Lumbosacral Radiculoplexus Neuropathy Research Articles

Bruns Garland Syndrome as the first presentation of type 2 diabetes: two case reports and a practical approach to diagnosis

BackgroundDiabetes is a global health problem causing a significant burden on the healthcare systems both due to the disease itself and associated complications. Diabetic radiculoplexus neuropathies or Bruns–Garland syndrome constitutes a rare form of microvascular complications, more commonly affecting the lumbosacral plexus and, very rarely, the cervical plexus. We describe two Sri Lankan males who presented with diabetic lumbosacral radiculoplexus neuropathy and diabetic cervical radiculoplexus neuropathy as the initial manifestation of diabetes.Case descriptionCase 1: a 49-year-old Sri Lankan hotel chef presented with subacute painful weakness and wasting of the left upper arm for 3 months and weight loss. Left upper limb proximal muscles were wasted with diminished power and reflexes. A nerve conduction study showed comparative amplitude reduction. An electromyogram revealed positive sharp waves, frequent fibrillations, and high amplitude polyphasic motor unit potentials with reduced recruitment in proximal muscles of left upper limb. Case-2: a 47-year-old Sri Lankan carpenter presented with subacute progressive asymmetrical painful weakness and wasting of bilateral thighs for 5 months and weight loss. Lower limb proximal muscles were wasted with reduced power and knee jerks. The nerve conduction study was normal. The electromyogram was similar to case 1 involving both quadratus femoris muscles, which was more prominent on the left side. The work up for an underlying etiology revealed only elevated fasting blood glucose and HbA1c, suggesting a new diagnosis of diabetes associated with neurological symptoms. Patient 1 was diagnosed with diabetic cervical radiculoplexus neuropathy and patient 2 with diabetic lumbosacral radiculoplexus neuropathy. Both showed significant improvement following optimization of glycemic control together with symptomatic treatment and physiotherapy.ConclusionDiagnosis of diabetic radiculoplexus neuropathy requires a comprehensive workup to rule out other sinister pathologies. This case report has a dual importance; it describes diabetic radiculoplexus neuropathy as the very first manifestation of two previously healthy people, giving rise to a new diagnosis of diabetes and, at the same time, reporting on diabetic cervical radiculoplexus neuropathy, which is extremely rare and has never been previously reported in Sri Lanka.

Clinicopathologic Findings in Patients With Paraneoplastic Neuropathies and Antibodies Strongly Associated With Cancer.

Pathologic descriptions of peripheral nerve involvement in paraneoplastic neuropathies are sparse, mostly from autopsies focusing on CNS and dorsal root ganglia tissues. Here, we describe the clinicopathologic features of peripheral nerve biopsies in patients with paraneoplastic neurologic syndromes to expand the currently limited knowledge. Retrospective review of the Mayo Clinic electronic medical record from 1995 to 2022 for patients identified to have subacute onset neuropathy with paraneoplastic antibodies identified in our neuroimmunology laboratory having available nerve biopsies performed at the time of diagnosis. Patients with another cause of neuropathy not linked to their subacute onset were excluded. Nineteen patients met inclusion criteria: 4 with amphiphysin antibodies, 6 with antineuronal nuclear antibody (ANNA)-1 only, 3 with both ANNA-1 and collapsin response-mediator protein 5 (CRMP-5), 2 with ANNA-2, and 4 with CRMP-5 antibodies only. Fifteen biopsies had reduced the density of myelinated nerve fibers-4 with multifocality. Subperineurial edema was present in 17 biopsies. Prominent epineurial perivascular inflammation was present in 3 biopsies, all belonging to patients with a lumbosacral radiculoplexus neuropathy (LRPN) phenotype. Axonal loss, subperineurial edema, and an absence of prominent inflammation are the most common findings in nerve biopsies of patients with paraneoplastic antibodies strongly associated with cancer. The LRPN phenotype was the only subset with inflammatory collections. Paraneoplastic autoantibody testing should be considered in patients with subacute onset neuropathies, with or without interstitial inflammatory findings.

Painless nondiabetic lumbosacral radiculoplexus neuropathy with complete recovery: a case report

BackgroundLumbosacral radiculoplexus neuropathy, also known as amyotrophy, is an uncommon monophasic disorder characterized by inflammation of the lumbosacral nerve roots and plexuses. Lumbosacral radiculoplexus neuropathy is usually associated with diabetes mellitus, is typically painful at presentation, and often associated with long-term residual neurologic deficits. We report a case of painless, nondiabetic lumbosacral radiculoplexus neuropathy in a young Chinese woman, who made a full recovery after treatment with intravenous immunoglobulin, adding an atypical case to the scarce literature on lumbosacral radiculoplexus neuropathy.Case presentationA 35-year-old Chinese woman presented to our emergency department with 1-week history of painless left lower limb weakness and numbness. Examination revealed weakness confined to the left lower limb but spanning various nerves and myotomes, with abnormal sensation. Clinical localization to the lumbosacral plexus was supported by neurodiagnostic tests, and magnetic resonance imaging of the lumbosacral plexus showed that the nerve roots were also involved. After treatment with intravenous immunoglobulin for nondiabetic lumbosacral radiculoplexus neuropathy, the patient had a full recovery.ConclusionOur patient’s case highlights that lumbosacral radiculoplexus neuropathy, an already rare disorder, can occur in the absence of diabetes mellitus and pain, making it even harder to recognize. A systematic and meticulous clinical approach, supported by intelligent selection of adjunctive tests, is required for localization and diagnosis. With an accurate diagnosis, our case also demonstrates that appropriate and prompt treatment can lead to complete recovery, despite previous reports suggesting a high prevalence of long-term residual deficits after lumbosacral radiculoplexus neuropathy.

Diabetic Neuropathies.

This article provides an up-to-date review of the diagnosis and management of the most common neuropathies that occur in patients with diabetes. The prevalence of diabetes continues to grow worldwide and, as a result, the burden of diabetic neuropathies is also increasing. Most diabetic neuropathies are caused by hyperglycemic effects on small and large fiber nerves, and glycemic control in individuals with type 1 diabetes reduces neuropathy prevalence. However, among people with type 2 diabetes, additional factors, particularly metabolic syndrome components, play a role and should be addressed. Although length-dependent distal symmetric polyneuropathy is the most common form of neuropathy, autonomic syndromes, particularly cardiovascular autonomic neuropathy, are associated with increased mortality, whereas lumbosacral radiculoplexus neuropathy and treatment-induced neuropathy cause substantial morbidity. Recent evidence-based guidelines have updated the recommended treatment options to manage pain associated with distal symmetric polyneuropathy of diabetes. Identifying and appropriately diagnosing the neuropathies of diabetes is key to preventing progression. Until better disease-modifying therapies are identified, management remains focused on diabetes and metabolic risk factor control and pain management.

Femoral Neuropathy: A Comprehensive Clinical and Electrodiagnostic Review (P13-8.001)

<h3>Objective:</h3> The aim of this comprehensive study was to gain better understanding of the risk factors, etiologies, electrodiagnostic (EDX) features, and clinical course of femoral neuropathy. <h3>Background:</h3> Femoral neuropathies can cause severe and prolonged debility. Previous literature has reviewed lateral femoral cutaneous neuropathy as well as the femoral predominance in lumbosacral radiculoplexus neuropathy, but there are limited studies addressing femoral neuropathy itself. <h3>Design/Methods:</h3> We identified patients evaluated at Mayo Clinic Rochester between January 1, 1999 and July 31, 2019 with possible new femoral neuropathy ascertained via ICD 9/10 diagnosis codes presenting within 6 months of symptom onset. A retrospective review of 1084 records was performed. <h3>Results:</h3> We identified 165 cases of femoral neuropathy for inclusion. The most common femoral neuropathy etiologies included: compressive (38%) from retroperitoneal/inguinal lesions or prolonged positioning; stretch (35%) post-operatively; and toxic/inflammatory (10%). Presenting symptoms included weakness (96%), sensory loss (73%), and pain (53%). Presenting motor physical exam findings demonstrated moderate weakness (34%) or no activation (26%) of knee extension and mild (32%) or moderate (35%) weakness of hip flexion. 121 (73%) patients underwent EDX testing including 23 with femoral motor nerve conduction studies. Treatment often involved physical therapy (89%) and was otherwise etiology-specific. In patients with follow-up data available (160), 80% experienced subjective clinical improvement at last follow-up with mean time to initial improvement 3.3 months and mean time to recovery at final follow up 14.7 months. 28 (17.5%) of patients experienced no symptomatic improvement at final follow up. <h3>Conclusions:</h3> In our cohort, the most common etiologies of femoral neuropathy are compression or stretch. Although initial morbidity is high, motor recovery is common but often prolonged. <b>Disclosure:</b> Dr. Santilli has nothing to disclose. Dr. Martinez-Thompson has nothing to disclose. Dr. Speelziek has nothing to disclose. Dr. Laughlin has nothing to disclose.

Chapter 24 - Autoimmune polyneuropathies

The autoimmune peripheral neuropathies with prominent motor manifestations are a diverse collection of unusual peripheral neuropathies that are appreciated in vast clinical settings. This chapter highlights the most common immune-mediated, motor predominant neuropathies excluding acute, and chronic inflammatory demyelinating polyradiculoneuropathy (AIDP and CIDP, respectively). Other acquired demyelinating neuropathies such as distal CIDP and multifocal motor neuropathy will be covered. Additionally, the radiculoplexus neuropathies, resulting from microvasculitis-induced injury to nerve roots, plexuses, and nerves, including diabetic and nondiabetic lumbosacral radiculoplexus neuropathy and neuralgic amyotrophy (i.e., Parsonage-Turner syndrome), will be included. Finally, the motor predominant peripheral neuropathies encountered in association with rheumatological disease, particularly Sjögren's syndrome and rheumatoid arthritis, are covered. Early recognition of these distinct motor predominant autoimmune neuropathies and initiation of immunomodulatory and immunosuppressant treatment likely result in improved outcomes.

The Role of Caudal Epidural Steroid Injections in Diabetic Lumbosacral Radiculoplexus Neuropathy: A Case Report

BACKGROUND: Diabetic lumbosacral radiculoplexus neuropathy (DLSRPN) or diabetic amyotrophy, although relatively uncommon and typically self-resolving, often leads to a period of severe compromise in quality of life. CASE REPORT: We present the case of a 46-year-old woman with 6 months of bilateral lower extremity weakness and neuropathic pain, diagnosed with diabetic lumbosacral plexopathy. Her recovery course was significantly improved by receiving a caudal epidural steroid injection (ESI) to address her pain and decreased function that was not sufficiently controlled by neuropathic agents and oral opioids. CONCLUSIONS: Caudal ESI may have a beneficial role treatment of DLSRPN to facilitate participation in a functional rehabilitation program. KEY WORDS: Diabetic lumbosacral plexopathy, diabetic lumbosacral radiculoplexus neuropathy, diabetic amyotrophy, Bruns-Garland syndrome, proximal diabetic neuropathy, caudal epidural steroid injection, epidural steroid injection, chronic pain

Risk factors for lumbosacral radiculoplexus neuropathy.

Recently, our group found an association between diabetes mellitus (DM) and lumbosacral radiculoplexus neuropathy (LRPN) in Olmsted County, Minnesota; we found a higher risk (odds ratio [OR], 7.91) for developing LRPN in diabetic compared with nondiabetic patients. However, the influence of other comorbidities and anthropomorphic variables was not studied. Demographic and clinical data from 59 LRPN patients and 177 age/sex-matched controls were extracted using the Rochester LRPN epidemiological study. Differences between groups were compared by chi-square/Fisher exact test or Wilcoxon rank-sum test. Uni- and multivariate logistic regression analysis were performed. Factors predictive of LRPN on univariate analysis were DM (OR, 7.91; 95% confidence interval [CI], 4.11-15.21), dementia (OR, 6.36; 95% CI, 1.13-35.67), stroke (OR, 3.81; 95% CI, 1.32-11.01), dyslipidemia (OR, 2.844; 95% CI, 1.53-5.27), comorbid autoimmune disorders (OR, 2.72; 95% CI, 1.07-6.93), hypertension (OR, 2.25; 95% CI, 1.2-4.13), obesity (OR, 2.05; 95% CI, 1.11-3.8), body mass index (BMI) (OR, 1.1; 95% CI, 1.04-1.15), and weight (OR, 1.02; 95% CI, 1.009-1.037). On multivariate logistic regression analysis only DM (OR, 8.03; 95% CI, 3.86-16.7), comorbid autoimmune disorders (OR, 4.58; 95% CI, 1.45-14.7), stroke (OR, 4.13; 95% CI, 1.2-14.25), and BMI (OR, 1.07; 95% CI, 1.01-1.13) were risk factors for LRPN. DM is the strongest risk factor for the development of LRPN, followed by comorbid autoimmune disorders, stroke, and higher BMI. Altered metabolism and immune dysfunction seem to be the most influential factors in the development of LRPN.

Diabetic Amyotrophy (Bruns-Garland Syndrome): A Narrative Review.

Bruns-Garland syndrome (BGS) continues to be a contentious topic even 100 years after its discovery. Its lifelong incidence is 1% amongst diabetic individuals and affects middle aged-elderly individuals with type 2 diabetes mellitus (usually not poorly controlled). The data presented in this review was collated from studies published on PubMed, MEDLINE and Google Scholar in October 2021. The search words included: "Bruns-Garland syndrome", "diabetic amyotrophy" and "diabetic lumbosacral radiculoplexus neuropathy" and "proximal diabetic neuropathy". The exact pathophysiology is debatable but an ischemic pathology (non-systemic microvasculitis) is most plausible. Its cardinal symptoms include acute onset of severe proximal lower extremity pain followed by weakness and wasting, some sensory loss, weight loss and autonomic symptoms. The prognosis is good as most patients improve to near- normal strength with pain cessation within 18 months of onset.

Symposium 7: Peripheral neuropathy SY7.1. Inflammatory neuropathies and diabetes

Inflammatory neuropathies may occur with diverse presentations in subjects with diabetes. Separating these neuropathies from typical diabetic polyneuropathy is important as prognosis and treatment differ. Diabetic lumbosacral radiculoplexus neuropathy and diabetic cervical radiculoplexus neuropathy are common and need to be readily recognized as require pain relief but no immunotherapy, and as are of generally good prognosis. Prompt diagnosis is important in vasculitic neuropathy in diabetes which requires similar diagnostic and treatment strategies as in non-diabetic subjects. Chronic inflammatory demyelinating polyneuropathy (CIDP) in diabetes has been the subject of controversy and uncertainty in recent years with divergent results from different non-population based and epidemiological studies. Some suggested an increased risk of CIDP with diabetes, while others did not. The clinical features take priority when considering CIDP in diabetic patients. Electrophysiology, although helpful for diagnostic confirmation, may however in practice pose problems with regards to technique and interpretation. This may result both in under- and overdiagnosis of CIDP in subjects with underlying diabetic polyneuropathy. Recent studies from different the U.K., Serbia and Italy, of 650 subjects with “definite” or “probable” CIDP, appear to confirm a higher prevalence of diabetes in CIDP populations, which is of later onset compared to subjects without diabetes. Data from these recent studies also suggest typical CIDP may be the predominant form in diabetic subjects, with however persistent uncertainty remaining with regards to disability levels and treatment response compared to subjects without diabetes. This implies that treatment strategies for subjects with CIDP and diabetes should not differ from those in subjects without diabetes, except for precautionary measures with regards diabetic control and vascular risk.

Lumbosacral Radiculoplexus Neuropathy: Neurologic Outcomes and Survival in a Population-Based Study.

To determine whether patients in the community with lumbosacral radiculoplexus neuropathy (LRPN) have milder neuropathy than referral patients, we characterized the outcomes and survival of population-based compared to referral-based LRPN cohorts. Previously, we found that the incidence of LRPN is 4.16/100,000/y, a frequency greater than other inflammatory neuropathies. The survival of patients with LRPN is uncharacterized. Sixty-two episodes in 59 patients with LRPN were identified over 16 years (2000-2015). Clinical findings were compared to previous referral-based LRPN cohorts. Survival data were compared to those of age- and sex-matched controls. At LRPN diagnosis, median age was 70 years, median Neuropathy Impairment Score (NIS) 22 points, 92% had pain, 95% had weakness, 23% were wheelchair-bound, and median modified Rankin Scale score (mRS) was 3 (range 1-4). At last follow-up, median NIS improved to 17 points (p < 0.001) with 56% having ≥4 points improvement, 16% were wheelchair-bound, and median mRS was 2. Compared to referral-based LRPN cohorts, community patients with LRPN had less impairment, less bilateral disease (37% vs 92%), and less wheelchair usage (23% vs 49%). LRPN survival was 86% at 5 years and 55% at 10 years. Compared to age- and sex-matched controls, patients with LRPN had 76% increased risk of death (p = 0.016). In multivariate analysis, diabetes, age, stroke, chronic kidney disease, peripheral artery disease, and coronary artery disease were significant mortality risk factors but LRPN was not. LRPN is a painful, paralytic, asymmetric, monophasic, sometimes bilateral pan-plexopathy that improves over time but leaves patients with impairment. Although having LRPN increases mortality, this increase is probably due to comorbidities (diabetes) rather than LRPN itself.

Autonomic Dysfunction in Diabetic and Non-diabetic Lumbosacral Radiculoplexus Neuropathy (5293)

Autonomic Dysfunction in Diabetic and Non-diabetic Lumbosacral Radiculoplexus Neuropathy (5293)

Acute diabetic neuropathy following improved glycaemic control: a case series and review.

SummaryWe present three cases of acute diabetic neuropathy and highlight a potentially underappreciated link between tightening of glycaemic control and acute neuropathies in patients with diabetes. Case 1: A 56-year-old male with poorly controlled type 2 diabetes (T2DM) was commenced on basal-bolus insulin. He presented 6 weeks later with a diffuse painful sensory neuropathy and postural hypotension. He was diagnosed with treatment-induced neuropathy (TIN, insulin neuritis) and obtained symptomatic relief from pregabalin. Case 2: A 67-year-old male with T2DM and chronic hyperglycaemia presented with left lower limb pain, weakness and weight loss shortly after achieving target glycaemia with oral anti-hyperglycaemics. Neurological examination and neuro-electrophysiological studies suggested diabetic lumbosacral radiculo-plexus neuropathy (DLPRN, diabetic amyotrophy). Pain and weakness resolved over time. Case 3: A 58-year-old male was admitted with blurred vision diplopia and complete ptosis of the right eye, with intact pupillary reflexes, shortly after intensification of glucose-lowering treatment with an SGLT2 inhibitor as adjunct to metformin. He was diagnosed with a pupil-sparing third nerve palsy secondary to diabetic mononeuritis which improved over time. While all three acute neuropathies have been previously well described, all are rare and require a high index of clinical suspicion as they are essentially a diagnosis of exclusion. Interestingly, all three of our cases are linked by the development of acute neuropathy following a significant improvement in glycaemic control. This phenomenon is well described in TIN, but not previously highlighted in other acute neuropathies.Learning points:A link between acute tightening of glycaemic control and acute neuropathies has not been well described in literature.Clinicians caring for patients with diabetes who develop otherwise unexplained neurologic symptoms following a tightening of glycaemic control should consider the possibility of an acute diabetic neuropathy.Early recognition of these neuropathies can obviate the need for detailed and expensive investigations and allow for early institution of appropriate pain-relieving medications.

Update on classification, epidemiology, clinical phenotype and imaging of the nonsystemic vasculitic neuropathies.

Single-organ vasculitis of the peripheral nervous system (PNS) is often designated nonsystemic vasculitic neuropathy (NSVN). Several variants or subtypes have been distinguished, including migratory sensory neuropathy, postsurgical inflammatory neuropathy, diabetic radiculoplexus neuropathies, skin-nerve vasculitides, and, arguably, neuralgic amyotrophy. NSVN often presents as nondiabetic lumbosacral radiculoplexus neuropathy (LRPN). This review updates classification, clinical features, epidemiology, and imaging of these disorders. A recent study showed the annual incidence of LRPN in Olmstead County, Minnesota to be 4.16/100 000:2.79/100 000 diabetic and 1.27/100 000 nondiabetic. This study was the first to determine the incidence or prevalence of any vasculitic neuropathy. In NSVN, ultrasonography shows multifocal enlargement of proximal and distal nerves. In neuralgic amyotrophy, MRI and ultrasound reveal multifocal enlargements and focal constrictions in nerves derived from the brachial plexus. Histopathology of these chronic lesions shows inflammation and rare vasculitis. Diffusion tensor imaging of tibial nerves in NSVN revealed decreased fractional anisotropy in one study. Single-organ PNS vasculitides are the most common inflammatory neuropathies. Neuralgic amyotrophy might result from PNS vasculitis, but further study is necessary. The usefulness of focal nerve enlargements or constrictions in understanding pathological mechanisms, directing biopsies, and monitoring disease activity in NSVN should be further investigated.

Nondiabetic Lumbosacral Radiculoplexus Neuropathy with Abnormal Magnetic Resonance Neurography Findings

Nondiabetic Lumbosacral Radiculoplexus Neuropathy with Abnormal Magnetic Resonance Neurography Findings

Electrodiagnostic Case Study of Diabetic Amyotrophy: Variant of Diabetic Neuropathy

The neuropathies are very common in diabetes and one of the most important presentations is proximal neuropathy which is called as diabetic amyotrophy. Diabetic amyotrophy is a condition presented as a proximal motor neuropathy which is asymmetric and with weakening of the proximal muscles supplied by the femoral and obturator nerves and sometimes there may be distal polyneuropathy. The diabetic amyotrophy includes various elements of lumbosacral plexus and electrodiagnostic study often gives the diagnosis of neurogenic lesion affecting at the level of root called as lumbosacral radiculopathy, or involving plexus as plexopathy, or proximal neuropathy. Thus, the disorder is called lumbosacral radiculoplexus neuropathy. This article is a case study related with neurophysiological findings of diabetic amyotrophy which shows asymmetric proximal motor neuropathy.

Lumbosacral Radiculoplexus Neuropathy: A Case Series

Lumbosacral radiculoplexus neuropathy (LRPN) is an acute or subacute painful neuropathy that is probably underrecognized; in diabetic patients, this

Lumbosacral radiculoplexus neuropathy: Incidence and the association with diabetes mellitus.

To determine the previously unknown incidence of lumbosacral radiculoplexus neuropathy (LRPN) and its association with diabetes mellitus (DM). LRPN defined by clinical and electrophysiologic criteria was identified among Olmsted County, Minnesota, residents during a 16-year period (2000-2015) using the unique facilities of the Rochester Epidemiology Project. DM was ascertained using American Diabetes Association criteria. Of 1,892 medical records reviewed, 59 patients (33 men, 26 women) were identified as having LRPN. The median age was 70 years (range 24-88 years) and the median time of onset of symptoms to diagnosis was 2 months (range 1-72 months). DM was more frequent in patients with LRPN than in controls (39/59 vs 35/177, p < 0.001) but not in those with pre-DM (10/20 vs 55/142, p = 0.336). LRPN recurred in 3 patients with DM resulting in 62 LRPN episodes during the study period. The overall incidence of LRPN was 4.16/100,000/y (95% confidence interval [CI] 3.13-5.18). The incidences of LRPN among DM and non-DM groups were 2.79/100,000/y (95% CI 1.94-3.64) and 1.27/100,000/y (95% CI 0.71-1.83), respectively. The odds of LRPN among patients with DM and pre-DM was 7.91 (95% CI 4.11-15.21) and 1.006 (95% CI 1.004-1.012), respectively. LRPN incidence in Olmsted County of 4.16/100,000/y makes LRPN a common inflammatory neuropathy and is higher than that of other immune-mediated neuropathies (acute or chronic inflammatory demyelinating polyradiculoneuropathy, brachial plexus neuropathy) assessed within the same population. DM is a major risk factor for LRPN and thus justifies the continued classification of LRPN into diabetic and nondiabetic forms.

Cervical and Lumbosacral Radiculoplexus Neuropathy Following Influenza Vaccination

Acute cervical radiculoplexus neuropathy contralateral to influenza vaccination preceded proximal and distal leg weakness, sensory loss and corresponding deficits of lumbosacral radiculoplexus neuropathy. Treatment with pulse corticosteroids followed by tapering oral corticosteroids and monthly low dose intravenous immune globulin was associated with eventual improvement.

Diabetic amyotrophy: a painful radiculoplexus neuropathy

Diabetic lumbosacral radiculoplexus neuropathy is a monophasic syndrome of diffuse pain and weakness that typically affects the lower limbs asymmetrically and is often associated with significant weight loss. Recovery can...