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Ohtahara syndrome

Diseases of the Nervous System

Ohtahara syndrome is a severe epileptic encephalopathy that begins in the first few months of life. Its outward signs are tonic spasms and partial seizures, and its name comes from a distinctive pattern of burst activity that shows up on an electroencephalogram, the recording of the brain's electrical rhythm. It is also known as early infantile developmental and epileptic encephalopathy, abbreviated EIDEE. The condition is described as a progressive neurological disorder that is extremely debilitating, involving seizures that resist treatment and severe intellectual disability. No single cause has been identified, although in many cases structural damage to the brain is present. This entry follows the summary of a general reference article and records the condition as a description of what is known, not as medical advice.

Facts
Classification
Body System
Nervous System 1
ICD-10 Chapter
VI: Diseases of the Nervous System 1
Communicable or Non-communicable
Non-Communicable 1
Transmission Route
Non-Transmissible 1
Causative Agent
Causative Agent (category)
Idiopathic / Multifactorial 1
First Described
1976 1
Sources
1. Ohtahara syndrome (Wikipedia)
  • Lead paragraph
    It is an extremely debilitating progressive neurological disorder
  • lead sentence 1
    Ohtahara syndrome (OS), also known as early infantile developmental and epileptic encephalopathy (EIDEE) is a progressive epileptic encephalopathy.
  • Article text - era-first-described
    It is named for the Japanese neurologist Shunsuke Ohtahara (1930-2013), who identified it in 1976.
  • Lead
    Ohtahara syndrome (OS), also known as early infantile developmental and epileptic encephalopathy (EIDEE) is a progressive epileptic encephalopathy.
  • Introduction
    No single cause has been identified, although in many cases structural brain damage is present.
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