Worster-Drought Syndrome

[Guest guest]

> I found this site the other day. I was looking for additional info

> on CBPS. The article mentioned that CBPS is now know to be similar to

> Worster-Drought Syndrome.

> What do ya think?

> Patty, mom to twelve year old Wesley(CBPS, myoclonic jerks right now)

Patty

I found this article on Pubmed that suggests they are the same

condition.............

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Worster-Drought syndrome, a mild tetraplegic perisylvian cerebral palsy. Review

of 47 cases.

M, Carr L, Reilly S, Neville BG.

Institute of Child Health (UCL) and Great Ormond Street Hospital for Children

NHS Trust, London, UK. b.neville@...

A retrospective case-note analysis was undertaken of 47 children with a

congenital upper motor neurone bulbar palsy (excluding pure speech dyspraxia) to

clarify the phenotype of Worster-Drought syndrome (WDS) and to record its

associated features and complications. The results revealed that the study

children had significant bulbar problems (with 80% still needing a modified diet

and a similar number using augmentative communication methods at last review).

There were also high rates of predictable bulbar complications (86% had

dribbling, 60% had glue ear, gastro-oesophageal reflux in 40%, history of poor

nutrition in 40% and aspiration in 40%). Most of the children had additional

complex impairments (91% had mild pyramidal tetraplegia, 81% learning

difficulties, 60% congenital defects, 41% neuropsychiatric problems and 28%

epilepsy). Over half of the children had significant medical problems in the

first year, but mean age at diagnosis was 6 years. There were no obvious causes

in pregnancy or birth. Six children had a family history of WDS and 32% (12/37)

had abnormal neuroimaging including five with bilateral perisylvian

polymicrogyria. In our experience, WDS is not uncommon, is relatively easily

diagnosed and is crucial not to miss as the management of these children's

multiple impairments is complex and requires a careful team approach. WDS falls

clearly within the cerebral palsies as a syndrome that includes motor impairment

arising from static damage to the brain in early life. The common presence of

cognitive, behavioural and seizure impairments strongly supports the cerebral

cortical (presumably perisylvian) localization. Its core elements are a

suprabulbar paresis, a mild spastic tetraplegia and a significant excess of

cognitive and behavioural impairments and epilepsy. The complete overlap in

phenotype between WDS and the bilateral perisylvian syndrome leads us to propose

that they are the same condition. WDS is startlingly absent from epidemiological

studies of the cerebral palsies and rarely diagnosed, presumably because of lack

of clinical awareness of the condition and lack of major gross motor

impairments.

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cheers

Sally, Jon, (ILS Grade 4a) and , Cambridge, UK

Lissencephaly Launch Pad http://homepage.ntlworld.com/foliot/liss/