BPSU - PIND (Progressive Intellectual and Neurological Deterioration)

As a BPSU study, surveillance of Progressive Intellectual and Neurological Deterioration (PIND) in Children (including Creutzfeldt-Jakob Disease) began in May 1997 with the main aim of determining whether or not any child has developed variant Creutzfeldt Jakob disease (vCJD). As the clinical presentation of vCJD is not typical of classical CJD, the aim is to detect suspected cases by looking at a broader group of conditions.

Leads of BPSU study in PIND

Lead investigator 

Dr Chris Verity
Consultant Paediatric Neurologist
Addenbrooke’s Hospital
Hills Road
Cambridge
CB2 0QQ

Overview

  • Active prospective surveillance of UK children with progressive intellectual and neurological deterioration (PIND) commenced in May 1997.
  • The main aim is to determine whether or not any child has developed variant Creutzfeldt Jakob disease (vCJD).
  • vCJD has appeared in patients as young as 12 years of age and could occur in younger children.
  • Although paediatric cases have not appeared since 2001, vCJD has not gone away.
  • As the clinical presentation of vCJD is not typical of classical CJD and could be different in children, the aim is to detect suspected cases by looking at a broader group of conditions. This group needs to be large enough to include all possible cases of CJD hence the need to perform surveillance for all children with PIND.

Case definition

Any child under 16 years of age at onset of symptoms who fulfils all of the following three criteria:

  • Progressive deterioration for more than three months, with
  • Loss of already attained intellectual/developmental abilities, and
  • Development of abnormal neurological signs.

Excluding:

  • Static intellectual loss e.g. after encephalitis, head injury or near drowning
Including:
  • Children who meet the case definition even if specific neurological diagnoses have been made.
  • Metabolic disorders leading to neurological deterioration.
  • Seizure disorders if associated with progressive deterioration.
  • Children that have been diagnosed as having neurodegenerative conditions but who have not yet developed symptoms

Reporting restricted to:

Cases seen in the last month but including those whose conditions began earlier (ie including 'old cases' of children in follow-up if seen in that month).

Reporting instructions

Please report any child seen in the last month who meets the case defintion, including those who have already been given a specific diagnosis.

More information

Duration: May 1997 to May 2018

Funding: Department of Health.

Ethical approval: Approved by Addenbrooke’s NHS Trust and the PHE     

Study contact details

AnneMarie Winstone / Alison Powell, Research Coordinator
Box 267
Addenbrooke’s Hospital
Hills Road
Cambridge
CB2 0QQ

Email: annemarie.winstone@addenbrookes.nhs.uk / alison.powell@addenbrookes.nhs.uk

Protocol  

Protocol card (PDF, 97KB)

Support groups

Creutzfeldt-Jakob Disease Support Network: Birchwood, Heath Top, Ashley Heath, Market Drayton, TF9 4QR

Batten Disease Family Association: c/o Heather House, Heather Drive, Tadley, Hampshire, RG26 4QR. Tel:0791 4060742

The Society for Mucopolysaccharide Diseases: MPS House, Repton Place, White Lion Road, Amersham, Bucks, HP7 9LP

Climb: (formerly the Research Trust for Metabolic Diseases in Children (RTMDC.), The Quadrangle, Crewe Hall, Weston Road, Crewe, CW2 6UR

ALD Family Support Trust: 30-32 Morley House, 320 Regent Street, London, W1R 5AB

Niemann Pick Disease Group: Kingslaw House, East Brae, East Wemyss, Fife KY1 4RS, Scotland