H - 38 Neuropsychological Evaluation and Cognitive Profile of Stiff Person Syndrome (SPS) in a Predominantly Spanish-Speaking Older Adult: Is Cognition Really Affected?

Abstract

Stiff Person Syndrome (SPS) is a rare autoimmune disorder that affects approximately one in every million people. It is characterized by progressive muscle rigidity and intense, episodic muscle spasms triggered by environmental and psychosocial (i.e., emotional stress) factors. While original diagnostic criteria for classical SPS included the absence of frank cognitive impairment (aside from what would be expected from seizures), more recent literature suggests a number of neurocognitive difficulties potentially associated with reduced GABA levels. The following case study presents a 73-year-old, predominantly Spanish-speaking man with 14years of education. His medical history is notable for over 20years with diagnosed SPS, hypertension well managed with medications, and the absence of other medical conditions that may contribute to cerebrovascular burden. He completed a comprehensive neuropsychological evaluation in Spanish. Overall, his profile was notable for mild inefficiencies in frontal-subcortical functions, with a relative weakness in rote learning and retention. He performed remarkably well relative to his level of motor compromise. There was no evidence of either a mild or major neurocognitive disorder, and his clinical interview and behavioral inventories were suspicious for a personality disorder. Recent literature proposes SPS is related to impairment in verbal learning, retention, fluency, processing speed, and attention. The present case corroborated, at most, mild inefficiencies in these previously documented areas. Careful consideration should be used in the evaluation of studies on SPS and cognition, particularly in determining whether samples had high degrees of comorbid conditions (i.e., cerebrovascular burden) that could help explain positive findings related to cognition.