AUTHOR AND EDITOR INFORMATION

Section 1 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

INTRODUCTION

Section 2 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

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 is often asymmetrical. This condition occurs in both type 1 and type 2 DM. Bruns first described this condition in patients with DM in 1890.¹ In 1955, Garland coined the term diabetic amyotrophy, so the name Bruns-Garland syndrome also is used to describe the condition.²

Diabetic amyotrophy is a disabling illness distinct from other forms of diabetic neuropathy. Most commonly, onset is in middle age or later, although it may occur in youth. Concomitant distal, predominantly sensory, neuropathy may be present. Electrodiagnostic studies most often are consistent with neurogenic lesion attributable to lumbosacral radiculopathy, plexopathy, or proximal crural neuropathy.

Pathophysiology

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

Frequency

United States

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

Mortality/Morbidity

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

Race

No predilection exists for any particular race.

Sex

No predilection exists for either sex.

Age

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.³ 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.⁴ DLP is rare in children, and only 3 cases of DLP in children aged 13-16 years have been reported in the literature.

CLINICAL

Section 3 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

History

The following findings commonly are reported in the history of patients with DLP:

• 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 or may not 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 sudden, unilateral onset. This symptom sometimes is the initial presenting feature of DM.



• Significant recent weight loss frequently is reported. History of loss of 10-40 pounds is common.

Physical

Common findings during the physical examination 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.



• Knee-jerk reflex is absent, with commonly preserved ankle jerks; however, ankle jerks also may be absent with underlying DSPN.



• Features may be localized to the lumbosacral plexus or upper lumbar roots.

Causes

The exact cause of DLP is not known. Features associated with the condition include the following:

• DM, type 1 or 2



• Poor glycemic control



• Significant weight loss

DIFFERENTIALS

Section 4 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Other Problems to be Considered

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

WORKUP

Section 5 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Lab Studies

• Laboratory studies (eg, fasting blood glucose, hemoglobin A1c) should be performed to diagnose or evaluate control of DM.



• 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.

Procedures

• Electromyography (EMG) and nerve conduction studies (NCS) should be performed.
• • In patients without 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 asymmetric amplitudes.
  
  
  
  • Paraspinal muscle needle EMG may show fibrillations and positive sharp waves, but 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 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

Section 6 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Rehabilitation Program

Physical Therapy

Neurologic recovery is slow for patients with DLP. The physical therapist (PT) can assist in improving 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, reacher, elevated toilet seat, 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 adjustment of medication for diabetes (eg, oral agents, insulin) is of paramount importance. Education on proper diet and exercise also is important.

Surgical Intervention

No surgical intervention is needed for this condition.

Consultations

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

MEDICATION

Section 7 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Intravenous human immunoglobulin (IVIg) may hasten recovery in patients with DLP, although this treatment has not been proven in controlled studies and remains controversial. 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.

Drug Category: Blood products

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

Drug Category: Tricyclic antidepressants

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

Drug Category: Antiepileptic drugs

Used for neuropathic type of pain.

FOLLOW-UP

Section 8 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Further Inpatient Care

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

Further Outpatient Care

• Patients may derive further benefit from a course of outpatient physical therapy to achieve their maximum potential with functional mobility and lower extremity strength.

Prognosis

• Good functional recovery is expected in 60% of patients within 12-24 months.
• 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.



• During rehabilitation, the patient should be taught exercises to regain strength in the affected muscle groups, to improve functional recovery.

MISCELLANEOUS

Section 9 of 10  

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

Medical/Legal 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 to avoid unnecessary surgery.



• Misdiagnosis can cause significant concerns for the patient and the family and could trigger a medicolegal lawsuit.

REFERENCES

Section 10 of 10

• Authors and Editors
• Introduction
• Clinical
• Differentials
• Workup
• Treatment
• Medication
• Follow-up
• Miscellaneous
• References

1. Bruns L. Uberneuritsche lahmungen beim diabetes mellitus. Berl Klin Wochenschr. 1890;27:509-515.
2. Garland H. Diabetic amyotrophy. Br Med J. 1955;2:1287-1290.
3. Casey EB, Harrison MJ. Diabetic amyotrophy: a follow-up study. Br Med J. Mar 11 1972;1(5801):656-9. [Medline]. [Full Text].
4. Bastron JA, Thomas JE. Diabetic polyradiculopathy: clinical and electromyographic findings in 105 patients. Mayo Clin Proc. Dec 1981;56(12):725-32. [Medline].
5. Asbury AK. Proximal diabetic neuropathy. Ann Neurol. Sep 1977;2(3):179-80. [Medline].
6. 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].
7. Brown MJ, Asbury AK. Diabetic neuropathy. Ann Neurol. Jan 1984;15(1):2-12. [Medline].
8. Dyck PJ, Norell JE, Dyck PJ. Microvasculitis and ischemia in diabetic lumbosacral radiculoplexus neuropathy. Neurology. Dec 10 1999;53(9):2113-21. [Medline].
9. Dyck PJ, Windebank AJ. Diabetic and nondiabetic lumbosacral radiculoplexus neuropathies: new insights into pathophysiology and treatment. Muscle Nerve. 2002;25(4):477-91.
10. Fernandes Filho JA, Nathan BM, Palmert MR. Diabetic amyotrophy in an adolescent responsive to intravenous immunoglobulin. Muscle Nerve. Dec 2005;32(6:818-20.
11. Harrison MJ, Casey EB. Diabetic amyotrophy. Br Med J. Jul 29 1972;3(5821):293. [Medline]. [Full Text].
12. Locke S, Lawrence DG, Legg MA. Diabetic amyotrophy. Am J Med. 1963;34:775-785.
13. 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].
14. Pascoe MK, Low PA, Windebank AJ. Subacute diabetic proximal neuropathy. Mayo Clin Proc. Dec 1997;72(12):1123-32. [Medline].
15. Raff MC, Asburry AK. Ischemic mononeuropathy and mononeuropathy multiplex in diabetes mellitus. N Eng J Med. 1968;279:17-22.
16. Sander HW, Chokroverty S. Diabetic amyotrophy: current concepts. Semin Neurol. Jun 1996;16(2):173-8. [Medline].
17. Subramony SH, Wilbourn AJ. Diabetic proximal neuropathy. Clinical and electromyographic studies. J Neurol Sci. Feb 1982;53(2):293-304. [Medline].
18. Taylor BV, Dunne JW. Diabetic amyotrophy progressing to severe quadriparesis. Muscle Nerve. Oct 2004;30(4):505-9.
19. Williams IR, Mayer RF. Subacute proximal diabetic neuropathy. Neurology. Feb 1976;26(2):108-16. [Medline].

Article Last Updated: Apr 12, 2007