Clinical presentation of rapidly progressive familial Danish dementia

Familial Danish dementia (FDD) is a rare, autosomal dominant neurodegenerative disorder characterized by progressive hearing loss, cataracts, progressive ataxia, and dementia. While multiple pathophysiological studies exist in the literature, clinical case presentations are currently limited. We present a case of young-onset dementia in a 47-year-old patient with Danish heritage who was subsequently diagnosed FDD through genetic testing. Cognitive impairment was his initial symptom, followed by Parkinsonian symptoms, and mood disturbances. The patient experienced rapid decline over only 19 months. Increased awareness and understanding of familial forms of dementia (i.e., FDD) can contribute to an enhanced provision of care for patients with such conditions.