The Gowers sign is the clinical evidence of a decrease in the strength of the proximal muscles of the lower limbs in patients with muscular dystrophy. This sign is present in any pathology that is associated with weakness of the proximal muscles or of the pelvic girdle, such as Duchenne muscular dystrophy, Becker muscular dystrophy and spinal muscular atrophy, among others.
To observe it, the patient should be asked to lie down or sit on the floor and try to get up without using their hands. If you need to support both hands on the floor or on your legs to gain the momentum and strength necessary to reach a standing position, the sign is considered positive.
Process to confirm the Gowers sign. By William Richard Gowers (1845–1915) - Gowers WR. Clinical lecture on pseudohypertrophic muscular paralysis. Lancet 1879; ii, 73-5., Public Domain, In general, the age of diagnosed patients varies between 2 and 7 years, since it is the age at which parents realize the disadvantage in strength and mobility of the child with respect to their peers.
In some inflammatory diseases, such as polymyositis, the sign can be completely reversed with proper treatment.
In the medical literature the eponymous can be found to describe signs of other pathologies such as pupillary asymmetry found in syphilis and pain in the sciatic nerve with passive ankle dorsiflexion. However, it is more used to describe decreased strength in muscular dystrophy.
History
The Gowers sign was described by the English neurologist Sir William Richard Gowers (1845-1915), who is remembered as the greatest clinical neurologist of all time, in the words of the president of the world neurological association, Macdonald Critchley, in 1949.
Sir William Gowers. Public Domain, Between 1870 and 1890, Gowers published a two-volume book titled Manual of Diseases of the Nervous System, which is still used in clinical practice and in which most of his contributions are found.
In 1886, Dr. Gowers described a sign that he observed in 21 patients between the ages of 2 and 5 to diagnose muscle diseases that caused weakness of the muscles of the hip and pelvic girdle. The patients in his study suffered from Duchenne dystrophy.
In the medical literature, some authors describe other Gowers signs in pathologies other than muscular ones, such as: the pupillary Gowers sign, which is observed in syphilis as an asymmetric closure of the pupils in light, and the neurological Gowers sign. which refers to pain in the path of the sciatic nerve to passive dorsiflexion of the ankle.
Although these descriptions correspond to the same eponymous, most authors refer to the Gowers sign associated with muscular dystrophy.
It is to be noted that Sir William Gowers was against the use of eponyms in medical writing, and in this regard he wrote the following:
Gowers sign
In the book Manual of diseases of the central nervous system, Dr. William Gowers described the sign that bears his name and the maneuver to show it.
The patient is asked to sit on the floor and try to get up using only the legs. Any object that she may use to help herself should be removed. If the patient manages to get up without help, the sign is negative.
But often, patients with muscular dystrophy do not have enough knee strength to lift the weight of the trunk on the legs. Therefore, they seek to help themselves by leaning on the floor and "walking" with their hands in the direction of the body.
Positive Gowers sign. By Internet Archive Book Images - https://www.flickr.com/photos/internetarchivebookimages/14595108429/Source book page: https://archive.org/stream/diseasesofchildr00mcco/diseasesofchildr00mcco#page/n278/mode/1up, No restrictions, Once the knees are extended, patients with severe disease cannot find the strength to erect their back. Thus, they will continue to support their hands on their knees and thighs until they are fully standing.
Subsequent research has studied the sign in older patients, between 3 and 7 years old, on the basis that children under 3 years of age may have difficulty getting up due to age-related lack of ability.
Importance
Although the Gowers sign is not specific for any disease, it does show a decrease in muscle strength in the proximal muscles of the lower limbs, which allows the doctor to make a diagnostic approach based on other data.
It is also an easy test to perform, which does not require any special equipment or further training, and which can be included in the routine neurological evaluation, in case some type of dystrophy is suspected.
Clinical considerations
The Gowers sign is associated with muscular dystrophies that cause progressive deterioration with decreased muscle strength.
The most frequent diseases in which it can be evidenced are:
- Duchenne muscular dystrophy
- Becker muscular dystrophy
- Spinal muscular atrophy
- Sacroglycanopathies
- Polymyositis
- Discitis
- Juvenile idiopathic arthritis.
It is usually seen in children older than 2 years, since it is the age at which standing and jumping ability begins. At this stage, parents may notice the difficulty or inability of the child to perform these movements.
When the Gowers sign is positive in a patient between 2 and 3 years old, this should be monitored every 3 to 6 months by neurological consultation to evaluate its evolution.
If no improvement is observed after 3 years, complementary neurological examinations are indicated to study the problem in depth.
Treatment
In general, the diseases in which the Gowers sign is observed are controllable but do not have a treatment that points towards their definitive cure. The therapies are based on slowing the progression of dystrophy and improving the patient's quality of life.
However, there are conditions in which there may be a positive Gowers sign that completely reverses with treatment.
This is the case of poliomyositis, an autoimmune inflammatory myopathy, which can be treated very effectively with high doses of steroids, with a satisfactory evolution for the patient.
Similarly, discitis is a pediatric disease in which the Gowers sign can be positive for reasons other than muscular dystrophy.
Patients with this condition have inflammation of the intervertebral disc of the L4-L5 lumbar vertebrae, due to an infection that has migrated from a primary site, for example a urinary tract or respiratory tract infection.
Discitis in a 2-year-old patient By Hospital for Sick Children, Toronto - Hospital for Sick Children, Toronto, CC BY-SA 3.0, The sign is usually positive because the child has pain when standing and moving the lower limbs, but not because of weakness or decreased muscle strength.
Treatment with antibiotics, anti-inflammatories and analgesics, completely improves the inflammation by reversing the sign.
References
- Shrestha, S; Munakomi, S. (2019). Gowers Sign. StatPearls. Treasure Island (FL). Taken from: ncbi.nlm.nih.gov
- Wallace, G. B; Newton, RW (1989). Gower's sign revisited. Manchester, Great Britain. Taken from: adc.bmj.com
- Pearce, JMS (2000). Gowers' sign. Journal of Neurology, Neurosurgery & Psychiatry. Taken from: jnnp.bmj.com
- Chaustre, D; Chona, W. (2011). Duchenne muscular dystrophy: perspectives from rehabilitation. Revista Med. Taken from: scielo.org.co
- Do, T. (2018). What is the Gower sign in patients with muscular dystrophy? Taken from: medscape.com
- Chang, R. F; Mubarak, SJ (2012). Pathomechanics of Gowers 'sign: a video analysis of a spectrum of Gowers' maneuvers. Clinical orthopedics and related research. Taken from: ncbi.nlm.nih.gov