G31.82
BillableLeigh's disease
HCC Category Mapping
V28HCC 127 — Dementia, Mild or Unspecified
0.464RxHCCHCC 112 — Dementia and Other Specified Brain Disorders
0.000What This Code Means
Leigh's disease is a rare inherited neurological disorder affecting energy production in cells, causing progressive brain and muscle deterioration, typically starting in infancy.
Coding Tips
- •This is a mitochondrial disorder; document genetic confirmation when available
- •Code associated symptoms such as seizures, respiratory issues, and cardiac involvement separately
Clinical Significance
Leigh's disease (subacute necrotizing encephalomyelopathy) is a severe inherited mitochondrial disorder affecting cellular energy production. It causes progressive neurological deterioration including loss of motor skills, breathing difficulties, and organ dysfunction. The diagnosis reflects one of the most serious pediatric neurological conditions, requiring intensive multidisciplinary management.
Documentation Requirements
- ✓Documentation specifically identifying Leigh's disease or subacute necrotizing encephalomyelopathy
- ✓Neuroimaging (MRI) showing characteristic bilateral symmetric lesions in basal ganglia and brainstem
- ✓Genetic or metabolic testing confirming mitochondrial dysfunction
- ✓Documentation of progressive neurological symptoms (developmental regression, hypotonia, respiratory compromise)
- ✓Metabolic workup results including lactate levels
- ✓Current treatment plan addressing nutritional, respiratory, and neurological management
Commonly Confused Codes
G31.81 — Alpers disease; another mitochondrial disorder but primarily with seizures and liver failureG23.3 — Hypomyelination with atrophy of basal ganglia and cerebellum; different pathology and imaging patternG31.89 — Other specified degenerative diseases of nervous system; too nonspecific when Leigh's disease is confirmedE88.40 — Mitochondrial metabolism disorder, unspecified; captures the metabolic aspect but not the neurological disease