CALIBER phenotype

person Phenotype Rare or unspecified dementia
person Type Disease or syndrome
person Data sources Primary care (CPRD), hospital admission data (HES)
person Clinical Terminologies Read, ICD-9, ICD-10
person Valid event date range 01/01/1999 - 01/07/2016
person Sex Female/Male
person Agreed 23.11.2012 (Revision 2)
person Authors Mar Pujades-Rodriguez, Valentina Assi, Arturo Gonzalez-Izquierdo, Tim Wilkinson, Christian Schnier, Cathie Sudlow, Harry Hemingway, William N. Whiteley

Primary Care (incident and prevalent)

In the Clinical Practice Research Datalink (CPRD, primary care data) we ascertained cases by searching for Read terms related to:

Rare dementia
Eu02400	[X]Dementia in human immunodef virus [HIV] disease
Eu02100	[X]Dementia in Creutzfeldt-Jakob disease
Eu02200	[X]Dementia in Huntington's disease
Eu02000	[X]Dementia in Pick's disease
F111.00	Pick's disease
Eu02300	[X]Dementia in Parkinson's disease
Eu02500	[X]Lewy body dementia
F116.00	Lewy body disease
Unspecified dementia
E002z00	Senile dementia with depressive or paranoid features NOS
Eu02z11	[X] Presenile dementia NOS
Eu02y00	[X]Dementia in other specified diseases classif elsewhere
E001000	Uncomplicated presenile dementia
E001100	Presenile dementia with delirium
E012.00	Other alcoholic dementia
Eu04100	[X]Delirium superimposed on dementia
Eu02z13	[X] Primary degenerative dementia NOS
E001300	Presenile dementia with depression
E001200	Presenile dementia with paranoia
Eu02z16	[X] Senile dementia, depressed or paranoid type
E001z00	Presenile dementia NOS
E002.00	Senile dementia with depressive or paranoid features
E003.00	Senile dementia with delirium
Eu10711	[X]Alcoholic dementia NOS
E012.11	Alcoholic dementia NOS
E041.00	Dementia in conditions EC
E002000	Senile dementia with paranoia
Eu02.00	[X]Dementia in other diseases classified elsewhere
E002100	Senile dementia with depression
E001.00	Presenile dementia
9hD0.00	Excepted from dementia quality indicators: Patient unsuitabl
Eu02z14	[X] Senile dementia NOS
E000.00	Uncomplicated senile dementia
Eu02z00	[X] Unspecified dementia
E00..11	Senile dementia
E00..12	Senile/presenile dementia
E02y100	Drug-induced dementia
Read terms are hierarhically organized in top-level chapters i.e. chapter G....00 is related to Circulatory System Diseases and sub-headings i.e. heading G2...00 is related to Hypertensive Heart Disease while G3...00 is related to Ischaemic Heart Disease.

Secondary Care (incident and prevalent)

In Hospital Episode Statistics (HES, hospital admission data) we used ICD-10 terms (see below) for diagnosis when marked as the primary diagnosis i.e. the main condition treated or investigated during the relevant episode of healthcare. We used the date of admission to hospital as the date of the event. We additionally searched for OPCS-4 terms indicating releted procedures.

Rare dementia
F020A	Dementia in Pick's disease
F021A	Dementia in Creutzfeldt-Jakob disease
F022A	Dementia in Huntington's disease
F023A	Dementia in Parkinson's disease
F024A	Dementia in human immunodef virus [HIV] disease
F028A	Dementia in other specified diseases classified elsewhere
046.19	Creutzfeld Jacob
333.4	Huntingdon's
331.1	Frontotemporal dementia
Unspecified dementia
F02	Dementia in other diseases classified elsewhere
F03X	Unspecified dementia
F051	Delirium superimposed on dementia
290.0	Senile dementia, uncomplicated
290.1x	Presenile dementia
290.2	Senile dementia with delusional features
290.3	Senile dementia with delirium

Mortality data

In mortality data, we used ICD-9 and ICD-10 terms (see below) at any position (underlying or not):

F00	Dementia in Alzheimer's disease
F000A	Dementia in Alzheimer's disease with early onset
F001A	Dementia in Alzheimer's disease with late onset
F002A	Dementia in Alzheimer's disease, atypical or mixed type
F009A	Dementia in Alzheimer's disease, unspecified
G30	Alzheimer’s disease
G30.0	Alzheimer’s disease with early onset
G30.1	Alzheimer’s disease with late onset
G30.8	Other Alzheimer disease, unspecified
G30.9	Alzheimer's disease unspecified
331.0	Alzheimer's disease        

Combining evidence across sources to define and date phenotypes

We defined dementia as the record of one or more diagnostic codes in any of the three data sources at any time and in any position (i.e. dementia was any of the recorded diagnosis in hospital admission or death record). We defined people with corroborating evidence of diagnosis if they had dementia with: (i) more than one record of dementia in the same data source, on different dates; or (ii) a record of dementia in 2 or 3 data sources; or (iii) a record of falls, confusion, memory problems or nursing home admission; or (iv) dementia monitoring codes in primary care; or (v) a referral to a dementia speciality (geriatrics, care of the elderly, psychiatry); or (v) more than one prescription of rivastigmine, galantamine, donepezil, memantine, which are typically used to treat patients with Alzheimer’s disease and sometimes patients with dementia in Parkinson’s disease and dementia with Lewy bodies.
Pujades-Rodriguez, Mar, Valentina Assi, Arturo Gonzalez-Izquierdo, Tim Wilkinson, Christian Schnier, Cathie Sudlow, Harry Hemingway, and William N. Whiteley. "The diagnosis, burden and prognosis of dementia: A record-linkage cohort study in England." PloS one 13, no. 6 (2018): e0199026.
Pujades-Rodriguez, Mar, Valentina Assi, Arturo Gonzalez-Izquierdo, Tim Wilkinson, Christian Schnier, Cathie Sudlow, Harry Hemingway, and William N. Whiteley. "The diagnosis, burden and prognosis of dementia: A record-linkage cohort study in England." PloS one 13, no. 6 (2018): e0199026.
Pujades-Rodriguez, Mar, Valentina Assi, Arturo Gonzalez-Izquierdo, Tim Wilkinson, Christian Schnier, Cathie Sudlow, Harry Hemingway, and William N. Whiteley. "The diagnosis, burden and prognosis of dementia: A record-linkage cohort study in England." PloS one 13, no. 6 (2018): e0199026.