scholarly journals MON-674 Diabetic Amyotrophy; A Rare Cause of Muscle Weakness

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Duaa Abdallah ◽  
Trek Langenhan ◽  
Jarod Speer

Abstract Background: Diabetic amyotrophy is a rare complication of type 2 diabetes mellitus. There is little existing evidence contributing to projected outcomes for patients recovering from diabetic amyotrophy.Clinical Case: A 42 year-old man presented with lower extremity muscle pain and progressive proximal muscle weakness over 8 months. He developed asymmetrical muscle weakness in the lower extremities with diffuse pain and sensitivity to touch. He also had 80 pounds weight loss, diarrhea, and erectile dysfunction over the same time period. He had a past medical history of asthma, chronic migraines, and type II diabetes mellitus with A1c 7.1. His medications included high dose prednisone to treat his chronic migraines and asthma. Exam revealed generalized muscle atrophy, asymmetric proximal weakness, areflexia, with sensory loss in bilateral lower limbs.ESR, CRP, ANA, anti-HMG CoA reductase, CK, aldolase, SPEP, and myomarker panel were all within normal limits. Treponema pallidum and Bartonella serologies were negative. CSF evaluation was not suggestive of any demyelinating or neuromuscular disease. Full body STIR MRI demonstrated muscle edema in abductor, gluteus minimus, and paraspinal muscles bilaterally. EMG testing revealed acute to subacute active asymmetrical polyradiculoneuropathy and evidence of chronic proximal myopathy.Based on clinical presentation, EMG findings, and lack of evidence to support alternative diagnoses, he was diagnosed with diabetic amyotrophy and was started on IVIG and methylprednisolone with improvement in pain but very minimal improvement in weakness. Unfortunately, the expected clinical course following a diagnosis of diabetic amyotrophy is one of minimal improvement with treatment, as was the case in our patient.Conclusion: Diabetic amyotrophy is a rare complication of type 2 diabetes mellitus which typically presents with muscle weakness followed by severe pain in the thighs, hips, and buttocks. Compared with other neurologic complications of diabetes, amyotrophy is relatively uncommon, affecting approximately 1 percent of patients. This low prevalence and the broad differential for proximal muscle weakness makes it challenging to diagnose. It remains a diagnosis of exclusion, though EMG studies showing polyradiculoneuropathy in the proximal leg musculature is suggestive. Clinical improvement is slow and often incomplete. Physical and occupational therapy are a mainstay of treatment which may also include IVIG and steroids aimed at treating associated pain. Endocrinologists should have a high clinical suspicion for diabetic amyotrophy in the appropriate clinical context. When considering the diagnosis and discussing treatment options with patients, this case highlights the important role of endocrinologists discussing expectations associated with projected outcomes while attempting to manage diabetic amyotrophy.

2019 ◽  
Vol 3 (Supplement_1) ◽  
Author(s):  
Muhamad Jasim ◽  
Jafar Ibrahim ◽  
William Scotton ◽  
Francesco Manfredonia ◽  
Margaret Timmons ◽  
...  

Abstract Introduction Statins are frequently prescribed, following or in order to prevent cardiovascular events. They inhibit 3-hydroxy-3-methylglutaryl coenzyme A reductase (HMGCoA), an enzyme involved in cholesterol synthesis. Up to 20% of patients experience myalgia which resolve after the drug is stopped. We highlight a more serious and potentially life-threatening complication: statin-induced autoimmune necrotising myositis (SIANM). Recently SIANM has been differentiated from inflammatory polymyositis. Patients present with bilateral proximal muscle weakness, elevated creatinine kinase, a muscle biopsy with necrosis and a positive HMGCoA reductase antibody. The latter has been found to be a specific and sensitive investigation for SIANM. Case description Given the rarity of SIANM, no guidelines available recommend a best course of treatment, here we highlight 3 successfully treated patients. Case 1: 72-year-old man with hypercholesterolaemia, type 2 diabetes and hypertension presented with progressive proximal symmetrical weakness for 6 months. He started 20mg atorvastatin a year earlier and stopped this 2 months before admission. Examination revealed 4/5 muscle strength proximally in all 4 limbs and the patient struggled to stand from sitting. CK was elevated at 8223 IU/L (30-200). EMG confirmed a myopathic process and MRI thighs showed active inflammation. A muscle biopsy and HMGCoA antibodies confirmed SIANM and the patient commenced IV and then oral steroids. The patient deteriorated rapidly over the subsequent days with progressive weakness and dysphonia. He developed bilateral pneumonias and was admitted to ITU. Here we commenced the patient on IV immunoglobulin (IVIG) and rituximab. With this he improved significantly, with increasing power and a normalised CK. Case 2: 55-year-old old man with a background of previous MI in 2013 (after which he commenced atorvastatin), type 2 diabetes, hypercholesterolemia and hypertension presented with progressive bilateral proximal muscle weakness. Serum CK found to be 8413, his statin was stopped and the patient underwent extensive investigation. Once again investigations confirmed the diagnosis of SIANM. The patient commenced steroid treatment but despite initial improvement in his power, this soon plateaued as did his CK. He was commenced on IVIG and methotrexate and found significant benefit with these treatments. Case 3: 60-year-old lady presented with a 5-month history of generalised aches and pains with difficulty standing from sitting. She had been on atorvastatin for many years but her symptoms did not improve despite having stopped this 5 months previously. Investigations confirmed a SIANM. The patient was commenced on steroids and methotrexate with good effect. Discussion Patients presenting on statins with proximal symmetrical weakness and a raised CK should have HMGCOA antibodies checked as part of a myositis screen. Though statins should always be stopped, patients with SIANM can continue to deteriorate despite drug discontinuation and steroid treatment. Such patients should be considered for immunosuppression. The 3 cases described show positive response to a combination of methotrexate, IVIG and/or rituximab. This seems to mirror the growing clinical experience in other published case reports. Key learning points Patients presenting on statins with proximal symmetrical weakness and a raised CK should have HMGCOA antibodies checked as part of a myositis screen. Withdrawal of the statin and steroid treatment alone is often insufficient to successfully treat SIANM. Close monitoring of a patient’s power and CK levels are required even after withdrawal of a statin and treatment with steroid as patients can continue to deteriorate. In such cases, additional treatment with methotrexate, IVIG and/ or rituximab appears to have the best outcomes. Conflicts of interest The authors have declared no conflicts of interest.


2010 ◽  
Vol 21 (1) ◽  
pp. 29-35 ◽  
Author(s):  
Diogo Machado Kaminski ◽  
Beatriz D’Agord Schaan ◽  
Antônio Marcos Vargas da Silva ◽  
Pedro Paulo Soares ◽  
Rodrigo Della Méa Plentz ◽  
...  

2020 ◽  
pp. 94-103
Author(s):  
James W. Albers ◽  
Ryan D. Jacobson ◽  
David L. Smyth

Diabetic amyotrophy is a rare complication of diabetes compared to distal symmetric polyneuropathy, but can occasionally be encountered in clinical practice, particularly as the incidence of diabetes increases. The distinctive history of unilateral neuropathic symptoms followed rapidly by atrophy and weakness is typical of the disorder. This complication most commonly occurs in cases of well-controlled Type 2 diabetes mellitus. While the underlying pathophysiology is known to be microvasculitic in nature, the diagnosis is often based on clinical and electrodiagnostic grounds and tissue biopsy is not typically performed. Attempts at corticosteroid administration during immunotherapy should be carefully considered on a patient-by-patient basis. Better recognition of this disorder is likely to result in more rapid diagnosis, counselling, and subspecialty referral.


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