Congenital bilateral perisylvian syndrome synonyms

Congenital bilateral perisylvian syndrome (CBPS), also known as Worster-Drought syndrome, is a rare neurological disorder characterized by the inability to control the muscles of the mouth, tongue, and throat. Because the condition has been described through various clinical lenses over the last century, it is frequently referenced by several synonyms, though "Congenital bilateral perisylvian syndrome" remains the preferred clinical term for its descriptive accuracy regarding the brain's perisylvian region.

What are the primary synonyms for Congenital bilateral perisylvian syndrome?

In medical literature, Congenital bilateral perisylvian syndrome is most commonly identified by the eponymous term Worster-Drought syndrome. This name honors the physician who first described the clinical presentation of pseudobulbar palsy in children. Other terms you may encounter in older medical records or international literature include:

• Congenital suprabulbar paresis


• Bilateral perisylvian polymicrogyria (often used when the diagnosis is confirmed via MRI)


• Congenital opercular syndrome


• Perisylvian syndrome

Why does this condition have so many different names?

The variety of names for Congenital bilateral perisylvian syndrome stems from the evolution of diagnostic technology and clinical observation. Historically, clinicians used the term "Worster-Drought syndrome" to describe the outward symptoms—specifically, the difficulty with speech and swallowing known as pseudobulbar palsy. As neuroimaging (MRI) became standard, researchers identified that these symptoms were caused by malformations in the perisylvian region of the brain, leading to the more anatomically precise term, Congenital bilateral perisylvian syndrome. The term "bilateral perisylvian polymicrogyria" is often used specifically by neuroradiologists to describe the cortical brain folding pattern associated with the condition.

How is the condition classified in official medical databases?

Navigating medical records can be difficult due to varying classification systems. To ensure you are looking at the most relevant data, note the following identifiers used by major institutions:

• Orphanet: Lists the condition under ORPHA:79275, primarily referencing Congenital bilateral perisylvian syndrome.


• OMIM (Online Mendelian Inheritance in Man): The condition is often cross-referenced with entries related to perisylvian polymicrogyria (OMIM #300267), highlighting the genetic heterogeneity of the disorder.


• ICD-10/11: There is no single specific code for the syndrome; it is often coded under broader categories of congenital malformations of the brain or pseudobulbar palsy.

Which name should I use when speaking with specialists?

While "Worster-Drought syndrome" is still widely recognized by neurologists and speech-language pathologists, Congenital bilateral perisylvian syndrome is currently the preferred terminology in modern academic and clinical settings. Using the full name helps specialists immediately identify that the diagnosis involves both the neurological symptoms and the underlying structural brain findings. At DiseaseMaps.org, our community of 45 members who share experiences with Congenital bilateral perisylvian syndrome typically uses the full name to facilitate clearer communication with their multidisciplinary care teams.

Next steps

• Consult with a pediatric neurologist or a specialist in neurogenetics to ensure your medical records reflect the most accurate diagnosis.


• If you are seeking support, join the DiseaseMaps.org community to connect with other families navigating the complexities of Congenital bilateral perisylvian syndrome.


• Request a copy of your brain MRI report and ask your radiologist if the term "polymicrogyria" is explicitly mentioned, as this is a key diagnostic marker.


• Work with a speech-language pathologist specializing in pediatric feeding and swallowing disorders.

Medical disclaimer: This information is for educational purposes only and does not constitute medical advice, diagnosis, or treatment; always seek the advice of your physician or other qualified health provider with any questions regarding a medical condition.

References

• Orphanet: Portal for rare diseases and orphan drugs (ORPHA:79275).


• NIH Genetic and Rare Diseases (GARD) Information Center: Congenital bilateral perisylvian syndrome.


• OMIM (Online Mendelian Inheritance in Man): Entry #300267 (Perisylvian polymicrogyria).


• Worster-Drought, C. (1974). An unusual form of acquired aphasia in children. Developmental Medicine & Child Neurology.