LMNB1-related autosomal dominant leukodystrophy

This code identifies an inherited neurological disorder characterized by progressive demyelination of the central nervous system, specifically linked to mutations in the LMNB1 gene. It results in a range of symptoms including motor dysfunction, cognitive decline, and spasticity, typically with an adult onset.

Use this code when documentation confirms a diagnosis of LMNB1-related autosomal dominant leukodystrophy, often supported by genetic testing results. This applies to patients presenting with the characteristic clinical features and a confirmed genetic basis for the condition.

• Ensure genetic testing results or a clear clinical diagnosis from a neurologist explicitly state "LMNB1-related" to support this specific code.
• Do not confuse this with other types of leukodystrophies without a confirmed LMNB1 mutation; use more general or specific codes as appropriate.
• Sequence this code as a primary diagnosis when it is the reason for the encounter, or as a secondary diagnosis when managing complications or manifestations of the condition.