AHA Coding Clinic® for ICD-9 - 1984 November - December

Rett's Syndrome

The code assigned for Rett's syndrome is 330.8, Other specified cerebral degenerations in childhood. Code also 294.1, Dementia in conditions classified elsewhere. Note: As of October 1, 2000, code 294.1 has been expanded to the fifth digit level to indicate whether or not there is behavioral disturbances Rett's syndrome is a progressive deterioration of brain functions, seemingly exclusive to girls. It is characterized by autistic behavior, dementia, walking difficulty, loss of facial expression, and stereotyped use of hands. The hand problem includes "hand washing" or "hand wringing" movements in front of the mouth and chest, a...

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