What is Gowers sign in a young male patient with suspected Duchenne Muscular Dystrophy (DMD)?

What is Gowers Sign

Gowers sign is a clinical maneuver where a patient uses their hands to "climb up" their own thighs when rising from the floor to standing position, compensating for weakness of the pelvic girdle and paravertebral muscles. 1

Clinical Description and Mechanism

The sign reflects proximal muscle weakness and involves a characteristic sequence of movements:

• The patient assumes a prone crawl position and uses one or both hands on the thigh to push themselves upward 2
• This compensatory mechanism reduces the hip extension moment and keeps forces anterior to the knee joint, improving balance during the rise 2
• The maneuver allows patients to overcome weakness of the gluteus maximus, gluteus medius, and paravertebral muscles that would normally power standing from a floor position 3, 4

Spectrum of Severity

Gowers sign exists on a spectrum from mild to severe presentations:

Mild Presentation

• Prolonged rise time or single-hand action on the thigh 2
• Exaggerated torso flexion, wide base, and equinus posturing are the earliest changes 2

Moderate Presentation

• Forming prone crawl position and using one or two hands on thigh 2
• Wide hip abduction, shifts in pelvic tilt, and lordosis become apparent 2

Severe (Classic) Presentation

• More than two thigh maneuvers, rising with additional aid, or inability to rise 2
• Exaggeration of all compensatory mechanisms described above 2

Clinical Significance in DMD

In the context of suspected Duchenne Muscular Dystrophy, Gowers sign is a key diagnostic indicator:

• The presence of Gowers sign in a male child should trigger immediate diagnostic investigation for DMD, especially if accompanied by a waddling gait 1
• The sign is particularly significant in children under 5 years of age and warrants creatine kinase testing 1
• According to the European Society of Cardiology, Gowers sign is one of the cardinal features that should raise suspicion for DMD in children without a family history 5

Early Detection Importance

• The earliest postural change in DMD gait is increased lumbar lordosis secondary to gluteus maximus weakness, which precedes the full Gowers sign 3
• Gowers sign appears in the early stage of DMD when patients still have relatively competent quadriceps but demonstrate gluteus maximus weakness 3
• More subtle forms including mild hand pressure against the thigh should be recognized by clinicians to initiate additional diagnostic testing 2

Important Caveats

Gowers sign is not pathognomonic for DMD and can occur in other conditions:

• Lumbar disc space infection in children can produce Gowers sign as patients avoid painful lumbar spine motion 4
• Various other neuromuscular diseases can manifest this sign 2
• The sign disappears after treatment in non-neuromuscular causes like discitis 4

Diagnostic Pathway When Gowers Sign is Present

When Gowers sign is identified in a male child:

• Immediately measure serum creatine kinase levels, which are markedly elevated (>10,000 U/L) in DMD 1, 5
• Proceed to genetic testing for dystrophin gene deletions/duplications if CK is elevated 5, 6
• Consider muscle biopsy if genetic testing is negative to evaluate dystrophin protein expression 5, 6