G11.6
BillableLeukodystrophy with vanishing white matter disease
HCC Category Mapping
V28HCC 200 — Cerebellar Ataxia and Other Degenerative Diseases of Nervous System
0.262V24HCC 72 — Spinal Cord Disorders/Injuries
0.464ESRDHCC 72 — Spinal Cord Disorders/Injuries
0.000What This Code Means
Leukodystrophy with vanishing white matter disease is a rare genetic disorder where the protective coating of nerve fibers in the brain progressively deteriorates, causing neurological decline.
Coding Tips
- •Document the age of onset and progression pattern to support the diagnosis
- •Code any associated symptoms (e.g., seizures, cognitive decline, ataxia) separately if clinically relevant
Clinical Significance
Leukodystrophy with vanishing white matter disease is a progressive genetic disorder where white matter in the brain deteriorates, especially during febrile illness or minor head trauma. This condition leads to progressive neurological decline and is often fatal, requiring intensive neurological management and family support services.
Documentation Requirements
- ✓Brain MRI demonstrating vanishing white matter pattern (rarefaction of cerebral white matter)
- ✓Genetic testing results for EIF2B gene mutations if available
- ✓Documentation of episodic deterioration triggered by fever or trauma
- ✓Progressive neurological decline documented with timeline
- ✓Age of onset and rate of progression
- ✓Current functional status and supportive care needs
Commonly Confused Codes
G11.5 — 4H syndrome: different leukodystrophy with hypomyelination pattern, not vanishing white matterE75.25 — Metachromatic leukodystrophy: different enzymatic leukodystrophy with different MRI patternG37.0 — Diffuse sclerosis of central nervous system: an acquired demyelinating process, not geneticE75.23 — Krabbe disease: another leukodystrophy but with different genetic basis and clinical course
Code Hierarchy
└G11Hereditary ataxia└G11.6Leukodystrophy with vanishing white matter disease
└G11.6Leukodystrophy with vanishing white matter disease