Worster-Drought and congenital perisylvian syndromes-a continuum?
A 5-year-old female was evaluated because of severe speech and expressive language delay. On examination, she could hardly speak and communicated through gestures. She manifested severe dysarthria and difficulty in protruding and moving her tongue laterally. She lacked coordination of the swallowing process, with drooling and an increased mental reflex. Her cognitive development was normal, and no associated neurologic dysfunction of the limbs was noted. On follow-up, the child experienced two episodes of seizures at 6 years of age. Magnetic resonance imaging of the brain demonstrated perisylvian and frontal polymicrogyria. Clinical and radiologic findings demonstrated a similarity and continuum between congenital suprabulbar paresis (Worster-Drought syndrome) and perisylvian syndrome.
Institute for Child Development and the Pediatric Neurology Unit, Division of Pediatrics, Dana Children's Hospital, Tel Aviv, Israel.
Y
Y
Nevo
Y
Y
Segev
Y
Y
Gelman
I
I
Rieder-Grosswasser
S
S
Harel
D010244:Bulbar Palsy, Progressive / Q000145:classification / Q000151:congenital* / Q000175:diagnosis*; D002675:Child, Preschool; D003937:Diagnosis, Differential; D004827:Epilepsy / Q000209:etiology; D005260:Female; D005625:Frontal Lobe / Q000473:pathology*; D006801:Humans; D007805:Language Development Disorders / Q000209:etiology; D008279:Magnetic Resonance Imaging; D010487:Periaqueductal Gray / Q000473:pathology*; D013064:Speech Disorders / Q000209:etiology* / Q000473:pathology; D013577:Syndrome