Duchenne muscular dystrophy natural history, complications and prognosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Fahimeh Shojaei, M.D.

Overview

If left untreated, 100% of patients with Duchenne muscular dystrophy may progress to develop heart failure, respiratory failure, and death. Common complications of Duchenne muscular dystrophy include cardiomyopathy with heart failure, respiratory failure, cataracts, decreased movement, depression, contractures, mental impairment, scoliosis, and failure to thrive. Prognosis is generally poor, and the mortality rate of patients with Duchenne muscular dystrophy is approximately 100%.

Natural History, Complications, and Prognosis

Natural History

The symptoms of Duchenne muscular dystrophy usually develop in the first decade of life, and start with symptoms such as muscle weakness and gait abnormalities.^[1]
If left untreated, 100% of patients with Duchenne muscular dystrophy may progress to develop heart failure, respiratory failure, and death.

Complications

Common complications of Duchenne muscular dystrophy include:^[2]^[3]^[4]^[5]
- Cardiomyopathy with heart failure
- Respiratory failure
- Cataracts
- Decreased movement
- Depression
- Contractures
- Mental impairment
- Scoliosis
- Failure to thrive

Prognosis

Prognosis is generally poor, and the mortality rate of patients with Duchenne muscular dystrophy is approximately 100%.^[6]
The presence of low vital capacity or a sharp decline of vital capacity is associated with a particularly poor prognosis among patients with Duchenne muscular dystrophy.

References

↑ Liang WC, Wang CH, Chou PC, Chen WZ, Jong YJ (April 2018). “The natural history of the patients with Duchenne muscular dystrophy in Taiwan: A medical center experience”. Pediatr Neonatol. 59 (2): 176–183. doi:10.1016/j.pedneo.2017.02.004. PMID 28903883.
↑ Nigro, G.; Comi, L.I.; Politano, L.; Bain, R.J.I. (1990). “The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy”. International Journal of Cardiology. 26 (3): 271–277. doi:10.1016/0167-5273(90)90082-G. ISSN 0167-5273.
↑ Birnkrant DJ, Bushby K, Bann CM, Alman BA, Apkon SD, Blackwell A, Case LE, Cripe L, Hadjiyannakis S, Olson AK, Sheehan DW, Bolen J, Weber DR, Ward LM (April 2018). “Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management”. Lancet Neurol. 17 (4): 347–361. doi:10.1016/S1474-4422(18)30025-5. PMC 5889091. PMID 29395990.
↑ Fitzpatrick, Carol; Barry, Ciaran; Garvey, Criona (2008). “PSYCHIATRIC DISORDER AMONG BOYS WITH DUCHENNE MUSCULAR DYSTROPHY”. Developmental Medicine & Child Neurology. 28 (5): 589–595. doi:10.1111/j.1469-8749.1986.tb03900.x. ISSN 0012-1622.
↑ Smith AD, Koreska J, Moseley CF (August 1989). “Progression of scoliosis in Duchenne muscular dystrophy”. J Bone Joint Surg Am. 71 (7): 1066–74. PMID 2760082.
↑ Visser, Jeldican; van den Berg-Vos, Renske M.; Franssen, Hessel; van den Berg, Leonard H.; Wokke, John H.; Vianney de Jong, J. M.; Holman, Rebecca; de Haan, Rob J.; de Visser, Marianne (2007). “Disease Course and Prognostic Factors of Progressive Muscular Atrophy”. Archives of Neurology. 64 (4): 522. doi:10.1001/archneur.64.4.522. ISSN 0003-9942.

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[pmid28903883-1] Liang WC, Wang CH, Chou PC, Chen WZ, Jong YJ (April 2018). “The natural history of the patients with Duchenne muscular dystrophy in Taiwan: A medical center experience”. Pediatr Neonatol. 59 (2): 176–183. doi:10.1016/j.pedneo.2017.02.004. PMID 28903883.

[NigroComi1990-2] Nigro, G.; Comi, L.I.; Politano, L.; Bain, R.J.I. (1990). “The incidence and evolution of cardiomyopathy in Duchenne muscular dystrophy”. International Journal of Cardiology. 26 (3): 271–277. doi:10.1016/0167-5273(90)90082-G. ISSN 0167-5273.

[pmid29395990-3] Birnkrant DJ, Bushby K, Bann CM, Alman BA, Apkon SD, Blackwell A, Case LE, Cripe L, Hadjiyannakis S, Olson AK, Sheehan DW, Bolen J, Weber DR, Ward LM (April 2018). “Diagnosis and management of Duchenne muscular dystrophy, part 2: respiratory, cardiac, bone health, and orthopaedic management”. Lancet Neurol. 17 (4): 347–361. doi:10.1016/S1474-4422(18)30025-5. PMC 5889091. PMID 29395990.

[FitzpatrickBarry2008-4] Fitzpatrick, Carol; Barry, Ciaran; Garvey, Criona (2008). “PSYCHIATRIC DISORDER AMONG BOYS WITH DUCHENNE MUSCULAR DYSTROPHY”. Developmental Medicine & Child Neurology. 28 (5): 589–595. doi:10.1111/j.1469-8749.1986.tb03900.x. ISSN 0012-1622.

[pmid2760082-5] Smith AD, Koreska J, Moseley CF (August 1989). “Progression of scoliosis in Duchenne muscular dystrophy”. J Bone Joint Surg Am. 71 (7): 1066–74. PMID 2760082.

[Visservan_den_Berg-Vos2007-6] Visser, Jeldican; van den Berg-Vos, Renske M.; Franssen, Hessel; van den Berg, Leonard H.; Wokke, John H.; Vianney de Jong, J. M.; Holman, Rebecca; de Haan, Rob J.; de Visser, Marianne (2007). “Disease Course and Prognostic Factors of Progressive Muscular Atrophy”. Archives of Neurology. 64 (4): 522. doi:10.1001/archneur.64.4.522. ISSN 0003-9942.

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