PubMed Health⌕ Search

SEARCH · PubMed Health

Results for “Spasms, Infantile”

Explore indexed PubMed citations for clinical trials, systematic reviews and public health research. Read source abstracts and follow each citation to its original PubMed record.

Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.

At least 37 records · Page 2Linked to original sources
PubMed1999

Vigabatrin in the treatment of infantile spasms.

Infantile spasms (IS) have been conventionally treated with adrenocorticotropic hormone (ACTH), which is often associated with significant side effects. This study assessed the efficacy of vigabatrin (VGB) as an alternative in the treatment of IS and compared the efficacy of VGB in symptomatic vs cryptogenic patients. The study retrospectively reviewed 25 infants with IS (19 symptomatic, six cryptogenic) who were treated with VGB. Of the symptomatic group, 13 (68.4%) of 19 had clinical improvement, and 15 (78.9%) had electroencephalographic improvement. Three (50%) of six in the cryptogenic group had clinical improvement, and two (33%) had electroencephalographic improvement. Overall, three patients demonstrated clinical spasm control but electroencephalographic deterioration or persistence of hypsarrhythmia coupled with further cognitive decline. Four of the six partial clinical responders had deterioration of spasms with additional VGB dosage increases. VGB is comparable with ACTH in effectiveness for treatment of symptomatic IS. Higher doses of VGB may sometimes cause deterioration rather than further improvement, and therefore an optimum dosage of VGB needs to be titrated for every patient. Persistent electroencephalographic abnormalities and even electroencephalographic deterioration despite clinical control have been observed with VGB treatment; electroencephalographic monitoring during VGB treatment is recommended.

Adrenocorticotropic Hormone↗
PubMed1997

Long-term outcomes of conventional therapy for infantile spasms.

Infantile spasms (IS) is an age-specific epilepsy which responds to anticonvulsant therapy but has a generally poor prognosis for normal psychomotor development. The subgroup of infants with a cryptogenic aetiology or whose therapy is initiated promptly is thought to have a more favourable prognosis. We retrospectively reviewed 28 infants with IS treated between 1990 and 1996 with adrenocorticotropic hormone (ACTH), valproic acid (VPA), or both, in order to correlate therapeutic response with long-term outcome. Mean age at onset of treatment was 6.4 months, with 57% of patients started within 1 month of IS appearance. IS was considered cryptogenic in 39%. The majority of infants responded to ACTH or VPA with a reduction in spasms of 75% or more. Total remission of seizures occurred in 52%. Death occurred in eight patients; mean duration of follow-up for survivors was 55 months. All subgroups based on age, aetiology, or treatment had poor outcomes, commonly with residual epilepsy, cerebral palsy or mental retardation. Conventional treatment for IS, even when initially successful in reducing spasms, is inadequate when viewed from a long-term developmental perspective, suggesting the need for novel innovative approaches for treating IS.

Adrenocorticotropic Hormone↗
PubMed2006

[Treatment of infantile spasms].

Infantile spasms (IS) are characterised by neurodevelopmental regression, a unique type of seizures and a hypsarrhythmic EEG pattern. Studies recommend the medical treatment of IS as a positive short-term outcome with respect to the spasms and in the resolution of the hypsarrhythmia. However, the data are insufficient to recommend medical treatment with respect to improving the long-term outcome. The adrenocorticotropic hormone and prednisolone are recommended for treatment of IS, although side effects are common. Vigabatrin is also recommended; IS caused by tuberous sclerosis seems to respond especially well.

Adrenocorticotropic Hormone↗
PubMed1990

Infantile spasms.

Infantile spasms are a seizure disorder in young infants with diverse etiologies, suggesting that they arise from any disturbance of central nervous system function during susceptible periods of development. The prognosis for normal intellectual and neurologic development parallels that of the underlying etiology. Early and appropriate treatment with ACTH may lead to seizure control in a majority of patients. The treating physician must anticipate the side effects of this modality.

Anticonvulsants↗
PubMed2006

Treatment of infantile spasms.

Infantile spasms are associated with a diverse range of conditions, and treatment options are available. However, outcomes remain generally poor, particularly for those with symptomatic etiologies. First-line therapy is considered to be hormonal (adrenocorticotropic hormone; ACTH), which some evidence suggests is more effective when started early. However, side effects may place limits on its use acutely and long-term. There is additional evidence for vigabatrin, specifically for infantile spasms secondary to tuberous sclerosis complex. In refractory cases, candidacy for surgical management should be explored, along with new-generation anticonvulsants (eg, topiramate, zonisamide) and the ketogenic diet. There is urgent need for further treatment trials comparing anticonvulsants with ACTH and a satisfactory animal model for the study of spasms.

Journal Article↗
PubMed2002

Brain maturational aspects relevant to pathophysiology of infantile spasms.

Infantile spasms (IS) are so typically associated with West syndrome that the term IS, properly referred to as a seizure type, is currently used synonymously with this severe infantile epilepsy. This chapter reviews some clinical and experimental observations relevant to IS pathophysiology with particular regard to maturational aspects that may account for IS age-related expression. Neither the cortical nor the brain stem generator hypotheses account for all the clinical features of IS. It is suggested that IS are generated whenever a cortical discharge is able to influence some archaic circuit located putatively in the brain stem through either an excitatory or a disinhibitory mechanism. This may occur with the highest probability during a developmental window in which IS typically occur, when N-methyl-D-aspartate (NMDA) transmission is particularly facilitated due a reduced sensitivity of NMDA receptors to the Mg2+ block. Interestingly, in this age range (0-18 postnatal day in rat), flexion spasms can be elicited experimentally by NMDA administration. These data provide an important key to further investigating IS pathophysiology, thus allowing us to design more effective strategies aimed at counteracting this harmful infantile epilepsy.

Aging↗
PubMed2001

Randomized trial of vigabatrin in patients with infantile spasms.

BACKGROUND: Infantile spasms are a rare but devastating pediatric epilepsy that, outside the United States, is often treated with vigabatrin. The authors evaluated the efficacy and safety of vigabatrin in children with recent-onset infantile spasms. METHODS: This 2-week, randomized, single-masked, multicenter study with a 3- year, open-label, dose-ranging follow-up study included patients who were younger than 2 years of age, had a diagnosed duration of infantile spasms of no more than 3 months, and had not previously been treated with adrenocorticotropic hormone, prednisone, or valproic acid. Patients were randomly assigned to receive low-dose (18-36 mg/kg/day) or high-dose (100-148 mg/kg/day) vigabatrin. Treatment responders were those who were free of infantile spasm for 7 consecutive days beginning within the first 14 days of vigabatrin therapy. Time to response to therapy was evaluated during the first 3 months, and safety was evaluated for the entire study period. RESULTS: Overall, 32 of 142 patients who were able to be evaluated for efficacy were treatment responders (8/75 receiving low-dose vigabatrin vs 24/67 receiving high doses, p < 0.001). Response increased dramatically after approximately 2 weeks of vigabatrin therapy and continued to increase over the 3-month follow-up period. Time to response was shorter in those receiving high-dose versus low-dose vigabatrin (p = 0.04) and in those with tuberous sclerosis versus other etiologies (p < 0.001). Vigabatrin was well tolerated and safe; only nine patients discontinued therapy because of adverse events. CONCLUSIONS: These results confirm previous reports of the efficacy and safety of vigabatrin in patients with infantile spasms, particularly among those with spasms secondary to tuberous sclerosis.

Anticonvulsants↗
PubMed2000

Cortical visual impairment in children with infantile spasms.

BACKGROUND: Infantile spasms are often associated with cortical visual impairment. METHOD: We report a series of 10 patients with infantile spasms, hypsarrhythmic electroencephalograms, and developmental delay, (ie, West syndrome), who presented with severe visual inattention despite a normal ocular examination. RESULTS: At follow-up (14 months to 6 years), 5 patients (50%) had no improvement in their visual behaviour. Although some degree of improvement was observed in the others, their visual function remained abnormal. All patients had moderate or severe mental retardation. CONCLUSIONS: The clinical features of West syndrome are reviewed, and the cause of the associated cortical visual impairment is discussed. Cortical visual impairment with infantile spasms is an important association, which pediatric ophthalmologists should recognize.

Diagnosis, Differential↗
PubMed2005

[Infantile spasms].

BACKGROUND: Infantile spasms are an epileptic manifestation typical of infancy. The spasms may be associated with a wide spectrum of brain abnormalities and diseases, but coexisting pathology is not always found. Prognosis depends to a substantial extent on the underlying condition. Most of the traditional antiepileptics are not effective. Adrenocorticotropic hormone (ACTH) and more recently vigabatrin are the most common first-line medications. MATERIAL AND METHODS: We have performed a literature search mainly in the PubMed database on the treatment of infantile spasms. The results are discussed in relation to Norwegian practice, for which the Norwegian Paediatric Society's recommendations are the main guidelines. RESULTS/INTERPRETATION: Vigabatrin has emerged as an accepted first-line medication for infantile spasms in Norway and many other countries, although it has not been shown that this therapy in general is as effective as ACTH/steroids. Well-designed treatment studies have been difficult to carry out. Until the final results of such ongoing studies should give different signals, it seems reasonable to continue to give vigabatrin before hormones. Oral steroids can probably be tried before ACTH, although a firm conclusion about this has yet to be drawn.

Adrenocorticotropic Hormone↗
PubMed1976

The long-term prognosis infantile spasms--the present condition of cases of infantile spasms followed in school age.

A follow-up study has been made of 25 cases with infantile spasms, all of whom were six years old or more at review. Only four (16%) out of 25 cases made a full recovery and attended normal school. Spasms ceased in 96% of all cases, but fits other than spasms (grand mal, tonic seizure, atonic seizure, myoclonic seizure, atypical absence and psychomotor seizure) occurred subsequently in 11 cases (44%). The EEG became normal in two cases (8%), but still showed modified hypsarhythmia in three cases (12%), "epileptic non-hypsarhythmic" discharges in 17 cases (68%) and non-specific abnormalities in three cases (12%). The important factors associated with good prognosis were normal development before the onset of spasms, late onset (seven months old or over) and short duration of spasms, the absence of other types of fit following spasms and lack of neurological abnormality. A bad prognosis was associated with abnormal development prior to the onset of spasms, early onset and long duration of spasms, the presence of other types of fit following spasms and evidence of any neyrological abnormality This follow-up may confirm that the therapy with ACTH-A has no significant effect on final mental state.

Adolescent↗
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↗
PubMed1992

Treatment of infantile spasms: medical or surgical?

Although infantile spasms were initially described in 1841, remarkably little progress has been made in understanding the pathophysiology of this "peculiar form of infantile convulsions." Consequently, our ability to treat infantile spasms is limited. Infantile spasms are classified as a "generalized" seizure disorder in the international classification system, which suggests that the underlying brain abnormality causing the seizures also must be diffuse or generalized. As the classification suggests, there are many diffuse, or multifocal, brain disorders related to infantile spasms, e.g., inborn errors of metabolism, hypoxic-ischemic brain injury, and developmental brain defects such as tuberous sclerosis or Aicardi's syndrome. On the other hand, infantile spasms have been reported in which a localized brain abnormality was present, e.g., tumor, stroke, and trauma. On rare occasions, removal of a tumor has resulted in cessation of the generalized infantile spasms. This finding suggests that focal cortical abnormalities can cause infantile spasms and that removing the abnormality can stop the seizures. At University of California, Los Angeles, the Pediatric Epilepsy Surgery Program has developed new approaches to the treatment of infantile spasms. The principal underlying concepts are (a) children with medically refractory infantile spasms may have an area of cortical defect (called the zone of cortical abnormality) that causes the seizures and (b) infantile spasms are usually generalized seizures. Thus, the goal of the surgical assessment is not the identification of the focus of seizure onset but rather the identification of the zone of cortical abnormality.

Adrenocorticotropic Hormone↗
PubMed1991

[A prognostic study of cryptogenic infantile spasms].

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.

Adrenocorticotropic Hormone↗
PubMed1994

The treatment of infantile spasms by child neurologists.

Infantile spasms is a refractory seizure disorder for which a number of different treatment regimens are available. No information is available on which treatment regimens are most widely used and which would be of practical importance in designing clinical trials to determine efficacy of new treatments. We proceeded to gather data on the most commonly employed methods of treating infantile spasms. A survey was mailed in 1991 to all junior, active, and emeritus members of the Child Neurology Society asking details about the treatment of infantile spasms. Telephone follow-up on a random sample of nonresponders was made. The total response rate was 58.3%. Most respondents who treat infantile spasms use corticotropin (ACTH) as their drug of first choice (88%). The most frequently used dosage was 40 IU per day, and the most frequent duration of treatment was 1 to 2 months. The most frequently reported side effects of ACTH or oral corticosteroid treatment were cushingism, behavior changes or irritability, hypertension, topical infections, and systemic infections. Of those not using ACTH, valproic acid was the next most commonly employed agent, followed by oral corticosteroids. The results were similar for all respondents regardless of age, sex, type of practice, number of cases of infantile spasms seen, location (United States or abroad) or whether the survey was completed by mail or telephone. These data suggest that there is a relative uniformity among child neurologists in the management of infantile spasms despite the publication of many alternative treatment strategies.

Adrenal Cortex Hormones↗
PubMedDate not supplied

Low-dose topiramate is effective in the treatment of infantile spasms.

BACKGROUND: Management of infantile spasms is difficult because current treatment regimens, including many anticonvulsants and hormones, are often ineffective. We conducted this study to determine the effective dose of topiramate (TPM) in Taiwanese children with infantile spasms. METHODS: Fourteen patients with infantile spasms were given TPM at an initial dose of 12.5 mg/d, and the dose was raised by 12.5 mg every 2 approximately 3 days. If the seizure frequency did not decrease during the initial 2 weeks, the dose was raised more rapidly. Titration continued for < or = 12 weeks. Subjects were monitored by weekly visits to undergo titration. RESULTS: The etiology of the infantile spasms included a cryptogenic group (n = 3) and a symptomatic group (n = 11). Overall, spasms in 5 patients (38%) were completely controlled. A > or = 50% reduction in spasms was observed in 11 (85%) of 13 subjects during stabilization, while one patient quit the treatment. The mean dose of TPM during stabilization was 7.35 +/- 4.9 mg/kg/d. Among these, 6 patients achieved seizure control and 3 were free of seizures at TPM doses of lower than 6 mg/kg/d. CONCLUSIONS: Seizure control was achieved with lower doses of TPM therapy than suggested in previous studies.

Adrenocorticotropic Hormone↗
PubMed1977

Genetic study of infantile spasm with hypsarrhythmia.

Infantile spasms (IS) appear to be a distinct syndrome complicated by heterogeneous etiology. Family data support a multifactorial model involving a polygenic determination of susceptibility to IS but requiring environmental factors such as anoxia, birth trauma, or immunization to precipitate seizures. The empiric recurrence risk among siblings was estimated to be 15 +/- 3 and for all first degree relatives as 7 +/- 5 per 1,000. These risks should be interpreted with caution since possible heterogeneity of IS may result in the occurrence of families in which cases are presumably totally environmental, and other rare families which may be segregating for an autosomal recessive disorder. Careful review of involved medical and family histories and a thorough physical examination should permit discrimination among these possibilities.

Epilepsy↗
PubMed1982

Use of valproic acid in treatment of infantile spasms.

Nineteen babies with infantile spasms were treated with valproic acid over a two-year period. Eleven of the patients were also treated with purified corticotropin, but in no cases were the two drugs used simultaneously. Approximately 40% of the patients had good control of their infantile spasms with valproic acid. Side effects from valproic acid in these babies were not frequent and were, for the most part, minor. Though the percentage of infants with infantile spasms responding to valproic acid may be less than the percentage responding to corticotropin, the incidence and severity of side effects are less with valproic acid.

Adrenocorticotropic Hormone↗