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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Note:  The following article synopsis was NOT provided by the AHA. It was created by Find-A-Code/innoviHealth.

Article Overview

This article explains the topic of Rett's syndrome from a coding and clinical context. It is relevant to coders, auditors, and other healthcare professionals who need to understand how the condition is referenced in diagnosis coding guidance and what broad clinical features are associated with it. The article also notes an update tied to a date-specific expansion of one referenced diagnosis code and discusses general features, progression, and diagnostic considerations.

Why This Topic Matters

Rett's syndrome is a complex neurologic condition with specific diagnosis coding references and time-sensitive coding guidance. Understanding the article helps users recognize the relevant diagnosis categories and the general clinical context without needing to consult the full premium content.

What You Will Learn

  • The diagnosis coding references associated with Rett's syndrome.
  • The general clinical picture and progression described for the condition.
  • A date-specific update affecting one referenced diagnosis code.
  • The broad types of disorders that were considered in the evaluation discussed.

Who Should Read This

  • Medical coders
  • Coding auditors
  • Clinical documentation improvement staff
  • Healthcare billers
  • Compliance professionals
  • Healthcare clinicians

Codes Discussed

  • ICD-9-CM: 330.8
  • ICD-9-CM: 294.1

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