ICD-10 Code A81.82 – Gerstmann-Sträussler-Scheinker syndrome (2026): Diagnosis, Symptoms & Billing Guide
2026 ICD-10-CM Diagnosis Code A81.82 – Gerstmann-Sträussler-Scheinker syndrome
What it is
A81.82 identifies Gerstmann-Sträussler-Scheinker syndrome, a rare inherited prion disease that causes progressive brain degeneration. Use it when the record specifically names this diagnosis.
Clinical signs
Typical findings include progressive ataxia, cognitive decline, and other neurological deterioration. Clinical features vary; refer to documentation.
When to use this code
Use A81.82 when the clinician documents Gerstmann-Sträussler-Scheinker syndrome as the confirmed diagnosis or the principal reason for care. It may also apply when the chart clearly states a family history or genetic diagnosis tied to this syndrome, if that is the documented condition being coded. Check documentation for specificity.
Do not use for
Do not use this code for nonspecific dementia, ataxia, or other prion diseases unless Gerstmann-Sträussler-Scheinker syndrome is documented. Do not guess from symptoms alone.
Coding tip
Code the exact documented prion disorder name; if the note is vague, query the provider before assigning A81.82.