If a child suffers from a serious disease, this is particularly difficult to bear. Despite worries and insecurity, a decision needs to be taken about the optimum treatment for the child. For this purpose, it is very important and helpful to get easy to understand information about the symptoms and the disease pattern, to have a medic you trust and to get help and support from your family and friends.  

West’s syndrome is a rare type of infant epilepsy. The disease was named after the medic James West, who had observed the symptoms at his own 4 months old son, already in 1841. The generic term epilepsy describes a disease pattern with at least one spontaneously occurring spasmodic seizure that is not caused by a notable previous event (i.e. an acute inflammation, an electric shock or poisoning).  

Children affected by the West's syndrome are generally infants at the age of 3 to 12 months.  

The causes or sources of West’s syndrome differ from case to case and it is often the case that they cannot be exactly determined in detail. However, in approximately 90% of the cases an organic traumatism of the brain is the underlying cause. Such traumatism may take place before, during or after birth.  

Children with West’s syndrome are often observed to display development not according to age, even before the first occurrence of spasmodic seizure. Children display a general psychomotor retardation of development with various manifestations, such as impaired communication ability, abnormal eye movement, impairment of hearing, muscular weakness, facial grimacing and/or apathy, on the one hand, and distinct restlessness, on the other hand.   

The epileptic seizures that can be observed in infants suffering from West's syndrome may be classified as 3 types of seizure. „Jack-knife" seizures (sudden convulsions of the entire body), “nodding” spasms (contraction of the cervical and neck muscles) and “salaam” convulsions (convulsions similar to the Oriental salutation).

The seizures occur independently of external stimuli and may often take place shortly after waking up or shortly before falling asleep. Although the seizures are not painful and consciousness is maintained during this state, it often is the case that children cry during and after the seizures as these are very strenuous.  

Initially, the diagnosis is made by means of EEG (Electroencephalography; Greek – encephalon = brain; graphein = to write). By this method the electrical activity of the brain is measured; it is deduced absolutely pain-free via electrodes on the head. In case West’s syndrome is present in a patient the medic will see a typical modification in the EEG.  

In addition to the measurement of the electronic activity of a child's brain, it is recommended for the diagnosis to carry out an analysis of the blood and urine as part of laboratory diagnostics in order to be able to rule out other diseases. 

Other auxiliary methods for examination and analysis are ultrasonic examination, magnetic resonance therapy or computer tomography.  

Although some people may have the impression that such seizures are harmless due to short duration and unobtrusiveness, they may lead to severe and in parts permanent impairment of the physical and intellectual development of the child if they are not treated or in case of resistance towards the therapy. For this reason, therapy is indispensable and should be commenced as soon as possible upon diagnosis.   

In the most cases, therapy is with a particular sub-group of medication from the group of the so-named "anticonvulsants". The therapy concept is to start with well-tolerated medicinal preparations and dosages with as few side effects as possible and to possibly increase or adjust medication, if the seizures cannot be reduced after a determined control period. Multiple change-over in medication are not rare scenarios.  The active medical agents employed for treatment of West's Syndrome include the following (in alphabetical order), among others: ACTH; benzodiazepines; felbamate; glycocorticoides; sultiame; topiramate; valproate; vigabatrin. 

Monitoring of the therapy is particularly carried out by regular examination of the EEG and by means of blood analyses. 

Unfortunately, the side and adverse effects of the medical preparations are very strenuous and exhausting for the child’s organism.  

In order to antagonise and prevent retardation of the physical and intellectual development of the child supportive therapy with medical and educational effects may be carried out as auxiliary measures, such as evaluation and training of developmentally developed infants, motion therapy, physiotherapy and speech therapy. Preferably, such supportive measures should be carried out in agreement with the attending neurologist and paediatrist.   

Unfortunately, general prognoses on the process and course of the disease are hardly possible due to the large variability of causes and sources and due to the differences in the characteristics and manifestation of West's syndrome. Therefore, it is always the individual child and his/her particular development in connection with the disease that must be considered.