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Diabetic Amyotrophy

Diabetic amyotrophy is a complication of diabetes mellitus. Affected individuals suffer from painful, progressive damage to the lumbosacral plexus and consequent neurological deficits.


Presentation

Initially, DA manifests in form of severe, unilateral pain in buttocks, hips, and thighs. Similar symptoms subsequently affect the contralateral side. Patients then develop a progressive, asymmetric weakness of the lower limbs. As the disease progresses, patients may experience bouts of acute exacerbation and distal parts of the legs may become involved [2] [7]. Paresthesia may be noted in distal portions of the legs. In the long term, muscle atrophy can be observed in affected regions. In severe cases, patients may become wheelchair-dependent.

Autonomic function impairment has repeatedly been described in DA patients and may comprise orthostatic hypotension, diarrhea or constipation, and sexual dysfunction. Compromise of the brachial plexus, cervical and thoracic radiculopathy have been reported [8] [9]. Neurological symptoms are often accompanied by general weight loss. Of note, patients may also claim polyuria and polydipsia, recurrent infections and retarded wound healing as well as diabetic foot ulcer, diabetic retinopathy, and diabetic nephropathy. These symptoms are not characteristic of DA but are frequently observed in diabetes mellitus patients.

After several weeks of illness, symptoms usually subside spontaneously. However, some patients do not achieve complete recovery.

Weight Loss
  • A patient with weight loss and weakness presents a diagnostic challenge. Drs Moeser and Kent describe an unusual case of diabetic amyotrophy that required extensive workup to arrive at the diagnosis and rule out more serious disease.[ncbi.nlm.nih.gov]
  • It is usually seen in patients with poorly controlled or previously unrecognized diabetes mellitus, and severe weight loss is a common concomitant.[e-arm.org]
Wheelchair Bound
  • A 49-year-old woman with diabetes mellitus developed progressive weakness and atrophy of both thighs rendering her wheelchair-bound within two months.[ncbi.nlm.nih.gov]
  • Diabetic Amyotrophy Symptoms and Treatment Because this condition involves the wasting away of certain muscle groups, particularly in the leg and thigh, many patients afflicted with this condition are wheelchair bound or forced to ambulate with the assistance[diabeteshealthresource.com]
  • At tertiary evaluation, approximately one-half of patients in both cohorts were wheelchair-bound and most were still on continuous pain medication.[brain.oxfordjournals.org]
Italian
  • Diabetic Amyotrophy ( C0271685 ) Concepts Disease or Syndrome ( T047 ) MSH D003929 SnomedCT 190349003, 267472008, 193211001, 154683002, 39058009 Italian Amiotrofia diabetica Japanese 糖尿病性筋萎縮, トウニョウビョウセイキンイシュク Portuguese Amiotrofia Diabética, Amiotrofia[fpnotebook.com]
Difficulty Climbing Stairs
  • A steady decline led to mobilisation with a Zimmer frame and great difficulty climbing stairs. He complained of a painful burning sensation in his left shin and numbness of the left leg. There had been a 12 kg weight loss in four months.[pmj.bmj.com]
Walking with a Cane
  • Immediately after intravenous immunoglobulin (IVIg) therapy (20 g x 3 days), she became able to walk with a cane. After the next course of the therapy, she could walk without assistance.[ncbi.nlm.nih.gov]
Polydipsia
  • Of note, patients may also claim polyuria and polydipsia, recurrent infections and retarded wound healing as well as diabetic foot ulcer, diabetic retinopathy, and diabetic nephropathy.[symptoma.com]
Thrombosis
  • Deep vein thrombosis was diagnosed and anticoagulation therapy was started. The patient reported sharp excruciating pain in the left groin and anteromedial thigh with radiation to the medial knee.[healio.com]
Muscle Weakness
  • A 56 year-old man with diabetes mellitus and pain, muscle weakness and wasting in both thighs, of three months duration, had a diagnosis of diabetic amyotrophy.[ncbi.nlm.nih.gov]
  • While amyotrophy will cause muscle weakness and shooting pains, focal neuropathy will cause pain only in specific areas of the legs. Autonomic neuropathy affects the autonomic nervous system.[glucosecontrolnet.com]
  • This enhances Diabetic Amyopathy The symptoms are typically unilateral and sometimes bilateral which include: Severe pain and muscle weakness in your hip and thigh or buttock (occasionally the arms and abdomen) Proximal muscle weakness and wasting in[desimd.com]
  • Diabetic amyotrophy is a syndrome consisting of bilateral, but often asymmetrical, proximal muscle weakness and atrophy primarily affecting the lower extremities accompanied by pain in the back or the thighs.[e-arm.org]
Muscular Atrophy
  • SMN1 and NAIP genes deletions in different types of spinal muscular atrophy in Khuzestan province, Iran.[connection.ebscohost.com]
  • Nerve conduction and EMG is used to rule out other conditions and diagnose diabetes muscular atrophy. Treatment of diabetic amyotrophy Muscle atrophy, diabetes is reversible. This can take a variety of measures.[whydiabetes.blogspot.com]
  • Biros I, Forrest S (1999) Spinal muscular atrophy: Untangling the knot? J Med Genet 36: 1-8.[omicsonline.org]
  • Treating of spinal muscular atrophy A form of the disease differ in severity, so treatment is adjusted individually.[health-factbook.com]
  • Abstract Spinal muscular atrophy is an autosomal recessive neurodegenerative disease caused by homozygous mutation to the survival motor neuron 1 (SMN1) gene.[ncbi.nlm.nih.gov]
Thigh Weakness
  • DISCUSSION Diabetic amyotrophy was first cited by Bruns1 in 1890 in 3 patients with diabetes mellitus who had progression of proximal thigh weakness and pain.[healio.com]
Atrophy of the Thigh Muscles
  • This condition is often characterized by difficulty standing from a sitting position, sudden and severe pain in the buttocks, thigh or hip, atrophied and weak thigh muscles, swelling in the abdomen and sudden and unexplained weight loss.[diabeteshealthresource.com]
Muscle Swelling
  • This condition is often characterized by difficulty standing from a sitting position, sudden and severe pain in the buttocks, thigh or hip, atrophied and weak thigh muscles, swelling in the abdomen and sudden and unexplained weight loss.[diabeteshealthresource.com]
Loss of Initiative
  • Although initial symptomatology is typically unilateral, by the time of clinical assessment there is usually evidence of bilateral involvement, the latency to involvement of the second leg ranging from three days to eight months. 3 The onset with severe[pmj.bmj.com]
Polyuria
  • Of note, patients may also claim polyuria and polydipsia, recurrent infections and retarded wound healing as well as diabetic foot ulcer, diabetic retinopathy, and diabetic nephropathy.[symptoma.com]
Peripheral Neuropathy
  • Proximal limb pain was not invariable, a distal sensory peripheral neuropathy was common, and diabetic control at diagnosis was likely to be good. No prognostic factors were identified.[ncbi.nlm.nih.gov]
  • Aside from amyotrophy, the other three types of neuropathy include peripheral neuropathy, autonomic neuropathy, and mononeuropathy. Peripheral neuropathy is by far the most common type.[diabeteshealthresource.com]
  • It is the second most common kind of Diabetic Neuropathy, the most common being diabetic peripheral neuropathy. Elevated levels of blood sugar cause injury to nerve fibers throughout the body.[dovemed.com]
  • On the contrary, about 50% of patients with diabetes mellitus all around the world suffer from DPN (Diabetic Peripheral Neuropathy).[desimd.com]
Limb Weakness
  • , weakness that is not confined to one nerve or nerve root distribution and exclusion of other causes of lumbosacral radiculopathies and plexopathy.[ncbi.nlm.nih.gov]
  • ; weakness that is not confined to one nerve or nerve root distribution; and exclusion of other causes of lumbosacral radiculopathies and plexopathy.[cochrane.org]
Myoclonic Jerking
  • He responded well to steroids with marked improvement in strength of the lower limb muscles and complete resolution of myoclonic jerks. Diabetic amyotrophy presenting as myoclonic jerks has been rarely reported before.[ncbi.nlm.nih.gov]

Workup

The observation of characteristic symptoms in a diabetic patient is the basis of the diagnosis of DA. DA may well be the first symptom of diabetes mellitus and thus, some patients may not have been previously diagnosed with this disease. In any case, measurements of fasting blood glucose and hemoglobin A1C (HbA1C) levels should be performed. Results may demonstrate poor glycemic control or prompt a suspicion of undiagnosed diabetes mellitus. As for the latter, glucose tolerance tests are indicated to establish the respective diagnosis.

Complementary diagnostic measures may be conducted as follows:

  • Electrophysiological studies may be carried out to confirm the neuropathic origin of weakness and wasting. In DA patients, such studies often yield asymmetrically reduced motor and sensory amplitudes [8]. Neurological deficits don't follow myotome patterns of distribution and result from injury to lumbosacral roots, plexus, and nerves.
  • If cerebrospinal fluid specimens are obtained, enhanced protein contents may be indicative of DA [10].
  • Although not usually required, biopsy specimens may be obtained for histopathological analysis. Typical findings comprise an inflammation of small, perineural vessel and polymorphonuclear infiltrates. Nerve fiber loss and post-injury neuroma formation may be observed. Immunohistochemical staining may reveal immunoglobulin-M (IgM) and complement deposits in lumen and walls of affected vascular structures [2].
  • Diagnostic imaging may be employed to rule out skeletal anomalies and neoplasms.
Hyponatremia
  • In most diabetic patients, the presence of hyponatremia is usually ascribed to severe hyperglycemia, hypertriglyceridemia, oral hypoglycemic agents, or other drugs.[ncbi.nlm.nih.gov]
Liver Biopsy
  • We identified a 5778-bp deletion (8214-13991) in mitochondrial DNA from the muscle and liver biopsy specimens by the primer shift PCR and PCR-direct sequence methods.[ncbi.nlm.nih.gov]

Treatment

  • Rapid clinical, functional, and electrical improvement followed treatment with intravenous immunoglobulin.[ncbi.nlm.nih.gov]

Prognosis

  • Involvement of the upper limbs is unusual, and prognosis is said to be good. We describe two patients, each with type II diabetes mellitus, who presented with diabetic amyotrophy progressing to severe quadriparesis.[ncbi.nlm.nih.gov]
  • Tricyclic antidepressants seem to be effective for symptomatic relief and it usually has a good prognosis. To cite this abstract: Karmacharya P, Pathak R, Ghimire S, Shrestha P, Ghimire S, Poudel D, Aryal M, Doomra S, Alweis R.[shmabstracts.com]
  • […] therapy may help in controlling the muscle weakness or pain Please find comprehensive information on Diabetic Proximal Neuropathy regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis[dovemed.com]
  • The outlook (prognosis) is usually good. Most patients recover well, although some symptoms may remain. The chances of making a good recovery - and remaining well (as the condition can relapse) - are improved by maintaining good diabetes control.[patient.info]

Etiology

  • Certain conditions have both an underlying etiology and multiple body system manifestations due to the underlying etiology.[icd10coded.com]
  • Possible etiologic factors have been considered in a review of animal experiments and clinical observations in human patients. Received December 23, 1964. Accepted February 18, 1965.[pediatrics.aappublications.org]
  • Brachial plexopathy in this patient was likely related to his diabetes because there was no other obvious etiology; pain and weakness were prominent with relative paucity of sensory symptoms, and there was preceding weight loss, both characteristic features[care.diabetesjournals.org]
  • (Etiology) The following factors may be responsible for Proximal Neuropathy due to Diabetes: Poor glycemic control is a key factor contributing to Diabetic Proximal Neuropathy.[dovemed.com]
  • Diabetic neuropathy: clinical features, etiology, and therapy. Curr Neurol Neurosci Rep 2004;4(1):55–61. Dyck PJB, Windebank AJ. Diabetic and nondiabetic lumbosacral radiculoplexus neuropathies: new insights into pathophysiology and treatment.[journals.lww.com]

Epidemiology

  • […] types IV & V collagen: Especially in innermost lamellae Endoneurial microvessles Increased types IV, V and VI collagen around microvessels CNS: Spinal cord atrophy reported with diabetes DIABETIC NEUROPATHIES: SYMMETRIC Chronic Distal: Sensory Autonomic Epidemiology[neuromuscular.wustl.edu]
  • Sciatica: a review of history, epidemiology, pathogenesis, and the role of epidural steroid injection in management.[explainmedicine.com]
  • Determination of peripheral neuropathy prevalence and associated factors in Chinese subjects with diabetes and pre-diabetes - ShangHai Diabetic Neuropathy Epidemiology and Molecular Genetics Study (SH-DREAMS).[scielo.br]
  • The Strengthening the Reporting of Observational Studies in Epidemiology (STROBE) guidelines have been followed in the study [ 5 ]. All the patients were living in Spain at the time of the study.[ojrd.biomedcentral.com]
Sex distribution
Age distribution

Pathophysiology

  • Diabetic amyotrophy has the features of a metabolic myopathy and similarities between the ultrastructural changes and clinical course of amyotrophy, thyrotoxicosis and the myopathy of corticosteroid therapy suggest that common pathophysiologic mechanisms[insights.ovid.com]
  • Considerable evidence that diabetic amyotrophy is a distinct clinical entity with a defined clinical spectrum and pathophysiology has emerged over the last several years.[medlink.com]
  • The next description of the disorder was in 1953 by Garland and Tavemer.2 They reported a series of 5 patients and used the term "diabetic myelopathy" due to their uncertainty regarding spinal cord involvement in the pathophysiology of the disorder.[healio.com]
  • New insights into pathophysiology and treatment. Muscle Nerve, 25, 477–491. Courtney, A. E., McDonnell, G. V., Patterson, V. H. (2001). Human Immunoglobulin for Diabetic Amyotrophy- a Promising Prospect? Postgraduate Medical Journal, 77, 326–328.[en.wikipedia.org]

Prevention

  • Furthermore, this report highlights the preventive as well as therapeutic role of strict glycemic control, warranting population-based monitoring and education of patients for diabetic amyotrophy, especially in Pakistan.[ncbi.nlm.nih.gov]
  • Preventing complications from Diabetic Amyotrophy: One should control diabetes and other factors like smoking and alcohol consumption, which will help delay, prevent or slow down the progression of diabetic amyotrophy or diabetic neuropathy DCCT (The[desimd.com]
  • But you can prevent and even reverse it. Diabetic proximal neuropathy is only one of the diabetic neuropathies. Like all the others it is the result of long-term high blood sugar.[a-diabetic-life.com]
  • Diabetic Nephropathy: Diagnosis, Prevention, and Treatment.[explainmedicine.com]
  • This will prevent further deterioration in the condition and enhance healing process. Pain is controlled with various medicines available. Antidepressants, anticonvulsants, are generally used to reduce pain. Patient should live healthy lifestyle.[tandurust.com]

Summary

Diabetic amyotrophy (DA) is also known as proximal diabetic neuropathy or lumbosacral radiculoplexus neuropathy and refers to a complication of diabetes mellitus type 2 or, less commonly, diabetes mellitus type 1 [1]. About 1% of individuals suffering from diabetes mellitus develop DA [3]. This condition is assumed to result from an immune-mediated vasculopathy and ensuing nerve ischemia [2]. However, the precise cause of the presumed immune disorder remains unknown. DA patients don't usually have a medical history of autoimmune disease.

DA affects the lumbosacral plexus and emerging nerves, and thus interferes with motor control and sensation in the pelvic region and proximal parts of the legs. Contrary to the more common distal neuropathy of diabetes mellitus patients, motor deficits prevail in individuals affected by DA. Due to the underlying inflammation, the condition is painful. Treatment primarily consists of an appropriate glycemic control, although anti-inflammatory medication has occasionally been administered [4] [5]. Patients may also benefit from physical therapy [6].

References

Article

  1. Said G. Diabetic neuropathy. Handb Clin Neurol. 2013; 115:579-589.
  2. Kelkar P, Masood M, Parry GJ. Distinctive pathologic findings in proximal diabetic neuropathy (diabetic amyotrophy). Neurology. 2000; 55(1):83-88.
  3. Pasnoor M, Dimachkie MM, Barohn RJ. Diabetic neuropathy part 2: proximal and asymmetric phenotypes. Neurol Clin. 2013; 31(2):447-462.
  4. Dyck PJ, Norell JE, Dyck PJ. Methylprednisolone may improve lumbosacral radiculoplexus neuropathy. Can J Neurol Sci. 2001; 28(3):224-227.
  5. Tamburin S, Magrinelli F, Favaro F, Briani C, Zanette G. Long-term response of neuropathic pain to intravenous immunoglobulin in relapsing diabetic lumbosacral radiculoplexus neuropathy. A case report. Pain Pract. 2014; 14(2):E85-90.
  6. Thaisetthawatkul P, Dyck PJ. Treatment of diabetic and nondiabetic lumbosacral radiculoplexus neuropathy. Curr Treat Options Neurol. 2010; 12(2):95-99.
  7. Naddaf E, Dyck PJ. Vasculitic Neuropathies. Curr Treat Options Neurol. 2015; 17(10):374.
  8. Tracy JA, Engelstad JK, Dyck PJ. Microvasculitis in diabetic lumbosacral radiculoplexus neuropathy. J Clin Neuromuscul Dis. 2009; 11(1):44-48.
  9. Massie R, Mauermann ML, Staff NP, et al. Diabetic cervical radiculoplexus neuropathy: a distinct syndrome expanding the spectrum of diabetic radiculoplexus neuropathies. Brain. 2012; 135(Pt 10):3074-3088.
  10. Imtiaz KE, Lekwuwa G, Kaimal N, Rai M, Nafeez M, Majeed T. Elevated cerebrospinal fluid protein in diabetic lumbosacral radiculoplexus neuropathy. Qjm. 2012; 105(11):1119-1123.

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Last updated: 2019-07-11 21:14