The Lennox-Gastaut syndrome: electroencephalographic characteristics, clinical correlates, and follow-up studies.

Electroencephalographic and clinical findings are reported for 100 patients with the Lennox-Gastaut (LGS) triad of slow bilateral spike and wave (BSW), retardation and multiple seizures. Neurological and mental deficits were frequently observed, especially in patients who developed seizures before age 1 yr. More than half of the patients had focal epileptiform discharges that peaked in occurrence at age 4-6 yrs. EEG follow-up showed that background frequency slowed when patients developed the LGS pattern, and increased after recovery. Only 2 patients developed normal EEGs on follow-up, although 22 patients no longer showed the LGS pattern after an average of 3 yrs 3 mos follow-up.

Acute hypertensive encephalopathy in minimal change nephrotic syndrome.

A 3-year-old child with minimal change nephrotic syndrome (MCNS) developed an acute hypertensive encephalopathy characterized by coma, focal seizures, right hemiparesis, global aphasia and cortical blindness. Episodic hypertension and seizures persisted for 24 h despite intervention with antihypertensive and anticonvulsant therapy. Clinical suspicion of cortical blindness was confirmed by visual-evoked potential studies. CT scans performed 14 and 21 days after the acute episode demonstrated symmetric occipital white matter lucencies compatible with ischemia and/or associated edema. Hypertensive encephalopathy with cortical blindness and symmetric white matter hypodense lesions visualized on CT scan have recently also been described in eclampsia of pregnancy. This report documents an unusual acute hypertensive encephalopathy in childhood MCNS, unassociated with membranoproliferative glomerulonephritis, or progressive focal glomerulosclerosis.

The measurement of performance in childhood cancer patients.

The performance status of the child with cancer is an important outcome consideration in pediatric oncology research and practice. However, no single measure for children has been available. This is a report of the development and standardization of such a scale. The play-performance scale for children is a parent-rated instrument which records usual play activity as the index of performance. Performance status ratings were obtained on three groups of children: patients (n = 98), patients' siblings (n = 29), and an independent sample of hospital employees' children (n = 40). Children with all types and stages of childhood malignant neoplasms were represented. Test results established the parent as a competent, reliable rater and demonstrated the validity of the scale. Interrater reliability was examined using correlational statistics and percentage agreement. Agreement between parents was good, and there were no systematic rater biases. In addition, parents' ratings significantly discriminated differences in levels of functioning (mean score, patients 75.4 versus siblings 97.4). Correlational and analysis of variance (ANOVA) procedures demonstrated that the play-performance scale was significantly related to the global performance measures of experienced clinicians and was sensitive to change. Inpatients received a mean score of 42.3, outpatients 90.7, and normals 98.2. These findings indicate that the scale is both feasible and effective. It is concise, can be administered repeatedly even to extremely ill patients, and uses parents as observer reporters. The play-performance scale for children provides quantifiable, reproducible, and meaningful data, which is necessary for effective monitoring and management of the child with cancer.

Pitfalls in diagnosing brain death in infancy.

A 3-year-old child with phenotypic trisomy 18 syndrome survived 26 days after a cardiopulmonary arrest, secondary to an acute viral illness. The child was deeply comatose. No barbiturates, other sedatives, or aminoglycoside antibiotics had been recently administered. The child was normothermic with adequate cardiovascular function. Brain stem function was absent, as assessed by testing of brain stem reflexes. Serial cerebral radionuclide angiograms (CRAG) documented intact cerebral blood flow while electrocerebral silence (ECS) was present on two consecutive EEG recordings within 24 hours. Preservation of intracranial circulation was confirmed by rapid rotational computed tomographic (CT) scans. Cranial CT scans also revealed communicating hydrocephalus, and bilateral basal ganglia hemorrhages. This unusual case illustrates discordance between apparent irreversible loss of cortical function as indicated by electrocerebral silence with preserved cerebral blood flow. The implications of these apparent paradoxical events will be discussed in the context of defining brain death in children.

Normal cerebral radionuclide angiogram in a child with electrocerebral silence.

Pediatric neurologists agree that the determination of brain death in children, and especially retarded children, is difficult and that the criteria used in adult brain death may not be sufficient in pediatric cases. An unusual case of sustained electrocerebral silence on electroencephalogram (EEG) in a three-year-old retarded comatosed child with preserved intracerebral perfusion documented by a series of cerebral radionuclide angiograms (CRAG) is presented. The EEG showing electrocerebral silence represents loss of cerebrum (cortex) function (Barlow 1976). This absence of cortical function is demonstrated even though intracranial circulation is shown to be intact. We believe that the correlative studies presented accurately document a discordance between apparent loss of cortical function in a child as indicated by electrocerebral silence in the face of preserved cerebral blood flow. It is suggested that when evaluating brain death in retarded children with known cerebral atrophy, special emphasis should be placed on the CRAG and that the EEG should be read with caution.

Toward the development of a play performance scale for children (PPSC).

Performance scales (i.e., Karnofsky), as they measure quality of life, have been used effectively as an integral part of repeated assessment of adult cancer patients for the last several years. An equally concise measure of performance has not been developed for children. The task of developing a scale to assess performance in infants, toddlers, school-age children, and adolescents is formidable, as the activity measured should be of equal merit at each age level. Although all childhood cancer patients could benefit from a simple-to-administer, rapid assessment, children with brain tumors have the greatest need for a repeated measure of performance. The goal, then, is to develop a simplified set of criteria that can be used for assessment of children with brain tumors during hospitalization, at the time of clinic visits, and/or at the time of diagnostic procedures when the patient is in a reasonable state of health. The assessment should be able to performed by nonprofessional persons.

Central nervous system prophylaxis. Studies showing impairment in verbal skills and academic achievement.

School attendance and school achievement were the parameters studied to assess the pediatric cancer patient's ability to learn and keep pace with their peers. Effects of CNS prophylaxis, as either intrathecal methotrexate (IT) alone or intrathecal methotrexate given in addition to cranial radiation (CRT), were studied in two groups. A third group of cancer patients who received no CNS prophylaxis, and two comparison groups, siblings and a matched sample of children, also participated in the study. Impairment in central nervous system function was measured by means of psychological testing, neurological examination, and computer-assisted tomography. Patients who received central nervous system prophylactic treatments at an early age had poorer performance on verbal IQ scores, with comprehension and arithmetic subscores being most affected. Patients who received both cranial radiotherapy plus intrathecal methotrexate showed a decrease in six out of seven categories of instruction when grades from the year prior to diagnosis were compared to those obtained 3 years after diagnosis. The combined groups of patients with leukemia had a lower grade point average and poorer school attendance than did the comparison groups.

Application of three-dimensional computer reconstruction from computerized tomography to intracranial tumors in children.

A three-dimensional surface reconstruction algorithm was devised from contour information based upon anatomic structures identified by computed tomographic (CT) scanning. The triangular 'tiling' method from which the algorithm was derived accurately reconstructs the surfaces between contours of both supra- and infra-tentorial anatomic sites. Volume and surface area of the lateral ventricles and tumor site were calculated from the surface reconstruction algorithm in four children with intracranial mass lesions. Reconstruction of three-dimensional surfaces from CT head scans could be utilized to plan the surgical approach to supra-tentorial and posterior fossa lesions. The incorporation of this technique may greatly assist neurosurgeons and neuro-oncologists in planning multi-modality therapy and also for localization of small and relatively inaccessible lesions which may not be adequately assessed by conventional methods. Furthermore, the efficacy of radiation therapy and/or chemotherapy in brain tumors can accurately be assessed.

Hemiplegic migraine in childhood: diagnostic and therapeutic aspects.

Focal neurologic deficits, particularly hemiplegia, are occasionally observed in the pediatric migrainous population during the headache attack and are often mistaken for other neurologic or neurosurgical conditions. Clues to the correct diagnosis, illustrated by three patients in this report are: (1) rapid spontaneous recovery from the acute neurologic deficit, (2) striking electroencephalographic abnormalities, frequently consisting of focal slow waves during the acute stage, with rapid resolution, (3) significant past history of recurrent episodes, and (4) positive family history of migraine. Review of previously reported cases indicates that cerebral arteriography in the acute stage should be avoided if the clinical syndrome is typical. Preliminary results of prophylactic treatment with propranolol are encouraging; smaller dosage than those previously recommended sometimes can be effective.

Facial nerve schwannoma in a 16-year-old girl.

A 16-year-old girl with unilateral facial nerve schwannoma in the cerebellopontine angle is presented in this report. The characteristic computerized tomography (CT) findings described in schwannomas and the patient's clinical findings established the preoperative diagnosis of a rare but benign tumor in the pediatric age group. The value of early diagnosis of potentially resectable benign tumors is emphasized.

Hypothermic total body washout and intracranial pressure monitoring in Stage IV Reye syndrome.

The number of children in this report treated with either TBW or exchange transfusions is small. Case mortality rates among children with Reye syndrome in Stage IV coma tends to be exceedingly high, varying from 50 to 100%. Intracranial pressure monitoring with the subarachnoid screw may have been an additional factor in increasing our survival data in three patients in the TBW group, since it provided continuous monitoring of ICP and allowed judicious administration of mannitol intravenously. Survival of five of six patients without neurologic sequelae in the present series has encouraged us to coninue utilization of TBW in children with Stage IV Reye syndrome.

Mycotic aneurysm of the internal carotid artery in an unusual intra-cranial location.

A child is reported who developed a mycotic aneurysm of the cavernous portion of the internal carotid artery following staphylococcal cellulitis and septicemia. The unique feature of this case was the conspicuous absence of clinical or arteriographic evidence of carvernous sinus thrombosis. The pathogenesis of mycotic aneurysms is discussed.