Diabetic Amyotrophy: From the Basics to the Bedside

Abstract

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 (Bruns-Garland Syndrome)

“…For this reason, multiple other nomenclature have been described for it: Proximal diabetic neuropathy,[ 6 7 8 ] diabetic proximal amyotrophy,[ 9 ] diabetic lumbosacral plexopathy,[ 10 11 ] femoral-sciatic neuropathy[ 12 ] and femoral neuropathy,[ 13 14 ] and diabetic lumbosacral radioculo-plexo-neuropathy. [ 15 ] There is some correctness in all of these, but we support the eponym Bruns-Garland syndrome. This not only prevents future confusion, but also follows the working precedent established for diseases like Guillain-Barré syndrome and Emery-Dreifuss muscular dystrophy, where descriptive terms are misleading.…”

“…It occurs within a few years of diagnosis of diabetes mellitus (median: 3 years). [ 15 ] Occasionally, its presence sometimes led to the diabetes diagnosis and is commoner with type 2 diabetes. The blood sugar control is usually good with no concomitant microvascular complications (nephropathy and retinopathy) are present.…”

“…Its cardinal symptoms included acute onset of severe proximal lower extremity pain followed by weakness, mild-to-moderate sensory loss, weight loss, and autonomic symptoms. [ 15 ] Pain involves the thigh, hip, or back and is unilateral in most cases (approximately 70%) and is asymmetric in bilateral cases. It is severe in intensity and deep aching in character (with or without paresthesia).…”

“…Progression in the involved limb to cause distal weakness occurs in 60% of cases. [ 15 ] The afflicted region has loss of reflexes. Distal symmetric sensory involvement is also common, but not clinically useful because of its nonlocalizing value.…”

“…However, approximately 10% patients might continue to be dependent for ambulation with incomplete recovery after 2 years of onset. [ 15 ]…”