(Kiosk 3) A Working Diagnosis of Frontotemporal Dementia Presenting in the Third Decade of Life: A Case Report

Case Diagnosis: 30-year-old female with 5-year history of progressive neurocognitive disorder presented to acute inpatient rehabilitation following hospitalization for acute functional decline.

Case Description or Program Description: Patient is a college graduate who was previously healthy until age 25 when she began to develop compulsive behaviors and cognitive decline. This progressed to binge-eating then complete food aversion with swallowing dysfunction, eventually necessitating gastrostomy tube placement to maintain nutrition. Her communication skills regressed into mutism. She underwent extensive inpatient and outpatient diagnostic workup which was largely unrevealing other than imaging evidence of cerebral atrophy predominantly affecting the bilateral frontal and temporal lobes. She was assigned a working diagnosis of frontotemporal dementia (FTD) with behavioral disturbance. Her physical decline was less pronounced, remaining independently ambulatory for multiple years. She re-presented to acute care after a rapid onset of lethargy and generalized weakness without clear precipitant. After additional unrevealing workup she was admitted to acute inpatient rehabilitation (AIR).

Setting: Acute Inpatient Rehabilitation

Assessment/Results: During AIR stay, patient participated in physical, occupational and speech therapy and progressed to her recent functional and cognitive baseline. Speech therapy sessions focused on communication strategies; she remained mute but inconsistently followed commands or engaged in tasks requiring working memory. She was also noted to inconsistently communicate yes/no responses by foot tapping and participate with use of basic functions on a customized augmentative communication device.

Discussion (relevance): Our patient presents a unique case of neurocognitive decline of unclear origin, perhaps early onset FTD with behavioral disturbance versus an undiagnosed condition. During her AIR stay, a multidisciplinary approach facilitated identification of new avenues for possible communication to be utilized by patient and family going forward.

Conclusions: Despite unclear underlying neurologic diagnosis, AIR provided our patient with a setting conducive to functional recovery and mild improvement from baseline with regard to communication strategies.