Frontotemporal dementia

Description

Frontotemporal dementia (FTD) is a group of primary neurodegenerative disorders primarily affecting the frontal and temporal lobes. Onset is typically insidious with a gradual and worsening course. Several syndromic variants (some with an identified genetic basis or familiality) are described that include presentations with predominantly marked personality and behavioral changes (such as executive dysfunction, apathy, deterioration of social cognition, repetitive behaviours, and dietary changes), predominantly language deficits (that include semantic, agrammatic/nonfluent, and logopenic forms), predominantly movement-related deficits (progressive supranuclear palsy, corticobasal degeneration, multiple systems atrophy, or amyotrophic lateral sclerosis), or a combination of these deficits. Memory function often remains relatively intact, particularly during the early stages of the disorder.

Coding note

This category should never be used in primary tabulation. The codes are provided for use as supplementary or additional codes when it is desired to identify the presence of dementia in diseases classified elsewhere. When dementia is due to multiple aetiologies, code all that apply.

Coding note from above levels

This category should only be used for primary tabulation, if the aetiology of the dementia is unknown. If the aetiology of the dementia is known code to the aetiology of the dementia for primary tabulation. When dementia is due to multiple aetiologies, code all that apply.

Exclusions from above levels

• 6A00 – Disorders of intellectual development
• 6D70 – Delirium
• http://id.who.int/icd/entity/1516623224 – Neurodevelopmental disorders