INS NYC 2024 Program

Poster	Poster Session 08 Program Schedule 02/16/2024 01:45 pm - 03:00 pm Room: Shubert Complex (Posters 1-60) Poster Session 08: Cognition \| Cognitive Reserve Variables Final Abstract #25 CLIPPERS Syndrome: A Neuropsychological Case Study McKenna Garland, University of Tulsa, Tulsa, United States Brian Boatwright, Private Practice, Tulsa, United States Category: Medical/Neurological Disorders/Other (Adult) Keyword 1: autoimmune disorders Keyword 2: medical disorders/illness Objective: CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) syndrome is a rare inflammatory disorder of the central nervous system that primarily affects the brainstem, spinal cord, and cerebellum. Since the syndrome was first described in 2010, less than 100 patients have been diagnosed worldwide. While a number of reports have designated cognitive impairment as a clinical feature of the syndrome, the detailed neuropsychological outcomes of patients with CLIPPERS have only been documented in a single case study. Thus, we aim to describe the neuropsychological profile of an additional patient with CLIPPERS. Participants and Methods: A 61-year-old, right-handed, Caucasian, man with 12 years of education and a diagnosis of CLIPPERS syndrome was referred by neurology for neuropsychological evaluation secondary to changes in his cognitive functioning. The evaluation consisted of a clinical interview; medical record review; and neuropsychological measures examining attention, memory, executive functions, verbal and visuospatial abilities, cognitive and psychomotor speed, academic achievement, and emotional/psychological health. Results: Clinical interview indicated a two-year history of subjective declines in writing, spelling, word-finding, and name recall abilities. Additional examples provided included difficulties operating a television remote control and tying a tie. Medical history was notable for history of three hemorrhagic strokes, hyperlipidemia, hypothyroidism, and untreated sleep apnea. Neuropsychological testing results revealed lower than expected performances within tasks of learning, recall, and recognition of auditory-verbal information, as well as sensory motor and cognitive processing speed. Conclusions: The current work reflects only the second comprehensive neuropsychological case study of a patient with CLIPPERS syndrome. The patient’s diagnosis of CLIPPERS syndrome was associated with several cerebrovascular events, which resulted in cognitive changes that were largely consistent with performances typically seen in individuals with vascular presentations. These deficits may be related to the location and extent of brainstem lesions and/or the inflammatory process itself. However, further research is needed to better understand and characterize the neuropsychological symptoms associated with CLIPPERS syndrome.

Poster

Poster Session 08 Program Schedule

02/16/2024
01:45 pm - 03:00 pm
Room: Shubert Complex (Posters 1-60)

Poster Session 08: Cognition | Cognitive Reserve Variables

Final Abstract #25

CLIPPERS Syndrome: A Neuropsychological Case Study

McKenna Garland, University of Tulsa, Tulsa, United States
Brian Boatwright, Private Practice, Tulsa, United States

Category: Medical/Neurological Disorders/Other (Adult)

Keyword 1: autoimmune disorders
Keyword 2: medical disorders/illness

Objective:

CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) syndrome is a rare inflammatory disorder of the central nervous system that primarily affects the brainstem, spinal cord, and cerebellum. Since the syndrome was first described in 2010, less than 100 patients have been diagnosed worldwide. While a number of reports have designated cognitive impairment as a clinical feature of the syndrome, the detailed neuropsychological outcomes of patients with CLIPPERS have only been documented in a single case study. Thus, we aim to describe the neuropsychological profile of an additional patient with CLIPPERS.

Participants and Methods:

A 61-year-old, right-handed, Caucasian, man with 12 years of education and a diagnosis of CLIPPERS syndrome was referred by neurology for neuropsychological evaluation secondary to changes in his cognitive functioning. The evaluation consisted of a clinical interview; medical record review; and neuropsychological measures examining attention, memory, executive functions, verbal and visuospatial abilities, cognitive and psychomotor speed, academic achievement, and emotional/psychological health.

Results:

Clinical interview indicated a two-year history of subjective declines in writing, spelling, word-finding, and name recall abilities. Additional examples provided included difficulties operating a television remote control and tying a tie. Medical history was notable for history of three hemorrhagic strokes, hyperlipidemia, hypothyroidism, and untreated sleep apnea. Neuropsychological testing results revealed lower than expected performances within tasks of learning, recall, and recognition of auditory-verbal information, as well as sensory motor and cognitive processing speed.

Conclusions:

The current work reflects only the second comprehensive neuropsychological case study of a patient with CLIPPERS syndrome. The patient’s diagnosis of CLIPPERS syndrome was associated with several cerebrovascular events, which resulted in cognitive changes that were largely consistent with performances typically seen in individuals with vascular presentations. These deficits may be related to the location and extent of brainstem lesions and/or the inflammatory process itself. However, further research is needed to better understand and characterize the neuropsychological symptoms associated with CLIPPERS syndrome.