Neuropsychological functioning following cardiac transplant in Danon disease

To present a unique case involving a 31-year-old male with Danon disease (diagnosed at 14) who received cardiac transplant and subsequent cardiac re-transplant.

Brief report/case study.

Serial neuropsychological assessment across a 23-year span along with a review of school records and prior psychoeducational assessment.

A consistent pattern of higher level cognitive impairment from childhood through adulthood was found. This pattern is interpreted in light of the sparse literature regarding cognitive and adaptive functioning related to Danon disease.

The noteworthy aspects of this case include the preservation of some academic abilities and an unexpected level of functional independence given cognitive concerns. This case study further explores the nature of the deficits related to Danon disease and highlights the benefits of neuropsychological evaluation to guide functional interventions and maximize level of independence across the life span.