Infantile_Spasms_Flashcards
What are infantile spasms?
Infantile spasms, or West syndrome, is a type of childhood epilepsy typically presenting in the first 4 to 8 months of life, more commonly in males. They are often linked to serious underlying conditions and generally have a poor prognosis.
What are the characteristic features of infantile spasms?
Infantile spasms are characterized by ‘salaam’ attacks, which involve flexion of the head, trunk, and arms followed by extension of the arms. These spasms last only 1-2 seconds but may repeat up to 50 times.
What are common findings on investigations for infantile spasms?
The EEG typically shows hypsarrhythmia in two-thirds of infants, while CT scans demonstrate diffuse or localized brain disease in 70% of cases, such as tuberous sclerosis.
What is the management approach for infantile spasms?
Infantile spasms have a poor prognosis. Vigabatrin is considered first-line therapy, and ACTH is also used for treatment.
summarise
Infantile spasms
Infantile spasms, or West syndrome, is a type of childhood epilepsy which typically presents in the first 4 to 8 months of life and is more common in male infants. They are often associated with a serious underlying condition and carry a poor prognosis
Features
characteristic ‘salaam’ attacks: flexion of the head, trunk and arms followed by extension of the arms
this lasts only 1-2 seconds but may be repeated up to 50 times
progressive mental handicap
Investigation
the EEG shows hypsarrhythmia in two-thirds of infants
CT demonstrates diffuse or localised brain disease in 70% (e.g. tuberous sclerosis)
Management
poor prognosis
vigabatrin is now considered first-line therapy
ACTH is also used
A 6-month-old boy is brought into your practice by his adoptive parents, complaining of an unusual pattern of movements recently in their son. They were able to catch this on camera, and on watching the video you notice subtle symmetrical contracting of his neck and drawing up of his legs, followed by extending of his arms. He repeats this movement around 50 times before stopping.
What is the most likely diagnosis in this case?
Infantile colic
Tonic-clonic seizure
Focal aware seizure
Focal impaired awareness seizure
Infantile spasms
Infantile spasms
Infantile spasms - classically characterised by repeated flexion of head/arms/trunk followed by extension of arms
Although an easily missed diagnosis in clinical practice, this case illustrates a classic presentation of infantile spasms (West syndrome). This is a type of epilepsy presenting at 4-8 months, which typically has repeated flexion and extension movements (Salaam attacks) repeated up to 50 times. On EEG, this would show a dramatic hypsarrhythmia appearance. This diagnosis confers a poor prognosis.
Infantile colic can have drawing up of the legs, but this would not be in the repeated nature seen here.
Tonic-clonic seizures are characterised by a contracting of the musculature and then a rapid shaking of the muscles.
This description of spasms involves the entire body and therefore is not a focal seizure.
Which one of the following statements regarding infantile spasms is incorrect?
EEG shows hypsarrhythmia in the majority of children
Carries a good prognosis
More common in male children
Typically presents in the first 4 to 8 months
Causes characteristic ‘salaam’ attacks
Carries a good prognosis
The correct answer is ‘Carries a good prognosis’. Infantile spasms, also known as West syndrome, generally do not carry a good prognosis. The condition is associated with poor developmental outcomes and can progress to other forms of epilepsy in many cases. According to UK guidelines, the prognosis depends on the underlying etiology and early treatment initiation but overall, it remains poor.
The statement ‘EEG shows hypsarrhythmia in the majority of children’ is correct. Hypsarrhythmia is a specific EEG pattern characterised by high amplitude and irregular waves and spikes that is seen in most cases of infantile spasms.
The statement ‘More common in male children’ is also correct. Although infantile spasms can occur in both genders, there is a slight male predominance reported in various studies.
‘Typically presents in the first 4 to 8 months’ is another accurate statement. Infantile spasms usually begin between the ages of 4 to 8 months, although they may start anytime during the first two years of life.
Finally, ‘Causes characteristic ‘salaam’ attacks’ is true as well. The term ‘salaam’ attacks or ‘jackknife seizures’ refers to the sudden bending forward and stiffening of the body, arms, and legs that are characteristic of infantile spasms.
