Nerve biopsy findings in different patterns of proximal diabetic neuropathy

Saïd, Gérard; Goulon‐Goeau, Catherine; Lacroix, Catherine; Moulonguet, A

doi:10.1002/ana.410350509

Cited by 247 publications

(150 citation statements)

References 24 publications

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“…Unlike NSVN with proximal involvement, the diabetic radiculoplexus neuropathies are always self-limited and develop in a stereotypical manner, commencing in one region and spreading to another, even as the first site is resolving 11,[49][50][51][52][53][54][55][56][57][58] . The mechanisms responsible for this distinctive behaviour of diabetesrelated vasculitis are poorly understood.…”

Section: Variantsmentioning

confidence: 99%

“…Diabetic radiculoplexus neuropathy has a propensity to affect lumbosacral nerve roots and peripheral nerve trunks and is then labelled as diabetic lumbosacral radiculoplexus neuropathy (DLSRPN) 11,[49][50][51][52][53][54][55][56][57][58] . DLSRPN affects 1% of patients with diabetes mellitus 59 , typically type 2.…”

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

“…ESR is elevated in 20% of patients, and CSF protein levels are high in 85%. Symptoms progress for 1 week to 3 years (median four months), then recede over many months (median 15 months) 11,[49][50][51][52][53][54][55][56][57][58] . Residual weakness persists, especially distally, in more than 50% of patients, and 10-15% of patients relapse.…”

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

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The nonsystemic vasculitic neuropathies

2017

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The vasculitides are a clinically diverse group of diseases with the histopathological signature of blood vessel-centred inflammation that results in vascular damage and ischaemic injury to the affected tissues 1 . Vasculitis can be caused by drugs, infections and cancers, but diagnostic workup reveals no trigger in most patients. Autoimmune mechanisms are active in all vasculitides, except those caused by direct infection of vessel walls. Most vasculitides are systemic and involve multiple organs and tissues. Those affecting small-to medium-sized vessels often manifest with a vasculitic neuropathy. For example, neuropathies occur in 60-70% of patients with polyarteritis nodosa, eosinophilic granulomatosis with polyangiitis and cryoglobulinaemic vasculitis, and 40-50% of those with microscopic polyangiitis and rheumatoid vasculitis 2 .Vasculitic neuropathy can also occur without systemic vasculitis. This single-organ vasculitis of the PNS has commonly been referred to as nonsystemic vasculitic neuropathy (NSVN), but other forms of clinically isolated PNS vasculitis are now recognized and can be considered variants of NSVN 3 . Although NSVN is classically an acute, relapsing, multifocal neuropathy, it can present as a slowly progressive neuropathy without distinct asymmetries. As such, it should be considered as a possible cause of any progressive axonal neur opathy. NSVN can be diagnosed only with a nerve biopsy, with or without a concomitant muscle or skin biopsy; as nerve biopsies are seldom performed, the condition is under-recognized. The disorder must be distinguished from many other causes of multifocal neuropathy, the definition of which is itself not standardized and requires clarification.A Peripheral Nerve Society guideline group reviewed the literature on vasculitic neuropathy and published consensus recommendations on the classification, diagnosis and immunosuppressive treatment of NSVN in 2010 (REF. 4) Abstract | Nonsystemic vasculitic neuropathy (NSVN) is an under-recognized single-organ vasculitis of peripheral nerves that can only be diagnosed with a nerve biopsy. A Peripheral Nerve Society guideline group published consensus recommendations on the classification, diagnosis and treatment of NSVN in 2010, and new diagnostic criteria for vasculitic neuropathy were developed by the Brighton Collaboration in 2015. In this Review, we provide an update on the classification, diagnosis and treatment of NSVN. NSVN subtypes include Wartenberg migratory sensory neuropathy and postsurgical inflammatory neuropathy. Variants include diabetic radiculoplexus neuropathy and -arguably -neuralgic amyotrophy. NSVN with proximal involvement is sometimes termed nondiabetic lumbosacral radiculoplexus neuropathy. Cutaneous polyarteritis nodosa and other skin-nerve vasculitides overlap with NSVN clinically. Three patterns of involvement in NSVN have been identified: multifocal neuropathy, distal symmetric polyneuropathy, and overlapping multifocal neuropathy (asymmetric polyneuropathy). These patterns lack standard defi...

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Section: Variantsmentioning

confidence: 99%

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

Section: Diabetic Lumbosacral Radiculoplexus Neuropathymentioning

confidence: 99%

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The nonsystemic vasculitic neuropathies

2017

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“…The finding of pathogenic anti-L-periaxin antibody in sera of two of our diabetic neuropathy patients, while unexpected, does suggest a broader significance of these findings to other neurological disease groups. Clinical observations of endoneurial mononuclear infiltrates in peripheral nerves of diabetic neuropathic patients (Said et al 1994) and clinical improvement of diabetic neuropathy with immune-modulating therapies (Krendel et al 1995) have raised the possibility of an immune component to this disorder. As additional evidence from in vitro studies emerges (Milicevic et al 1997;Shigeta et al 1997;Rosoklija et al 2000), an antibody-mediated mechanism of nerve injury in diabetes mellitus merits further evaluation.…”

Section: Discussionmentioning

confidence: 99%

“…In addition, a role of autoimmunity in peripheral nerve disorders associated with metabolic disturbances, such as diabetic neuropathy, has been entertained (Said et al 1994;Krendel et al 1995). However, the antigenic specificities Abbreviations used: CMAP, compound muscle action potential; D/P, distal (ankle) to proximal (sciatic notch) ratio; IgG, immunoglobulin gamma; IgM, immunoglobulin mu; L-periaxin, large isoform of periaxin; MAG, myelin-associated glycoprotein; MGUS, monoclonal gammopathy of undetermined significance; PAGE, polyacrylamide gel electrophoresis.…”

mentioning

confidence: 99%

Antibodies to L‐periaxin in sera of patients with peripheral neuropathy produce experimental sensory nerve conduction deficits

Lawlor¹,

Richards

Vries

et al. 2002

Journal of Neurochemistry

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L-Periaxin is a PDZ-domain protein localized to the plasma membrane of myelinating Schwann cells and plays a key role in the stabilization of mature myelin in peripheral nerves. Mutations in L-periaxin have recently been described in some patients with demyelinating peripheral neuropathy, suggesting that disruption of L-periaxin function may result in nerve injury. In this study, we report the presence of autoantibodies to L-periaxin in sera from two of 12 patients with diabetes mellitus (type 2)-associated neuropathy and three of 17 patients with IgG monoclonal gammopathy of undetermined significance (MGUS) neuropathy, an autoimmune peripheral nerve disorder. By comparison, anti-L-periaxin antibodies were not present in sera from nine patients with IgM MGUS neuropathy or in sera from 10 healthy control subjects. The effect of anti-L-periaxin serum antibody on peripheral nerve function was tested in vivo by intraneural injection. Sera containing anti-L-periaxin antibody, but not sera from age-matched control subjects, injected into the endoneurium of rat sciatic nerve significantly (p < 0.005, n ¼ 3) attenuated sensory-evoked compound muscle action potential (CMAP) amplitudes in the absence of temporal dispersion. In contrast, motor-evoked CMAP amplitudes and latencies were not affected by intraneural injection of sera containing anti-L-periaxin antibody. Light and electron microscopy of anti-L-periaxin serum-injected nerves showed morphologic evidence of demyelination and axon enlargement. Depleting sera of anti-L-periaxin antibodies neutralized the serum-mediated effects on nerve function and nerve morphology. Together, these data support anti-L-periaxin antibody as the pathologic agent in these serum samples. We suggest that anti-L-periaxin antibodies, when present in sera of patients with IgG MGUS-or diabetesassociated peripheral neuropathy, may elicit sensory nerve conduction deficits. Keywords: antibody, diabetes, MGUS, neuropathy, periaxin. Peripheral neuropathies are a heterogeneous group of nerve disorders often characterized by a mixture of sensory and motor disturbances (Adams et al. 1997). Although the etiology of the peripheral neuropathy is often obscure, some patients exhibit strong familial histories consistent with an autosomal dominance, autosomal recessive, or X-linked pattern of inheritance. In other patients, the peripheral neuropathy is acquired such as in monoclonal gammopathy of undetermined significance (MGUS) neuropathy (Kyle 1992) or is secondary to a metabolic disturbance such as diabetes mellitus.Serum autoantibodies have been implicated as potential pathogenic agents in several autoimmune-mediated peripheral nerve disorders (Ho et al. 1998;Quarles and Weiss 1999). In addition, a role of autoimmunity in peripheral nerve disorders associated with metabolic disturbances, such as diabetic neuropathy, has been entertained (Said et al. 1994;Krendel et al. 1995). However, the antigenic specificities Abbreviations used: CMAP, compound muscle action potential; D/P, distal (ankle) to...

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Successful Treatment of Neuropathies in Patients With Diabetes Mellitus

Krendel¹,

Costigan²,

Hopkins³

1995

Archives of Neurology

221

100

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Neuropathies responsive to anti-inflammatory and/or anti-immune therapy in patients with diabetes mellitus include (1) multifocal axonal neuropathy caused by inflammatory vasculopathy, predominantly in patients with non-insulin-dependent diabetes mellitus, indistinguishable from diabetic proximal neuropathy or mononeuropathy multiplex, and (2) demyelinating neuropathy indistinguishable from chronic inflammatory demyelinating polyneuropathy, predominantly in patients with insulin-dependent diabetes mellitus.

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Nerve biopsy findings in different patterns of proximal diabetic neuropathy

Cited by 247 publications

References 24 publications

The nonsystemic vasculitic neuropathies

The nonsystemic vasculitic neuropathies

Antibodies to L‐periaxin in sera of patients with peripheral neuropathy produce experimental sensory nerve conduction deficits

Successful Treatment of Neuropathies in Patients With Diabetes Mellitus

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