Diabetic Lumbosacral Plexopathy 

  • Author: Divakara Kedlaya, MBBS; Chief Editor: Robert H Meier III, MD   more...
 
Updated: Apr 4, 2011
 

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 is also 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 the presence of a neurogenic lesion that could be associated with lumbosacral plexopathy, radiculopathy, or proximal crural neuropathy.[5, 6, 7, 8] However, the exact cause of diabetic lumbosacral plexopathy is not known.[9]

For more information, see Type 1 Diabetes Mellitus, Type 2 Diabetes Mellitus, Diabetic Neuropathy, and Electrophysiology.

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Pathophysiology

The underlying pathogenesis of diabetic lumbosacral plexopathy and the site of the lesion are not clearly understood and remain subjects of controversy.[5, 10] The condition is most likely caused by inflammatory, immune-mediated vascular radiculoplexopathy.[11, 12, 13, 14] 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.[15]

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Epidemiology

In the United States, the overall prevalence of diabetic lumbosacral plexopathy among individuals with diabetes is 0.08%, although the condition occurs more frequently in persons with type 2 diabetes (1.1%) than in those with the type 1 disease (0.3%).

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.

No race or sex predilection exists for diabetic lumbosacral plexopathy; however, the condition 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.[16] In a large series of 105 patients with diabetic amyotrophy reported by Bastron and Thomas, the age of onset ranged from 36 to 83 years; symptoms progressed over an average of 6.2 months, with 9.5% of patients having painless muscle weakness.[17] Diabetic lumbosacral plexopathy is rare in children; only 3 cases of the condition in children aged 13-16 years have been reported in the literature.

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Prognosis

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

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Patient Education

The patient should be educated in the importance of good glycemic control in conjunction with proper diet and exercise.[18] During rehabilitation, in order to improve functional recovery, the patient should be taught exercises to regain strength in the affected muscle groups.

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Contributor Information and Disclosures
Author

Divakara Kedlaya, MBBS  Clinical Associate Professor, Department of Physical Medicine and Rehabilitation, Loma Linda University School of Medicine; Medical Director, Physical Medicine and Rehabilitation and Pain Management, St Mary Corwin Medical Center

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.

Specialty Editor Board

Teresa L Massagli, MD  Professor 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.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Senior Pharmacy Editor, eMedicine

Disclosure: eMedicine Salary Employment

Michael T Andary, MD, MS  Professor, Residency Program Director, 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; Pfizer Honoraria Speaking and teaching

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.

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]. [Full Text].

  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].

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

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

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

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

  15. 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].

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

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

  18. Gulve EA. Exercise and glycemic control in diabetes: benefits, challenges, and adjustments to pharmacotherapy. Phys Ther. Nov 2008;88(11):1297-321. [Medline].

  19. Russell JW, Berent-Spillson A, Vincent AM, Freimann CL, Sullivan KA, Feldman EL. Oxidative injury and neuropathy in diabetes and impaired glucose tolerance. Neurobiol Dis. Jun 2008;30(3):420-9. [Medline]. [Full Text].

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

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

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

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

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

  25. Kawagashira Y, Watanabe H, Oki Y, Iijima M, Koike H, Hattori N, 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]. [Full Text].

  26. Wada Y, Yanagihara C, Nishimura Y, Oka N. 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].

  27. Chan YC, Lo YL, Chan ES. Immunotherapy for diabetic amyotrophy. Cochrane Database Syst Rev. Jul 8 2009;CD006521. [Medline].

  28. Kilfoyle D, Kelkar P, Parry GJ. Pulsed methylprednisolone is a safe and effective treatment for diabetic amyotrophy. J Clin Neuromuscul Dis. Jun 2003;4(4):168-70. [Medline].

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