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PubMed1991

[Infantile spasms (infantile myoclonic encephalopathy with hypsarrhythmia)].

The paper reports on a very important aspect in a pediatric practice, referring to one of the most severe forms of epilepsy of the infant, known in the modern specialty literature as infantile spasms. The paper reviews the etiologic data of the disease in its secondary form, with emphasis on the form of the disease better studied in the last years, i.e. the so-called infantile benign epileptic spasms. The paper also analyzes the clinical and electroencephalographic aspects, discuss the most important aspects of the differential diagnosis that includes some entities recently individualized and appreciates, on the basis of the most topical literature data, the nature of the different treatment means used, and of the evolution and prognosis.

Brain↗

PubMed1989

Infantile spasms.

Infantile spasms constitute a relatively rare disorder of infancy and early childhood; their onset is usually within the first 6 to 8 months of life. A large percentage of patients with this disorder (85-90 per cent) show various degrees of retardation. Infantile spasms typically occur in clusters immediately on arousal, or soon thereafter, but rarely occur while the infant is actually asleep. The usual interictal EEG pattern associated with infantile spasms is hypsarrhythmia, but infantile spasms may occur in the absence of this EEG pattern. The pathophysiology of infantile spasms is not known, but recent evidence suggests that certain regions in the brain stem that are associated with sleep cycling may be responsible for the clinical and EEG manifestations of this disorder. At present, the only known effective treatment for infantile spasms is ACTH or corticosteroids. The therapeutic efficacy of these two agents is relatively equal, and one drug may be effective if the other drug fails. The effectiveness of certain traditional anticonvulsants (valproic acid and the benzodiazepines) and pyridoxine in the treatment of infantile spasms has not been adequately assessed. The long-term mental and developmental outcome of patients with infantile spasms is poor. The only factor that appears to be important in terms of long-term outcome is whether the patient is initially classified as cryptogenic or symptomatic, with the cryptogenic patients having the better outcomes. Approximately half of the infantile spasm patients will continue to have other types of seizures after their spasms stop.

Adrenal Cortex Hormones↗

PubMed1991

Therapeutic trial of vigabatrin in refractory infantile spasms.

Infantile spasms usually start during the first year of life and constitute one of the most difficult types of epilepsy to treat. They carry a very poor prognosis for both epilepsy and mental development. Seventy children, including 47 infants, with intractable infantile spasms were entered into an open study with vigabatrin as add-on therapy to the usual anticonvulsant treatment. All were resistant to previous treatments, including corticosteroids (43 patients), carbamazepine, benzodiazepines, and sodium valproate. Two children withdrew from the study because of intolerance to vigabatrin (hypotonia or hypertonia) before evaluation of efficacy could be made. Of the remaining 68 children, 29 (43%) showed complete suppression of spasms. Forty-six children had a greater than 50% reduction in spasms. The best response was observed in those with tuberous sclerosis (12/14 compared with 12/18 with symptomatic infantile spasms of other origin and 22/36 with cryptogenic infantile spasms). Following the initial response to treatment of these patients (n = 68), a long-term response was confirmed in 75% of children with symptomatic infantile spasms and 36% of children with cryptogenic infantile spasms. In eight children, all other anticonvulsant medication could be definitively withdrawn. Tolerability appeared excellent, with 52 of 70 patients reporting no side effects. Somnolence, hypotonia, weight gain, excitation, and insomnia were the most common problems at the beginning of the study and were usually transient. Given the poor prognosis of this type of childhood epilepsy, vigabatrin appears to be a very interesting advance in the management of drug-resistant infantile spasms.

Adolescent↗

PubMed1990

Discharge planning for the child with infantile spasms.

Infantile spasms is a type of seizure disorder usually occurring within the first 24 months of life. The standard treatment is intramuscular injections of repository corticotropin (HP ACTHAR gel). The child with infantile spasms is at risk for developmental delay, even if the seizures are controlled. The parents' grief reaction to this prognosis may affect their ability to learn and implement the treatment plan. Discharge planning for children and families is extensive and must begin at the time of the child's admission to the hospital.

Adaptation, Psychological↗

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↗

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↗

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↗

PubMedDate not supplied

Vitamin B6 and valproic acid in treatment of infantile spasms.

Twenty patients with infantile spasms were treated with high doses of vitamin b6, valproic acid, or both. Three of 13 patients (23%) treated initially with high doses of vitamin B6 demonstrated a definite reduction in seizures; 2 patients had no improvement on electroencephalography. Vitamin B6 therapy alone was continued in a single patient (8%) who remained seizure-free during the 15-month follow-up period. Initial treatment with vitamin B6 and valproic acid improved the electroencephalogram significantly more (P less than 0.05) than initial vitamin B6 treatment alone. The group which had valproic acid added to vitamin B6 therapy had significantly fewer seizures (P less than 0.05) and better electroencephalograms (P less than 0.01) than did the group treated initially with vitamin B6 alone. There were no significant differences among the group treated initially with vitamin B6, the group treated initially with valproic acid, and the group in which valproic acid was substituted for vitamin B6. ACTH was more effective in abolishing seizures than was valproic acid or vitamin B6 and valproic acid. ACTH had an excellent effect on seizures in 86% of patients who did not respond well to vitamin B6, valproic acid, or both; however, many of these patients had later recurrence of infantile spasms. The combination of vitamin B6 and valproic acid is effective and safe in the treatment of infantile spasms.

Adrenocorticotropic Hormone↗

PubMed1991

The epidemiology and natural history of infantile spasms.

Few population-based studies of infantile spasms have been done, and most reports have not included comparison groups. In spite of these limitations, this review attempts to summarize what is known about the distribution, etiology, and natural history of infantile spasms in populations; discusses the limitations of current data; and includes suggestions for further population-based research. Most estimates of the incidence of infantile spasms are between 0.25 and 0.42 per 1000 live births per year. Among children less than 10 years of age, the annual prevalence of infantile spasms is 0.14 to 0.19 per 1000. The peak age at onset of spasms is 4 to 6 months, and there appears to be a slight excess of male cases. The etiology of infantile spasms is unknown for 40% to 50% of affected children. Selected syndromes (eg, Aicardi syndrome, Down syndrome, etc) and inherited disorders (eg, tuberous sclerosis) may account for as many as one third of cases. Other factors, such as intrauterine infection, "birth injury," and head trauma have not been systematically evaluated, and thus, their contribution to the etiology of infantile spasms is uncertain. Areas for future etiologic research include controlled studies of immunologic factors and in utero and postnatal infections and further exploration of the interrelationship between infantile spasms and Lennox-Gastaut syndrome.

Cross-Cultural Comparison↗

PubMed1991

Simultaneous infantile spasms and partial seizures.

We report 11 infants with infantile spasms who had partial seizures that occurred concurrently with the infantile spasms. We studied this phenomenon with time-locked video electroencephalography. The partial seizures began before the infantile spasms and continued after one or more infantile spasms in seven patients. An infantile spasm occurred at the beginning of the partial seizure in five patients. (One patient had both seizure types simultaneously.) The clinical descriptions of these combinations of seizures were confusing, since they did not match known seizure types. The genesis of these seizures may involve an interaction between processes of focal cortical epileptogenesis (partial seizures) and brain-stem synchronization (infantile spasms).

Electroencephalography↗

PubMed1977

Critical evaluation of the role of immunization as an etiological factor of infantile spasms.

One hundred and ten cases of infantile spasms with detailed information about immunization available were selected as the masterial to evaluate the significance of immunization as an etiological factor of infantile spasms. In 80% of the cases, immunization could not be considered to have any relation with causation of infantile spasms, because 44 cases (40%) never had innoculations as yet, and other 44 cases (40%) had been immunized by some vaccines over one month before or after the onset of the disease. The remaining 22 cases, in whom immunization had been performed within one month before the onset of the disease, constituted the candidates for further study. The age of onset of the disease of the candidate group ranged from 3 to 9 months of age with peak incidence at 4 to 5 months old, exactly identical with that of patients with infantile spasms in general. The kind of vaccines concerned included DPT triple vaccines in 15 cases, smallpox vaccine in 4, antipolio live vaccine in 1, anti-Japanese encephalitis vaccine in 2. Causal relationship of immunization with infantile spasms in each patient was evaluated on the basis of the following three apsects: 1. Any other causal factors except immunization should be absent. 2. Psychomotor development before onset of the disease should be normal. 3. Interval from immunization to onset of the disease should be lied: a) Within 48 hours, in the case of pertussis vaccine, b) within 18 days, in the case of smallpox vaccine or anti-Japanese encephalitis vaccine or antipolio vaccine. Only five cases (4.8% of the total) were able to classify into the compatible group which should fulfill all the above three criteria. The small figure may easily be explicable on the assumption that the natural onset of spasm is chronologically superposed by chance over immunizations which have to be done within the first year of life.

Age Factors↗

PubMed1986

[Benign epileptic infantile spasms].

Among 100 infants with infantile spasms studied before treatment, 14 had idiopathic spasms with a favourable outcome. They had a normal development, including reaching for objects before the age of 5 months and moderate regression without loss of eye following. Although it was hysarhythmic, the EEG tracing showed identifiable basic activity and sleep spindles; there was no slow waves focus even after diazepam administration and the spasms were "independent" even during a cluster. This type of infantile spasms seems to be a particular type of non-lesional epilepsy. It contrasts with idiopathic spasms of unfavourable outcome that apparently result from a preexisting and overlooked focal lesion responsible of later cognitive troubles.

Child, Preschool↗

PubMedDate not supplied

Studies on CSF tryptophan metabolism in infantile spasms.

Cerebrospinal fluid from 8 patients with infantile spasms (mean age: 6.1 months) was collected before treatment. The concentration of cerebrospinal fluid tryptophan metabolites was analyzed using high-performance liquid chromatography and compared to metabolite concentrations in cerebrospinal fluid from 20 age-matched controls (mean age: 5.8 months). The levels of cerebrospinal fluid serotonin, 5-hydroxyindoleacetic acid, and kynurenine were significantly lower in infantile spasm patients compared to controls (P less than .05). In contrast, the levels of cerebrospinal fluid 3-hydroxykynurenine were significantly higher in infantile spasm patients than in controls (P less than .05). There were no significant differences in the levels of cerebrospinal fluid tryptophan and 5-hydroxytryptophan. Although the study population was small, these findings suggest that the presence of seizures in infantile spasms is associated with a decrease in serotonergic metabolites which, in turn, may indicate a decrease in serotonergic activity, altered clearance of these metabolites, or altered turnover in the direction of 3-hydroxykynurenine. The perturbance caused by increased 3-hydroxykynurenine and decreased kynurenine in the homeostatic balance between these 2 tryptophan metabolites could further contribute to the pathogenesis of infantile spasms.

5-Hydroxytryptophan↗

PubMedDate not supplied

Treatment of infantile spasms.

Although the syndrome of infantile spasms has been known for 150 years and its treatment described since 1958, controversy still surrounds the appropriate therapy for this devastating disorder. The rationale, dosage, and side effects of ACTH treatment of infantile spasms is described. The recommended treatment regimen is placed in the context of the literature on the therapy of spasms and its relation to outcome.

Adrenocorticotropic Hormone↗

PubMed1992

[Infantile spasms associated with a brain tumor].

BACKGROUND: Infantile spasms are exceptionally associated with a focal cerebral lesion such as a brain tumor. CASE REPORT: A 15 month-old girl was treated with hydrocortisone and valproic acid for infantile spasms that had appeared a few days earlier. Her psychomotor development was considered normal and the CT scan was normal. Clinical symptoms disappeared within 10 days and the EEG became normal except for a few epileptiform discharges in the frontal areas. The girl developed partial seizures at the age of 5 years. Imaging techniques showed a brain tumor in the right frontal lobe. Surgical excision was followed by radiation therapy. Analysis of the tumor showed an astrocytoma. The girl is normal 18 months after intervention. CONCLUSION: It is difficult to determine a relationship between the infantile spasms and the brain tumor in this case. The moderately asymmetrical pattern of the EEG after infantile spasms, the fact that these spasms appeared relatively late and the persistence of discharges, lead to repeated specialized investigations.

Astrocytoma↗