Diffuse sclerosis of central nervous system

This code represents a rare, progressive neurological disorder characterized by widespread demyelination within the central nervous system. It involves the destruction of the myelin sheath, leading to impaired nerve signal transmission and a variety of neurological deficits. This condition is distinct from multiple sclerosis due to its diffuse and often more severe nature.

Assign this code when documentation explicitly states "diffuse sclerosis of central nervous system" or a synonymous term like "Schilder's disease" or "encephalitis periaxialis diffusa." This diagnosis is typically made based on clinical presentation, neuroimaging findings (e.g., MRI showing extensive demyelination), and sometimes biopsy. Ensure the physician's notes clearly differentiate this from other demyelinating diseases.

• Do not code with X-linked adrenoleukodystrophy (E71.52-) due to the Excludes1 note.
• Distinguish carefully from multiple sclerosis (G35) or other localized demyelinating conditions.
• Look for specific diagnostic terms in the physician's documentation to support this rare diagnosis.