Enjoying her swing (Education Read West Syndrome – Infantile Spasms)
Image by Event Essentials Photography & Decor
Paige was diagnosed with West syndrome or West’s Syndrome (September 2010) it is an uncommon to rare epileptic disorder in infants.
The syndrome is age-related, generally occurring between the third and the twelfth month, generally manifesting around the fifth month. There are various causes ("polyetiology"). The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal, perinatal (caused during birth) or postnatal.
It is either Cryptogenic or Idiopathic.
When a direct cause cannot be determined but the child has other neurological disorder, the case is referred to as cryptogenic West syndrome, where an underlying cause is most likely but, even with modern means, cannot be detected. Currently the cryptogenic group is often combined with idiopathic while referred to as "cryptogenic".
Sometimes multiple children within the same family develop West syndrome. In this case it is also referred to as cryptogenic, in which genetic and sometimes hereditary influences play a role. There are known cases in which West syndrome appears in successive generations in boys; this has to do with X-chromosomal heredity.
Occasionally the syndrome is referred to as idiopathic West syndrome, when a cause cannot be determined. Important diagnostic criteria are:
Regular development until the onset of the attacks or before the beginning of the therapy no pathological findings in neurological or neuroradiological studies no evidence of a trigger for the spasms
Those are becoming rare due to modern medicine.
The epileptic seizures which can be observed in infants with West syndrome fall into three categories, collectively known as infantile spasms. Typically, the following triad of attack types appears; while the three types usually appear simultaneously, they also can occur independently of each other:
Lightning attacks: Sudden, severe myoclonic convulsions of the entire body or several parts of the body in split seconds, and the legs in particular are bent (flexor muscle convulsions here are generally more severe than extensor ones).
Nodding attacks: Convulsions of the throat and neck flexor muscles, during which the chin is fitfully jerked towards the breast or the head is drawn inward.
Salaam or jackknife attacks: a flexor spasm with rapid bending of the head and torso forward and simultaneous raising and bending of the arms while partially drawing the hands together in front of the chest and/or flailing. If one imagined this act in slow motion, it would appear similar to the oriental ceremonial greeting (Salaam), from which this type of attack derives its name.
Paige was first put on Vigabatrin which she outgrew a month later. She was admitted again into Kingston General Hospital to begin a treatment called ACTH.
Compared with other forms of epilepsy, West syndrome is difficult to treat. To raise the chance of successful treatment and keep down the risk of longer-lasting effects, it is very important that the condition is diagnosed as early as possible and that treatment begins straight away. However, there is no guarantee that therapy will work even in this case.
Insufficient research has yet been carried out into whether the form of treatment has an effect upon the long-term prognosis. Based on what is known today, the prognosis depends mainly on the cause of the attacks and the length of time that hypsarrhythmia lasts. In general it can be said that the prognosis is worse when the patient does not react as well to therapy and the epileptic over-activity in the brain continues. Treatment differs in each individual case and depends on the cause of the West syndrome (etiological classification) and the state of brain development at the time of the damage.
Due to their side-effects, two drugs are currently being used as the first-line treatment: ACTH and Vigabatrin.
ACTH – Use primarily in United States
Side effects are: Weight gain, especially in the trunk and face, hypertension, metabolic abnormalities, severe irritability, osteoporosis, sepsis, and congestive heart failure.
Vigabatrin (Sabril) – Approved in several countries, including most of Europe, Canada, Mexico, and more recently the United States.
Side effects are: Somnolence, headache, dizziness, fatigue, weight gain, decreased vision or other vision changes
Vigabatrin is known for being effective, especially in children with tuberous sclerosis, with few and benign side effects. But due to some recent studies showing visual field constriction (loss of peripheral vision), it was not approved in the United States until mid-2009. It is currently debated that a short use (6 months or less) of Vigabatrin will not affect vision. Also, considering the effect of frequent seizures on day to day life and mental development, some parents prefer to take the risk of some vision loss.
When those two are proving ineffective, other drugs may be used in conjunction or alone. From those, corticosteroids (prednisone) are often used. In Japan, there is a good experience with pyridoxine therapy. Further, topiramate (Topamax), lamotrigine (Lamictal), levetiracetam (Keppra) and zonisamide (Zonegran) are amongst those drugs most widely used.
The ketogenic diet has been shown to be effective in treating infantile spams, up to 70% of children having a 50% or more reduction in seizure.
You can read more on Infantile Spasms by going to : en.wikipedia.org/wiki/West_syndrome
You can also view my youtube channel:
I have uploaded videos for those who wonder what the Syndrome is and looks like.
I posted pictures of my beautiful baby girl because people need to understand being chubby or overweight may not always be caused by overfeeding. My daughters was caused by the ACTH treatment which is a steroid.