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PubMed1981

Cerebellar hemorrhage complicating isovaleric acidemia: a case report.

Isovaleric acidemia (IVA) is an inborn error of leucine metabolism, resulting in an accumulation of isovaleric acid in the body fluids. The neuropathologic findings in an 11-day-old infant with IVA consisted of diffuse cerebral edema, massive cerebellar hemorrhage, upward transtentorial herniation, and focal degeneration of clusters of glial cells in white and gray matter. Although abnormal myelination has been described in different aminoacidopathies, the above findings have not been reported previously in IVA.

Acidosis↗

PubMedDate not supplied

Evaluation of congenital extracranial masses by waterpath sonography.

Congenital scalp masses pose a diagnostic and management challenge. In the past the relationship of the lesion to the skull and intracranial contents have been assessed by skull x-rays and computed cranial tomography. Waterpath sonography is a non-invasive technique that utilizes no ionizing radiation for evaluating and monitoring congenital scalp masses. In two illustrative cases this method of imaging assisted in assessing the size, contents, and relationship of the congenital scalp masses to the intracranial contents. It enabled the surgeon to anticipate the avascular cystic nature of the congenital scalp masses, the intact underlying calvarium, and non-violation of the CSF pathways prior to surgery. The technique was also useful for monitoring progress after surgery.

Cysts↗