William Berenberg

Cerebrovascular accidents in infants and children with cyanotic congenital heart disease

Cerebrovascular accidents unrelated to surgery, brain abscess or subacute bacterial endocarditis remain a serious complication in patients with cyanotic congenital heart disease. To determine the risk factors for cerebrovascular accidents, we reviewed our experience in 29 consecutive cases and in a control group of 220 cases. The overall incidence of cerebrovascular accidents in patients with cyanotic congenital heart disease was 1.6 percent. Of 30 cerebrovascular accidents, 21 occurred in infants and children between 5 months and 4 years of age and 9 in older patients. Ninety percent of the accidents occurred in patients with tetralogy of Fallot and dextrotransposition of the great arteries. Hemiplegia was precipitated by acute febrile illness in six patients, cardiac catheterization in two and hyperpneic spells in two. Seven patients recovered completely, and 20 had residual hemiplegia. A seizure disorder developed in five patients and mental retardation in four. Three patients died (mortality rate 10 percent). Cerebrovascular accidents in patients less than 4 years of age were associated with anemia (low mean corpuscular hemoglobin concentration) and hypoxemia. By contrast, cerebrovascular accidents in older patients were associated with polycythemia and hypoxemia. The data suggest that spontaneous cerebrovascular accidents may be prevented in infants and young children with cyanotic congenital heart disease by medical treatment of anemia and surgical relief of the hypoxemia. Since the cardiovascular malformations in 90 percent of our patients who had a cerebrovascular accident are amenable to surgical correction, an aggressive surgical approach with early correction of the malformations may significantly reduce the incidence of this complication.

Epedimic and endemic HLA-B and DR associations in mucocutaneous lymph node syndrome

HLA-A,B and DR antigens were evaluated in 51 patients with mucocutaneous lymph node syndrome (Kawasaki disease) during both endemic and epidemic periods in the Boston area. Although 80% of patients were HLA-B5 (70% HLA-Bw51) during endemic periods, there were no HLA-B5 individuals during the epidemic period. There was, however, an increased incidence of HLA-Bw44 during the Boston epidemic. Such different associations in endemic versus epidemic periods suggest possible different, as yet unknown, inciting agents.

Ganglioneuroma presenting with differentiated skeletal metastases: report of a case

The authors report the case of a child with retroperitoneal ganglioneuroma and cytodifferentiated skeletal metastases. The primary tumor was surgically resected, and the child is alive and well 2 years later without additional therapy. This rarely documented phenomenon can be explained by spontaneous cytomaturation within both primary and metastatic tumor.

Pseudoprecocious Puberty in Girls as a Result of Estrogen Ingestion

THE purpose of this paper is to report 2 cases of precocious secondary sexual development resulting from the ingestion of estrogenic substances by young girls, to point out the clinical peculiariti...

Changing Motor Patterns in Cerebral Palsy

Three patients are reported in whom athetosis became an increasing problem late in childhood. Both a traumatic birth history and a preceding motor deficit were well defined in each case. This report emphasizes the concept that a brain injury incurred at birth may worsen or alter its clinical expression late in the first or early in the second decade. The differential diagnosis and théories of the mechanism for such an évolution are discussed.

Behr's Syndrome: Familial Optic Atrophy, Spastic Diplegia and Ataxia

Two sisters with Behrs syndrome are described. A relatively non‐progressive spinocerebellar degeneration of infantile onset, Behrs syndrome consists of ataxia, spasticity (particularly of the lower extremities), bilateral optic atrophy, posterior column sensory loss, peripheral neuropathy and mental retardation. In the two patients described here, Behrs syndrome appears to have been inherited as an autosomal recessive trait.

Toward the prevention of neuromotor dysfunction. Presidential address to the American Academy for Cerebral Palsy, 1968.

THE founding fathers of this Academy defined several major objectives. Many of these goals have been shared and supported through the years in a variety of Presidential Addresses. Although the attempt to forecast the shape of future needs has often been difficult, the concepts were most often clear and sharp, even when their execution proved to be complex. Understandably, therapeutic methods and the evaluation of their effect have attracted maximal interest and indeed they merit even more concern. However, there is also need for increased effort to prevent brain damage. We have made certain advances in this area, despite which the incidence of cerebral palsy remains at least unchanged, if not increased. Improved, but less than optimal, therapy of some conditions has dramatically reduced mortality only to increase residual morbidity. The most commonly recognized incidence of cerebral palsy today is in children of low birthweight. The incidence of cerebral palsy increases 80-fold in children weighing 4 pounds or less at birth. For every two weeks that the fetus remains in utero, between the 30th to 40th week of pregnancy, the incidence of

The Congenital Hyperammonemic Syndrome

Clinical and laboratory findings are described in a patient with an apparent defect of the urea cycle in whom a favorable response to protein restriction has been observed.

735 HLA ANTIGENS IN MUCOCUTANEOUS LYMPH NODE SYNDROME (MLNS)

Associations of specific histocompatibility antigens with certain human diseases may elucidate basic mechanisms of disease. Since there is evidence for a disordered immune response in MLNS, HLA antigens were evaluated by NIGH standard technique in 27 patients (3 Oriental, 1 black) fulfilling CDC criteria for MLNS. In contrast to previous Japanese studies, we found no incidence of HLA-Bw22. We did, however, find a significant increase in HLA-Bw51: CHANCES OF DISTRIBUTION OF HLA PHENOTYPES IN CAUCASIANSTo our knowledge, this is only the second disease associated with HLA-B5 specificity. In preliminary studies, 10/17 Caucasian patients with MLNS were DR-2 (frequency in control population 23%, p<0.01). In other studies (Geha, et al), suppressor T-cells(OKT8) were found decreased in the acute phase of MLNS. Thus, MLNS, like systemic lupus erythmatosus and multiple sclerosis, is associated with both decreased suppressor cells and DR-2. Such findings may have genetic implications regarding inter-human variation in immune responsiveness. Infectious agents, toxins, or endogenous immune stimulation may trigger, in genetically susceptible persons, an inappropriate immune response.