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Walker Warburg syndrome

Radswiki et al.

The Walker-Warburg syndrome (WWS) (sometimes known as the HARDE syndrome) is an extremely rare lethal form of congenital muscular dystrophy. It is primarily characterised by

fetal hydrocephalus : almost always present
neural migrational anomalies : agyria (cobblestone lissencephaly / lissencephaly type II)
Dandy Walker continuum
retinal dysplasia
encephalocoele
cerebellar malformations
congenital muscular dystrophy

Additional anomalies include

Pathology

Genetics

It is considered by many to be inherited in an autosomal recessive manner.

Etymology

Initially described by Warburg et.al in 1971

Treatment and prognosis

The overall prognosis is poor with most infants dying within the 1^styear of life. There is no specific treatment and management is mainly supportive.

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References

Synonyms & Alternative Spellings

Synonyms or Alternative Spelling	Include in Listings?
Walker-Warburg syndrome (WWS)	✗
HARDE syndrome	✓
HARD syndrome	✗
Cerebro-ocular dysplasia and muscular dystrophy	✓
Cerebro-ocular dysplasia and muscular dystrophy syndrome	✗