G71.11

Applicable To

• Dystrophia myotonica [Steinert]

• Myotonia atrophica

• Myotonic dystrophy

• Proximal myotonic myopathy PROMM)

• Steinert disease

Approximate Synonyms

• Myotonic dystrophy

• Steinert myotonic dystrophy syndrome

Clinical Information

• An autosomal dominant neuromuscular disorder which usually presents in early adulthood, characterized by progressive muscular atrophy most frequently involving the hands, forearms, and face), myotonia, frontal baldness, lenticular opacities, and testicular atrophy. Cardiac conduction abnormalities, diaphragmatic weakness, and mild intellectual disability may also occur. Congenital myotonic dystrophy is a severe form of this disorder, characterized by neonatal muscle hypotonia, feeding difficulties, respiratory muscle weakness, and an increased incidence of intellectual disability. from Adams et al., Principles of Neurology, 6th ed, pp1423-5; Joynt, Clinical Neurology, 1997, ch16, pp16-7)

• An inherited progressive disorder affecting the muscles. It is characterized by muscle wasting and hypotonia, cataracts, heart conduction defects and endocrinopathies.

• Autosomal dominant neuromuscular disorder which usually presents in early adulthood, characterized by progressive muscular atrophy most frequently involving the hands, forearms, and face), myotonia, frontal baldness, lenticular opacities, and testicular atrophy; cardiac conduction abnormalities, diaphragmatic weakness, and mild mental retardation may also occur; congenital myotonic dystrophy is a severe form of this disorder.

Details

• G71.11 is a billable/specific ICD-10-CM code that can be used to indicate a diagnosis for reimbursement purposes.

• The 2023 edition of ICD-10-CM G71.11 became effective on October 1, 2022.

• This is the American ICD-10-CM version of G71.11 – other international versions of ICD-10 G71.11 may differ.