ICD-11 code : 8B60.4;
Preferred Label : Primary lateral sclerosis;
ICD-11 definition : 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.;
ICD-11 inclusion : PLS - [primary lateral sclerosis]; lateral sclerosis;
Origin ID : 1686688462;
UMLS CUI : C0085084;
Automatic exact mappings (from CISMeF team)
- Currated CISMeF NLP mapping
- ICD-10 Mapping
- Semantic type(s)
- UMLS correspondences (same concept)
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.
