Ohtahara syndrome, also known as early infantile epilepsy encephalopathy with suppression bursts, is an extremely rare syndrome that starts in very young babies, usually below the age of 3 months. In the vast majority of cases, the baby will have a structural brain abnormality to which this syndrome can be attributed. These abnormalities can be either genetic and passed through the genes or as the result of trauma before, during or after childbirth.
As previously mentioned, seizures will often begin before the baby is 3 months old. In fact, in many cases it’s within the first 10 days of life. It’s possible that the baby had already been having seizures during pregnancy.
The seizure types can vary, but will commonly include tonic spasms whereby the baby will stiffen their arms and legs. This will usually last a few seconds before they relax and may happen multiple times. Focal (partial) seizures are also common with ohtahara syndrome, which will result in the jerking of one limb or one side of the body, this can often last several minutes. Finally, generalised seizures may begin to occur as the baby gets older and after several months seizures may develop into infantile spasms, otherwise known as West syndrome.
An electroencephalogram (EEG) test will be used to record electrical activity in the brain, which in the case of ohtahara syndrome will display abnormal discharges or alternating periods between spike and wave to flat and featureless patterns, which is known as a type of record called a suppression burst. In addition, anecdotal evidence and a full account of how the seizure presents in the child will be key to making an accurate diagnosis of ohtahara syndrome.
Seizures associated with ohtahara syndrome are often resistant to anti-epilepsy medications (AEDs), which can make treatment difficult. Older AEDs are often the first to be tried, with some seeing success using phenobarbital, for example. Clobazam, clonazepam and certain cases possibly vigabatrin may also be considered. In rare cases, epilepsy surgery may be an option where the area of the brain with the abnormality can be removed to prevent seizures.
Babies with ohtahara syndrome are often described as being ‘floppy’ and sleep more. They will also often have difficulty feeding and develop stiffness (spasticity) in the limbs. Sadly, many babies with ohtahara syndrome do not have a long lifespan and many die within their first 2 years of life. Those that do survive will often have severe disabilities and rarely become seizure-free.
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