ICD-10 Code A50.42 – Late congenital syphilitic encephalitis (2026): Diagnosis, Symptoms & Billing Guide

The ICD-10 code for Late congenital syphilitic encephalitis is A50.42.
2026 ICD-10-CM Diagnosis Code A50.42 – Late congenital syphilitic encephalitis

What it is

A50.42 identifies late congenital syphilitic encephalitis, a neurologic complication of congenital syphilis that appears later in childhood. It indicates brain involvement from untreated or inadequately treated maternal infection.

Clinical signs

Clinical features vary; refer to documentation. You may see neurologic impairment, developmental delay, seizures, or other signs of central nervous system involvement in a child with confirmed congenital syphilis.

When to use this code

Use this code when the provider documents late congenital syphilitic encephalitis as the diagnosis. It fits cases where congenital syphilis has progressed and the encephalitic manifestation is specifically identified. If the record only says congenital syphilis without neurologic involvement, check documentation.

Do not use for

Do not use A50.42 for acquired syphilis, nonspecific encephalopathy, or congenital syphilis without encephalitis. If the chart does not clearly link the neurologic findings to congenital syphilis, you should not assume this code.

Coding tip

Confirm that the provider documents both congenital syphilis and late encephalitic involvement before assigning A50.42.

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