Robert M. Shuman

The neuropathology of the nonketotic and ketotic hyperglycinemias Three cases

Abnormal dysmyelination constitutes a pathoanatomic basis for the mental retardation in two different aminoacidopathies, nonketotic hyperglycinemia and ketotic hyperglycinemia. In both conditions myelin is decreased in amount and vacuolated. Similar patterns of dysmyelination in different aminoacidopathies suggest that abnormal myelination results from inadequate synthesis of myelin proteins.

Optic Nerve Hypoplasia: One Part of a Spectrum

In this issue of Journal of Child Neurology, Ouvrier and Billson review optic nerve hypoplasia Their review illustrates our continuing need for knowledge about the natural history of many disorders. Optic nerve hypoplasia may represent one component of a multifaceted congenital malformation affecting an entire embryogenic field. This field defect may have associated complex neurologic, endocrinologic, neuroradiologic, and morphologic abnormalities. Conversely, optic nerve hypoplasia could be viewed as but one part of a heterogeneous constellation of abnormalities with little in common. However discrete

Topical exposure of neonates to hexachlorophene: long-standing effects on mating behavior and prostatic development in rats.

Abstract Male and female rats were washed with pHisoHex (3% hexachlorophene, HCP) during the first 8 days of life, the “critical period” of neuroendocrine differentiation, in order to investigate potential alterations in sexual development. Female rats treated in such a manner had a normal onset of puberty, regular 4 to 5-day estrous cycles, and were fertile when tested at 4.5 months of age. Seven-month-old male rats exposed to pHisoHex had significantly reduced fertility. When exposed to female rats at 11 months of age the males mounted and intromitted, but did not ejaculate. Many of the animals exposed to pHisHex had prostatic cysts and fibrosis. Serum testosterone concentrations were normal, testicular weights were not affected, and epididymal smears had abundant motile sperm. The infertility of adult male rats was the result of their inability to ejaculate. HCP may be responsible for these results, however, other data suggest that HCP may be contaminated with dioxins, toxic substances with androgen-like activity. Since other work shows that dissociation of intromission and ejaculation occurs in properly primed androgen-treated rats, it is possible that dioxin, as an “androgenic” contaminant of HCP, is responsible for permanent disruption of the CNS-integrated ejaculatory reflex.

The Journal of Child Neurology and Child Neurology: Growing Together

as a formal discipline, 15 years since the first American Board of Psychiatry and Neurology certifications in child neurology, and just over 10 years since the founding of the Child Neurology Society. n2 Through that quarter century our fledgling specialty had spoken only when permitted by the sufferance of its neurologic parents and older siblings. The Child Neurology Society had become affiliated with the prestigious Annals of Neurology. This affiliation was (and continues