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OHTAHARA SYNDROME

What is Ohtahara Syndrome ? : Early infantile epileptic encephalopathy (EIEE) or Ohtahara
syndrome, is one of the most severe forms of age-related epileptic encephalopathies,
characterized by the onset of tonic spasms within the first 3 months of life that can be
generalized or lateralized, independent of the sleep cycle and that can occur hundreds of
times per day, leading to psychomotor impairment and death

What are the Causes ? :
1. Structural brain abnormalities
2. Metabolic disorders
3. Brain malformations that may or not be genetic in origin

What are the Symptoms ? :
1. tonic seizures (Can appear and last up to 10 seconds, the pattern of these spasms
remains unchanged during wakefulness and sleep and they can occur hundreds of times
per day)

2. psychomotor development delay (delay in development of mental and motor skills)
3. there may development of other neurological difficulties such as:

 diplegia (paralysis of like parts on either side of the body)

 hemiplegia (paralysis/weakness of one side of the body)

 tetraplegia (spasticity and weakness of all four limbs)

 ataxia (a lack of balance and coordination)

 dystonia (abnormal movements with increased tone)

How is it Diagnosed ? :
1. Clinical sign

2. EEG “burst suppression”

3. MRI

4. Metabolic Screening

HOW IS IT TREATED ?
Pharmacotherapy :
1. Anticonvulsant drugs (such as benzodiazepines, valproate, levetiracetam, zonisamide
and phenobarbital) - even it’s not usually very effective for this disorder.

2. Corticosteroids (prednisolone or ACTH) are occasionally helpful

Seizures become more frequent. . or stomach or lung problems. Complication Chest infection : Bronchitis. irritability. Other Treatments : They are symptomatic and supportive. low carbohydrate) Treatment for selected patients : In cases where there is a focal brain lesion (damage or abnormal development of one area/side of the brain). As they grow. these can include tratments for abnormal muscle tone.Therapy : Ketogenic diet (high fat. Some children will die in infancy. others will survive but be profoundly handicapped. accompanied by delays in physical and cognitive development. Pneumonia TAMBAHAN JIKA CUKUP Prognosis The course of Ohtahara syndrome is severely progressive. some children will progress into other epilepsy syndromes. surgery may be beneficial.