[Worster-Drought syndrome--a specific cerebral palsy syndrome--why is the diagnosis frequently overlooked?].

Abstract

Worster-Drought syndrome (WDS) is a developmental disorder presenting as mild tetraplegic cerebral palsy (CP) with severe pseudobulbar palsy, manifested as articulation problems or anarthria, chewing, and swallowing difficulties and severe drooling. Epilepsy, learning disabilities, behavioral disturbances, and other congenital anomalies are common. WDS accounts for about 1% of CP cases. On brain imaging, migrational abnormalities are frequently found, mainly in the opercular area, however normal brain imaging does not exclude the diagnosis. Although children with WDS have serious, early-onset developmental disabilities, the specific diagnosis is frequently delayed or overlooked. There is a lack of awareness of the syndrome and an overlap with similar congenital syndromes, such as the Opercular Syndrome and Congenital Bilateral Perisylvian Syndrome. Two children with WDS are presented, emphasizing the clinical symptoms, natural history of the disorder, etiology and imaging. The similarity between WDS and other specific congenital syndromes will be discussed with the suggestion to unite them all under the name WDS.

• Authors: Dorit Shmueli, Varda Gross-Tsur
• Journal: Harefuah (Harefuah) Vol. 146 Issue 10 Pg. 755-8, 815 (Oct 2007) ISSN: 0017-7768 [Print] Israel
• PMID: 17990388 (Publication Type: Case Reports, Journal Article)

Topics

• Cerebral Palsy (complications, diagnosis)
• Child
• Diagnosis, Differential
• Epilepsy (epidemiology)
• Humans
• Learning Disabilities (epidemiology)
• Male
• Mental Disorders (epidemiology)
• Quadriplegia (complications)
• Syndrome