About jackknife convulsion
What is jackknife convulsion?
West syndrome is a type of epilepsy characterized by spasms, abnormal brain wave patterns called hypsarrhythmia and sometimes mental retardation. The spasms that occur may range from violent jackknife or "salaam" movements where the whole body bends in half, or they may be no more than a mild twitching of the shoulder or eye changes. These spasms usually begin in the early months after birth and can sometimes be helped with medication. They can also occur rarely in older patients; if this happens, they are called "epileptic spasms" rather than infantile spasms. There are many different causes of West syndrome and if a specific cause can be identified, a diagnosis of symptomatic West syndrome can be made. If a cause cannot be determined, a diagnosis of cryptogenic West syndrome is made.
What are the symptoms for jackknife convulsion?
The following conditions are symptoms of West syndrome:
- Hypsarrhythmia (abnormal brain waves detected by an electroencephalogram [EEG])
- Mental retardation
- Infantile spasms (seizures), some of which are termed “jackknife seizures” because the infant suddenly bends forward at the waist.
What are the causes for jackknife convulsion?
Any type of brain damage can cause West syndrome, for example:
- Severe brain injury at birth
- Brain malformations
- Metabolic disease
- Chromosome abnormalities
- Tuberous sclerosis (a genetic condition)
Cryptogenic West syndrome is the term used when the cause of the syndrome cannot be identified.
What are the treatments for jackknife convulsion?
- Since there are so many potential causes of West syndrome, treatment is based on the cause.
- There is no cure for West syndrome, so unfortunately, the only treatment is to reduce symptoms.
- West syndrome treatments usually include a course of prednisolone and/or an anti-epileptic medication.
- In a few individuals, surgery in the brain may help reduce symptoms.
What are the risk factors for jackknife convulsion?
West Syndrome is an age related disease in infants. Symptoms of the syndrome appear between 3 and 12 months of age, usually occurring at the fifth month.
Is there a cure/medications for jackknife convulsion?
- Due to the various causes of West syndrome, each individual will have a somewhat different prognosis. However, children that can become free of attacks with appropriate medication (about 50% of all individuals with West syndrome) have a fair prognosis.
- Individuals that do not have EEG abnormalities and show early response to treatment have a better prognosis. Unfortunately, up to about 90% of all children with West syndrome still suffer severe cognitive and physical impairments.
- Life expectancy for West syndrome is variable. About five in every 100 infants and children with West syndrome do not survive beyond five years of age. A study of 214 Finnish children over 25 years of age showed about 61% dying at or before age 10 years.