A-71: Neuropsychological Profile of Good Syndrome: A Case Study of Frontal-Subcortical Dysfunction in a 36-Year-Old Female

Abstract

Objective: Good Syndrome (GS) is a rare adult-onset immunodeficiency involving thymoma, hypogammaglobulinemia, and increased susceptibility to infections. GS and associated opportunistic CNS infections have been associated with cognitive and motor declines. While GS typically affects older adults, a recent longitudinal study suggests that younger patients may be symptomatic but unrecognized with delayed diagnosis of GS. Thus, age-stratified presentation of GS remains poorly understood. In this study, we present the case of a 36-year-old woman diagnosed with GS in her early 30’s referred for outpatient neuropsychological evaluation for concerns about worsening executive dysfunction and motor symptoms. Method/Data Selection: A right-handed female with a medical history of GS, CVID, SPAD, and thymectomy, presented for outpatient neuropsychological evaluation. Presenting concerns included chronic pain, fatigue, changes in balance, intermittent diplopia, “motor tics,” and a three-year decline in short-term memory, initiation, organization, and IADLs.Prior head CT and MRI were unremarkable (~6-8 years). Her most recent neurological work-up was unavailable for direct review. Results/Data Synthesis: Cognitive testing revealed deficits localizing to the bilateral frontal-subcortical regions, with subtle parietal involvement. Specific impairments included reduced performances across measures of sustained attention, acquisition and recall of verbal-auditory material, bilateral fine motor dexterity (L>R), mental flexibility/set-shifting, verbal associative reasoning, visual planning/organization, and bilateral motor-sequencing/monitoring (L>R). Conclusions: Neuropsychological profile was broadly consistent with the clinical presentation of GS in older adults. These findings expand upon the limited GS literature by characterizing the neurocognitive presentation of GS in a younger, symptomatic patient. Additional details from the patient’s history and implications of outcome are discussed.