Agenesis of the corpus callosum is associated with feeding difficulties.

Our objective was to characterize the common occurrence of feeding and swallowing disorders noticed by our speech therapy department among patients with agenesis of the corpus callosum. All patients with suspected or presumed agenesis of the corpus callosum undergoing therapy for feeding and/or swallowing disorders, including oral and/or pharyngeal dysphagia and oral-sensory disorder, were identified. Their brain magnetic resonance imaging (MRI) studies and charts were reviewed in detail. Seven patients with striking oral-motor and oral-sensory disorder met the criteria for agenesis of the corpus callosum. Particular difficulties noted include oral-sensory defensiveness in five of these seven patients, oral dysphagia/weakness in all seven patients, and pharyngeal dysphagia with risk of aspiration in three of these seven patients. The oral-motor weakness observed in all of the patients affected the strength and coordination necessary for functional oral-phase swallowing. We conclude that feeding and swallowing disorders are significant in children with agenesis of the corpus callosum. Although the pathophysiologic mechanism is not known, recognition of this phenomenon is important because early detection and intervention can facilitate functional feeding and swallowing patterns earlier in these patients, therefore avoiding the adverse and more severe disorders that can arise when dysphagias and/or oral-sensory disorder persist over extended periods of time.

Athabascan brainstem dysgenesis syndrome.

We report a new disorder with diverse neurological problems resulting from abnormal brainstem function. Consistent features of this disorder, which we propose should be called the Atabascan brainstem dysgenesis syndrome, include horizontal gaze palsy, sensorineural deafness, central hypoventilation, and developmental delay. Other features seen in some patients include swallowing dysfunction, vocal cord paralysis, facial paresis, seizures, and cardiac outflow tract anomalies. All affected children described are of Athabascan Indian heritage, with eight children from the Navajo tribe and two patients who are of Apache background. The disorder can be distinguished from the Moebius syndrome by the pattern of central nervous system findings, especially the sensorineural deafness, horizontal gaze palsy, and central hypoventilation. Recognition of children with some features of Athabascan brainstem dysgenesis syndrome should prompt investigation for other related abnormalities. Published 2003 Wiley-Liss, Inc.

Cerebellar infarction: an unrecognized complication of very low birthweight.

We evaluated 13 children with cerebral palsy who had birthweights under 1085 g. A magnetic resonance image (MRI) of the head was obtained, the findings were compared, and the neonatal records were reviewed. The individual children were classified as to the type of cerebral palsy. On MRI, all had severe injury to the inferior cerebellar hemispheres, mostly symmetric, and in some there was injury to the inferior vermis. The average birthweight was 668 g, and the gestational ages were 24 to 27 weeks. No other outstanding prenatal or postnatal problems were identified. The children had different types of severe cerebral palsy, with only 3 being able to walk. Almost all were mentally retarded and microcephalic. All had visual problems. This report defines a previously underappreciated injury to the cerebellum in extremely premature infants. Further clinical, laboratory, and pathologic studies are needed to better define the underlying mechanisms.

Erb's palsy contrasted with Klumpke's and total palsy: different mechanisms are involved.

OBJECTIVE: The purpose of this study was to examine the available evidence regarding the nature of the forces that were involved in the production of a lower plexus palsy or a total (whole arm) palsy, as contrasted with the nature of the forces that resulted in an upper plexus palsy. STUDY DESIGN: This was a review of studies that dealt with specific mechanisms that were supplemented by reports of total palsy that were gleaned from the literature and case reports from the clinical and medical legal cases of the authors. RESULTS: Studies of the forces involved in brachial plexus injury in adults and from cadaver studies in infants who were stillborn or who died in the newborn period attest to the nature of the forces needed to damage the lower plexus. This evidence is reinforced by the case reports. CONCLUSION: Forces other than simple widening of the head-shoulder angle are necessary to disrupt the roots or cords of the lower brachial plexus. The position of the arm and direction of the forces that are applied determine the nature of the lesion.