Diabetic 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.

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

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.

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.

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...

A Rare Cause of Proximal Thigh Discomfort and Weakness with Type 2 Diabetes Mellitus

Diabetic amyotrophy also known as Bruns-Garland syndrome or Diabetic Lumbosacral Radiculoplexus neuropathy is diabetic neuropathy subtype affecting the lumbrosacral nerve roots and peripheral nerves1. There is an ongoing debate on the pathophysiology behind this condition whether is it ischemic, metabolic (hyperglycemia) or inflammatory1. We think that the likely cause responsible is due to an ischemic injury from a nonsystemic microvasculitis. There is a series of studies involving diabetic amyotrophy conducted with 33 patients, nerve biopsy specimens showed features of multifocal fiber loss, perineurial thickening, neovascularization and abortive regeneration of nerve fibers forming microfasciculi2. These findings are suggestive of an ischemic process due to microvascuitis2

Atypical presentation of a patient clinically diagnosed with diabetic lumbosacral radiculoplexus neuropathy

Background: Diabetic lumbosacral radiculoplexus neuropathy (DLSRPN) occurs in approximately 1% of diabetic patients, more commonly with mild type 2 diabetes mellitus. This syndrome is characterized by disabling pain, weakness and atrophy affecting usually the proximal thigh muscles asymmetrically.

A limited form of diabetic lumbosacral radiculoplexus neuropathy; Predominant s1 root involvement or " diabetic S1 radiculitis"

Background: Diabetic lumbosacral radiculoplexus neuropathy (LSRPN) is a distinct clinical condition characterized by severe pain with nocturnal worsening and weakness/wasting commonly affecting the proximal thigh muscles (L3/L4) asymmetrically in the context of poor glycaemic control.

Diabetic lumbosacral radiculoplexus neuropathy after bariatric surgery

Diabetic lumbosacral radiculoplexus neuropathy after bariatric surgery

Vasculitic Neuropathies.

From pathological standpoint, we divide vasculitic neuropathies in two categories: nerve large arteriole vasculitides and nerve microvasculitis. It is also important to determine whether a large arteriole vasculitis has an infectious etiology as it entails different treatment approach. Treatment of non-infectious large arteriole vasculitides consists initially of induction therapy with corticosteroids. Adding an immunosuppressant, mainly cyclophosphamide, is often needed. Treatment of infectious large arteriole vasculitides needs a multidisciplinary approach to target both the underlying infection and the vasculitis. Corticosteroids are the first-line therapy for classic non-systemic vasculitic neuropathy. Stable or improving patients without biopsy evidence of active vasculitis can be either observed or treated. Currently, adding an immunosuppressant is only indicated for patients who continue to progress on corticosteroids alone or patients with a rapidly progressive course. The treatment of the radiculoplexus neuropathies such as diabetic lumbosacral radiculoplexus neuropathy, lumbosacral radiculoplexus neuropathy (in non-diabetic patients), and diabetic cervical radiculoplexus neuropathy, as well as painless diabetic motor neuropathy, is not well established yet. We treat patients, if they present early on in the disease course or if they have severe disabling symptoms, with IV methylprednisolone 1g once a week for 12weeks.

Immune activation and autoimmunity in chronic pain conditions and response to immunoglobulin G.

Immune activation and autoimmunity in chronic pain conditions and response to immunoglobulin G.

Managing inflammatory diabetic neuropathies

SUMMARY Most diabetic neuropathies are felt to be related to metabolic and microvascular complications from prolonged periods of hyperglycemia, the classical example being diabetic polyneuropathy. Diabetic polyneuropathy is a symmetric slowly progressive distal predominant, sensory greater than motor neuropathy, commonly seen in neurological practice. However, there are subtypes of diabetic neuropathy, inflammatory in origin, which are important to identify, as the fundamental approach to treatment is different. The main categories of inflammatory diabetic neuropathy, which we will discuss in detail, including clinical presentation, evidence for an inflammatory pathophysiology, and the treatment strategies, are the different forms of radiculoplexus neuropathies (diabetic lumbosacral radiculoplexus neuropathy, diabetic cervical radiculoplexus neuropathy, diabetic thoracic radiculoneuropathy and painless motor and lower limb predominant neuropathy) and diabetic chronic inflammatory demyelinating polyradicu...

Diabetic lumbosacral radiculoplexus neuropathy in an older patient

A 79-year-old man with type-2 diabetes, treated with metformin, presented with significant weight loss and marked pain and weakness in the left thigh persisting for 3 months, thus, requiring him to use a cane. The pain arose from his lower back and radiated into the anterior thigh. The left lower limb showed patellar areflexia and weakness in the psoas, quadriceps, and buttocks muscles. Significant left quadricipital amyotrophy was identified (Fig. 1A). Sensitivity was preserved. Neurological examination of the other limbs was normal. Cranial nerves were intact. HbA1c was 10.1%; other laboratory findings were unremarkable. Antibodies to extractible nuclear antigens, rheumatoid factor, and Lyme serology were negative. Magnetic resonance imaging of the lumbosacral plexus was normal, showing no compressive mass, and that of the thoracolumbar spine was reassuring. Computed tomography of the thigh confirmed reduced muscle trophicity (Fig. 1B). Electromyography showed peripheral denervation from L1–L4, with reduced recruitment in the left thigh myotomas without abnormal rest activity. Cerebrospinal fluid showed an increased protein rate of 0.86 g/L (nL < 0.45), without nucleated cells. The poorly-controlled diabetes, electromyographic results, absence of nerve root compressive signs, and lumbar puncture results led to a diagnostic assumption of diabetic lumbosacral radiculoplexus neuropathy (DLSRPN). Oral methylprednisolone 64 mg/day, with reduced-dose regimen, was started, and insulin therapy replaced the metformin. Improvement was noted within days, with marked reduction in disability. Follow-up confirmed this finding and the amyotrophy

Long‐Term Response of Neuropathic Pain to Intravenous Immunoglobulin in Relapsing Diabetic Lumbosacral Radiculoplexus Neuropathy. A Case Report

Diabetic lumbosacral radiculoplexus neuropathy (DLRPN) is a rare painful peripheral neuropathic complication of diabetes mellitus. The clinical features of DLRPN include severe neuropathic pain, weakness, atrophy, and sensory loss in the lower limbs with asymmetrical distribution. Nerve ischemia due to inflammation and microvasculitis has been suggested as the pathophysiological mechanism for DLRPN. Analgesics and drugs for neuropathic pain often cannot achieve adequate pain control in DLRPN. Some reports suggest that intravenous immunoglobulin (IVIg) may reduce pain in DLRPN, but the mechanisms of this effect are unclear. We report a patient with relapsing DLRPN who was followed up for 8 years and whose pain improved after IVIg on nine occasions. We measured serum cytokines before and after IVIg; serum tumor necrosis factor α was increased when the patient reported pain and normalized after IVIg in parallel with pain improvement. Our data extend the notion that some types of pain, including peripheral neuropathic pain, may respond to IVIg and give some clue on the mechanism of this therapeutic effect. They are also consistent with the suggested role of the immune system in the pathophysiology of neuropathic pain and offer support to the hypothesis that cytokines may contribute to the pathogenesis of neuropathic pain.

Diabetic cervical radiculoplexus neuropathy: a distinct syndrome expanding the spectrum of diabetic radiculoplexus neuropathies

Diabetic lumbosacral radiculoplexus neuropathy is a subacute painful, asymmetrical lower limb neuropathy due to ischaemic injury and microvasculitis. The occurrence of a cervical diabetic radiculoplexus neuropathy has been postulated. Our objective was to characterize the clinical features and pathological alterations of diabetic cervical radiculoplexus neuropathy, to see if they are similar to diabetic lumbosacral radiculoplexus neuropathy and due to ischaemic injury and microvasculitis. We identified patients with diabetic cervical radiculoplexus neuropathy by review of the Mayo Clinic database from 1996 to 2008. We systematically reviewed the clinical features, laboratory studies, neurophysiological findings, neuroimaging and pathological features and compared the findings with a previously published diabetic lumbosacral radiculoplexus neuropathy cohort. Eighty-five patients (56 males, 67 with Type 2 diabetes mellitus) were identified. The median age was 62 years (range 32-83). The main presenting symptom was pain (53/85). At evaluation, weakness was the most common symptom (84/85), followed by pain (69/85) and numbness (56/85). Neuropathic deficits were moderate (median motor neuropathy impairment score 10.0 points) and improved at follow-up. Upper, middle and lower brachial plexus segments were involved equally and pan-plexopathy was not unusual (25/85). Over half of patients (44/85) had at least one additional body region affected (30 contralateral cervical, 20 lumbosacral and 16 thoracic) as is found in diabetic lumbosacral radiculoplexus neuropathy. Recurrent disease occurred in 18/85. Neurophysiology showed axonal neuropathy (80/80) with paraspinal denervation (21/65), and abnormal autonomic (23/24) and sensory testing (10/13). Cerebrospinal fluid protein was elevated (median 70 mg/dl). Magnetic resonance imaging showed brachial plexus abnormality in all (38/38). Nerve biopsies (11 upper and 11 lower limbs) showed ischaemic injury (axonal degeneration, multifocal fibre loss 15/22, focal perineurial thickening 16/22, injury neuroma 5/22) and increased inflammation (epineural perivascular inflammation 22/22, haemosiderin deposition 6/22, vessel wall inflammation 14/22 and microvasculitis 5/22). We therefore conclude that (i) diabetic cervical radiculoplexus neuropathy is a predominantly monophasic, upper limb diabetic neuropathy with pain followed by weakness and involves motor, sensory and autonomic fibres; (ii) the neuropathy begins focally and often evolves into a multifocal or bilateral condition; (iii) the pathology of diabetic cervical radiculoplexus neuropathy demonstrates ischaemic injury often from microvasculitis; and (iv) diabetic cervical radiculoplexus neuropathy shares many of the clinical and pathological features of diabetic lumbosacral radiculoplexus neuropathy, providing evidence that these conditions are best categorized together within the spectrum of diabetic radiculoplexus neuropathies.

Elevated cerebrospinal fluid protein in diabetic lumbosacral radiculoplexus neuropathy

An 86-year-old ex-sailor with Type 2 diabetes was admitted in September 2010, with a 3-year history of weakness and pain in his legs, ataxia, recurrent falls, significant difficulties in walking and getting up, intermittent paraesthesia in his feet with strong jerks in the lower limbs and unintentional weight loss of 4 stones over the last few years. He was an ex-smoker and had previous history of chronic pancreatitis, Stage 3 chronic kidney disease, right shoulder pain with impingement syndrome, bilateral Dupuytren's contracture, right carpal tunnel release operation, bilateral cataracts surgery and early dry macular degeneration. He underwent left hemicolectomy for Dukes’ B adenocarcinoma of the splenic flexure with no distal metastases. His medication included Metformin and Creon. Three years earlier, he was investigated in the neurology clinic, with similar complaints of falls and ataxia. He had stopped drinking excessive amounts of alcohol by 2006. Previous investigations revealed normal or negative results including haematinics, erythrocyte sedimentation rate, autoantibody screening, antineutrophilic cytoplasmic antigen (ANCA: c and p), serum immunoglobulins, paraneoplastic antibodies (negative for HU, YO, RI, MA, TA and TR), blood and cerebrospinal fluid (CSF) treponemal antibodies, HIV 1 and 2 antibodies, coeliac disease screening, rheumatoid factor, complement and prostatic specific antigen. CSF showed normal cytology, glucose and no oligoclonal bands were detected; CSF protein was high at 2.84 g/l while CSF serology for Lyme disease was negative. Previous Computerized tomogram (CT) of abdomen and pelvis was consistent with calcified chronic pancreatitis. Previous nerve conduction study/Electromyography (NCS/EMG) showed axonal sensory and motor generalized neuropathy; repeat study in 2008 showing extensive polyradiculoneuropathy, with prolonged F wave latences in the upper limbs, some degree of demyelination in the nerve roots but no electrophysiological evidence of distal demyelination at this stage. He was initially treated in 2007–08 with diazepam, baclofen and clonazepam as an inpatient for …

P4.20 Pain response to repeated administration of intravenous immunoglobulin in a patient with diabetic lumbosacral radiculoplexus neuropathy. Evidence for proinflammatory cytokine activation

P4.20 Pain response to repeated administration of intravenous immunoglobulin in a patient with diabetic lumbosacral radiculoplexus neuropathy. Evidence for proinflammatory cytokine activation