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Biomedical subjects

E J Horton

Publications and source records attributed to E J Horton.

12 recordsLinked to original sources
PubMed1996

High-dose corticotropin (ACTH) versus prednisone for infantile spasms: a prospective, randomized, blinded study.

OBJECTIVE: To compare the efficacy of corticotropin (ACTH) (150 U/m2/day) and prednosone (2 mg/kg/day) given for 2 weeks, in suppressing clinical spasms and hypsarrhythmic electroencephalogram (EEG) in infantile spasms (IS). AACTH and prednisone are standard treatments for IS. ACTH at high doses causes severe dose- and duration-dependent side effects, but may be superior to prednisone, based on retrospective or uncontrolled studies. Blinded prospecive studies have shown equal efficacy of prednisone and low-dose ACTH, and low versus high-dose ACTH. DESIGN: A prospective, randomized, single-blinded study. SUBJECTS AND METHODS: Patient population consisted of consecutive infants fulfilling entry criteria, including the presence of clinical spasms, hypsarrhythmia (or variants) during a full sleep cycle video-EEG, and no prior steroid/ACTH treatment. Response required both cessation of spasms and elimination of hypsarrhythmia by the end of the 2-week treatment period, as determined by an investigator "blinded" to treatment. Treatment of responders was tapered off over 12 days; those failing one hormone were crossed-over to the other. RESULTS: OF 34 eligible infants, 29 were enrolled. Median age of patients was 6 months. Twenty-two infants were "symptomatic" with known or suspected cause, and seven were cryptogenic (two normal). Of 15 infants randomized to ACTH, 13 responded by EEG and clinical criteria (86.6%); Seizures stopped in an additional infant, but EEG remained hypsarrhythmic (considered a failure). Four of the 14 patients given prednisone responded (28.6%,, with complete clinical-EEG correlation), significantly less than with ACTH, (chi2 test). CONCLUSIONS: Using a prospective, randomized approach, a 2-week course of high-dose ACTH is superior to 2 weeks of prednsone for treatment of IS, as assessed by both clinical and EEG criteria.

Adrenocorticotropic Hormone↗
PubMed1995

Clinical utility of video-EEG monitoring.

This study investigated the efficiency of simultaneous video-electroencephalography (EEG) monitoring in documenting paroxysmal events, the value in clinical diagnosis, and the effect on treatment. In this retrospective review, 230 children underwent this procedure between January, 1990 and December, 1992. The data demonstrated that video-EEG monitoring can be used as a daytime procedure with a high success rate (80%) in detecting and differentiating the nature of recurrent paroxysmal behaviors that have occurred on a daily basis. Video-EEG monitoring has a high diagnostic rate in differentiating seizure versus nonseizure events (70%), in classifying seizure types (88%), and in evaluating the candidacy for epilepsy surgery (64%). Video-EEG diagnosis resulted in an alteration of clinical management in 45% of patients. Continuous video-EEG monitoring is an efficient and valuable procedure in the diagnosis and management of paroxysmal disorders in children.

Anticonvulsants↗
PubMed1995

Cerebrospinal fluid corticotropin and cortisol are reduced in infantile spasms.

Infantile spasms respond to ACTH, and levels of the hormone in cerebrospinal fluid of untreated infants with this disorder were found to be lower than in age-matched controls. In this study we analyzed cerebrospinal fluid cortisol and ACTH using improved immunoassays in a larger cohort of infants with infantile spasms. Analysis of 20 patients and 15 age-matched controls revealed significantly lower levels of both ACTH and cortisol in the cerebrospinal fluid. These data, combined with the efficacy of ACTH and glucocorticoids for infantile spasms, support an involvement of the brain-adrenal-axis in this disorder.

Adrenocorticotropic Hormone↗
PubMed1995

Abnormal somatosensory evoked potentials in patients with classic galactosemia: correlation with neurologic outcome.

In classic galactosemia, long-term neurologic sequelae can include low cognitive functioning and a curious neurologic syndrome with tremors, dysmetria, and ataxia. An abnormal white-matter signal on cerebral magnetic resonance imaging (MRI) is present in almost all patients; some have mild cerebral or cerebellar atrophy and focal white-matter lesions. The present study was undertaken to assess the integrity of myelinated pathways by recording somatosensory evoked potentials. Results were correlated with age at diagnosis, severity of illness, age at evoked potentials, neurologic examination, MRI studies and cognitive outcome as measured by the Woodcock-Johnson Revised Standard Cognitive Battery. Evoked potentials were abnormal in 17 (28%) of 60 patients who had median nerve, and 26 (77%) of 34 patients who had posterior tibial nerve studies. Abnormalities of the central rather than the peripheral nervous system were most common. Evoked potentials correlated with severity of presenting symptoms (P = .011), age at evoked potential testing (P = .029), and presence of focal white-matter lesions on MRI (P = .049). Results of neurophysiologic testing showed no correlation with the Woodcock-Johnson Battery. Patients with classic galactosemia may have abnormal conduction along myelinated pathways that is associated with other central deficits. Myelin, which contains galactose, may be adversely affected in this inborn error of metabolism.

Adolescent↗
PubMed1992

Depressed interferon synthesis in skin fibroblasts from lung cancer patients.

Skin fibroblast cell cultures, derived from male adult lung cancer patients, an adult control population, and a newborn population were examined for their susceptibility to transformation with Kirsten murine sarcoma virus and their ability to respond to an interferon inducer (poly I.poly C). An association between sensitivity to viral transformation and induction of interferon was observed. Cultures derived from lung cancer patients demonstrated an increased sensitivity to virus transformation and a decreased ability to respond to interferon induction as compared with age-matched controls and newborns.

Adult↗
PubMed1992

Brain-adrenal axis hormones are altered in the CSF of infants with massive infantile spasms.

Massive infantile spasms (MIS), a seizure disorder unique to infants, is considered an age-dependent response of the immature brain to various insults and stressors. The seizures improve with ACTH and glucocorticoids, both major components of the brain-adrenal axis. We hypothesized that CNS levels of these hormones are abnormal in infants with MIS and studied CSF from 14 infants with MIS and 13 age-matched controls by analysis for corticotropin-releasing hormone (CRH), ACTH, cortisol, and interleukin-1-beta. ACTH levels in CSF of patients were significantly lower than those of controls, but differences in cortisol levels between patients and controls were not statistically significant. CRH levels in both groups were similar and fluctuated diurnally. These results indicate an alteration of specific CNS components of the brain-adrenal axis in MIS.

Adrenocorticotropic Hormone↗
PubMed1990

Rhythmic coma in children.

We describe a syndrome of rhythmic coma in children that consists of an invariant, nonreactive, diffuse cortical activity of a specific frequency, such as alpha, beta, spindle, or theta, recorded from a comatose child. We report 11 cases of children who were found to be in rhythmic coma during their acute illnesses. Their ages ranged from 2 to 15 years, and their diagnoses included encephalitis, head trauma, seizures, near drowning, brain tumors, stroke, and metabolic derangements. The specific frequency of the electroencephalographic pattern, ie, alpha, beta, spindle, or theta, did not influence the outcome. The clinical outcome appeared to depend on the primary disease process rather than the electroencephalographic finding. The prognosis of alpha-frequency rhythmic coma as well as of rhythmic coma in general was better in children than in adults. The pathophysiology in children may be similar, ie, the interruption of reticulothalamocortical pathways by metabolic or structural abnormalities, but the expression of this deafferentation may be more varied in the developing brain. Thus, we propose the term rhythmic coma as a unified concept for alpha, beta, spindle, and theta coma in children.

Adolescent↗
PubMed1985

Depressed interferon synthesis in skin fibroblasts from lung cancer patients.

Skin fibroblast cell cultures, derived from male adult lung cancer patients, an adult control population, and a newborn population were examined for their susceptibility to transformation with Kirsten murine sarcoma virus and their ability to respond to an interferon inducer (poly I X poly C). An association between sensitivity to viral transformation and induction of interferon was observed. Cultures derived from lung cancer patients demonstrated an increased sensitivity to virus transformation and a decreased ability to respond to interferon induction as compared with age-matched controls and newborns.

Adult↗
PubMed1980

Inhibitory effects of an aromatic retinoid (Ro 10-9359) on viral tumorigenesis.

Prophylactic treatment of hamsters and chickens with an aromatic retinoid (Ro 10-9359) inhibited Rous sarcoma virus (RSV) tumorigenesis. Retinoid administration to chickens with RSV-induced tumors resulted in tumor regression and/or confinement to the primary site. The retinoid also exerted a therapeutic effect on Shope virus papilloma development in the skin of rabbits.

Animals↗
PubMedDate not supplied

Compulsive somatosensory self-stimulation inducing epileptic seizures.

Three children who had been diagnosed as having infantile spasms with major psychomotor retardation were referred for the persistence of series of spasm-like seizures, which were resistant to antiepileptic drug treatment, beyond infancy. Serial seizures were elicited by a compulsive self-stimulating behavior that was documented by long-term video-polygraphy. These behaviors implicated proprioceptive inputs: tapping on the chin in one case, flexion and external rotation of the leg with dislocation of the femur in one case, a particular posturing of the body followed by rhythmic shaking of the head in the last case. This clinical picture seems to constitute a particular type of outcome for infantile spasms and is particularly resistant to therapy.

Cerebral Cortex↗