A - 101 A Neuropsychological Case Study of Progressive Bulbar Palsy.

Abstract

There has been limited literature regarding cognitive dysfunction associated with progressive bulbar palsy. This case study presents the neuropsychological profile of a 66-year-old White male diagnosed with progressive bulbar palsy (evolving primary lateral sclerosis) with speech production difficulties. The patient was referred by his neurologist for a neuropsychological evaluation of progressively worsening cognitive changes over the past two years, including difficulties with reading directions, spelling, structuring sentences, thinking of words to write down, and counting. He also described progressively worsening speech production difficulties. During the evaluation, the patient communicated solely through written means. Available MRI results demonstrated subtle evidence of anterior temporal atrophy bilaterally. Additional relevant history includes chronic obstructive pulmonary disease and a remote history of recreational opioid use. Given the patient's speech production difficulties, measures were selected to include those that did not require verbal responses. For the few measures that did require verbal responses, administration was adjusted to allow him to provide written responses. On evaluation, he demonstrated intact orientation, processing speed, receptive language, and visuospatial functioning. In contrast, he demonstrated relative weaknesses on measures of attention, executive functioning (i.e., problem solving and cognitive flexibility), and free recall of information (with intact recognition). Behavioral features were also noted during the evaluation, including impulsivity and disorganization. The case provides preliminary data regarding possible cognitive profiles associated with progressive bulbar palsy, including possible areas of cognitive strengths and weaknesses. This case also highlights the need for further investigation of cognitive profiles of the condition which may assist with treatment planning.