Infantile spasms are convulsions, which are characterized by sudden bending of the arms, forward flexion of the trunk and extending the legs.
The attacks last from a few seconds and repeat several times a day. They occur mostly <1 year in children. The cramps may disappear at an age of about 5 years, spontaneous, but they are often replaced by other seizure types.
Infantile spasms are convulsions, which are characterized by sudden bending of the arms, forward flexion of the trunk and extending the legs. The attacks last from a few seconds and repeat several times a day. They occur mostly <1 year in children. The cramps may disappear at an age of about 5 years, spontaneous, but they are often replaced by other seizure types. The pathophysiology is unknown but infantile spasms may reflect abnormal interactions between the cortex and the brain stem. Causes Usually occur infantile spasms in infants with severe brain disorders and developmental disorders that have often been detected. These diseases may include injuries to the brain during the neonatal period metabolic disorders brain malformations tuberous sclerosis is a common cause; the prognosis is better sometimes when the seizures are caused by this disorder than when the seizures have another identifiable cause. Sometimes no cause can be determined. Symptoms and cramps begin with a sudden, quick, tonic contraction of the trunk and limbs, sometimes a few seconds. Seizures range from subtle nod to the contraction of the whole body. You can choose from flexion, extension, or, more often, consist of a mixture thereof. The cramps usually occur in series, often several dozen after another in rapid succession on. They typically occur after the kids wake up or sometimes even during sleep. Sometimes they are mistaken first for starting up. There are developmental disorders usually. In the early stages of the disease regression may occur in development (eg. As may aufhöhen children to smile or the ability to sit or lose to roll to the side). The premature mortality rate is 5-31% and is related to the etiology of infantile spasms. Diagnosis waking and sleep EEG neuroimaging, preferably MRI testing to identify the cause, unless a significant underlying neurological disorder has yet been identified, the medical history (eg. As hypoxicischemic encephalopathy of the newborn) and / or symptoms and signs point at some children out the diagnosis. Physical and neurological examinations are performed, but often no pathognomonic findings are except for tuberous sclerosis identified. A wake and sleep EEG is performed to confirm the diagnosis and to review certain anomalies. Typically, the interictal pattern is hypsarrhythmia (chaotic, high-polymorphic delta and theta waves with superimposed multifocal spike discharges). Several variations (z. B. focal or asymmetric hypsarrhythmia) are possible. The ictal pattern is normally a sudden, marked and diffuse attenuation of the electrical activity. Neuroimaging, preferably MRI is performed, if it has not been performed recently. Tests to determine the cause, if the cause is not clear by imaging methods or medical history, among the tests to determine this: Laboratory tests (e.g., blood counts with differential analysis, measurement of serum glucose, electrolytes, urea, creatinine. , sodium, calcium, magnesium, phosphorus, amino acids in the serum and organic acids in the urine; liver function tests), when a metabolic disorder is suspected genetic testing CSF-analysis, to check for metabolic diseases treatment Parenteral ACTH vigabatrin (Sometimes oral particular in tuberous sclerosis) corticosteroids Infantile spasms do not respond to typical anticonvulsants. ACTH is the most effective treatment. Both high-dose (150 units / m2) ACTH and low-dose (20 units / m2) ACTH, i.m. daily be administered, have been used, and the evidence suggests that the higher doses act better, are not final; However, higher doses are normally if lower doses do not stop the spasms within 2 weeks, are used. The ACTH therapy is continued usually at the effective dose for 2 to 3 weeks and then died away slowly over 6 to 9 weeks. Vigabatrin is the only anticonvulsant with proven efficacy; it is the method of choice if the cramps are caused by tuberous sclerosis, and is often used in children with an established pre-existing severe brain injury or -fehlbildung and among those who ACTH nihct tolerate or respond to it. The dosage of vigabatrin is 25 mg / kg 2 times daily, gradually increased up to 75 mg / kg 2 times a day, if necessary. There are not available enough evidence that any other anticonvulsant or the ketogenic diet are effective. Corticosteroids (e.g., prednisone p.o. 2 mg / kg / day) is sometimes given for 4 to 7 weeks as an alternative to ACTH. In some patients with resistant spasms focal cortical resection can eliminate the seizures. There is evidence that effective treatment is initiated sooner, the better is the result of neurodevelopment, particularly when no cause has been identified. Important points spasms in children are ongoing for a few seconds and can repeatedly recur in the day; they can disappear at an age of about 5 years, spontaneous, but they are often replaced by other seizure types. Usually infantile spasms occur in infants with severe brain disorders and developmental disorders that have often been recognized; tuberous sclerosis is a common cause. Perform EEG during wakefulness and sleep EEG by to confirm the diagnosis and to check for certain anomalies; imaging techniques (preferably MRI), if not done recently, should be performed. The most effective treatment is with ACTH, but vigabatrin is the drug of choice be for cramps caused by tuberous sclerosis, and is often used in children with an established pre-existing severe brain injury or -fehlbildung.