Ronald J. Lemire

Coma scale for use in brain-injured children

The association between admission coma score and eventual outcome was assessed using a coma scale developed for children with a variety of central nervous system injuries. As opposed to the Glasgow coma scale, this scale does not demand assessment of verbalization, and thus can be applied to the preverbal or previously intubated child. Cortical function is graded from 6 (purposeful, spontaneous movements) to 0 (flaccid), and brainstem function is graded from 3 (intact) to 0 (absent and apneic). Maximum total score is 9.In 91 children treated for intracranial hypertension, the association was moderately good. The scale was better in predicting the outcome of patients with hypoxic encephalopathy and head trauma than that of patients with Reyes syndrome, meningitis, or encephalitis. No child with a score of less than 3 survived in spite of intensive therapy. Most of these children were flaccid with depressed or absent brainstem reflexes. No child with flaccidity on admission survived.

Skin-covered sacrococcygeal masses in infantsand children

Although the typical myelomeningocele presents no difficulty in diagnosis, differentiation of masses over the caudal spine of infants may be difficult when the lesion is covered by intact skin. This paper presents the clinical and radiologic findings in 31 such cases and reviews parameters by which the major diagnostic groups can be separated. A few simple clinical observations, when combined with standard radiographs, should allow the clinician to arrive at a preoperative diagnosis with increased certainty.

Predominance of hemoglobin Gower 1 in early human embryonic development.

Hemoglobin Gower 1, the structure of which is thought to be ε4, is the predominant hemoglobin in early human embryonic life. This finding suggests that the production of ε-chains initially exceeds that of other known (α, β, γ, and δ) chains.

Hemangioblastoma of the conus medullaris associated with cutaneous hemangioma

A 6-month-old infant is reported with a spinal cord hemangioblastoma located in the conus medullaris associated with an overlying congenital dermal sinus and cutaneous capillary hemangioma. There were no neurologic deficits either preoperatively or following removal of the tumor. The skin and spinal cord lesions were believed to represent an isolated vascular malformation. This spinal cord hemangioblastoma is unusual because of the age of the patient, manner of clinical presentation, location in the caudal spinal cord, and pathologic characteristics. We review the literature and discuss the associations of spinal cord hemangioblastomas with cutaneous and other lesions.

Neural tube defects.

Neural tube defects (NTDs) are a group of malformations of the brain and spinal cord that originate at various times during gestation. The most common NTDs are anencephaly and meningomyelocele, which arise during the process of neurulation, a well-defined period between the 17th and 30th day after ovulation. These NTDs are clinically apparent by being open, ie, leaving nervous tissue exposed, in contrast to postneurulation NTDs that are skin covered. The incidence of NTDs is declining in several areas of the world, including the United States. While the reasons for this are unclear, there seems to be some effect from prenatal diagnosis, genetic counseling, and possibly nutritional supplementation.