What is Otahar Syndrome?
The syndrome is described in 1976, is determined by the very early onset of the disease, is rare.
Causes of Otahar Syndrome
The disease refers to symptomatic generalized epilepsy of a non-specific etiology. Often there are family cases of the disease, which indicates some kind of metabolic disorder.
Symptoms of Otahar Syndrome
Start in the first few months of life with frequent fragmented myoclonus. Then – partial seizures, massive myoclonus or tonic spasms.
Diagnosis of Otahar Syndrome
Based on clinical features and EEG data. On EEG – suppressive-explosive activity, which can go into hypsarrhythmia.
Differential diagnosis
West Syndrome.
Forecast
Adverse. At the age of 4-6 months, a transition to West syndrome is often noted. The course is very difficult and rapid dramatic development. Psychomotor development stops and death may occur in the first year.
Treatment for Otahar Syndrome
Marked resistance to AK treatment.