Familial motor neuron disease

This code identifies a progressive neurodegenerative disorder characterized by the degeneration of motor neurons, leading to muscle weakness, atrophy, and spasticity, with a clear hereditary pattern. It encompasses various genetic forms of motor neuron disease where a familial link is established. The condition typically results in increasing disability and often affects both upper and lower motor neurons.

Assign this code when documentation explicitly states a diagnosis of familial motor neuron disease, indicating a genetic predisposition or inherited form of the condition. Supporting documentation should include genetic testing results, family history, and neurological examination findings consistent with motor neuron degeneration. Use this code for patients presenting with symptoms of motor neuron disease where a familial component has been confirmed or strongly suspected by the treating physician.

• Ensure documentation clearly distinguishes familial forms from sporadic motor neuron disease.
• Do not use this code for sporadic amyotrophic lateral sclerosis (ALS) unless a familial link is specified.
• Consider sequencing this code as the primary diagnosis when it is the main reason for the encounter.