G40.82

Applicable To

• Infantile spasms

• Salaam attacks

• West’s syndrome

Clinical Information

• A rare autosomal recessive inherited neurodegenerative disorder caused by mutations in the pla2g6 gene. It is characterized by the development of swellings called spheroids along the axons of the central nervous system. Signs and symptoms appear early in life and include movement difficulties, muscle hypotonia and spasticity, and dementia.

• An epileptic syndrome characterized by the triad of infantile spasms, hypsarrhythmia, and arrest of psychomotor development at seizure onset. The majority present between 3-12 months of age, with spasms consisting of combinations of brief flexor or extensor movements of the head, trunk, and limbs. The condition is divided into two forms: cryptogenic idiopathic) and symptomatic secondary to a known disease process such as intrauterine infections; nervous system abnormalities; brain diseases, metabolic, inborn; prematurity; perinatal asphyxia; tuberous sclerosis; etc.). from menkes, textbook of child neurology, 5th ed, pp744-8)

• Myoclonus epilepsy of infancy and early childhood, characterized by seizures involving the muscles of the neck, trunk, and limbs, and manifested by nodding of the head and flexion of the arms. The seizures are associated with brain abnormalities and frequent mental retardation.

Details

• G40.82 should not be used for reimbursement purposes as there are multiple codes below it that contain a greater level of detail.

• The 2023 edition of ICD-10-CM G40.82 became effective on October 1, 2022.

• This is the American ICD-10-CM version of G40.82 – other international versions of ICD-10 G40.82 may differ.