G31.81
BillableAlpers disease
HCC Category Mapping
V28HCC 127 — Dementia, Mild or Unspecified
0.464RxHCCHCC 112 — Dementia and Other Specified Brain Disorders
0.000What This Code Means
Alpers disease is a rare inherited neurological disorder that causes progressive brain degeneration, seizures, and liver dysfunction, typically beginning in infancy or early childhood.
Coding Tips
- •This is a specific genetic disorder; ensure documentation confirms Alpers disease diagnosis
- •Code any associated seizures and liver involvement separately with appropriate ICD-10 codes
Clinical Significance
Alpers disease is a rare, severe mitochondrial disorder causing progressive brain degeneration, intractable seizures, and liver failure. It typically presents in infancy or early childhood and is ultimately fatal. The diagnosis carries extremely high resource utilization including intensive neurology, hepatology, and palliative care management, making accurate capture critical for risk adjustment.
Documentation Requirements
- ✓Documentation specifically identifying Alpers disease (also known as Alpers-Huttenlocher syndrome)
- ✓Genetic testing confirming POLG gene mutation if available
- ✓Clinical documentation of the triad: progressive neurological degeneration, intractable seizures, and liver dysfunction
- ✓EEG findings if available showing characteristic patterns
- ✓Documentation of associated liver involvement with liver function test results
- ✓Current treatment plan including seizure management and supportive care
Commonly Confused Codes
G31.82 — Leigh's disease; another mitochondrial disorder but with different clinical presentation and imaging findingsG31.89 — Other specified degenerative diseases of nervous system; less specific and should not be used when Alpers is confirmedG40.019 — Intractable epilepsy; may be coded additionally but does not capture the underlying Alpers diseaseG31.80 — Leukodystrophy, unspecified; different pathological mechanism (white matter vs. gray matter)