Visual Diagnosis Week 3

1. What is the diagnosis of a 2yo child with this EEG? How would you treat this child?

Dx: Hypsarrhythmia (aka West Syndrome, infantile spasms)
West Syndrome has 3 components: infantile spasms, an interictal EEG pattern termed hypsarrhythmia (above), and mental retardation. Developmental retardation is common. Spasms can be flexor, extensor, or a mixture of flexion and extension. Spasms lead to an arrest or regression of developmental milestones. 
Tx: ACTH, Vigabantrin (expensive and only found in Canada/Europe and may cause blindness)
Less commonly used: vit B6, prednisone

2.  A 6 month old comes to your office with this rash. It has developed over the past month, as the child has been weaning from breast milk. What is the treatment?

Answer: Dx: Acrodermatitis enteropathica
This disease is characterized by the simultaneous dermatitis and diarrhea. The skin on the cheeks, elbows and knees and tissue about the mouth and anus are inflamed. There is also balding and delayed wound healing, as well as recurrent bacterial and fungal infections due to immune deficiency.  Acrodermatitis enteropathica is typically due to an abnormally low blood zinc level, reflecting impaired zinc uptake. Treatment with zinc by mouth is curative.  This disorder usually occurs at the time of weaning from breastmilk.