Developmental brain disabilities

Intrauterine seizures may occur. The electroencephalogram often displays a hypsarrhythmia or a burst-suppression pattern. Patients display myoclonic jerks, hiccuping and a profound hypotonia. The few patients who survive past the first week usually sustain profound mental retardation and neurological disability. Brain imaging shows atrophy and a loss of myelin. Rarely, patients present later in life with psychomotor retardation and growth failure. Others have had initial normal development followed by a progressive loss of developmental milestones. Some patients have manifested spinocerebellar degeneration and other symptoms of motor dysfunction [27],... 

It is not surprising that infant L. suffered diffuse encephalopathy (brain disorder), a cerebral infarction, and seizures during the neonatal period (Yager, 2002). Both asphyxia and hypoglycemia are injurious to the brain. The treatment for seizures consists of providing normal metabolic substrates (e.g., glucose) and appropriate anticonvulsant therapy (phenobarbital), as was done in the present case. The long-term treatment for the child s developmental disabilities is complex and involves the skills of many members of the health care team. 

At this age, Roberto s brain is irreversibly damaged. He s attending a school for developmentally disabled children. He s not suffering from classical autism, but will likely be living for the rest of his life with various cognitive disabilities that will remain a severe handicap. 

The other major area of manganese involvement in developmental processes is the nervous system [14]. A number of authors [486-488] have provided details on the effects of the metal on brain development. Evidence supports a role for excess Mn(II) in inhibiting neurite extension and formation of neuromuscular synapses [489] and for normal Mn(II) levels to be involved in the development of action potentials in explanted cortical neurons [490]. Collipp et al. [491] have reviewed the relationship between manganese in infant formulae and learning disabilities. In the ageing process, some correlation has been found between changes in brain concentrations of Mn(II) and Alzheimer s disease [492]. 

Head circumference is often used in clinical settings as an overall, nonspecific indicator of brain growth. It has limited usefulness in screening for potential developmental or neurological disabilities, but it is useful in comparison with other anthropometries to assess proportionality. The ratio of mid-arm to head circumference is a less commonly used index of proportionality. 

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