Pandemic A/H1N1 2009 influenza vaccination, preceding infections and clinical findings in UK children with Guillain-Barré syndrome.

OBJECTIVE: To record clinical findings in all new cases of Guillain-Barré syndrome (GBS) or Fisher syndrome (FS) in UK children in the 2 years following September 2009 and determine the proportion temporally associated with recent infections, pandemic H1N1 (2009) strain influenza vaccination or seasonal influenza vaccination. DESIGN: A prospective UK-wide epidemiological study using the British Paediatric Surveillance Unit system. PATIENTS: Children aged 16 years or less meeting the Brighton Collaboration criteria for GBS or FS. RESULTS: 112 children with GBS (66 boys and 46 girls) and 3 boys with FS were identified in 2 years. All but one recovered sufficiently to go home. The annual UK incidence rate of GBS in patients less than 15 years old was 0.45/100 000, similar to other countries. There was evidence of infection in the 3 months preceding onset in 92/112 GBS and 3/3 FS cases. Of those living in England, 7 cases received pandemic A/H1N1 2009 influenza vaccination before GBS symptom onset (3/7 were within 6 months including 1 within 3 months); 2 children received 2010/2011 seasonal influenza vaccination within 6 months of GBS onset. The numbers vaccinated were not significantly greater than expected by chance. CONCLUSIONS: The outcome for childhood GBS and FS after 6 months was better than reported in adults. Most UK GBS and FS cases had infections in the preceding 3 months. When considering the children living in England, there was no significantly increased risk of GBS after pandemic A/H1N1 2009 influenza vaccination or 2010/2011 seasonal influenza vaccination.

No clinical evidence of hidden vCJD in UK children.

Between May 1997 and November 2004 this national prospective surveillance study identified 1007 children with "progressive intellectual and neurological deterioration" (PIND). In most cases specific diagnoses were made, but of 92 undiagnosed children with PIND 46 had died and only four underwent full necropsy. There was no clinical evidence of variant Creutzfeldt-Jakob disease (vCJD) in these undiagnosed cases, but without necropsy it is not possible to exclude vCJD completely.

Is variant Creutzfeldt-Jakob disease in young children misdiagnosed as Alpers' syndrome? An analysis of a national surveillance study.

BACKGROUND: There has been concern that children with variant Creutzfeldt-Jakob disease (vCJD) might be misdiagnosed as cases of Alpers' syndrome, as a spongiform degeneration of the brain is seen in both conditions. OBJECTIVE: To report a national prospective surveillance study of children with progressive intellectual and neurological deterioration, designed to detect any children in the United Kingdom with vCJD, to see whether this misdiagnosis is occurring. METHODS: A monthly surveillance card is sent by the British Paediatric Surveillance Unit to all consultant paediatricians in the UK. The card lists the disorders currently under surveillance. Paediatricians are asked to return the card, reporting cases seen in the previous month. The BPSU office informs the surveillance groups about reported cases, and they obtain clinical information from the notifying paediatrician. RESULTS: After 5 years and 8 months of surveillance, 1244 children had been reported to the study. Alpers' syndrome was confirmed in two, although this was the suggested diagnosis in 11 children at the time of initial notification. CONCLUSIONS: The results show that Alpers' syndrome is rare and it is unlikely that vCJD cases are being misdiagnosed as Alpers' syndrome.

Variations in neurodegenerative disease across the UK: findings from the national study of Progressive Intellectual and Neurological Deterioration (PIND).

AIMS: To identify any UK children with variant Creutzfeldt-Jakob disease (vCJD) and obtain information about the causes of progressive intellectual and neurological deterioration (PIND) and the geographical distribution of cases. METHODS: The PIND Study uses the monthly surveillance card that is sent to all UK paediatricians by the British Paediatric Surveillance Unit. Case details are obtained from the reporting paediatricians by telephone interview, site visit, or self completion of a questionnaire. A paediatric neurology expert group then classifies the anonymised cases. The Communicable Disease Surveillance Centre (CDSC) provides mapping support. RESULTS: After five years and five months of surveillance, 1400 children had been reported. In the UK the majority of PIND cases had a confirmed diagnosis (comprising 99 different conditions); 505 "no cases" and 97 "outstanding" cases were excluded. A total of 798 PIND cases were included as follows: 577 with a confirmed underlying diagnosis; six with definite or probable vCJD, 51 who had undiagnosed PIND but were not thought to have vCJD, and 164 cases who were still under investigation. In some districts there were unexpectedly high numbers of PIND cases with a heterogeneous mixture of underlying diagnoses. In the five districts with the largest numbers of resident cases the majority not only came from a particular ethnic group but also had high reported rates of consanguinity. CONCLUSIONS: In districts with large numbers of PIND cases there are major resource implications. These children and their families have complex problems and they need access to diagnostic facilities and appropriate service provision.

Variant Creutzfeldt-Jakob disease in UK children: a national surveillance study.

BACKGROUND: Variant Creutzfeldt-Jakob Disease (vCJD) was first reported in 1996; the youngest patient developed symptoms at 16 years of age. We have done 3 years of prospective active surveillance for progressive intellectual and neurological deterioration (PIND) in UK children, and have searched for vCJD among the children who were reported. METHODS: Since May, 1997, there has been active surveillance for patients younger than 16 years old with PIND by means of a monthly card sent to all UK consultant paediatricians by the British Paediatric Surveillance Unit. Clinical'details of cases of PIND are obtained from reporting paediatricians by telephone interview or site visit, and an expert group of paediatric neurologists then classifies the cases. FINDINGS: After 3 years, 885 patients with suspected PIND have been reported. Among them were two fatal cases of definite vCJD and one case of probable vCJD; all were reported in 1999. One girl was age 12 years at onset--the youngest ever case of vCJD. No other children with the clinical features of vCJD were identified. The expert group has discussed 655 cases, of which 360 have a confirmed underlying cause, being categorised into 88 known neurodegenerative diseases. INTERPRETATION: That this prospective active surveillance in the UK has found few children with suspected vCJD is relatively reassuring. However, 3 years is a short time to survey a disease with an unknown incubation period. Since one probable and two definite cases of vCJD were reported to the study in 1999, there is concern that more childhood cases may appear.