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Biomedical subjects

C M Foley

Publications and source records attributed to C M Foley.

9 recordsLinked to original sources

The epileptiform significance of intermittent rhythmic delta activity in childhood.

Intermittent rhythmic delta activity is reported in various disorders and is classified as a nonspecific abnormal electroencephalographic pattern. We have investigated its clinical and electroencephalographic features in childhood. Intermittent rhythmic delta activity was identified in 54 children over a period of 48 months. Epilepsy was present in 81%, 4% had only a single generalized tonic-clonic seizure, and 15% had no seizures. Generalized seizures were more common than partial seizures (83% versus 13%; 4% were mixed). The largest group of patients had idiopathic epilepsy. Epileptiform features were present in 70%. No patient identified prospectively has had a space-occupying lesion. Intermittent rhythmic delta activity should be considered an epileptiform pattern in children, most commonly occurring as an interictal pattern in primary generalized epilepsy.

Adolescent

Cerebral edema causing death in children with maple syrup urine disease.

Four children with the classic form of maple syrup urine disease (MSUD) died of cerebral edema during an intercurrent infection that caused severe dehydration and acidosis. The diagnosis of MSUD had been established during the neonatal period in all four patients, on day 1 of life in three of them. All were in satisfactory control before the intercurrent illness. Two patients underwent peritoneal dialysis. Signs of brain-stem compression occurred after treatment, when biochemical abnormalities were improving. Computed tomography of the head, which was done in two patients, revealed cerebral edema; one of these patients also had subarachnoid hemorrhage. Autopsy in one case revealed cerebral edema with herniation. Our experience documents that cerebral edema may occur in the older child with MSUD as well as in the neonate. The pathogenesis of cerebral edema in MSUD remains unclear. Early treatment of dehydration and acidosis may prevent the catastrophic consequences that we have observed.

Acute Disease

Olfactory hamartomas in tuberous sclerosis.

Gross or microscopic glial hamartomas were found in the anterior olfactory lobe and olfactory germinal layer of three babies, two of them newborns, with tuberous sclerosis. In two cases microscopic hamartomas were seen in the anterior olfactory lobe, and in one of them there was a prominent nodular tumor of the olfactory tract and trigone. In addition, in both of these cases there were bilateral germinal layer tumors between striatum and septum, at the junction of the obliterated olfactory recess and the frontal horn of the lateral ventricle. Only microscopic hamartomas were present in the olfactory germinal layer of the third case. Typical subependymal germinal layer tumors were also present elsewhere in all cases; however, cortical tubers were recognized in only two of them. In all three patients, the clinical presentation and death were due to cardiac rhabdomyomas. The findings suggest that olfactory hamartomas might be relatively common in tuberous sclerosis. Involvement of olfactory structures is not surprising because the lesions seem to originate in the germinal layer, a region of the brain which is prominently involved in the disease.

Brain Neoplasms

Classifying multidimensional stimuli: stimulus, task, and observer factors.

When observers decide how to classify stimuli, they often employ one of two types of information: identity along one particular dimension or overall similarity. The present studies examined interrelations among the factors which determine the use of these types of information. Participants' classifications of certain types of materials (e.g., size and brightness, length and density) revealed strong individual differences, were related to the individual's response tempo and selective processing ability, and were influenced by task demands. Classifications of other materials (e.g., saturation and brightness) did not reveal individual differences, were not affected by response tempo and selective processing ability, and were unaffected by changes in task demands. The former, but not the latter, types of materials have also been found to be influenced by developmental differences. The results are consistent with the idea that differences in response tempo and selective processing ability underlie observer differences (both individual and developmental) and that certain types of stimuli which are not susceptible to such influences set boundary conditions for observer differences. The results are discussed within an integral-to-separable model of processing.

Adult

Encephalopathy in children with chronic renal failure.

The progressive encephalopathy observed in 5 children with chronic renal failure was clinically similar to the so-called dialysis encephalopathy of adults, except that it was not related to dialysis therapy. Renal osteodystrophy is more prevalent in children than in adults and often more severe. The attempt to control the crippling deformities of renal osteodystrophy in growing children with renal insufficiency has led to the use of large quantities of aluminum containing antacids. The encephalopathy observed in children with chronic renal failure may be related to the oral ingestion of aluminum containing compounds in the presence of persistent secondary hyperparathyroidism. We suggest that alternative methods for the adequate control of serum phosphorus levels should be sought and indications for parathyroidectomy in children reevaluated. During the past 18 mos we have lowered the dose of aluminum containing compounds to 50 to 100 mg/Kg/day in our patients with progressive renal failure and recommend parathyroidectomy. No new cases of the encephalopathy have occurred.

Age Factors

Value of plasmapheresis in hepatic encephalopathy.

Plasmapheresis is used for treating the complications of liver failure. We performed plasmapheresis on 6 children with hepatic encephalopathy resulting from acute hepatic failure and prospectively assessed its effects on neurologic and electrophysiologic (electroencephalography and evoked potentials) function. Clinical improvement was observed in 3 of 6 patients; changes in the serum ammonia value or the results of initial electrophysiologic tests did not predict the patient response. Two patients underwent transplantation after neurologic improvement was produced by plasmapheresis; however, despite plasmapheresis, 4 patients progressed to brain death. Our data demonstrate that plasmapheresis may transiently improve the encephalopathy of acute hepatic failure but is not curative alone. Therefore, plasmapheresis may be a useful adjunct in the treatment of liver failure, potentially improving the pretransplantation status of the patient.

Brain Stem