Gower's sign

Gower's sign is a medical sign that indicates weakness of the proximal muscles, particularly those of the lower limb. The sign is named after British neurologist Sir William Richard Gower (1845–1915), who first described it. Gower's sign is most commonly associated with Duchenne Muscular Dystrophy (DMD), but it can also be observed in other conditions that cause proximal muscle weakness.

Presentation

Gower's sign is evident when a person attempts to rise from a sitting or squatting position. Due to weakness in the proximal muscles of the legs, individuals with this sign cannot get up without assistance. The classic maneuver to observe Gower's sign involves the person using their hands and arms to "walk" up their own body from the knees to the thighs, in order to push themselves into a standing position. This is often referred to as the "Gower's maneuver."

Pathophysiology

The underlying cause of Gower's sign is a significant weakness in the proximal muscles of the lower limbs. In conditions like Duchenne Muscular Dystrophy, there is progressive degeneration and weakness of muscle tissue, which makes it difficult for affected individuals to perform tasks that require muscle strength, such as standing up from a sitting position. The use of the hands to assist in standing is a compensatory technique to overcome this weakness.

Associated Conditions

While Gower's sign is most commonly associated with Duchenne Muscular Dystrophy, it can also be seen in other muscular dystrophies and conditions that lead to proximal muscle weakness. These conditions include, but are not limited to:

• Becker Muscular Dystrophy
• Spinal Muscular Atrophy
• Polymyositis
• Dermatomyositis

Diagnosis

Observation of Gower's sign is a critical component in the physical examination of individuals suspected to have muscular dystrophy or other neuromuscular disorders. The presence of this sign prompts further diagnostic evaluation, including genetic testing, muscle biopsy, and electromyography (EMG), to identify the specific type of muscular dystrophy or neuromuscular condition.

Treatment

There is currently no cure for many of the conditions associated with Gower's sign, including Duchenne Muscular Dystrophy. Treatment focuses on managing symptoms, slowing the progression of muscle weakness, and improving quality of life. This may include physical therapy, occupational therapy, use of assistive devices, and medications such as corticosteroids to help slow muscle degeneration.

Prognosis

The prognosis for individuals with Gower's sign varies depending on the underlying condition. For example, individuals with Duchenne Muscular Dystrophy typically experience progressive muscle weakness leading to loss of ambulation and the need for ventilatory support. Early intervention and comprehensive management can help improve quality of life and extend lifespan.