8A40.1

ICD-11 MMS 2026-01

Primary progressive multiple sclerosis

Description

Disease progression from onset, with occasional plateaus and temporary minor improvements allowed.

Coding note

This category is to be used to indicate Primary progressive multiple sclerosis which is progressive from onset but includes progressive - relapsing, or is progressive from onset with a single relapse

Index terms

  • Primary progressive multiple 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

  • 8A40.0 – Relapsing-remitting multiple sclerosis
  • 8A40.2 – Secondary progressive multiple sclerosis
  • 8A40.Y – Other specified multiple sclerosis
  • 8A40.Z – Multiple sclerosis, unspecified

Foundation entity

Primary progressive multiple sclerosis – http://id.who.int/icd/entity/1020720762

Allowed postcoordination axes

Part of: 8A40: Multiple sclerosis › 08: Diseases of the nervous system