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Box 1 Inclusion and exclusion criteria for childhood dementia definition*

From: The psychosocial impact of childhood dementia on children and their parents: a systematic review

Inclusion: any child (under 18 years of age at symptom onset) which has any illness that fulfils all the following criteria:

• Multiple losses of already attained cognitive developmental skills

• Duration of illness greater than 3 months

• Skill loss most likely due to CNS dysfunction

• Evidence of generalised (not merely focal) brain dysfunction

• Has a condition which will in the future, in all probability, lead to progressive deterioration as above

Exclusion:

• Conditions associated with static intellectual losses (e.g., infectious, traumatic, or anoxic insults) (a)

• Conditions mainly associated with episodic cognitive impairment (e.g., in the context of acute metabolic crises)

• Conditions with primary cognitive decline because of epilepsy [1, 7] (b)

  1. *Adapted from the only published case definitions identified for childhood dementia
  2. a. Static neurocognitive decline was excluded as the label of static skill loss as dementia was not acceptable to clinicians [1]. This is the rationale for excluding any forms of static or transient cognitive decline such as in infectious or toxic encephalopathies, acute metabolic crises (phenylketonuria, urea cycle disorders), or traumatic and anoxic injuries (head trauma, drowning).
  3. b. Whilst the epileptic encephalopathies can cause episodic cognitive decline, this is not currently seen as temporally progressive neurodegeneration [37,38,39].
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Orphanet Journal of Rare Diseases

ISSN: 1750-1172