Program Operations Manual System (POMS)
TN 30 (08-20)
COMPASSIONATE ALLOWANCES INFORMATION
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OHTAHARA
SYNDROME |
ALTERNATE NAMES
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Early Infantile Epileptic Encephalopathy with Burst Suppression; EIEE
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DESCRIPTION
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Ohtahara Syndrome (OS) is a rare neurological disorder characterized by onset of seizures within the first
three months of life. Infants primarily have tonic seizures, but may also experience
partial seizures (also called focal seizures), or myoclonic seizures. OS is most commonly
caused by metabolic disorders or structural abnormalities in the brain; although for
many cases, the cause cannot be determined. Most infants with the disorder show significant
abnormalities of the cerebral hemispheres. Males are more often affected than females.
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DIAGNOSTIC TESTING, PHYSICAL FINDINGS, AND ICD-9-CM/ICD-10-CM
CODING
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Diagnostic testing:
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EEG shows the characteristic “burst suppression” pattern;
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Physical findings: Infants with OS often appear excessively sleepy with limited muscle control. As seizures
become more frequent, they develop stiffness (spasticity) in the limbs.
ICD-9: 345.11
ICD-10: G40.319
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PROGRESSION
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OS is a very progressive seizure disorder that is associated with high mortality and
morbidity. As the disease progresses, seizures become more frequent, accompanied by
physical impairments and developmental delay. Some children will die in infancy within
weeks or months from onset due to chest infections or pneumonia; others will have
severe permanent mental and neurological deficits.
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TREATMENT
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There is no cure for this disorder. Treatment is symptomatic and supportive. Anti-seizure
medications are prescribed to control seizures with limited effectiveness. Corticosteroids
are occasionally helpful. In cases where there is a focal abnormal area of the brain,
surgery may be beneficial.
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SUGGESTED PROGRAMMATIC ASSESSMENT*
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Suggested MER for Evaluation:
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Clinical history and examination that describes the diagnostic features of the impairment;
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EEG with evidence of burst suppression pattern;
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Imaging studies such as CT or MRI; and
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Metabolic laboratory studies.
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Suggested Listings for
Evaluation: |
DETERMINATION
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LISTING
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REMARKS
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Meets |
11.02
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111.02
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Equals |
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* Adjudicators may, at their discretion, use the Medical Evidence of Record or the
listings suggested to evaluate the claim. However, the decision to allow or deny the
claim rests with the adjudicator.
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