Congenital suprabulbar palsy: a distinct clinical syndrome of heterogeneous aetiology.
Congenital suprabulbar palsy is clinically characterized by problems of feeding, swallowing, drooling, and dysarthria. Epilepsy, delayed motor, cognitive, and language development, as well as learning disabilities may co-exist. Aetiology of the syndrome is diverse, yet studies often attribute it to specific entities. We report on nine patients (seven males, two females; age range 2 to 20 years), highlighting the heterogeneous causes of suprabulbar palsy using neuroimaging and emphasizing the need for systematic investigation for early detection and management. We identified patients with symmetrical infarcts involving the perisylvian region, apart from already-recognized conditions, such as congenital bilateral perisylvian syndrome (CBPS; a neuronal migration disorder) and Worster-Drought syndrome. CBPS simulates Foix-Chavany-Marie syndrome in adults because of staged stroke but differs in many respects. Anoxia or ischemia to the developing brain could be a common plausible aetiology. Studies with large groups of patients are required to differentiate the various subgroups and identify essential criteria for diagnosis.
Suresh
Poovathinal A
PA
Department of Neurology, Institute for Communicative and Cognitive Neurosciences, Kavalappara, Shoranur, Kerala, India. icconssrr@sify.com
Deepa
Cherukunnathu
C
D000293:Adolescent; D000328:Adult; D001072:Apraxias / Q000209:etiology; D010244:Bulbar Palsy, Progressive / Q000151:congenital / Q000473:pathology* / Q000503:physiopathology*; D002648:Child; D002675:Child, Preschool; D003680:Deglutition Disorders / Q000209:etiology; D002658:Developmental Disabilities / Q000209:etiology; D004401:Dysarthria / Q000209:etiology; D004827:Epilepsy / Q000209:etiology; D005260:Female; D006801:Humans; D007859:Learning Disabilities / Q000209:etiology; D008297:Male; D013577:Syndrome