Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
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May 31, 2018
Opercular Syndrome due to Bilateral Perisylvian Polymicrogyria
- Background:
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Opercular syndrome is a rare clinical entity, also known as bilateral perisylvian syndrome, Worster-Drought syndrome, or Foix‑Chavany‑Marie syndrome, though controversies regarding nomenclature exist.
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It is characterized by facial, lingual, pharyngeal, and laryngoglossal palsies.
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Various etiologies of opercular syndrome are suggested, including perinatal insult, cardiovascular disease, infections, and other inflammatory causes.
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- Clinical Presentation:
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Patients present with classical features of impaired voluntary movements of the facial, lingual, pharyngeal, and masticatory muscles, with preservation of automatic and reflex movements.
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May be associated with developmental delay, mental retardation, or seizures
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- Key Diagnostic Features:
- An appropriate clinical history, along with MRI findings, helps to diagnose this entity.
- MRI is the imaging modality of choice.
- MRI findings include encephalomalacic changes, polymicrogyria, and FLAIR/T2 hyperintensity in bilateral perisylvian regions with a wide sylvian fissure and dysplastic and thickened opercula.
- Differential Diagnoses:
- Pseudobulbar palsy: impairment of automatic and emotional movements and absence of classical imaging features help to differentiate from opercular syndrome
- Intrauterine CMV infection: can present with similar radiologic findings; however, classical clinical features of corticobulbar palsy are absent in intrauterine CMV infections
- Treatment:
- Treatment is mostly symptomatic and directed toward the underlying etiologic factor.
- Clinical improvement is poor.
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