Ohtahara syndrome
Ohtahara Syndrome
Ohtahara Syndrome (pronounced: oh-tah-ha-ra sin-drome), also known as Early Infantile Epileptic Encephalopathy (EIEE), is a rare neurological disorder characterized by frequent, severe seizures and developmental delays. The syndrome is named after the Japanese neurologist, Dr. Shunsuke Ohtahara, who first described the condition in 1976.
Etymology
The term "Ohtahara Syndrome" is derived from the name of the Japanese neurologist, Dr. Shunsuke Ohtahara, who first described the condition. The term "syndrome" is derived from the Greek word "syndromē", which means "concurrence of symptoms" or "running together".
Symptoms
The primary symptom of Ohtahara Syndrome is the presence of frequent, severe seizures, which often begin within the first few days or weeks of life. Other symptoms may include developmental delays, muscle stiffness, and feeding difficulties.
Causes
Ohtahara Syndrome is typically caused by structural abnormalities in the brain, genetic mutations, or metabolic disorders. However, in many cases, the exact cause of the syndrome is unknown.
Diagnosis
Diagnosis of Ohtahara Syndrome is based on the presence of characteristic symptoms, a detailed patient history, a thorough clinical evaluation, and a variety of specialized tests, including electroencephalogram (EEG) and magnetic resonance imaging (MRI).
Treatment
Treatment of Ohtahara Syndrome is symptomatic and supportive, and may include antiepileptic drugs, ketogenic diet, and in some cases, surgery.
Prognosis
The prognosis for individuals with Ohtahara Syndrome is generally poor, with most children experiencing severe developmental delays and a shortened lifespan.
See Also
External links
- Medical encyclopedia article on Ohtahara syndrome
- Wikipedia's article - Ohtahara syndrome
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