Subacute sclerosing panencephalitis

This code represents subacute sclerosing panencephalitis (SSPE), a rare, progressive, and fatal neurodegenerative disease of the central nervous system. It is a late complication of measles infection, characterized by inflammation and demyelination of the brain.

Use this code for documented cases of SSPE, typically presenting years after an initial measles infection. Documentation should clearly indicate the diagnosis of SSPE, often supported by neurological findings, EEG abnormalities, and elevated measles antibody titers in CSF and serum.

• Ensure documentation explicitly states "subacute sclerosing panencephalitis" to support this specific code.
• Do not confuse with acute encephalitis or other forms of panencephalitis; SSPE has a distinct etiology and progression.
• Consider sequencing with a history of measles (V12.01) if documented and relevant to the patient's history.