Aysel; Riva, Nilo; Quattrini, Angelo

doi:10.1111/jns.12462

Cited by 2 publications

(3 citation statements)

References 1 publication

(1 reference statement)

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“…Peripheral nerves are composed of axons, cytoplasmic processes, and Schwann cells (SCs) (a type of glial cell) ( 23 ). Glial cells are important for nerve conduction velocity because they are characteristic of insulating and provide the conditions for rapid, saltatory conduction of action potentials over long distances ( 24 , 25 ). Myelinated nerve fibers have glial cells wrapped around the periphery of axons, while small axons form non-myelinating Remak bundles ( 26 ).…”

Section: Mechanismmentioning

confidence: 99%

Diabetic peripheral neuropathy: pathogenetic mechanisms and treatment

Zhu,

Hu,

Luo

et al. 2024

Front. Endocrinol.

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Diabetic peripheral neuropathy (DPN) refers to the development of peripheral nerve dysfunction in patients with diabetes when other causes are excluded. Diabetic distal symmetric polyneuropathy (DSPN) is the most representative form of DPN. As one of the most common complications of diabetes, its prevalence increases with the duration of diabetes. 10-15% of newly diagnosed T2DM patients have DSPN, and the prevalence can exceed 50% in patients with diabetes for more than 10 years. Bilateral limb pain, numbness, and paresthesia are the most common clinical manifestations in patients with DPN, and in severe cases, foot ulcers can occur, even leading to amputation. The etiology and pathogenesis of diabetic neuropathy are not yet completely clarified, but hyperglycemia, disorders of lipid metabolism, and abnormalities in insulin signaling pathways are currently considered to be the initiating factors for a range of pathophysiological changes in DPN. In the presence of abnormal metabolic factors, the normal structure and function of the entire peripheral nervous system are disrupted, including myelinated and unmyelinated nerve axons, perikaryon, neurovascular, and glial cells. In addition, abnormalities in the insulin signaling pathway will inhibit neural axon repair and promote apoptosis of damaged cells. Here, we will discuss recent advances in the study of DPN mechanisms, including oxidative stress pathways, mechanisms of microvascular damage, mechanisms of damage to insulin receptor signaling pathways, and other potential mechanisms associated with neuroinflammation, mitochondrial dysfunction, and cellular oxidative damage. Identifying the contributions from each pathway to neuropathy and the associations between them may help us to further explore more targeted screening and treatment interventions.

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Section: Mechanismmentioning

confidence: 99%

Diabetic peripheral neuropathy: pathogenetic mechanisms and treatment

Zhu,

Hu,

Luo

et al. 2024

Front. Endocrinol.

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“…8 Three patients reported in abstract form alone and a fourth briefly described in an image atlas were excluded due to inadequate information. [92][93][94] Our searches yielded 19 cases (14 from database searches, 5 from hand searches) matching this clinicopathological definition. These were supplemented by an unreported case personally observed by M.C.…”

Section: Selection Criteriamentioning

confidence: 99%

“…Only patient 13 in the series reported by Sorenson and colleagues was included, as the other 12 had “epineurial vasculitis” or no significant perineurial inflammation 8 . Three patients reported in abstract form alone and a fourth briefly described in an image atlas were excluded due to inadequate information 92–94 …”

Section: Literature Review Of Primary and Secondary Perineuritismentioning

confidence: 99%

Primary perineuritis, a rare but treatable neuropathy: Review of perineurial anatomy, clinicopathological features, and differential diagnosis

Collins,

Hadden,

Shahnoor

2023

Muscle and Nerve

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The perineurium surrounds each fascicle in peripheral nerves, forming part of the blood–nerve barrier. We describe its normal anatomy and function. “Perineuritis” refers to both a nonspecific histopathological finding and more specific clinicopathological entity, primary perineuritis (PP). Patients with PP are often assumed to have nonsystemic vasculitic neuropathy until nerve biopsy is performed. We systematically reviewed the literature on PP and developed a differential diagnosis for histopathologically defined perineuritis. We searched PubMed, Embase, Scopus, and Web of Science for “perineuritis.” We identified 20 cases (11 M/9F) of PP: progressive, unexplained neuropathy with biopsy showing perineuritis without vasculitis or other known predisposing condition. Patients ranged in age from 18 to 75 (mean 53.7) y and had symptoms 2–24 (median 4.5) mo before diagnosis. Neuropathy was usually sensory‐motor (15/20), painful (18/19), multifocal (16/20), and distal‐predominant (16/17) with legs more affected than arms. Truncal numbness occurred in 6/17; 10/18 had elevated cerebrospinal fluid (CSF) protein. Electromyography (EMG) and nerve conduction studies (NCS) demonstrated primarily axonal changes. Nerve biopsies showed T‐cell‐predominant inflammation, widening, and fibrosis of perineurium; infiltrates in epineurium in 10/20 and endoneurium in 7/20; and non‐uniform axonal degeneration. Six had epithelioid cells. 19/20 received corticosteroids, 8 with additional immunomodulators; 18/19 improved. Two patients did not respond to intravenous immunoglobulin (IVIg). At final follow‐up, 13/16 patients had mild and 2/16 moderate disability; 1/16 died. Secondary causes of perineuritis include leprosy, vasculitis, neurosarcoidosis, neuroborreliosis, neurolymphomatosis, toxic oil syndrome, eosinophilia‐myalgia syndrome, and rarer conditions. PP appears to be an immune‐mediated, corticosteroid‐responsive disorder. It mimics nonsystemic vasculitic neuropathy. Cases with epithelioid cells might represent peripheral nervous system (PNS)‐restricted forms of sarcoidosis.

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Normal structure and pathological features in peripheral neuropathies

Cited by 2 publications

References 1 publication

Diabetic peripheral neuropathy: pathogenetic mechanisms and treatment

Diabetic peripheral neuropathy: pathogenetic mechanisms and treatment

Primary perineuritis, a rare but treatable neuropathy: Review of perineurial anatomy, clinicopathological features, and differential diagnosis

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