Rapidly Progressive Frontotemporal Dementia With Amyotrophic Lateral Sclerosis in an Elderly Female

A 69-year-old female with a family history significant for early onset dementia and a past medical history significant for coronary artery disease, primary hypertension, type two diabetes mellitus, and Crohn's disease presents to our facility with rapidly progressive cognitive decline, delusions, hallucinations, and ambulatory dysfunction over the past two months. Neurological examination was remarkable for bilateral horizontal nystagmus, tongue fasciculations, bilateral upper extremity incoordination, and bilateral lower extremity spasticity, atrophy, and weakness. Laboratory and microbiological testing were remarkable for low serum thiamine levels. Computed tomography (CT) of the head without contrast showed significant brain atrophy in the frontal and temporal regions as compared to a CT without contrast of the head 5 years prior. Magnetic resonance imaging (MRI) of the head with and without contrast showed significant atrophy in the frontal and temporal regions as well as the cerebellum. Follow-up electromyography was consistent with lower motor neuron disease. The patient was given adequate thiamine supplementation for her thiamine deficiency and discharged on donepezil with instructions to follow up with the amyotrophic lateral sclerosis clinic for further monitoring and initiation of riluzole.