FOI 5399-Worster Drought Syndrome

Date: 25 March 2020 to 25 March 2023

Worster Drought Syndrome is a rare/ultra-rare disease that in 2019 Orphanet estimate to have a European prevalence of 3.7 cases per 100,000 population1.


Based upon this prevalence there are estimated to be 2,442 patients however some prevalence estimates are 9 in 100,000 population with up to 5,940 prevalent population in the UK today.


There is concern that WDS is under-diagnosed or misdiagnosed, there does not appear to any treatment pathway or recommendations for the referral, management or identifiable specialist centres for diagnosis and management in the UK.


The WDSSG are attempting to identify where diagnosis is taking place, the diagnosed prevalence of WDS and for potential similar syndromes/diseases where there could be misdiagnosis or no diagnosis at all in order to work with the NHS to provide education and hopefully better outcomes for patients.


There is not a specific ICD 10 code available to attribute to a WDS diagnosis, as a form of cerebral palsy (ICD10 G80). WDS is then included in the sub categories C80.8 (Other Cerebral Palsy) or C80.9 (Cerebral Palsy Unspecified)


The table below outlines the data that the WDSSG would like to know.




Age at diagnosis

ICD 10 code applied

Other co-morbidities and ICD codes

A primary diagnosis of Worster Drought over the last 3 years





Worster Drought prevalence (all ages) under your care





No of patients diagnosed with Congenital suprabulbar palsy/paresis





No of patients diagnosed with Congenital pseudobulbar palsy/paresis





No of patients diagnosed with bilateral perisylvian polymicrogyria





No of patients diagnosed with foix-chavany-marie syndrome





How many patients have had a diagnosis of WDS which has been re coded: to what disease/ICD10 code?





How many patients have been re coded to WDS





Any specific specialities/services/pathways/recommendations provided for WDS patients





  • Request ID:
    FOI 5399
  • Category:
    Patient - Activity Data
  • Response:

    Response attached