ICD-11 classes
08 Diseases of the nervous system
Spinal cord disorders excluding trauma
8B44 — Degenerative myelopathic disorders
8B60.4 — Primary lateral sclerosis

ICD-11 8B60.4 — Primary lateral sclerosis

Primary lateral sclerosis (PLS) is a rare motor neuron disease variant which presents with slowly progressive UMN signs, such as spastic gait, brisk deep tendon jerks, and extensor plantar responses. Onset is most commonly with spastic paraparesis, but patients typically progress to develop upper limb and bulbar involvement. The characteristic feature of PLS is the complete absence of involvement of lower motor neuron involvement. When LMN signs develop during the course of the disease, the diagnosis will change to ALS, and they are considered a spectrum of the same disorder.

The diagnosis includes nothing.

The diagnosis excludes nothing.

It has no clarifying diagnoses.

The diagnosis is coded elsewhere:

• Motor neuron disease #7177