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

  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]

  We report the case of 71-year-old male who was once diagnosed as having diabetic amyotrophy, because of pronounced wasting in proximal muscles, massive weight loss, and development of paresthesia in his legs. [ncbi.nlm.nih.gov]

  With control of the diabetes and weight gain the symptoms reverse. Key-words Depression Diabetic amyotrophy Diabetic neuropathic cachexia Leg pain Weight loss This is a preview of subscription content, log in to check access. [link.springer.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]

  Weakness in sciatic distribution is reported [1] but is less common early in the disease compared to the proximal weakness. [13], [19] Patients commonly complain of difficulty climbing stairs, rising from low chairs and buckling of the knee with weight-bearing [neurologyindia.com]

• Abdominal Obesity

  […] correlates with Severity of insulin resistance Reduced capacity for lipid oxidation Increased risk for obesity Subsarcolemmal mitochondrial electron transport chain activity is reduced in Obesity: 3.5 fold Type 2 diabetes: 7 fold TRIGLYCERIDES: Increased [neuromuscular.wustl.edu]

• Anemia

   Subcutaneous recombinant human erythropoietin effective in some patients with OH & anemia. Octreotide may improve OH by splanchnic vasoconstriction. Delayed gastric emptying relieved with metoclopramide Diabetic diarrhea treated with short courses [slideshare.net]

• 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]

  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]

  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]

• Muscular Atrophy

  SMN1 and NAIP genes deletions in different types of spinal muscular atrophy in Khuzestan province, Iran. [connection.ebscohost.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]

  The diagnosis of diabetic amyotrophy The electrical test is useful in diagnosis of diabetes, muscle atrophy. Nerve conduction and EMG is used to rule out other conditions and diagnose diabetes muscular atrophy. [whydiabetes.blogspot.com]

• 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]

  This was followed by a gradual improvement in weakness that was at times incomplete with residual thigh weakness. Despite residual weakness, disability was minor in the majority of patients. [neurologyindia.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 Tenderness

  Examination Symmetrical proximal muscle weakness. Muscle tenderness is very rare with myopathy. Fever with inflammatory causes. There is usually no wasting but there may be hypertrophy of muscle (atrophy is a late sign). [patient.info]

• Peripheral Neuropathy

  Neuropathies in association with diabetes mellitus are symmetrical peripheral neuropathy, mononeuropathy, radiculopathy, autonomic visceral neuropathy, and diabetic cranial neuropathy. [e-arm.org]

  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]

  In this Patient Guide, we’ll refer to it as peripheral diabetic neuropathy, or simply peripheral neuropathy. Peripheral neuropathy is the most common form of neuropathy caused by diabetes. [endocrineweb.com]

  The health care costs of diabetic peripheral neuropathy in the US. Diabetes Care. 2003;26(6):1790-5 [ Links ] 18 Thomas PK. Classification, differential diagnosis and staging of diabetic peripheral neuropathy. [scielo.br]

• Limb Weakness

  BACKGROUND: People with diabetes mellitus (DM) sometimes present with acute or subacute, progressive, asymmetrical pain and weakness of the proximal lower limb muscles. [ncbi.nlm.nih.gov]

  In diabetic amyotrophy - Causes, Symptoms and Treatment Diabetic Muscle atrophy is a condition in which patients had lower limb weakness, pain in. [whydiabetes.blogspot.com]

  Background: People with diabetes mellitus (DM) sometimes present with acute or subacute, progressive, asymmetrical pain and weakness of the proximal lower limb muscles. [cochrane.org]

• Tremor

  Children with the condition may: be able to sit up without help, but not stand or walk have weak arms or legs have shaking (tremors) in their fingers and hands later develop problems with their joints, such as an unusually curved spine (scoliosis) have [nhs.uk]

  Perioral fasciculations, hand tremors, noninsulin-dependent diabetes mellitus, and infertility are common. [emedicine.medscape.com]

• Neglect

  Its pathogenesis was ill understood, and its treatment was neglected with the anticipation that the patient would eventually recover, albeit over a period of some 1-2 years, suffering considerable pain, weakness and disability. [diabetesmanager.pbworks.com]

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.

OBJECTIVES: We aimed to review the evidence from randomised trials for the efficacy of any form of immunotherapy in the treatment of diabetic amyotrophy. [ncbi.nlm.nih.gov]

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]

There is a strong emphasis on patient and family education with respect to future care and prognosis. [pennmedicine.org]

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]

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]

"Lumbar spinal stenosis: an update on the epidemiology, diagnosis and treatment". [en.wikipedia.org]

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]

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]

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]

THE PATHOPHYSIOLOGY To better understand the pathophysiology of the disease, the Mayo researchers performed ipsilateral nerve biopsies on the 33 patients in their prospective study. [journals.lww.com]

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]

Prevention In most cases, complications of diabetes can be prevented by following certain practices. Controlling blood sugar levels is one of the most important thing that diabetics can do. [healthguideinfo.com]

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].

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.