Congenital suprabulbar palsy: a distinct clinical syndrome of heterogeneous aetiology

Abstract

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.

Assessment of dysphagia in infants with facial malformations

“…In nine of them, EMG during bottle feeding showed oral-pharyngeal incoordination and EM detected an associated esophageal dysfunction. This indicates the absence of cranial nerve nuclei lesions and suggests disturbances of the central swallowing pattern generator, which may be of bulbar or suprabulbar origin [17]. In 14 other patients, EMG showed neurogenic patterns in muscles of the face and/or soft palate associated with altered BRs, sucking-swallowing disorder, and EM abnormalities.…”

Bilateral Operculum Syndrome in Childhood

“…1 Worster-Drought reported on a similar condition of suprabulbar (pseudobulbar) paresis in children with communication disorders (Worster-Drought syndrome). [2][3][4] The pathology of these syndromes involves the cerebral opercula bilaterally. The operculum refers to portions of the frontal, parietal, and temporal lobes adjacent to the Sylvian fissure and overlying the insula.…”

“…6,7 Bilateral structural and functional, or only functional, impairment of the opercula can lead to suprabulbar (pseudobulbar) symptomatology associated with difficulties in feeding and speech development. [1][2][3][4]8 The etiology of the clinical symptoms can be congenital or acquired as well as persistent or intermittent and includes bilateral dysgenesis of the opercular cortex, vascular events, central nervous system infections (eg, encephalitis), and epileptic disorders. [1][2][3][4]8 There seems to be an overlap between the symptomatology of Foix-Chavany-Marie, Worster-Drought, and congenital bilateral perisylvian syndromes and speech and oromotor deficits of epileptic origin in benign childhood epilepsy with centrotemporal (Rolandic) spikes.…”

“…[1][2][3][4]8 The etiology of the clinical symptoms can be congenital or acquired as well as persistent or intermittent and includes bilateral dysgenesis of the opercular cortex, vascular events, central nervous system infections (eg, encephalitis), and epileptic disorders. [1][2][3][4]8 There seems to be an overlap between the symptomatology of Foix-Chavany-Marie, Worster-Drought, and congenital bilateral perisylvian syndromes and speech and oromotor deficits of epileptic origin in benign childhood epilepsy with centrotemporal (Rolandic) spikes. [1][2][3][4]8 We report on 2 children with severe bilateral anatomical defect in the operculum formation and suprabulbar paresis in addition to other neurological signs, and a third child with impairment of the operculum function in association with epileptic activity.…”

“…[1][2][3][4]8 There seems to be an overlap between the symptomatology of Foix-Chavany-Marie, Worster-Drought, and congenital bilateral perisylvian syndromes and speech and oromotor deficits of epileptic origin in benign childhood epilepsy with centrotemporal (Rolandic) spikes. [1][2][3][4]8 We report on 2 children with severe bilateral anatomical defect in the operculum formation and suprabulbar paresis in addition to other neurological signs, and a third child with impairment of the operculum function in association with epileptic activity. We prefer the term bilateral operculum syndrome to designate these conditions.…”