eMedicine Specialties > Physical Medicine and Rehabilitation > Plexopathy

Diabetic Lumbosacral Plexopathy

Divakara Kedlaya, MBBS, Clinical Associate Professor, Department of Physical Medicine and Rehabilitation, Loma Linda University School of Medicine

Updated: Oct 10, 2008

Introduction

Background

Proximal neuropathy in diabetes mellitus (DM) is a condition in which patients develop severe aching or burning and lancinating pain in the hip and thigh. This is followed by weakness and wasting of the thigh muscles, which often occur asymmetrically. This disabling condition occurs in type 1 and type 2 DM. Bruns first described the disorder in patients with DM in 1890.¹ In 1955, Garland coined the term diabetic amyotrophy, although the name Bruns-Garland syndrome also is used to describe the condition.^2,3,4

Diabetic amyotrophy, which is distinct from other types of diabetic neuropathy, usually has its onset during or after middle age (although it can occur in younger individuals). Concomitant distal, predominantly sensory, neuropathy may exist. The results of most electrodiagnostic studies are consistent with a neurogenic lesion that could be associated with lumbosacral plexopathy, radiculopathy, or proximal crural neuropathy.^5,6,7,8

Related eMedicine topics:
Diabetes Mellitus, Type 1 - A Review
Diabetes Mellitus, Type 2 - A Review
Diabetes Mellitus, Type 1 [Endocrinology]
Diabetes Mellitus, Type 2 [Endocrinology]
Diabetes Mellitus, Type 1 [Pediatrics: General Medicine]
Diabetes Mellitus, Type 2 [Pediatrics: General Medicine]
Diabetic Neuropathy
Neoplastic Lumbosacral Plexopathy
Radiation-Induced Lumbosacral Plexopathy

Pathophysiology

The underlying pathogenesis and the site of the lesion are not clearly understood and remain subjects of controversy.^5,9 The condition most likely is caused by inflammatory, immune-mediated vascular radiculoplexopathy.^10,11,12,13 Most authors now favor an immune vasculopathy as the cause of diabetic amyotrophy. Studies suggest a role for immunomodulating agents in certain types of diabetic neuropathy, including diabetic amyotrophy.¹⁴ Diabetic lumbosacral plexopathy often occurs in conjunction with weight loss and is associated with only mildly elevated serum glucose levels.

Frequency

United States

The overall prevalence of diabetic lumbosacral plexopathy (DLP) is 0.08% of individuals with diabetes; however, DLP is more frequent with diabetes type 2 (1.1%) than with type 1 (0.3%).

Mortality/Morbidity

Morbidity related to diabetic lumbosacral plexopathy is mainly secondary to pain, proximal muscle wasting, and weakness, causing difficulty getting up from a chair and climbing stairs.

Race

No race predilection exists for diabetic lumbosacral plexopathy.

Sex

No sex predilection exists for diabetic lumbosacral plexopathy.

Age

Diabetic lumbosacral plexopathy (DLP) occurs most commonly in patients aged 50 years or older. In a series of 12 cases reported by Casey and Harrison, no patient was younger than 50 years, and 10 patients were older than 60 years.¹⁵ In a large series of 105 patients with diabetic amyotrophy, reported by Bastron and Thomas, the age of onset ranged from 36-83 years; symptoms progressed over an average of 6.2 months, with 9.5% of patients having painless muscle weakness.¹⁶ DLP is rare in children, and only 3 cases of DLP in children aged 13-16 years have been reported in the literature.

Clinical

History

The following findings commonly are reported in the history of patients with diabetic lumbosacral plexopathy:

• Asymmetrical pain in the hip, buttock, or thigh is common.
• Proximal weakness in quadriceps, hip adductors, and iliopsoas muscles is characteristic.
• Poor blood sugar control generally is noted.
• Patients may have underlying distal symmetrical polyneuropathy (DSPN).
• Gradual onset with bilateral presentation is typical in patients with DSPN; patients usually are insulin dependent.
• Patients without DSPN usually have a sudden, unilateral onset. This symptom sometimes is the initial presenting feature of diabetes mellitus.¹⁷
• Significant recent weight loss frequently is reported. The patient's history commonly includes a loss of 10-40 pounds.

Physical

During the physical examination, common findings in patients with diabetic lumbosacral plexopathy may include the following:

• Proximal lower limb muscle weakness and wasting are characteristic. The patient has particular difficulty getting up from a squatting position.
• Minimal sensory loss is observed.
• The knee-jerk reflex is absent, with commonly preserved ankle jerks; however, ankle jerks also may be absent with underlying distal symmetrical polyneuropathy.
• Features may be localized to the lumbosacral plexus or the upper lumbar roots.

Causes

The exact cause of diabetic lumbosacral plexopathy is not known.¹⁸ Features associated with the condition include the following:

• Diabetes mellitus, type 1 or 2¹⁹
• Poor glycemic control
• Significant weight loss

Differential Diagnoses

Other Problems to Be Considered

Immune-mediated lichen planus - vasculitis
Hemorrhage
Hematoma
Intra-arterial injections
Ischemic lumbar plexopathy
Obstetric-gynecologic complications or complications following any pelvic surgery
Other causes of lumbosacral plexopathy
Lumbar radiculopathy

Workup

Laboratory Studies

• Laboratory studies (eg, fasting blood glucose, hemoglobin A _1c ) should be performed to diagnose or evaluate control of diabetes mellitus.
• Cerebrospinal fluid (CSF) proteins may be elevated, sometimes more than 1 g on lumbar puncture.
• Other lab studies to rule out other causes of neuropathy, as well as cancer and bleeding diathesis, are important.

Imaging Studies

• Lumbar spine and pelvic radiographs should be performed to evaluate for other causes.
• Computerized tomography (CT) scanning or magnetic resonance imaging (MRI) of the lumbosacral spine and pelvis may be indicated in some cases to rule out mass lesions.²⁰

Procedures

• Electromyography (EMG) and nerve conduction studies (NCS) should be performed.^21,22
  • In patients without distal symmetrical polyneuropathy (DSPN), needle EMG usually shows positive sharp waves and fibrillation potentials in iliopsoas, hip adductors, and quadriceps, but other muscles also may be involved.
  • In patients with underlying DSPN, in addition to the above findings, sural sensory nerve action potential (SNAP) is usually absent, and amplitudes in peroneal and tibial compound motor action potential (CMAP) are reduced.
  • Femoral nerve motor conduction studies may show asymmetrical amplitudes.
  • Paraspinal muscle needle EMG may show fibrillations and positive sharp waves, but the results are usually within the reference range.

Histologic Findings

Biopsies rarely are indicated, and systematic studies are lacking in the literature. Early in the disease course, epineurial and perivascular inflammation around the small vessels may be caused by infiltration by mononuclear cells, with or without polymorphonuclear cells. Endoneurium and subperineurial space immunoglobulin-M (IgM) deposition should be expected. Activated complement (C5b-9) deposition in the endothelium of small vessels also is common. Reduced numbers of myelinated and unmyelinated axons may be observed. Differential fascicular loss of axons also is characteristic.

Treatment

Rehabilitation Program

Physical Therapy

Neurologic recovery is slow for patients with diabetic lumbosacral plexopathy. A physical therapist (PT) can assist in improving a patient's functional mobility (eg, transfers, ambulation). The PT instructs the patient in the use of assistive devices when necessary. An exercise and range-of-motion program supervised by the PT also is helpful to maintain and improve function and avoid contractures.

Occupational Therapy

The occupational therapist can recommend appropriate adaptive equipment (eg, a reacher, an elevated toilet seat, a tub bench), depending on the amount of weakness, so that the patient can be independent in activities of daily living and perform self-care tasks in a seated position.

Medical Issues/Complications

Good glycemic control through the adjustment of diabetes medication (eg, oral agents, insulin) is of paramount importance. Education on proper diet and exercise also is essential.

Surgical Intervention

No surgical intervention is needed for diabetic lumbosacral plexopathy.

Consultations

Consider consultation with an endocrinologist (eg, with a diabetologist) to assist with the management of diabetes mellitus.

Medication

Intravenous human immunoglobulin (IVIg) may hasten recovery in patients with diabetic lumbosacral plexopathy (DLP), although this treatment has not been proven in controlled studies and remains controversial.^23,24,25 Other immunosuppressant agents, such as cyclophosphamide and methylprednisolone, also are thought to improve recovery. Two double-blinded, placebo-controlled trials of subjects with DLP have been initiated, one with intravenous methylprednisolone and the other with IVIg. The results of these studies have not yet been published.

Blood products

The administration of human immunoglobulins may improve the clinical and immunologic aspects of the disease. Blood products may decrease autoantibody production and increase solubilization and removal of immune complexes.

Immunoglobulins (Gamimune, Gammagard S/D, Sandimmune)

Immunoglobulin neutralizes circulating myelin antibodies through anti-idiotypic antibodies. It down-regulates proinflammatory cytokines, including INF-gamma; blocks Fc receptors on macrophages; suppresses inducer T-cells and B-cells and augments suppressor T-cells; blocks complement cascade; promotes remyelination; and may increase CSF IgG (10%).

Dosing

Adult

2 g/kg IV qmo for 3 mo

Pediatric

Not established

Interactions

Increases toxicity of live virus vaccine (MMR); do not administer within 3 mo of vaccine

Contraindications

Documented hypersensitivity; IgA deficiency; anti-IgE/IgG antibodies

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Check serum IgA before intravenous immune globulin; use an IgA-depleted product (eg, Gammagard S/D); infusions may increase serum viscosity and thromboembolic events; infusions may increase risk of migraine attacks, aseptic meningitis (10%), urticaria, pruritus, or petechiae (2-5 d postinfusion to 30 d); increases risk of renal tubular necrosis in elderly patients and in patients with diabetes, volume depletion, and preexisting kidney disease; lab result changes associated with infusions include elevated antiviral or antibacterial antibody titers for 1 mo, 6-fold increase in ESR for 2-3 wk, and apparent hyponatremia

Tricyclic antidepressants

These drugs have central and peripheral anticholinergic effects, as well as sedative effects, and block the active reuptake of norepinephrine and serotonin.

Amitriptyline (Elavil)

Analgesic for certain chronic pain.

Dosing

Adult

30-150 mg/d PO

Pediatric

Not established

Interactions

Phenobarbital may decrease effects; coadministration with CYP2D6 enzyme system inhibitors (eg, cimetidine, quinidine) may increase levels; inhibits hypotensive effects of guanethidine; may interact with thyroid medications, alcohol, CNS depressants, barbiturates, and disulfiram

Contraindications

Documented hypersensitivity; patients who have taken MAOIs in past 14 d; patients with history of seizures, cardiac arrhythmias, glaucoma, and urinary retention

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in cardiac conduction disturbances and history of hyperthyroidism, renal or hepatic impairment; avoid using in elderly patients

Nortriptyline (Aventyl, Pamelor)

Has demonstrated effectiveness in the treatment of chronic pain. By inhibiting the reuptake of serotonin and/or norepinephrine by the presynaptic neuronal membrane, this drug increases the synaptic concentration of these neurotransmitters in the CNS. Pharmacodynamic effects, such as the desensitization of adenyl cyclase and the down-regulation of beta-adrenergic receptors and serotonin receptors, also appear to play a role in its mechanisms of action.

Dosing

Adult

25-150 mg/d PO in divided doses

Pediatric

Not established

Interactions

Cimetidine may increase levels when used concurrently; may increase prothrombin time in patients stabilized with warfarin

Contraindications

Documented hypersensitivity; narrow-angle glaucoma; do not administer to patients who have taken MAOIs in past 14 d

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in cardiac conduction disturbances and history of hyperthyroidism and renal or hepatic impairment; due to pronounced effects in cardiovascular system, best to avoid in elderly patients

Doxepin (Sinequan, Adapin)

This agent inhibits histamine and acetylcholine activity and has proven useful in the treatment of various forms of depression associated with chronic and neuropathic pain.

Dosing

Adult

10-150 mg/d PO hs or divided bid/tid

Pediatric

Not established

Interactions

Decreases antihypertensive effects of clonidine but increases effects of sympathomimetics and benzodiazepines; effects increase with phenytoin, carbamazepine, and barbiturates

Contraindications

Documented hypersensitivity; urinary retention; acute recovery phase following myocardial infarction; glaucoma

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in cardiovascular disease, conduction disturbances, seizure disorders, urinary retention, hyperthyroidism, and patients receiving thyroid replacement

Desipramine (Norpramin)

May increase the synaptic concentration of norepinephrine in the CNS by inhibiting reuptake by presynaptic neuronal membrane. The drug may have effects in the desensitization of adenyl cyclase and in the down-regulation of beta-adrenergic receptors and serotonin receptors.

Dosing

Adult

25-100 mg/d PO; not to exceed 150 mg/d

Pediatric

Not established

Interactions

Decreases antihypertensive effects of clonidine but increases effects of sympathomimetics and benzodiazepines; effects of desipramine increase with phenytoin, carbamazepine, and barbiturates

Contraindications

Documented hypersensitivity; narrow-angle glaucoma, recent myocardial infarction; patients who currently are taking MAOIs or fluoxetine or who have taken them in the past 2 wk

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in cardiovascular disease, conduction disturbances, seizure disorders, urinary retention, hyperthyroidism, and patients receiving thyroid replacement

Antiepileptic drugs

These agents are used for the neuropathic type of pain.

Gabapentin (Neurontin)

Has anticonvulsant properties and antineuralgic effects; however, its exact mechanism of action is unknown. The drug is structurally related to GABA but does not interact with GABA receptors. Titration to effect can take place over several days (300 mg on day 1, 300 mg bid on day 2, and 300 mg tid on day 3).

Dosing

Adult

300-3600 mg/d PO in 3-4 divided doses

Pediatric

Not established

Interactions

Antacids may reduce bioavailability significantly (administer at least 2 h following antacids); may increase norethindrone levels significantly

Contraindications

Documented hypersensitivity

Precautions

Pregnancy

C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus

Precautions

Caution in patients with severe renal disease

Follow-up

Further Inpatient Care

• Because of the sudden onset of functional loss, some patients with diabetic lumbosacral plexopathy may need to be transferred to a subacute rehabilitation facility or a convalescent home for several months, until they recover strength.
• Most patients are able to avoid inpatient care for this condition.

Further Outpatient Care

• Patients with diabetic lumbosacral plexopathy may derive further benefit from a course of outpatient physical therapy, achieving their maximum potential in terms of functional mobility and lower extremity strength.

Prognosis

• Good functional recovery within 12-24 months is expected in 60% of patients with diabetic lumbosacral plexopathy.
• Mild weakness, discomfort, and stiffness often persist for years.
• Occasional relapses can occur.

Patient Education

• The patient should be educated in the importance of good glycemic control in conjunction with proper diet and exercise.²⁶
• During rehabilitation, the patient should be taught exercises to regain strength in the affected muscle groups, in order to improve functional recovery.

Related Medscape topics:
CME/CE Advances in Glucagon-like Peptides for the Treatment of Type 2 Diabetes
CME Managing Type 2 Diabetes: Developing Office-based Systems of Care
CME Weight Loss May Improve Glycemic Control and Blood Pressure in Type 2 Diabetes 

Miscellaneous

Medicolegal Pitfalls

• Familiarity with the clinical setting of this condition and prompt diagnosis are medicolegally important. It is especially important to distinguish this disorder from compressive radiculopathy in order to avoid unnecessary surgery.
• Misdiagnosis can cause significant concerns for the patient and his/her family and could trigger a medicolegal lawsuit.

References

1. Bruns L. Uberneuritsche lahmungen beim diabetes mellitus. Berl Klin Wochenschr. 1890;27:509-15.

2. Garland H. Diabetic amyotrophy. Br Med J. Nov 26 1955;2(4951):1287-90. [Medline]. [Full Text].

3. Asbury AK. Proximal diabetic neuropathy. Ann Neurol. Sep 1977;2(3):179-80. [Medline].

4. Locke S, Lawrence DG, Legg MA. Diabetic amyotrophy. Am J Med. Jun 1963;34:775-85. [Medline].

5. Sander HW, Chokroverty S. Diabetic amyotrophy: current concepts. Semin Neurol. Jun 1996;16(2):173-8. [Medline].

6. Tracy JA, Dyck PJ. The spectrum of diabetic neuropathies. Phys Med Rehabil Clin N Am. Feb 2008;19(1):1-26, v. [Medline].

7. Raff MC, Asbury AK. Ischemic mononeuropathy and mononeuropathy multiplex in diabetes mellitus. N Engl J Med. Jul 4 1968;279(1):17-21. [Medline].

8. Taylor BV, Dunne JW. Diabetic amyotrophy progressing to severe quadriparesis. Muscle Nerve. Oct 2004;30(4):505-9. [Medline].

9. Williams IR, Mayer RF. Subacute proximal diabetic neuropathy. Neurology. Feb 1976;26(2):108-16. [Medline].

10. Dyck PJ, Norell JE, Dyck PJ. Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy. Neurology. Dec 10 1999;53(9):2113-21. [Medline].

11. Harrison MJ, Casey EB. Diabetic amyotrophy. Br Med J. Jul 29 1972;3(5821):293. [Medline]. [Full Text].

12. Pascoe MK, Low PA, Windebank AJ. Subacute diabetic proximal neuropathy. Mayo Clin Proc. Dec 1997;72(12):1123-32. [Medline].

13. Kawamura N, Dyck PJ, Schmeichel AM, et al. Inflammatory mediators in diabetic and non-diabetic lumbosacral radiculoplexus neuropathy. Acta Neuropathol. Feb 2008;115(2):231-9. [Medline].

14. Dyck PJ, Windebank AJ. Diabetic and nondiabetic lumbosacral radiculoplexus neuropathies: new insights into pathophysiology and treatment. Muscle Nerve. Apr 2002;25(4):477-91. [Medline].

15. Casey EB, Harrison MJ. Diabetic amyotrophy: a follow-up study. Br Med J. Mar 11 1972;1(5801):656-9. [Medline]. [Full Text].

16. Bastron JA, Thomas JE. Diabetic polyradiculopathy: clinical and electromyographic findings in 105 patients. Mayo Clin Proc. Dec 1981;56(12):725-32. [Medline].

17. Barohn RJ, Sahenk Z, Warmolts JR. The Bruns-Garland syndrome (diabetic amyotrophy). Revisited 100 years later. Arch Neurol. Nov 1991;48(11):1130-5. [Medline].

18. Brown MJ, Asbury AK. Diabetic neuropathy. Ann Neurol. Jan 1984;15(1):2-12. [Medline].

19. Russell JW, Berent-Spillson A, Vincent AM, et al. Oxidative injury and neuropathy in diabetes and impaired glucose tolerance. Neurobiol Dis. Jun 2008;30(3):420-9. [Medline].

20. O'Neill BJ, Flanders AE, Escandon SL, et al. Treatable lumbosacral polyradiculitis masquerading as diabetic amyotrophy. J Neurol Sci. Oct 22 1997;151(2):223-5. [Medline].

21. Tataroglu C, Bicerol B, Kiylioglu N, et al. Proximal femoral conductions in patients with lumbosacral radiculoplexus neuropathy. Clin Neurol Neurosurg. Oct 2007;109(8):654-60. [Medline].

22. Subramony SH, Wilbourn AJ. Diabetic proximal neuropathy. Clinical and electromyographic studies. J Neurol Sci. Feb 1982;53(2):293-304. [Medline].

23. Fernandes Filho JA, Nathan BM, Palmert MR, et al. Diabetic amyotrophy in an adolescent responsive to intravenous immunoglobulin. Muscle Nerve. Dec 2005;32(6):818-20. [Medline].

24. Kawagashira Y, Watanabe H, Oki Y, et al. Intravenous immunoglobulin therapy markedly ameliorates muscle weakness and severe pain in proximal diabetic neuropathy. J Neurol Neurosurg Psychiatry. Aug 2007;78(8):899-901. [Medline].

25. Wada Y, Yanagihara C, Nishimura Y, et al. A case of diabetic amyotrophy with severe atrophy and weakness of shoulder girdle muscles showing good response to intravenous immune globulin. Diabetes Res Clin Pract. Jan 2007;75(1):107-10. [Medline].

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Keywords

diabetic lumbosacral plexopathy, diabetes mellitus, diabetes mellitus type 1, diabetes mellitus type 2, type 1 diabetes, diabetes type 1, type 2 diabetes, diabetes 1, diabetes 2, diabetic neuropathy, neuropathy, lumbosacral, lumbar sacral, plexopathy, lumbosacral plexopathy, proximal neuropathy, amyotrophy, diabetic amyotrophy, distal symmetrical polyneuropathy, DSPN, polyneuropathy, Bruns-Garland syndrome, diabetic proximal neuropathy, diabetic lumbosacral polyradiculopathy, polyradiculopathy, diabetic lumbosacral radiculoplexus neuropathy(DLRPN), diabetic femoral neuropathy, ischemic mononeuropathymultiplex associated with diabetes mellitus, proximal lower limb motor neuropathy

Contributor Information and Disclosures

Author

Divakara Kedlaya, MBBS, Clinical Associate Professor, Department of Physical Medicine and Rehabilitation, Loma Linda University School of Medicine
Divakara Kedlaya, MBBS is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Paraplegia Society, and Colorado Medical Society
Disclosure: Nothing to disclose.

Medical Editor

Teresa L Massagli, MD, Residency Director, Professor, Department of Rehabilitation Medicine and Pediatrics, University of Washington School of Medicine
Teresa L Massagli, MD is a member of the following medical societies: American Academy of Pediatrics, American Academy of Physical Medicine and Rehabilitation, and Association of Academic Physiatrists
Disclosure: Nothing to disclose.

Pharmacy Editor

Francisco Talavera, PharmD, PhD, Senior Pharmacy Editor, eMedicine
Disclosure: Nothing to disclose.

Managing Editor

Michael T Andary, MD, MS, Residency Program Director, Professor, Department of Physical Medicine and Rehabilitation, Michigan State University College of Osteopathic Medicine
Michael T Andary, MD, MS is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation, American Association of Neuromuscular and Electrodiagnostic Medicine, American Medical Association, and Association of Academic Physiatrists
Disclosure: allergan Honoraria Speaking and teaching

CME Editor

Kelly L Allen, MD, Regional Medical Director, IMX-Medical Management Services
Disclosure: Nothing to disclose.

Chief Editor

Robert H Meier III, MD, Director, Amputee Services of America; Active Medical Staff, Presbyterian/St Luke's Hospital, Spalding Rehabilitation Hospital, Select Specialty Hospital; Consulting Staff, Kindred Hospital
Robert H Meier III, MD is a member of the following medical societies: American Academy of Physical Medicine and Rehabilitation and Association of Academic Physiatrists
Disclosure: Nothing to disclose.

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