Epileptic spasms is an uncommon-to-rare epileptic disorder in infants, children and adults. One of the other names of the disorder, West syndrome, is in memory of the English physician, William James West (1793–1848), who first described it in an article published in The Lancet in 1841. The original case actually described his own son, James Edwin West (1840–1860). Other names for it are "generalized flexion epilepsy", "infantile epileptic encephalopathy", "infantile myoclonic encephalopathy", "jackknife convulsions", "massive myoclonia" and "Salaam spasms". The term "infantile spasms" can be used to describe the specific seizure manifestation in the syndrome, but is also used as a synonym for the syndrome itself. West syndrome in modern usage is the triad of infantile spasms, a pathognomonic EEG pattern (called hypsarrhythmia), and developmental regression – although the international definition requires only two out of these three elements. The syndrome is age-related, generally occurring between the third and the twelfth month, generally manifesting around the fifth month. There are various causes. The syndrome is often caused by an organic brain dysfunction whose origins may be prenatal, perinatal (caused during birth) or postnatal. Signs and symptoms
The epileptic seizures observed in infants with West syndrome, fall into three categories, collectively known as infantile spasms.
The International League Against Epilepsy (ILAE) in 2011 recommended abandoning these terms for reasons of clarity and instead trying to place individual cases into one of the following three groups: genetic, structural/metabolic, and unknown. The new terms are more immediately clear in their meaning, except that the structural and metabolic group includes cases that have a genetic component that does not always directly lead to the condition. Only the genetic grouping has a known direct genetic cause. "Unknown" cases may be of unknown genetic, structural, metabolic, or other unknown cause.The old terminology was defined by the ILAE as follows:
symptomatic:  the epilepsy is the consequence of a known or suspected disorder of the central nervous system. cryptogenic: this refers to a disorder whose cause is hidden or occult. Cryptogenic epilepsies are presumed to be symptomatic. idiopathic:  there is no underlying cause other than a possible hereditary predisposition.The remainder of this section will refer to the older terminology. Symptomatic
If a cause presents itself, the syndrome is referred to as symptomatic West syndrome, as the attacks manifest as a symptom of another problem. Almost any cause of brain damage could be associated, and these are divided into prenatal, perinatal, and post-natal. The following is a partial list:
In around one third of the children, there is evidence of a profound organic disorder of the brain. This includes:microcephaly
cortical dysplasia
cerebral atrophy
lissencephaly
bacterial meningitis
phakomatoses (e.g. tuberous sclerosis)
Aicardi syndrome
cephalhematoma and
vascular malformation. Furthermore, other causes increasingly being named in the literature are:
Incontinentia pigmenti
Foix–Chavany–Marie syndrome
Patau syndrome (trisomy 13)
Sturge–Weber syndrome
neurometabolic diseases
congential infections (e.g.