Primary lateral sclerosis

This code represents Primary Lateral Sclerosis (PLS), a rare, progressive neurodegenerative disease characterized by the gradual weakening and stiffness of voluntary muscles due to the degeneration of upper motor neurons. It primarily affects the motor cortex, brainstem, and spinal cord, leading to spasticity and difficulty with movement without significant muscle atrophy or sensory involvement.

Use this code for patients diagnosed with PLS, a distinct clinical entity from Amyotrophic Lateral Sclerosis (ALS) due to the absence of lower motor neuron signs. Documentation should clearly state a diagnosis of primary lateral sclerosis, often supported by neurological examination findings of spasticity, hyperreflexia, and pathological reflexes, with electromyography (EMG) showing no evidence of denervation.

• Differentiate carefully from Amyotrophic Lateral Sclerosis (ALS); PLS is characterized by only upper motor neuron involvement.
• Ensure documentation explicitly states "primary lateral sclerosis" to avoid confusion with other motor neuron diseases.
• This code is typically a primary diagnosis, reflecting the patient's underlying neurological condition.