John W. Benton

Subclinical central nervous system disease of neonates: A prospective study of infants born with increased levels of IgM

Umbilical cord sera obtained over a 1 year interval from all deliveries in a low income population were screened for elevated levels of IgM to determine the usefulness of this approach for diagnosis of subclinical congenital infection in newborns. There was a 30-fold or more increased infection rate in neonates born with elevated IgM values when compared with an appropriate control group. Forty-two systemic or localized infections, mostly subclinical forms, were detected in infants with increased cord IgM. Although symptoms were absent, persistent cerebrospinal fluid pleocytosis and elevated protein values were detected in over a half of infected newborns. Two of these with toxoplasma and 2 with cytomegaloviral infections have already developed signs of central nervous system damage during the first year. Data indicate that subclinical congenital infections with “silent” CNS involvement occur frequently in certain newborn groups and may be an important cause of brain damage that is currently unclassified. When coupled with methods for specific identification, screening of cord and neonatal sera for elevated IgM can be helpful in the search for and definition of this type of disease.

The bobble‐head doll syndrome: Report of a unique truncal tremor associated with third ventricular cyst and hydrocephalus in children

RECENTLY WE HAVE observed two children with to-and-fro bobbing or nodding of the head and trunk. The movement is reminiscent of that seen in dolls with weighted heads resting on a coiled spring, and thus it is named “bobble-head doll syndrome.” Each patient had a large cyst in the region of the third ventricle and dilated lateral ventricles. The disorder was unique in our experience, and no reference to a similar syndrome could be found in the literature.

Juvenile myasthenia gravis

We studied 32 children with myasthenia gravis over a period of 12 years. The mean age at onset was 7.7 years. Presentation was ocular in 63% of patients. Another major disease in addition to myasthenia occurred in 44% of patients; a seizure disorder was the most commonly associated disease. Serum IgG antibody to nicotinic acetylcholine receptor was present in 53% of patients and did not correlate with severity of disease or treatment. Medical management was effective in 63%; thymectomy was effective in only 28%. We conclude that myasthenia gravis appears commonly before age 10 and is associated with the risk of some disease other than hyperthyroidism. Serum IgG nicotinic acetylcholine receptor antibody is present less frequently than in normal adults, and vigorous medical management should be attempted before thymectomy.

Developmental sequelae in infants having suffered severe perinatal asphyxia

Abstract Fifteen of 20 infants surviving perinatal asphyxia (Apgar ≤ 3) necessitating resuscitation with positive-pressure oxygen and intravenous buffer were compared with 14 healthy mature nonasphyxiated infants 15 to 40 months after birth. Ten asphyxiated and 11 nonasphyxiated infants had normal neurologic examinations while two asphyxiated and three nonasphyxiated babies had borderline results. The three intrauterine growth-retarded babies in the asphyxia group all had neurologic deficits. Excluding these three badly damaged growth-retarded babies (mean I.Q. = 31), mean I.Q. scores of asphyxiated and nonasphyxiated groups were identical (96, asphyxiated; 96, nonasphyxiated). These preliminary data suggest a good prognosis for acutely asphyxiated infants managed with current techniques of resuscitation.

High resolution computerized tomography with coronal and sagittal reconstruction in the diagnosis of brain tumors in children.

We present 4 children with brain tumors that were missed or incompletely characterized conventional computerized tomography (CT) scanning. Using high resolution CT with high dose contrast and three-dimensional reconstruction, we were able to define the anatomy of each tumor and its associated aberrant vasculature. High resolution CT scanning with reconstruction is very helpful in diagnostic problems in which conventional CT scanning fails to provide a clear diagnosis.

Unilateral pupillary dilatation during and immediately following seizures

All cases of epilepsy and head trauma :idniitted to the neurological and neurosurgical services and all neurological consultations at the Massachusetts General Hospital for the years 1963 to 1965 were retrieved using an IBM sorter. (Identifying data, including diagnoses, are recorded routinely on IBM cards for every neurological and neurosurgical admission and for every neurological consultation. ) Approximately one thousand cases were reviewed for observations on pupiIlary size during and following seizures. Six examples of transient anisocoria related to seizures were found in this manner. Six additional cases had been observed a t various other times by one of the authors. These twelve cases form the basis of this report.

Cerebellopontine angle lipoma in a teenager

Lipomas of the cerebellopontine angle are very rare lesions. To date, 18 patients have been reported, 17 of whom were adults. A second child is described with cerebellopontine angle lipoma.

64 INTERDISCIPLINARY ASSESSMENT OF MORBIDITY OF INFANTS WITH BIRTHWEIGHTS ≤ 950 GRAMS

26/31(84%) surviving NICU infants with birthweights ≤950 g.born 4/74-1/77 were evaluated by an interdisciplinary team including pediatricians(2), psychologist, audiologist, optometrist, developmentalist, social worker, nutritionist and R.N. The study began in 1/76 with evaluations performed at ages 6, 12, 18, 24 and 36 mos. Mean age at last evaluation was 18 mos.(range 6-38 mos.). Population characteristics included mean birth weight 889 g.(range 650-940 g.); mean gestational age 28.4 wks.(range 24-33 wks., 7/26 small for gestational age); male:female 10:16; inborn:outborn 5:21; and Black:White:Other 13:12:1. Results were corrected for gestational age.Significant major morbidity was found in 9/26(34.6%)including stage V retrolental fibroplasia (RLF)(1); developmental delay (7 with DQ≥70); cerebral palsy(6); acquired hydrocephalus(2); extreme failure to thrive (FTT)(3). 6/9(67%) affected infants had multiple major handicaps. 11/17 infants without major handicaps had one or more minor disabilities including stage II RLF(1); recurrent lower respiratory disease(4); serous otitis media(11); mild conductive hearing loss(8); mild FTT(2); visual disorders(4) arrested hydrocephalus(3) and scoliosis(1). Although longitudinal followup may alter some of the results reported, it appears that survival without major morbidity is occurring in 65% of surviving infants with birthweights ≥950 g.