The phrase "Erbs atrophy" refers to a type of muscle atrophy that begins in the shoulder and typically affects the upper arm muscles. The spelling of this phrase can be explained using the International Phonetic Alphabet (IPA) as /ɜːbz ˈætrəfi/. This indicates that the first word is pronounced with a silent "h" (represented by the symbol "ː") and the second word is pronounced with a stressed first syllable (represented by the symbol "ˈ") and a short "a" sound (represented by the symbol "æ").
Erb's atrophy, also known as Erb's muscular dystrophy, refers to a rare genetic disorder characterized by progressive muscle weakness and wasting. It is named after Wilhelm Heinrich Erb, a German neurologist who first described the condition in the late 19th century.
Erb's atrophy primarily affects the muscles of the shoulder girdle and upper arm, leading to weakness in these areas. It is typically caused by mutations in the SMN1 gene, which plays a crucial role in the production of a protein called survival motor neuron (SMN). The lack of SMN protein results in the degeneration and reduction in size (atrophy) of the affected muscles.
The symptoms of Erb's atrophy usually manifest during early childhood, with affected individuals experiencing difficulty in performing activities requiring upper body strength, such as lifting objects, reaching overhead, and combing hair. Over time, muscle weakness may progress, leading to further limitations in mobility and functional abilities.
Although there is currently no cure for Erb's atrophy, treatment options focus on managing symptoms and promoting a better quality of life. Physiotherapy and occupational therapy can help maintain muscle strength and improve mobility. Assistive devices, such as braces or wheelchairs, may be necessary as the condition progresses. Additionally, genetic counseling can provide valuable information for affected individuals and their families regarding the inheritance and potential risks of the disorder.
In summary, Erb's atrophy is a genetic disorder characterized by progressive muscle weakness and wasting, primarily affecting the shoulder girdle and upper arm. While there is no cure, various interventions can help manage symptoms and improve the overall well-being of affected individuals.
Progressive muscular dystrophy.
A practical medical dictionary. By Stedman, Thomas Lathrop. Published 1920.
The term "Erbs atrophy" is derived from the combined names of two physicians who first described the condition - Wilhelm Heinrich Erb and Friedrich Gustav Jakob Ernst Schwalbe.
Wilhelm Heinrich Erb (1840-1921) was a German neurologist, known for his contributions in the field of neuromuscular disorders. Friedrich Gustav Jakob Ernst Schwalbe (1849-1916) was a German anatomist and pathologist. They collaborated in the late 19th century to investigate and describe a particular form of muscular atrophy that affects the shoulder and arm muscles, primarily in newborns.
The condition was later named "Erbs palsy" or "Erb-Duchenne palsy" after Erb himself. However, over time, the term "Erbs atrophy" began to be used to specifically refer to the muscular atrophy associated with this condition.