adrenocorticotropin-zinc and Spasms--Infantile

West syndrome (WS) is one of the catastrophic epileptic syndromes in infancy characterized by a triad of infantile spasms, psychomotor deterioration and hypsarrhythmic EEG pattern. WS is commonly associated with poor long-term outcome, especially in symptomatic cases, with development of other seizure types, impaired cognitive and psychosocial functioning. The aim of our study was to evaluate the efficacy of the control of infantile spasms using synthetic ACTH or vigabatrin in newly diagnosed cases and to correlate it with the underlyning causes, outcome and adverse effects.. The database of children with WS seen at the Neuropediatric Unit and followed at outpatient clinics from January 1, 1994 until December 31, 2003 were reviewed. The diagnosis of WS following the criteria of ILAE was made in 32 patients.. Data were collected for 32 children (9 girls and 23 boys). According to the etiology, 5 (15.6%) were cryptogenic, and 1 (3.1%) was idiopathic. In 26 (81.2%) symptomatic cases, hypoxic-ischemic encephalopathy (69.2%) was the most common etiologic factor, followed by central nervous system anomaly including malformation of cortical development (11.5%), and Sturge Weber syndrome (3.8%), and chromosomal translocation with Down syndrome (11.5%). In 65.1% of symptomatic cases birth occurred prematurely. The mean age at spasm onset was 5.8 months, and mean age at diagnosis and treatment 7.2 months. Between 1994 and 1996 synthetic ACTH was used for treatment of WS in 7 patients (1 cryptogenic and 6 symptomatic), spasm control was achieved in 6, hypsarrhythmia disappeared in 5, and vigabatrin was added after synthetic ACTH in 3 patients. In one child synthetic ACTH was stopped because of arterial hypertension. All children had Cushing syndrome. After 1996, vigabatrin was administrated to 5 children with cryptogenic and 20 children with symptomatic WS. In 22/32 spasm control was achieved within 15 days. Synthetic ACTH was added in 3 children with spasms and hypsarrhythmia disappeared in 1 child. There was no recurrence of WS. The mean follow-up in 27 children was 4.6 (0.5 to 9.9 years) whereas 5 were lost from follow-up. Of 6/27 children with cryptogenic WS, 1 had idiopathic WS, 3 had normal psychomotor development and 2 had psychomotor retardation, without epileptic fits and still receiving AED. Of 21/27 children with symptomatic WS 76.2% had severe psychomotor retardation, 42.8% had epilepsy, 23.8% had intractable epileptic fits, and 2 children with Down syndrome were without epilepsy and without AED. Lennox-Gastaut syndrome developed in 14.2% (3/21 children); 1 of them died at the age of 3.5 years from acute gastric bleeding during the administration of synthetic ACTH, and an other child died at the age of 5.5 years from infection and respiratory insufficiency. The mortality rate was 7.4% (2/27 children).. The cryptogenic etiology is associated with a very low risk of poor outcome in WS. In children with normal development and regular school performance an idiopathic etiology can be presumed. The children with Down syndrome had a relatively benign outcome with regard to seizure control compared with symptomatic infantile spasms in the general population. In symptomatic WS caused by hypoxic-ischemic encephalopathy the outcome was linked with coexistence of other forms of epilepsy and neurologic deficit. The poor prognosis concerning intractable nature of the seizures and serious neurologic deficit is recorded in children with malformation of cortical development and Sturge Weber syndrome. The outcome of these children is determined by the brain damage other than by epilepsy itself. Regarding the treatment with synthetic ACTH or vigabatrin, the control of WS was the same for cryptogenic and symptomatic forms, one drug may be effective if the other drug fails. Synthetic ACTH can have many side effects, even death. The visual field defect is associated with vigabatrin, but can be avoided with careful funduscopic follow-up. Vigabatrin can be suggested as the first drug for WS; if spasms persist after 15 days with a dose of 150 mg/kg, synthetic ACTH should be considered.

Topics: Anticonvulsants; Cosyntropin; Delayed-Action Preparations; Female; Humans; Infant; Infant, Newborn; Male; Prognosis; Spasms, Infantile; Vigabatrin

To evaluate the ocular changes and medical and surgical therapy after high-dose systemic steroid treatment in babies with infantile spasm and hypsarrhythmia.. Retrospective, noncomparative, interventional case series.. In 5 of the 9 (55%) babies with infantile spasm exposed to systemic corticosteroid treatment, an increase in intraocular pressure (IOP) and optic disc cupping was observed.. Ophthalmic examination under mild sedation was conducted 3 to 4 weeks after initiation of systemic therapy. Antiglaucoma treatment was given to the patients found to have high IOPs and cup-to-disc ratio changes. Routine follow-up was continued until systemic therapy was completed.. Controlled IOP with a decrease in cupping damage after antiglaucoma therapy.. Five patients required antiglaucoma treatment; one also underwent augmented trabeculectomy. Mean IOP decreased in this subgroup from 30.1 +/- 9.5 mmHg to 15.4 +/- 4.2 mmHg in the right eye (P = 0.043) and from 32.6 +/- 7.4 mmHg to 15.2 +/- 1.8 mmHg in the left eye (P = 0.043). Mean cup-to-disc ratio improved from 0.53 +/- 0.2 to 0.37 +/- 0.04 in the right eye (P = 0.06) and from 0.57 +/- 0.12 to 0.35 +/- 0.05 in the left eye (P = 0.042).. The rapid onset of IOP and cup-to-disc ratio changes in patients with infantile spasm and hypsarrhythmia treated by high-dose corticosteroids necessitates early and intensive monitoring to prevent anatomic ocular damage and visual impairment in the future.

Topics: Administration, Oral; Antihypertensive Agents; Cosyntropin; Female; Glucocorticoids; Humans; Infant; Injections, Intramuscular; Intraocular Pressure; Male; Ocular Hypertension; Optic Disk; Optic Nerve Diseases; Prednisone; Retrospective Studies; Spasms, Infantile; Tonometry, Ocular

Forty two children with West's syndrome who had been treated in the Clinic of Paediatrics, Higher Medical Institute, Plovdiv in the last 10 years were entered into the present study. Analysis is made of the aetiology of the disease, the results of treatment and development of the children. All children were followed up from 6 months to 10 years. The West's syndrome was idiopathic in four children and symptomatic in 38 children (90.5%). It had perinatal aetiology in 76.5% of the patients, prenatal in 21%, and postnatal in 2.6%. Complete seizure control was achieved in 17 children (40.5%) treated only with antiepileptic drugs. Synacthen was included in the treatment of the remaining 22 children in three therapeutic doses--0.0125 mg/kg/day (n = 8), 0.025 mg/kg/day (n = 8), and > or = 0.05 mg/kg/day (n = 6). Treatment with different doses of Synacthen showed no statistically significant differences in the three groups. The side effects of the treatment occurred more frequently and were more severe in the groups with a high-dose Synacthen treatment. The follow-up established mental retardation and/or neurological deficit in 88.1% of the children. One infant died during the treatment with Synacthen and another two with severe mental retardation--one year after treatment. In about one third of the cases transition was observed to other epileptic syndromes. Synacthen is concluded to be efficacious in the treatment of West's syndrome. If antiepileptic drugs fail to produce any effect Synacthen should be included in the therapy in due time, preferably in small doses in order to avoid severe and unwanted side effects.

Topics: Cosyntropin; Delayed-Action Preparations; Dose-Response Relationship, Drug; Echoencephalography; Electroencephalography; Female; Follow-Up Studies; Humans; Infant; Male; Prognosis; Retrospective Studies; Spasms, Infantile; Tomography, X-Ray Computed; Treatment Outcome

We report a patient who began to have clusters of seizures characterized by brief elevation of the right arm at 6 months of age. An interictal electroencephalogram (EEG) at 7 months revealed hypsarrhythmia without definite asymmetry. Simultaneous EEG and video recording disclosed that these focal spasms were associated with fast wave bursts superimposed on slow waves most markedly in the left centro-midtemporal region. The patient became seizure-free after synthetic ACTH therapy. The patient is developmentally normal at 3 years 5 months, but magnetic resonance imaging studies revealed findings suggestive of delayed myelination in the left frontal region. This patient is considered to have had an unusual variant of West syndrome associated with focal delayed myelination.

Topics: Adolescent; Child, Preschool; Cosyntropin; Delayed-Action Preparations; Dominance, Cerebral; Electroencephalography; Epilepsies, Partial; Evoked Potentials; Follow-Up Studies; Frontal Lobe; Humans; Infant; Magnetic Resonance Imaging; Nerve Fibers, Myelinated; Spasms, Infantile

Serum cortisol, prolactin (PRL), TSH, GH, LH and FSH levels were measured before and immediately after daily ACTH-Z therapy (0.01 mg/kg/day, 1-2 weeks) for 5 patients with infantile spasms and one patient with myoclonus epilepsy. Total number of ACTH-Z therapy were 8 times, and all patients became seizure free after ACTH-Z therapy. In 6 occasions, TRH, LH-RH and insulin tolerance tests were performed before and after daily ACTH-Z therapy. Serum cortisol levels were significantly increased after daily ACTH-Z therapy but all other hormone levels were significantly decreased. In TRH and LH-RH tolerance tests, peak levels and increments of PRL, LH and FSH were significantly decreased after daily ACTH-Z therapy and those of TSH were mildly decreased. In one case insulin tolerance test revealed an adequate decrease of blood glucose before and after ACTH-Z therapy, and there was a poor GH response after ACTH-Z therapy. Daily ACTH-Z therapy was thought to suppress secretion of anterior pituitary hormones.

Topics: Child, Preschool; Cosyntropin; Female; Humans; Infant; Male; Pituitary Gland, Anterior; Spasms, Infantile

Two infants developed hyperkalemia shortly after cessation of prolonged ACTH therapy for infantile spasms. We wish to call for cautious approach at time of cessation of prolonged ACTH therapy because of possible unexpected and only partially understood hazardous side effects such as hyperkalemia.

Topics: Cosyntropin; Electroencephalography; Female; Humans; Hyperkalemia; Infant; Long-Term Care; Male; Spasms, Infantile

Topics: Adrenocorticotropic Hormone; Cosyntropin; Humans; Hydrocortisone; Infant; Prognosis; Spasms, Infantile

Topics: Adrenocorticotropic Hormone; Cosyntropin; Female; Humans; Hydrocortisone; Infant; Male; Spasms, Infantile

A case of subdural hematoma following ACTH-Z therapy for infantile spasms was presented. A female baby of 5 months old showed little clinical evidence of cerebral dysfunction associated with subdural hematoma. There have been several reports about the relationship between steroid treatment and apparent brain atrophy on the CT brain scans. Then, we studied the CT brain scans before and after ACTH-Z therapy for infantile spasms, atonic seizure or Lennox syndrome and showed some relationship between apparent brain atrophy on the CT brain scans and ACTH-Z treatment. We also discussed the possible etiology of apparent brain atrophy and subdural hematoma, and stressed the necessity of extreme caution with long-term ACTH-Z administration.

Topics: Adrenocorticotropic Hormone; Atrophy; Brain; Cosyntropin; Electroencephalography; Female; Hematoma, Subdural; Humans; Infant; Spasms, Infantile; Tomography, X-Ray Computed