8B60.4

ICD-11 MMS 2026-01

Primary lateral sclerosis

Description

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.

Index terms

  • Primary lateral sclerosis
  • PLS - [primary lateral sclerosis]
  • lateral complete paralysis
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  • lateral incomplete paralysis
  • lateral paralysis
  • lateral sclerosis
  • lateral spinal sclerosis

Exclusions from above levels

  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 19 – Certain conditions originating in the perinatal period
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  • 22 – Injury, poisoning or certain other consequences of external causes

Sibling codes

  • 8B60.0 – Amyotrophic lateral sclerosis
  • 8B60.1 – Progressive bulbar palsy
  • 8B60.2 – Progressive pseudobulbar palsy
  • 8B60.3 – Progressive muscular atrophy
  • 8B60.5 – Amyotrophic lateral sclerosis-Plus
  • 8B60.6 – Monomelic amyotrophy
  • 8B60.7 – Madras type motor neuron disease
  • 8B60.Y – Other specified motor neuron disease
  • 8B60.Z – Motor neuron disease, unspecified

Foundation entity

Primary lateral sclerosis – http://id.who.int/icd/entity/1686688462

Allowed postcoordination axes

Part of: 8B60: Motor neuron disease › 08: Diseases of the nervous system