Alexander's disease: unique presentation.

Subacute necrotizing encephalomyelopathy (Leigh syndrome) refers to a nebulous disease entity characterized by lactic acidosis, a wide variety a clinical manifestations, and a consistent conglomeration of pathologic findings. Several abnormalities in metabolism have been delineated in association with Leigh syndrome, but many cases have no identified metabolic abnormality. We report a case that clinically, metabolically, and neuroradiologically appeared to be Leigh syndrome. In addition, our patient exhibited other unusual clinical findings, including ocular motility abnormalities. Neuropathologically, however, the diagnosis of Alexander's disease was confirmed. A review of the literature failed to find other cases of Alexander's disease reported with the metabolic abnormalities and clinical manifestations with which our patient presented.

The rise and fall of the plantar response in infancy.

To determine when the plantar response becomes reliably flexor in infants, 169 infants underwent serial evaluation from 2 weeks to 12 months of age during routine well-child visits. The plantar response, elicited in a standardized fashion, was extensor in 95.5%, 64.8%, 10.9%, 0.7%, and 0% of the infants at 2, 4, 6, 9, and 12 months, respectively. The plantar response becomes predominately flexor by 6 months of age in normal infants. This maturation of response correlates closely with that of other infantile reflexes.

The plantar response in normal newborn infants.

The neonatal plantar response has been reported as extensor in 90% of newborns and flexor in 93% of newborns, leading to uncertainty about its reliability and significance. To determine the normal neonatal plantar response we examined 349 healthy newborn infants, > 32 weeks gestation within 24 hours of birth. A supramaximal noxious stimulus was applied in a standardized manner to the lateral plantar surface of each foot. The plantar response was extensor in 90%, equivocal in 7%, and flexor in 3%. With proper physiologic technique, the normal neonatal plantar response is extensor.

Altered consciousness and shock in a malnourished child.

A 3-year 9-month-old child presented unresponsive, dehydrated, and in shock, a consequence of child neglect, abuse, and starvation. This scenario provides the vehicle for a discussion of three problems which can be precipitated by child neglect, specifically kwashiorkor, central pontine myelinolysis, and intellectual repercussions of malnutrition.