Appeal type: Medical necessity
Decision: Overturned

Diagnosis:  Central Nervous System/Neuromuscular Disorder - Seizures
Treatment:  Inpatient Hospital

The patient is a female infant with medical history significant for semilobar holoprosencephaly and diabetes insipidus, who was referred to the emergency department (ED) by an after-hours service because she had a cluster of seven brief episodes of abnormal movements.  The movements were described as neck flexion and arm stiffening.  The parents took a video and submitted it to Child Neurology, as well. The movements were concerning for infantile spasms, and an outpatient electroencephalogram (EEG) was ordered.  Because the episodes stopped, the family did not seek ED evaluation.

However, the episodes occurred again the following day, so the family presented to the ED.  Vital signs included temperature 37, heart rate 192, respiratory rate 29. Examination was significant for strong cry, no distress, active, moist mucous membranes, regular heart rate and rhythm, clear lungs, benign abdomen, and normal muscle tone.  Because of the increased frequency of the spells, she was admitted to Neurology for long-term monitoring rather than waiting for the outpatient work-up.  The neurologist's differential included infantile spasms versus myoclonic seizures.  A 24-hour EEG was performed, with no evidence of hypsarrhythmia indicative of infantile spasms and no epileptiform activity, only occasional bioccipital slowing.  She had episodes of arm extension during the EEG with no EEG changes, most likely non-epileptic.  Because of her brain abnormality and risk for infantile spasms, follow-up EEG in one to two weeks was recommended.  She was discharged home in stable condition.

The health plan's determination is overturned.

Infantile spasms is an age-specific syndrome of early infancy associated with abnormal seizure-like movements.  In addition to the spasms, patients may experience neurodevelopmental regression.  There is an associated specific EEG finding called hypsarrhythmia.  When all three components are present (spasms, regression, hypsarrhythmia), it is called West syndrome.  About two-thirds of infants with infantile spasms have neurologic abnormality.  More recent literature suggests a genetic underpinning with abnormalities in the development of GABAergic regions.  First line treatment alternatives include adrenocorticotropic hormone (ACTH) and vigabatrin.  West syndrome is particularly difficult to treat.  Early identification is crucial in improving neurodevelopmental outcome.

This young infant with prenatally-known brain abnormality and at risk for development of infantile spasms began having clusters of abnormal movements.  The family was able to obtain video, and the neurologist was concerned enough that they recommended admission for evaluation rather than waiting for outpatient evaluation.  The infant was subsequently admitted for monitoring and long-term telemetry with 24-hour EEG. Although there were abnormalities on the study, there was no evidence of seizure or infantile spasms.  Had the infant had evidence of hypsarrhythmia on EEG, she would likely have been started on medications.  If there was evidence of epileptiform activity, she may or may not have been started on routine anti-convulsant therapy.  There is concern in the young pediatric population of neurodevelopmental effects from both seizures and their treatment with anti-convulsants.  Based on the degree of urgency in this case, it was reasonable, appropriate, and medically necessary to admit the baby to the hospital for diagnostic studies and formulation of a plan of care.  The inpatient stay was medically necessary and appropriate.