[Epidemiology of progressive intellectual and neurological deterioration in childhood. A multicentre study in the Community of Valencia].
BACKGROUND
Progressive intellectual and neurological deterioration (PIND) in childhood has an incidence of 0.5 per thousand live births. Its impact on morbimortality is important. The aim of this work is to study the epidemiology of PIND and the most important causes of this process.


METHODS
A retrospective and multicentre study was carried out in different hospitals of the Valencian Community (Spain).


METHODS
age less than 18 years with a progressive loss of intellectual or development abilities, previously acquired for at least 3 months.


RESULTS
Nine hospitals participated in the study and a total of 85 cases were included. The mean age at the onset of symptoms was 1.8 years, and the mean age at diagnosis was 3.6 years. In 68% of cases there was an intellectual or developmental delay before the onset of symptoms. The mortality rate was 16.4%. A diagnosis was achieved in 74% of the cases. The most frequently found cause was Rett syndrome. More than two-thirds (68%) of diagnosed cases were due to a metabolic disease.


CONCLUSIONS
The existence of a previous development delay and the early onset of the PIND can make it difficult to identify. Like the rest of the series, the largest number of diagnosed cases involved a metabolic disease.


CONCLUSIONS
PIND has an early onset, and in a large percentage of cases the origin remains unknown and with a high mortality rate.
Unidad de Neuropediatría, Hospital Universitari i Politècnic La Fe, Valencia, Spain. tomas_mig@gva.es
M
M
Tomás Vila
I
I
Vitoria Miñana
F
F
Gomez Gosalvez
J
J
Pantoja Martinez
M
M
Revert Gomar
M D
MD
Teva Galan
D002648:Child; D002675:Child, Preschool; D002658:Developmental Disabilities / Q000453:epidemiology*; D018450:Disease Progression; D005260:Female; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000453:epidemiology*; D008297:Male; D009422:Nervous System Diseases / Q000453:epidemiology*; D012189:Retrospective Studies; D013030:Spain / Q000453:epidemiology