Rare case of diabetic neuropathic cachexia along with diabetic amyotrophy

A 65-year-old patient with background of alcohol excess and previous gunshot wounds was admitted with significant weight loss, leg cramps, dizziness and lethargy for the last 3 months. He was diagnosed with type 2 diabetes mellitus in July 2020 and was started on Metformin and Gliclazide by his in July; he was later commenced on alogliptin and empaglaflozin by diabetes specialist nurse in early August. He also had generalised muscle wasting, dorsal guttering in both hands and was cachectic when he presented to hospital. His haemoglobin A1c (HbA1c) was 124 mmol/mol in July 2020 and was 63 mmol/mol in September 2020. The patient had negative autoimmune and TB screen. CT abdomen/pelvis and CT lumbosacral spine that showed mild diverticular disease and bilateral L5 spondylolysis with L5-S1 spondylotic changes. Electrophysiological studies confirmed sensory motor peripheral neuropathy. Patient was diagnosed with diabetic neuropathic cachexia secondary to poorly controlled diabetes and was commenced on 30 units two times per day of NovoMix 30 insulin; this was adjusted to 24 units two times per day in endocrine clinic 3 months later, after gaining 10 kg in weight. Good glycaemic control is key to the management of such cases and, therefore, we recommend early referral to diabetes specialist input for consideration of insulin therapy.

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

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.

Diabetic Amyotrophy: From the Basics to the Bedside

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.

Diabetic amyotrophy, not your typical back pain

A 49-year-old man presented to the hospital for spinal cord decompression surgery with left buttock and left leg pain. The patient described an acute burning pain radiating down from his left buttock to left lateral leg. He also noted a 13.6 kg weight loss in recent months. Physical examination showed decreased muscle bulk of the left thigh, decreased strength of the left hip, left knee flexors and extensors. Recent MRI spine showed mild canal narrowing and cord flattening in the lower thoracic spine. Serologic testing showed an elevated glucose of 17.9 mmol/L and haemoglobin A1c of 9.8%. Electromyography showed denervation of scattered muscles of the left knee flexors, hip flexors and adductors. In the setting of newly diagnosed diabetes mellitus, he was diagnosed with diabetic amyotrophy, started on insulin therapy, and his surgery was cancelled.

Diabetic amyotrophy (Bruns-Garland syndrome)

Garland and Taverner first fully described diabetic amyotrophy as a clinical entity in 1950. Its distinctive features were a painful, markedly asymmetrical proximal weakness and wasting of the thighs and legs often with diminished or absent tendon reflexes. Motor signs dominated the picture, but autonomic and sensory nerves could be involved. Characteristically it occurred in poorly controlled diabetics in whom substantial if not always complete recovery was generally observed. A lumbosacral plexus neuropathy, associated with microvasculitis with secondary inflammatory perivascular mononuclear cell infiltrates is the underlying pathology.

Urgent Pain Problems

Chapter 15 addresses conditions that require prompt evaluation but are not generally in the group of conditions that are true emergencies. Included in this chapter are acute migraine, other severe nonemergent headaches, facial pain, severe low back pain, acute severe neuropathies such as shingles and diabetic amyotrophy, abdominal pain, and acutely painful muscle conditions requiring prompt attention, such as myositis and rhabdomyolysis. The differential diagnosis of headache with visual impairment is reviewed, as is the differential diagnosis of facial pain. Strategies for the evaluation, diagnosis, and treatment of atypical focal pains that can represent unusual presentations of common problems as well as uncommon conditions are discussed.