INFORMATION ON INFANTILE SPASMS

Infantile spasm is a specific type of seizure seen in an epilepsy syndrome of infancy and early childhood known as West Syndrome. It affects between 3-6 months causing bending forward and stiffening of the body, arms, and legs. Spasms tend to begin soon after arousal from sleep. Individual spasms typically last for 1 to 5 seconds and occur in clusters, ranging from 2 to 100 spasms at a time. Infantile spasms remain one of the most complex and challenging conditions in pediatric neurology
The syndrome is age-related, generally occurring between the third and the twelfth month, generally manifesting around the fifth month. The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal, perinatal or postnatal.

SYNONYMS OF INFANTILE SPASMS

1. Generalized Flexion Epilepsy
2. Infantile Epileptic Encephalopathy
3. Infantile Myoclonic Encephalopathy
4. jackknife convulsions
5. Massive Myoclonia
6. Hypsarrhythmia,
7. Developmental delay,
8. West syndrome,
9. Mental retardation,
10. Epilepsy syndrome

CAUSES OF INFANTILE SPASMS

The most common causes are tuberous sclerosis and perinatal asphyxia. In 40 percent of cases, there is no known cause and children have the best prognosis; causes that are symptomatic have the worst. As many as 20 percent die before age 5; 75 percent are mentally retarded; and more than 50 percent have persistent epilepsy. The number of neurological diseases that can result in infantile spasms is very large, but some of the major categories include intrauterine injury and infection, disorders caused by lack of blood flow to the fetal brain, developmental malformations of the cerebral cortex, metabolic disorders, other genetic or chromosomal defects, meningitis, and tumors.

SYMPTOMS OF INFANTILE SPASMS

The typical pattern is of a sudden flexion in a tonic fashion of the body, arms and legs. Sometimes the episodes are extensor but sometimes one side is affected more than the other. While single spasms may occur, infantile spasms usually occur in ‘runs’ of several in a row. It is common for babies who have infantile spasms to become irritable and for their development to slow up or even to go backwards until the spasms are controlled

DIAGNOSIS OF INFANTILE SPASMS

Diagnosis of infantile spasms is made by a combination of the typical features with a typical electroencephalogram which shows a very disorganized pattern termed ‘hypsarrhythmia’.
Information about the child's seizures and about the pregnancy, birth, and progress since birth will help the physician in making the diagnosis.
Most children with infantile spasms will need a number of tests electroencephalogram, brain scans, blood tests, urine tests and, sometimes, spinal fluid tests in order to try to identify the underlying cause

TREATMENT OF INFANTILE SPASMS

Due to the poor prognosis of infantile spasms, treatment is usually initiated quickly and aggressively after diagnosis, often at the risk of serious side effects, with the hope of changing the natural history of the disease
The main treatments used are steroids, sodium valproate, nitrazepam, clonazepam and vigabatrin. In the first instance, steroids are most commonly used.
Conventional anti-seizure drugs, valproate and nitrazepam have been shown to be effective as first-line therapy. In addition to medication, there are some potential surgical options for infantile spasms, although they may only be applicable to a small percentage of patients.