cosyntropin and Spasms--Infantile

Topics: Anticoagulants; Anticonvulsants; Aspirin; Cosyntropin; Female; Gangliosides; Humans; Infant; Infant, Newborn; Male; Myelin Sheath; Spasms, Infantile; Thrombosis; Thyroid Hormones; Tuberous Sclerosis; Vitamin B 6; Warfarin

Infantile spasms (West's Syndrome) is a syndrome that includes a peculiar type of epileptic seizure-the spasms-and an electroencephalographic (EEG) abnormality often called hypsarrhythmia. Psychomotor retardation is frequently found at follow-up. Approximately two-thirds of affected infants will have a detectable underlying neurological abnormality, but still little is known about the pathophysiological basis for infantile spasms, and treatment remains problematic.. To compare the effects of single pharmaceutical therapies used to treat infantile spasms in terms of control of the spasms, resolution of the EEG, relapse rates, psychomotor development, subsequent epilepsy, side effects, and mortality.. To identify published data, we searched the Cochrane Epilepsy Group Specialised Register (October 2012), CENTRAL (The Cochrane Library 2012, Issue 9), MEDLINE (1946 to September Week 4, 2012), EMBASE (1980 to March 2003), and the reference lists of all retrieved articles.To identify unpublished data, we searched the ISRCTN Register (www.controlled-trials.com), corresponded with colleagues and drug companies, and made requests at international conferences.. All randomised controlled trials (RCTs) of the administration of drug therapy to patients with infantile spasms.. Data collection from all relevant publications was independently undertaken by three review authors (before 2010) or by two review authors using a standard proforma. Analysis included assessment of study quality and a search for sources of heterogeneity.. We found 16 small RCTs (fewer than 100 patients enrolled) and 2 larger RCTs (more than 100 patients enrolled). These 18 studies looked at a total of 916 patients treated with a total of 12 different pharmaceutical agents. Overall methodology of the studies was poor, in part because of ethical dilemmas such as giving placebo injections to children. Two studies showed that placebo was not as good as active treatment in resolving the spasms. The strongest evidence suggested that hormonal treatment (prednisolone or tetracosactide depot) leads to resolution of spasms faster and in more infants than does vigabatrin. Responses without subsequent relapse may be no different. The same study suggests that hormonal treatments might improve the long-term developmental outcome compared with vigabatrin in infants not found to have an underlying cause for their infantile spasms.. To date, few well-designed RCTs have considered the treatment of infantile spasms, and the numbers of patients enrolled have been small. In the majority, methodology has been poor, hence it is not clear which treatment is optimal in the treatment of this epilepsy syndrome. Hormonal treatment resolves spasms in more infants than vigabatrin, but this may or may not translate into better long-term outcomes. If prednisolone or vigabatrin is used, high dosage is recommended. Vigabatrin may be the treatment of choice in tuberous sclerosis. Resolution of the EEG features may be important, but this has not been proven. Further research using large studies with robust methodology is required.

Topics: Anticonvulsants; Cosyntropin; Hormones; Humans; Infant; Prednisolone; Psychomotor Performance; Randomized Controlled Trials as Topic; Spasms, Infantile; Vigabatrin

Topics: Cosyntropin; Diagnosis, Differential; Electroencephalography; Humans; Infant; Infant, Newborn; Prognosis; Spasms, Infantile; Valproic Acid; Vitamin B 6

Infantile spasms constitute a severe form of epileptic encephalopathy. In the International Collaborative Infantile Spasms Study (ICISS), we showed that combining vigabatrin with hormonal therapy was more effective than hormonal therapy alone at stopping spasms between days 14 and 42 of treatment. In this planned follow-up, we aimed to assess whether combination therapy was associated with improved developmental and epilepsy outcomes at 18 months of age.. In ICISS, a multicentre, open-label, randomised controlled trial, infants were enrolled from 102 hospitals (three in Australia, 11 in Germany, two in New Zealand, three in Switzerland, and 83 in the UK). Eligible infants had a clinical diagnosis of infantile spasms and a hypsarrhythmic (or similar) electroencephalogram (EEG) no more than 7 days before enrolment. Participants were randomly assigned (1:1) by a secure website to receive hormonal therapy with vigabatrin or hormonal therapy alone. If parents consented, there was an additional randomisation (1:1) of type of hormonal therapy used (prednisolone or tetracosactide depot). Block randomisation was stratified for hormonal treatment and risk of developmental impairment. Parents and clinicians were not masked to therapy, but investigators assessing epilepsy and developmental outcomes at 18 months were masked to treatment allocation. Minimum doses were oral prednisolone 10 mg four times a day or intramuscular tetracosactide depot 0·5 mg (40 IU) on alternate days with or without oral vigabatrin 100 mg/kg per day. The primary outcome at 18 months was development as assessed by the Vineland Adaptive Behaviour Scales (VABS) composite score. Secondary outcomes were the presence or absence of epileptic seizures or infantile spasms in the previous 28 days, as recorded by parents and carers, and the use of any anti-epileptic treatment (including ketogenic diet) in the previous 28 days. Analysis was by intention to treat. The trial is registered with the ISRCTN registry, number 54363174, and EudraCT, number 2006-000788-27.. Between March 7, 2007, and May 22, 2014, 766 infants were screened and, of those, 377 were randomly assigned to hormonal therapy with vigabatrin (n=186) or hormonal therapy alone (n=191). 362 infants were assessed for developmental and epilepsy outcomes at 18 months, 181 in each treatment group. Mean VABS scores did not differ significantly between the combination therapy group and the hormonal therapy alone group (73·9 [SE 1·3] vs 72·7 [1·4], difference -1·2 [95% CI -4·9 to 2·6], p=0·55). Presence of epilepsy at the assessment at age 18 months was similar in both treatment groups (54 [30·0%] of 180 infants who received combination therapy vs 52 [29·2%] of 178 who received hormonal therapy alone; difference 0·8% [95% CI -8·8 to 10·4], p=0·90). Presence of spasms was also similar in both treatment groups (27 [15·0%] of 180 infants on combination therapy vs 28 [15·7%] of 178 on hormonal therapy alone; difference 0·7% [95% CI -6·9 to 8·3], p=0·85). At the 18-month assessment, 158 (44·1%) of 358 infants were on some form of anti-epileptic treatment. Initial control of spasms between days 14 and 42 of treatment was associated with higher mean VABS scores at 18 months (79·1 [SE 1·2] vs 63·2 [1·1], difference 15·9 [95% CI 12·4 to 19·5], p<0·001) and with higher likelihood of absence of seizures at 18 months (in 39 [17·0%] of 229 infants who achieved spasm cessation vs 67 [51·9%] of 129 who did not; difference 34·9% [24·8 to 45·0], p<0·001). Increasing lead-time to treatment was associated with lower VABS scores (analysis of variance: F[4,354]=6·38, p<0·001) and worse epilepsy outcomes (p=0·023).. Combination therapy did not result in improved developmental or epilepsy outcomes at 18 months. However, early clinical response to treatment was associated with improved developmental and epilepsy outcomes at 18 months. Longer lead-time to treatment was associated with poorer outcomes. Rapid diagnosis and effective treatment of infantile spasms could therefore improve outcomes.. The Castang Foundation, Bath Unit for Research in Paediatrics, National Institute of Health Research, the Royal United Hospitals Bath NHS Foundation Trust, BRONNER-BENDER Stiftung/Gernsbach, University Children's Hospital Zurich.

Topics: Cosyntropin; Drug Administration Schedule; Drug Therapy, Combination; Electroencephalography; Female; Humans; Infant; Male; Prednisolone; Spasms, Infantile; Vigabatrin

Infantile spasms constitutes a severe infantile epilepsy syndrome that is difficult to treat and has a high morbidity. Hormonal therapies or vigabatrin are the most commonly used treatments. We aimed to assess whether combining the treatments would be more effective than hormonal therapy alone.. In this multicentre, open-label randomised trial, 102 hospitals (Australia [three], Germany [11], New Zealand [two], Switzerland [three], and the UK [83]) enrolled infants who had a clinical diagnosis of infantile spasms and a hypsarrhythmic (or similar) EEG no more than 7 days before enrolment. Participants were randomly assigned (1:1) by a secure website to receive hormonal therapy with vigabatrin or hormonal therapy alone. If parents consented, there was an additional randomisation (1:1) of type of hormonal therapy used (prednisolone or tetracosactide depot). Block randomisation was stratified for hormonal treatment and risk of developmental impairment. Parents and clinicians were not masked to therapy, but investigators assessing electro-clinical outcome were masked to treatment allocation. Minimum doses were prednisolone 10 mg four times a day or intramuscular tetracosactide depot 0·5 mg (40 IU) on alternate days with or without vigabatrin 100 mg/kg per day. The primary outcome was cessation of spasms, which was defined as no witnessed spasms on and between day 14 and day 42 from trial entry, as recorded by parents and carers in a seizure diary. Analysis was by intention to treat. The trial is registered with The International Standard Randomised Controlled Trial Number (ISRCTN), number 54363174, and the European Union Drug Regulating Authorities Clinical Trials (EUDRACT), number 2006-000788-27.. Between March 7, 2007, and May 22, 2014, 766 infants were screened and, of those, 377 were randomly assigned to hormonal therapy with vigabatrin (186) or hormonal therapy alone (191). All 377 infants were assessed for the primary outcome. Between days 14 and 42 inclusive no spasms were witnessed in 133 (72%) of 186 patients on hormonal therapy with vigabatrin compared with 108 (57%) of 191 patients on hormonal therapy alone (difference 15·0%, 95% CI 5·1-24·9, p=0·002). Serious adverse reactions necessitating hospitalisation occurred in 33 infants (16 on hormonal therapy alone and 17 on hormonal therapy with vigabatrin). The most common serious adverse reaction was infection occurring in five infants on hormonal therapy alone and four on hormonal therapy with vigabatrin. There were no deaths attributable to treatment.. Hormonal therapy with vigabatrin is significantly more effective at stopping infantile spasms than hormonal therapy alone. The 4 week period of spasm cessation required to achieve a primary clinical response to treatment suggests that the effect seen might be sustained, but this needs to be confirmed at the 18 month follow-up.. The Castang Foundation, Bath Unit for Research in Paediatrics, National Institute of Health Research, the Royal United Hospitals Bath NHS Foundation Trust, the BRONNER-BENDUNG Stifung/Gernsbach, and University Children's Hospital Zurich.

Topics: Anticonvulsants; Cosyntropin; Drug Administration Schedule; Drug Therapy, Combination; Electroencephalography; Female; Follow-Up Studies; Hormones; Humans; Infant; Male; Prednisolone; Spasms, Infantile; Treatment Outcome; Vigabatrin

Infantile spasms is a severe infantile seizure disorder. Several factors affect developmental outcome, especially the underlying etiology of the spasms. Treatment also affects outcome. Both age at onset of spasms and lead time to treatment (the time from onset of spasms to start of treatment) may be important. We investigated these factors.. Developmental assessment using Vineland Adaptive Behaviour Scales (VABS) at 4 years of age in infants enrolled in the United Kingdom Infantile Spasms Study. Date of or age at onset of spasms was obtained prospectively. Lead time to treatment was then categorized into five categories. The effects of lead time to treatment, age of onset of spasms, etiology, and treatment on developmental outcome were investigated using multiple linear regression.. Age of onset ranged (77 infants) from <1 to 10 months (mean 5.2, standard deviation 2.1). Lead time to treatment was 7 days or less in 11, 8-14 days in 16, 15 days to 1 month in 8, 1-2 months in 15, >2 months in 21 and not known in 6. Each month of reduction in age at onset of spasms was associated with a 3.1 [95% confidence interval (CI) 0.64-5.5, p = 0.03] decrease, and each increase in category of lead time duration associated with a 3.9 (95% CI 7.3-0.4, p = 0.014) decrease in VABS, respectively. There was a significant interaction between treatment allocation and etiology with the benefit in VABS in those allocated steroid therapy being in children with no identified etiology (coefficient 29.9, p=0.004).. Both prompt diagnosis and prompt treatment of infantile spasms may help prevent subsequent developmental delay. Younger infants may be more at risk from the epileptic encephalopathy than older infants.

Topics: Age of Onset; Anticonvulsants; Child Development; Cosyntropin; Early Diagnosis; Humans; Infant; Infant, Newborn; Linear Models; Prednisolone; Prognosis; Spasms, Infantile; United Kingdom; Vigabatrin

Infantile spasms is the name given to a difficult to treat, severe infantile epilepsy with high morbidity. The United Kingdom Infantile Spasms Study (UKISS) showed that absence of spasms on days 13 and 14 after randomisation was more common in infants allocated hormonal treatments than vigabatrin. At 12-14 months, those with no identified aetiology allocated hormonal treatment had better development. However, epilepsy outcome was not affected by treatment allocated. It is not known if the difference in development persists as the infants grow.. Infants in UKISS were followed up blind to treatment allocation by telephone at a mean age of 4 years using the Vineland Adaptive Behaviour Scales (VABS) and an epilepsy questionnaire.. 9 of 107 enrolled infants had died. 77 were traced and consented to take part. The median (quartile) VABS scores were 60 (42, 97) for the 39 allocated hormonal treatment and 50 (36, 67) for the 38 allocated vigabatrin (Mann-Whitney U=575; p=0.091; median difference (95% CI): 8 (-1 to 19)). For those with no identified aetiology, VABS scores were 96 (52, 102) for the 21 allocated hormonal treatment and 63 (37, 92) for the 16 allocated vigabatrin (U=98.5; p=0.033; median difference (95% CI): 14 (1 to 42)).The proportions in each treatment group with epilepsy were similar.. For all 77 infants, development and epilepsy outcomes were not significantly different between the two treatment groups. The better development seen at 14 months in those with no identified aetiology allocated hormonal treatment was seen again at 4 years in this study.

Topics: Anticonvulsants; Child Development; Child, Preschool; Cosyntropin; Epidemiologic Methods; Glucocorticoids; Humans; Infant; Prednisolone; Severity of Illness Index; Spasms, Infantile; Treatment Outcome; Vigabatrin

Infantile spasms is a severe infantile seizure disorder that is difficult to treat and has a high morbidity. Absence of spasms on days 13 and 14 after randomisation is more common in infants allocated hormone treatments than in those allocated vigabatrin. We sought to assess whether early control of spasms is associated with improved developmental or epilepsy outcomes.. Infants enrolled in the United Kingdom Infantile Spasms Study (UKISS) were randomly assigned hormone treatment (n=55) or vigabatrin (n=52) and were followed up until clinical assessment at 12-14 months of age. We assessed neurodevelopment with the Vineland adaptive behaviour scales (VABS) at 14 months of age on an intention to treat basis.. Of 107 infants enrolled, five died and 101 survivors reached both follow-up assessments. Absence of spasms at final clinical assessment (hormone 41/55 [75%] vs vigabatrin 39/51 [76%]) was similar in each treatment group (difference 1.9%, 95% CI -18.3% to 14.4%; chi(2)=0.05; p=0.82). Mean VABS score did not differ significantly (hormone 78.6 [SD 16.8] vs vigabatrin 77.5 [SD 12.7]; difference 1.0, 95% CI -4.9 to 7.0; t(99)=0.35, p=0.73). In infants with no identified underlying aetiology, the mean VABS score was higher in those allocated hormone treatment than in those allocated vigabatrin (88.2 [17.3] vs 78.9 [14.3]; difference 9.3, 95% CI 1.2 to 17.3; t(95)=2.28, p=0.025).. Hormone treatment controls spasms better than does vigabatrin initially, but not at 12-14 months of age. Better initial control of spasms by hormone treatment in those with no identified underlying aetiology may lead to improved developmental outcome.

Topics: Adaptation, Psychological; Anti-Inflammatory Agents; Anticonvulsants; Child Development; Cosyntropin; Disease Progression; Epilepsy; Female; Humans; Infant; Infant, Newborn; Male; Prednisolone; Secondary Prevention; Spasms, Infantile; Treatment Outcome; United Kingdom; Vigabatrin

Combination therapy consisting of high-dose pyridoxal phosphate (40-50 mg/kg/day) and low-dose synthetic ACTH (0.01 mg/kg/day) was prescribed in 28 children with infantile spasms. Monotherapy with pyridoxal phosphate provided excellent seizure control in 3 of the 28 (11%) patients. ACTH was subsequently added to the regimen of the remaining 25 patients. As of 1 month after discontinuing the ACTH treatment, 21 of the 25 (84%) patients had experienced no seizures. The mean interval until seizure control was achieved was 4.1 days after the start of treatment with ACTH. The 21 patients have been monitored for a mean of 34.9 months (range 2-81 months); 6 patients (29%) have had recurrences of infantile spasms, and 10 (48%) have experienced normal development. Fourteen of the 28 patients (50%) have had transient increases in liver enzymes, but none of the patients developed more serious side effects.

Topics: Age of Onset; Anticonvulsants; Child, Preschool; Cosyntropin; Drug Administration Schedule; Drug Therapy, Combination; Female; Follow-Up Studies; Humans; Infant; Male; Pyridoxal Phosphate; Pyridoxine; Recurrence; Spasms, Infantile; Treatment Outcome

The authors systematically treated 94 patients with West syndrome using the same protocol of sodium valproate and steroids, starting with hydrocortisone (HC) orally for two weeks. If seizures stopped, HC was withdrawn; if they persisted, tetracosactrin (TA; synthetic ACTH) was administered for another two weeks then HC was slowly withdrawn. 90 per cent of the symptomatic cases were controlled by HC, the remainder by TA. 65 per cent of symptomatic cases were controlled by HC; this rose to 78 per cent if patients treated by HC then TA were included. At 31 months follow-up, the percentage of favourable results was 72 per cent for cryptogenic and 60 per cent for symptomatic cases. For the latter, best results were obtained in patients with periventricular leukomalacia, postnatal distress and porencephaly. Patients suffering from sequelae of full-term distress or encephalopathy of unknown aetiology were the most resistant.

Topics: Cosyntropin; Drug Administration Schedule; Drug Therapy, Combination; Humans; Hydrocortisone; Infant; Intelligence; Spasms, Infantile; Treatment Outcome; Valproic Acid

To report a prospectively planned analysis of two randomised controlled trials with embedded comparisons of prednisolone versus tetracosactide depot for the treatment of infantile epileptic spasms syndrome (IESS).. Individual patient data from patients randomly allocated to prednisolone or tetracosactide depot were analysed from two trials (UKISS, ICISS). The comparison was embedded within trials in which some patients also received vigabatrin but only patients receiving monotherapy with randomly allocated hormonal treatments are included in this analysis. The main outcome was cessation of spasms (Days 13-14 after randomisation). Lead time to treatment and underlying aetiology were taken into account. Cessation of spasms on Days 14-42 inclusive, electroclinical response (EEG Day 14), plus developmental and epilepsy outcomes (at 14 months in UKISS and 18 months in ICISS) are also reported. Minimum treatment was prednisolone 40 mg per day for two weeks or tetracosactide depot 0·5 mg IM on alternate days for two weeks, all followed by a reducing dose of prednisolone over two weeks.. 126 infants were included in this study. On tetracosactide depot, 47 of 62 (76%) were free of spasms on Days 13-14 compared to 43 of 64 (67%) on prednisolone (difference 9%, 95% CI -7·2% to +25·2%, chi square 1·15, p = 0·28). For Day 14-42 cessation of spasms, on tetracosactide depot, 41 of 61 (67%) were free of spasms compared to 35 of 62 (56%) on prednisolone (difference 11%, 95% CI -6·4% to +28·4%, chi square 1·51, p = 0·22). There was no significant difference in mean VABS score between infants who received prednisolone compared with those who received tetracosactide depot (74·8 (SD 18·3) versus 78·0 (SD 20·2) t = -0·91 p = 0·36). The proportion with ongoing epilepsy at the time of developmental assessment was 20 of 61 (33%) in the tetracosactide group compared with 26 out of 63 (41%) in the prednisolone group (difference 8%, 95% CI -9·2% to +25·2%, Chi [2] 0·95, p = 0·33).. With hormone monotherapy, either prednisolone or tetracosactide depot may be recommended for infantile epileptic spasms syndrome.

Topics: Anticonvulsants; Cosyntropin; Epilepsy; Humans; Infant; Prednisolone; Randomized Controlled Trials as Topic; Spasm; Spasms, Infantile; Syndrome; Treatment Outcome; Vigabatrin

To report the efficacy of intravenous (IV) synthetic ACTH (Tetracosactide) in the treatment of infantile spasms.. This is a retrospective chart review of patients with a diagnosis of infantile spasms conducted at the Pediatric Department of King Abdulaziz Medical City (KAMC) in Riyadh, Saudi Arabia, from 01-01-2005 to 31-12-2019.

Topics: Administration, Intravenous; Anticonvulsants; Cosyntropin; Humans; Infant; Retrospective Studies; Saudi Arabia; Spasms, Infantile; Treatment Outcome; Vigabatrin

To determine if treatment with ACTH for infantile spasms (IS) is associated with secondary adrenal insufficiency.. This is a retrospective study of patients diagnosed with infantile spasms and treated with ACTH between 2007 and 2018 at Soroka University Medical Center (SUMC). We reviewed the records of patients who had a post-hormonal laboratory assessment of their adrenal function; either a low dose ACTH test or a random morning cortisol level and looked for laboratory or clinical signs of adrenal insufficiency.. Between the years 2007 and 2018, 45 children were diagnosed with IS at our Pediatric Neurology Unit, 20 patients were treated with ACTH, of them 14 children met the inclusion criteria and had a post-treatment laboratory assessment of adrenal function by low dose ACTH test or morning cortisol level. Five children had a normal low dose ACTH test, two had normal morning cortisol level, five were not conclusive, and two had subnormal levels of cortisol. None of the children showed clinical signs of adrenal insufficiency.. Our study adds to the limited literature on this topic and in contrast to previous publications suggests that adrenal suppression should not occur after ACTH treatment.

Topics: Adrenal Glands; Adrenal Insufficiency; Cohort Studies; Cosyntropin; Female; Hormones; Humans; Infant; Male; Retrospective Studies; Spasms, Infantile

High dosages of natural adrenocorticotropic hormone are used in many centers in the United States for the treatment of infantile spasms. However, lower dosages of synthetic adrenocorticotropic hormone (tetracosactide) might be equally efficient as high dosages. We analyzed the treatment options for infantile spasms, especially regarding the adrenocorticotropic hormone dosage and the formulation (natural versus synthetic) and evaluated which options were more effective in a retrospective cohort from 1960 to 1976.. We compared the short-term response rates of patients treated with high dosages of natural adrenocorticotropic hormone (120 IU/day) (N = 31) (Group1) with those of patients treated with low-moderate dosages of natural adrenocorticotropic hormone (40 IU/day) (N = 52) (Group2). We also compared the short-term response rates of patients treated with natural adrenocorticotropic hormone (N = 83) with those of patients treated with synthetic adrenocorticotropic hormone, (N = 23) (Group3). The responses were evaluated clinically and by electroencephalography at two to three weeks after the onset of therapy.. A response was seen in 24 of 31 children treated with high dosages and in 43 of 52 children treated with low-moderate dosages of natural adrenocorticotropic hormone (P = 0.56). All children with an unknown etiology responded to both high and low-moderate dosages of natural adrenocorticotropic hormone. The proportion of children with a good early response to synthetic adrenocorticotropic hormone (16 of 23) did not differ from the proportion of children with a good early response treated with natural adrenocorticotropic hormone (67 of 83) (P = 0.25).. High dosages of adrenocorticotropic hormone are not more effective than low-moderate dosages in the short term for treating infantile spasms. Synthetic adrenocorticotropic hormone is equally effective as natural adrenocorticotropic hormone.

Topics: Adrenocorticotropic Hormone; Child, Preschool; Cosyntropin; Electroencephalography; Female; Humans; Infant; Infant, Newborn; Male; Outcome Assessment, Health Care; Retrospective Studies; Spasms, Infantile

Topics: Adrenocorticotropic Hormone; Cohort Studies; Cosyntropin; Diet, Ketogenic; Humans; Spasms, Infantile

Topics: Adrenocorticotropic Hormone; Cohort Studies; Cosyntropin; Diet, Ketogenic; Humans; Spasms, Infantile

West syndrome (WS), an intractable epileptic encephalopathy of infancy, is refractory to many antiepileptic drugs; however, adrenocorticotropic hormone (ACTH) is an effective treatment for WS. The mechanism behind the efficacy of ACTH is mediated by biochemical processes that remain unknown. We examined the effects of ACTH therapy with tetracosactide (TCS), a synthetic ACTH analogue, on brain metabolism in patients with WS, using (1)H magnetic resonance spectroscopy (¹H-MRS). In six patients with cryptogenic WS, we performed single-voxel ¹H-MRS at the occipital lobe cortex. Measurements were taken prior to TCS treatment, a few days after therapy, and several months after therapy. Data were also compared with subjects having only mild psychomotor delays. The metabolites measured were glutamine plus glutamate (Glx), N-acetylaspartate (NAA), choline (Cho), and myoinositol (mI); each was expressed as a ratio with creatine plus phosphocreatine (total creatine: tCr). The Glx/tCr ratio was significantly reduced after the TCS treatment. The NAA/tCr ratio was also significantly reduced after the treatment compared with the control group, although the change in NAA signal was heterogeneous among patients, correlating with respective outcomes. The Cho/tCr and mI/tCr ratios were not affected by TCS treatment. The reduction in Glx suggests a decrease in the glutamate-glutamine cycle, which plays a pivotal role in synthesizing neurotransmitters such as glutamate and GABA. TCS-induced Glx reduction may induce changes in synaptic signal transduction, thereby accounting for the effect of TCS on WS. The change in NAA indicates altered neuronal activity, which may be correlated with outcome in WS patients.

Topics: Adrenocorticotropic Hormone; Aspartic Acid; Choline; Cosyntropin; Electroencephalography; Female; Glutamic Acid; Glutamine; Humans; Infant; Inositol; Magnetic Resonance Spectroscopy; Male; Occipital Lobe; Spasms, Infantile

Topics: Adrenocorticotropic Hormone; Anticonvulsants; Cosyntropin; Cross-Cultural Comparison; Dose-Response Relationship, Drug; Drug Administration Schedule; Drug Therapy, Combination; Humans; Infant; Japan; Spasms, Infantile; Treatment Outcome; United States; Vigabatrin

To determine the dosage and factors influencing efficacy of adrenocorticotropic hormone (ACTH) for West syndrome.. A retrospective study of 135 patients receiving ACTH therapy with a synthetic analogue for initial effect, seizure outcome 1 year after therapy, and adverse effects. Efficacy and adverse effects were compared among the groups divided by clinical factors: dosage, treatment lag, onset age, and cause.. One hundred thirteen patients had seizure control with ACTH. For more than 1 year after ACTH, 59 remained seizure free. Adverse effects were observed in 57, and ACTH therapy was discontinued in 23. The lowest dosage group (0.0125 mg/kg/d) had fewer episodes of discontinuation (P<.05), whereas differences in efficacy between different dosages were insignificant. None of the clinical factors correlated with initial effect. The earlier-onset group (<4 months) showed unfavorable seizure outcome 1 year after ACTH (P<.01). The cryptogenic patients showed better seizure outcome (P<.05) compared with the symptomatic.. Synthetic ACTH therapy at a lower dosage is as effective as natural ACTH therapy at a higher dosage. Considering the adverse effects and the benefits for seizure control, the ACTH dosage of 0.0125 mg/kg/d (synthetic analogue) is more favorable than larger dosage.

Topics: Age of Onset; Cosyntropin; Dose-Response Relationship, Drug; Female; Humans; Infant; Infant, Newborn; Male; Retrospective Studies; Spasms, Infantile; Treatment Outcome

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

We analyzed the short- and long-term effects of adrenocorticotropic hormone (ACTH) therapy for patients with epileptic spasms (ESs) who did not meet the criteria of West syndrome (WS).. The subjects were 30 patients, including 13 boys and 17 girls, who had received ACTH therapy between 1970 and 2003. We excluded patients with WS, but included those with a history of WS who no longer showed hypsarrhythmia at the period of ACTH therapy. The age at onset of ESs and at ACTH therapy ranged from 2 to 82 months with a median of 18 months, and from 11 to 86 months with a median of 29 months, respectively.. Excellent and poor responses were obtained in 19 (63%) and 11 (37%) patients, respectively, as a short-term effect. Although the patients could be subclassified into five subgroups according to the previous reports, no difference was seen in short- term response to ACTH. Among 17 of the 19 patients with excellent short-term outcomes and a follow-up of >1 year after the ACTH therapy, eight patients have continued to be seizure free (29%; excellent long-term effect), whereas the remaining nine patients had a recurrence of seizures (complex partial seizures, four; generalized tonic seizures, three; ESs, two) at 9 months to 198 months (median, 49 months) after ACTH therapy. In addition, nine of the 17 patients demonstrated a localized frontal EEG focus after the ACTH therapy, although most of these had previously shown diffuse epileptic EEG abnormality.. ACTH therapy is worth trying for patients with resistant ESs, even without features of WS. However, the long-term effect is uncertain because recurrences of various types of seizures, including focal, were frequently observed.

Topics: Adolescent; Adult; Age of Onset; Aged; Child; Cosyntropin; Drug Administration Schedule; Electroencephalography; Epilepsy, Frontal Lobe; Female; Humans; Infant; Male; Middle Aged; Recurrence; Retrospective Studies; Spasms, Infantile; Survival Analysis; Treatment Outcome

To evaluate the outcome of children with cryptogenic infantile spasms treated with high-dose synthetic adrenocorticotropic hormone (ACTH) and the relation between early treatment, within 1 month of onset, and outcome.. We assessed the long-term cognitive and seizure outcomes of 37 patients with cryptogenic infantile spasms (onset, age 3 to 9 months) receiving standardized treatment regimen of high-dose tetracosactide depot, 1 mg IM every 48 h for 2 weeks, with a subsequent 8- to 10-week slow taper and followed by oral prednisone, 10 mg/day for a month, with a subsequent slow taper for 5 months or until the infant reached the age of 1 year, whichever came later. Development was assessed before treatment. Seizure outcomes were followed up prospectively. Cognitive outcomes were determined after 6 to 21 years and analyzed in relation to treatment lag and pretreatment regression.. Twenty-two infants were treated within 1 month of onset of infantile spasms, and 15 after 1 to 6.5 months. Normal cognitive outcome was found in all 22 (100%) patients of the early-treatment group, and in 40% of the late-treatment group. Normal cognitive outcome was found in all 25 (100%) patients who had no or only mild mental deterioration at presentation, including four in the late-treatment group but in only three of the 12 patients who had had marked or severe deterioration before treatment.. Early treatment of cryptogenic infantile spasms with a high-dose ACTH protocol is associated with favorable long-term cognitive outcomes. Once major developmental regression lasts for a month or more, the prognosis for normal cognitive outcome is poor. Further studies are needed on the optimal treatment regimen for this disorder.

Topics: Administration, Oral; Brain; Child; Child, Preschool; Cognition Disorders; Cohort Studies; Cosyntropin; Delayed-Action Preparations; Dose-Response Relationship, Drug; Drug Administration Schedule; Female; Humans; Infant, Newborn; Magnetic Resonance Imaging; Male; Neuropsychological Tests; Prednisone; Prospective Studies; Severity of Illness Index; Spasms, Infantile; Time Factors; Treatment Outcome

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

We examined the effects on astrocytes of ACTH, which is used to treat West syndrome. We stimulated cultured rat astrocytes with ACTH(1-24), corticotropin-releasing factor, and dexamethasone, and examined changes in neurotrophic factor mRNAs by reverse transcription-PCR. Down-regulation of ciliary neurotrophic factor mRNA expression was observed by stimulation with ACTH(1-24), but the expression of nerve growth factor, brain-derived neurotrophic factor, and nerotrophin-3 mRNAs was unaffected. Northern blot analysis revealed that the decrease in ciliary neurotrophic factor mRNA occurred 4 h after stimulation with more than 10 nM of ACTH(1-24). Up-regulation of nerotrophin-3 mRNA expression was found after stimulation with 1 mM dexamethasone. These results suggest that ACTH(1-24) administrated in West syndrome may influence the expression of neurotrophic factors in astrocytes in vivo.

Topics: Animals; Astrocytes; Base Sequence; Brain-Derived Neurotrophic Factor; Cells, Cultured; Ciliary Neurotrophic Factor; Corticotropin-Releasing Hormone; Cosyntropin; Cyclic AMP-Dependent Protein Kinases; Dexamethasone; DNA; Down-Regulation; Enzyme Inhibitors; Humans; Infant; Nerve Growth Factor; Neurotrophin 3; Protein Kinase C; Rats; RNA, Messenger; Spasms, Infantile

Most Japanese pediatric neurologists attempt other treatments before using adrenocorticotropic hormone (ACTH) therapy for West syndrome (WS), and even then, they use only a low-dose synthetic ACTH to avoid serious adverse effects. In this multi-institutional study, the authors analyzed the initial effects, adverse effects, and long-term outcome in patients treated with low-dose synthetic ACTH in Japan.. The medical records of 138 patients with WS, who were treated with low-dose synthetic ACTH therapy for the first time at the authors' institutions between 1989 and 1998, were analyzed.. At the end of ACTH therapy, excellent effect on seizures was noted in 106 of 138 (76%) patients, good effect in 23 (17%), and poor effect in 9 (7%). Initial effects on EEG were excellent in 53 of 138 (38%) patients, good in 76 (55%), and poor in 9 (7%). As for seizure prognosis at the time of follow-up, 51 of 99 (52%) patients were seizure-free, whereas 48 (48%) patients had seizures. Mental outcome was normal in 6 of 98 (6%) patients, mild mental retardation in 16 (16%), moderate mental retardation in 26 (27%), and severe mental retardation in 50 (51%). The initial effects of ACTH on seizures and long-term outcome were not dose dependent (daily dosage 0.005 to 0.032 mg/kg, 0.2 to 1.28 IU/kg; total dosage 0.1 to 0.87 mg/kg, 4 to 34.8 IU/kg). The severity of adverse effects correlated with total dosage of ACTH, and the severity of brain volume loss due to ACTH correlated well with the daily dosage and total dosage of ACTH.. Low-dose synthetic ACTH therapy is as effective for the treatment of WS as the higher doses used in previous studies. The dosage of synthetic ACTH used in the treatment of WS can be decreased as much as possible to avoid serious adverse effects.

Topics: Brain; Cosyntropin; Electroencephalography; Female; Follow-Up Studies; Humans; Infant; Intellectual Disability; Male; Retrospective Studies; Spasms, Infantile

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

Topics: Cosyntropin; Humans; Infant; Male; Opportunistic Infections; Pneumonia, Pneumocystis; Spasms, Infantile

The prognosis of infantile spasms is grim when a detectable brain lesion is present. In contrast, cryptogenic infantile spasms, in which there is no identifiable brain lesion, usually run a favorable course under treatment. Few studies have focused on the outcome in children with cryptogenic infantile spasms. Among 111 pediatric patients with a history of infantile spasms hospitalized over 18 consecutive years, 23 (21%) were given a diagnosis of cryptogenic infantile spasms. Follow-up ranged from 4 to 21 years. Outcome was as follows: the IQ was above 80 in 39% of cases and above 100 in 13% of cases; 42% of patients of school age were attending school but half of these had learning disabilities; 30% of patients had severe psychiatric disorders, and 22% had developmental delay and severe epilepsia. Early factors apparently associated with a good prognosis included the mild nature of psychomotor regression, persistence of spindles on EEGs recorded during NREM sleep, and prompt improvement of clinical status and EEG recordings under treatment. Conversely, severe regression, focalized EEG anomalies, failure of development to resume promptly after initiation of therapy, recurrence of spasms and hypsarrhythmia at discontinuation of treatment, and onset before 5 months or after 11 years of age were associated with a poor prognosis.

Topics: Adrenocorticotropic Hormone; Child, Preschool; Cosyntropin; Developmental Disabilities; Electroencephalography; Female; Follow-Up Studies; Humans; Hydrocortisone; Infant; Intellectual Disability; Intelligence; Male; Prognosis; Spasms, Infantile; Valproic Acid

Combination therapy of high-dose pyridoxal phosphate (PAL-P, 40-50 mg/kg/day) and low-dose ACTH beta 1-24-Z (tetracosactide acetate-Zn, Cortrosyn Z, 0.01 mg/kg/day) was instituted in 26 children suffering from West syndrome and related disorders--pretreated without success with high-dose PAL-P alone; 18 with West syndrome (14 with symptomatic and 4 with cryptogenic types), 2 with symptomatic Lennox-Gastaut syndrome, 5 with cerebral palsy with hypsarhythmia or diffuse slow spike-waves and one with myoclonic seizures (secondary generalized epilepsy). Clinical, electroencephalographic and neurochemical investigations were carried out. The results were summarized as follows. 1) Only one of 27 children with West syndrome and related disorders pretreated using high-dose PAL-P alone before ACTH showed a clinically excellent response. 2) Clinical seizures were completely suppressed in 19 of 21 children who initially had seizures (90%) after this combination therapy. 3) Twenty-one of the total 26 children (80%) had disappearance of hypsarhythmia or diffuse slow spike-waves in EEG after this therapy. 4) During PAL-P treatment alone transient increases in liver enzymes occurred in 37 percent. The brain shrinkage of CT and the significant rise in CSF NSE were seen in 95% and 78% after ACTH, respectively. 5) Twenty-three children have been followed for one to 29 months after tapering off of ACTH. No relapses were experienced in 11 of 18 who initially had seizures (61%) and 13 of 23 with hypsarhythmia or diffuse slow spike-waves (57%). 6) Postictal PRL elevations were suppressed during high-dose PAL-P. 7) No significant changes in the CSF levels of HVA and 5-HIAA were seen during this combination therapy. The CSF levels of HVA were significantly lower than the controls. 8) Daily ACTH therapy transiently suppressed the secretion of anterior pituitary hormones (GH, TSH, PRL, LH and FSH) and thyroid hormones (T3 free T3, T4 and free T4). It is recommended that the combination therapy of high-dose PAL-P and low-dose ACTH is a promising new method and should be tried in children with West syndrome and related disorders. The mechanism of action of this combination therapy remains obscure although some information has been obtained from our investigations.

Topics: Child, Preschool; Cosyntropin; Dose-Response Relationship, Drug; Drug Administration Schedule; Drug Therapy, Combination; Electroencephalography; Female; Follicle Stimulating Hormone; Homovanillic Acid; Humans; Hydroxyindoleacetic Acid; Infant; Luteinizing Hormone; Male; Pyridoxal Phosphate; Somatostatin; Spasms, Infantile

A new case of fatal systemic legionnaires' disease is reported in an infant. This 8 month-old boy was given a protracted treatment with adrenocorticotropic hormones for infantile spasms. Legionella pneumophila type I was found in tracheal secretions and there was multivisceral involvement at autopsy. The mode of contamination and the severity of the disease are discussed in the light of the immunosuppressive properties of the glucocorticoids administered over a period of 4 weeks.

Topics: Cosyntropin; France; Glucocorticoids; Humans; Infant; Legionnaires' Disease; Male; Prognosis; 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

The charts of 66 children suffering from West syndrome followed by the paediatric department of the University Central Hospital, Lille, between 1957 and 1984 were reviewed: 24 cases were followed for more than 10 years; 20 cases from 5 to 10 years; 22 cases from 1 to 5 years. Age at onset of the disease ranged from 3 to 5 months (37.8%) and 6 to 8 months (30.3%) with a slight male predominance. Prenatal causes were the most important (42.4%) with anomalies and cerebral malformations in 15.2% and neurocutaneous syndromes in 9.1% of cases. Etiology remained unknown in 27.3% of cases. Some clinical aspects were characteristic (flexion or extension spasms, psychomotor retardation) in 84.8% of cases. Hyparrhythmia occurred in 62.1% of cases. There were no significant differences in the results of the varying therapies used (ACTH, Synacten, hydrocortisone) or its associations. The best results were obtained with a treatment started as early as possible in the cryptogenic forms and in older infants (6 to 8 months).

Topics: Adrenocorticotropic Hormone; Anticonvulsants; Child, Preschool; Cosyntropin; Drug Therapy, Combination; Electroencephalography; Female; Follow-Up Studies; Humans; Hydrocortisone; Infant; Male; Prognosis; 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