Gelastic epilepsy

23rd July 2019

Gelastic epilepsy is a very rare syndrome that affects 1 out of every 1,000 children with epilepsy and is slightly more common in boys than girls. The syndrome takes its name from the Greek word, gelastikos, which means laughter because seizures will often start with unexplained laughter. The laughter is often described as being hollow or empty and not a typical pleasant laughter you might associate with a child. 

Causes

The most common cause of gelastic epilepsy is a small tumour located in the hypothalamus area of the brain. These tumours are most likely benign (non-cancerous) and grow very slowly. These tumours typically don’t spread to other parts of the brain. 

These tumours will either be a hamartoma or an astrocytoma. A hamartoma is a growth that’s made up of the cells and tissues that are normally found in the area in which it occurs. An astrocytoma is a nervous system tumour that grows from astrocytes. 

Symptoms

As previously mentioned, one of the core symptoms of gelastic epilepsy is seizures that begin with hollow, unpleasant laughter that comes on for no apparent reason. These seizures typically begin at around 3 or 4 years of age. Older children may even be able to recognise early warning signs of an oncoming seizure (focal aware seizure), although will likely be unable to explain what it feels like. 

The laughter is often short, lasting approximately 30 – 45 seconds before stopping abruptly. A focal seizure is likely to follow with automatisms that can vary from person to person, these might include lip-smacking, mumbling or fidgeting of the hands.  

Focal seizures are most common, however, other types associated with this particular syndrome are tonic-clonic and atonic. 

Find out more about the different types of seizures here

Diagnosis

Due to the unique nature of the seizures, diagnosis will often be made from a detailed account from someone who has witnessed them. However, it will likely be confirmed using an EEG test that will highlight focal and generalised abnormalities, as well as an MRI scan to locate the tumour. 

Treatment

There are a number of anti-epilepsy medications (AEDs) that may be effective in treating gelastic epilepsy. These include carbamazepine, clobazam, lamotrigine, lacosamide, levetiracetam, oxcarbazepine and topiramate. Unfortunately, these medications may be unlikely to control the seizures completely, however, they may reduce the severity or frequency. 

Surgery or radiotherapy may be an option for this syndrome. These treatments are only available from a select few specialist centres within the UK. 

Prognosis

Children who have gelastic epilepsy are likely to have learning or behavioural problems and it’s unlikely that the seizures can be controlled for more than a few weeks or perhaps months at a time. Successful surgery may improve their seizure control.

National Epilepsy Training can help

For more information on gelastic epilepsy, please call us on 01706 373075 or email admin@nationalepilepsytraining.co.uk