INS NYC 2024 Program

Poster	Poster Session 08 Program Schedule 02/16/2024 01:45 pm - 03:00 pm Room: Majestic Complex (Posters 61-120) Poster Session 08: Cognition \| Cognitive Reserve Variables Final Abstract #74 CREST and Cognition in Systemic Scleroderma Qingyang Zhu, University of Chicago Medical Center, Chicago, United States Maureen Lacy, University of Chicago Medical Center, Chicago, United States Category: Medical/Neurological Disorders/Other (Adult) Keyword 1: neuropsychological assessment Keyword 2: autoimmune disorders Keyword 3: cognitive functioning Objective: Scleroderma is a rare, rheumatologic disease with estimates of 17.6 in 100,000 individuals. Notably, there is a higher prevalence in women, particularly Black women. Simple Scleroderma typically affects connective tissue causing excessive collagen resulting in fibrosis in the skin. Systemic Scleroderma results in a more systemic constellation of systems driven by an autoimmune attack on connective tissue in addition to vasculature throughout the body. As such, patients are at elevated risk for cardiovascular, interstitial lung, and hematologic dysfunction. A 2023 survey (Chen et al 2023) found that over 50% of patients with systemic sclerosis endorsed significant cognitive impairment. Participants and Methods: A retrospective review was conducted of the medical record, neuroimaging reports, laboratory findings, and neuropsychological report of a woman referred by her neurologist due to mild cognitive (e.g., language retrieval, memory, and visuospatial impairment) and mood problems along with neuropathy of unclear etiology. Results: A 64-year-old right-handed White female reported slowly progressive distractibility, short-term memory, naming, and word retrieval problems over the last year. She also reported increased headaches, imbalance, dry eyes, and diffuse body pain in the context of a complicated medical history: 30s marked by Reynaud’s; 40s complicated by rising breathing issues with diagnoses of Asthma, Bronchitis, Pulmonary Hypertension, and Cardiac stenosis leading to stenting; 50s marked by worsening breathing, hypertension, pre-diabetes, thyroid cysts, elevated liver enzymes, and varied elevated immune antibodies; 60s marked by elevated APL enzymes and stable yet low platelets. Medical record review lists: scleroderma, coronary artery disease, hypertension, dyslipidemia, GERD, NASH, and pre-diabetes, along with recent thrombocytopenia. Family history includes migraines (son), diabetes (brother, son), and cancer (brother). Lab results were notable for high glucose and low platelets with high liver enzymes, reticulocyte count, kappa free light chain and C-reactive protein. B12 and TSH were normal. Prior labs revealed elevated anticardiolipin antibodies. MRI revealed “prominent sulci and ventricles” along with “mild chronic small vessel ischemic changes.” Emotionally she spoke of sadness and endorsed significant anxiety symptoms with worries around family stressors and fears of COVID-19 infection. Psychiatric history includes Major Depression and Seasonal Affective disorders treated with psychotherapy and pharmacological treatment for the last 5-8 years. Family psychiatric history is notable for depression (siblings) along with Autism Spectrum and Anxiety disorders (son). Inspection of test results revealed average scores on measures of intellect, learning, memory, language, and higher-order executive functioning with lower scores on measures of attention, processing speed, and visuoperceptual reasoning. Physically she demonstrated bilateral fine motor dexterity slowing and grip weakness. Conclusions: This is the first comprehensive neuropsychological assessment of a patient with systemic scleroderma with corresponding neuroimaging. The profile and constellation of symptoms are consistent with the systemic variant of scleroderma often referenced by “CREST” [Calcinosis, Raynaud’s syndrome, Esophageal problems, Sclerodactyly, and Telangiectasia]. A review (Amaral et al 2013) noted a high frequency of “white matter lesions in asymptomatic SSc patients, suggesting smaller branches and perforating arteries involvement”, which may explain the documented subtle executive dysfunction and neuroimaging findings seen in our patient. Neuropsychologists should be aware of this complicated syndrome.

Poster

02/16/2024
01:45 pm - 03:00 pm
Room: Majestic Complex (Posters 61-120)

Poster Session 08: Cognition | Cognitive Reserve Variables

Final Abstract #74

CREST and Cognition in Systemic Scleroderma

Qingyang Zhu, University of Chicago Medical Center, Chicago, United States
Maureen Lacy, University of Chicago Medical Center, Chicago, United States

Category: Medical/Neurological Disorders/Other (Adult)

Keyword 1: neuropsychological assessment
Keyword 2: autoimmune disorders
Keyword 3: cognitive functioning

Objective:

Scleroderma is a rare, rheumatologic disease with estimates of 17.6 in 100,000 individuals. Notably, there is a higher prevalence in women, particularly Black women. Simple Scleroderma typically affects connective tissue causing excessive collagen resulting in fibrosis in the skin. Systemic Scleroderma results in a more systemic constellation of systems driven by an autoimmune attack on connective tissue in addition to vasculature throughout the body. As such, patients are at elevated risk for cardiovascular, interstitial lung, and hematologic dysfunction. A 2023 survey (Chen et al 2023) found that over 50% of patients with systemic sclerosis endorsed significant cognitive impairment.

Participants and Methods:

A retrospective review was conducted of the medical record, neuroimaging reports, laboratory findings, and neuropsychological report of a woman referred by her neurologist due to mild cognitive (e.g., language retrieval, memory, and visuospatial impairment) and mood problems along with neuropathy of unclear etiology.

Results:

A 64-year-old right-handed White female reported slowly progressive distractibility, short-term memory, naming, and word retrieval problems over the last year. She also reported increased headaches, imbalance, dry eyes, and diffuse body pain in the context of a complicated medical history: 30s marked by Reynaud’s; 40s complicated by rising breathing issues with diagnoses of Asthma, Bronchitis, Pulmonary Hypertension, and Cardiac stenosis leading to stenting; 50s marked by worsening breathing, hypertension, pre-diabetes, thyroid cysts, elevated liver enzymes, and varied elevated immune antibodies; 60s marked by elevated APL enzymes and stable yet low platelets. Medical record review lists: scleroderma, coronary artery disease, hypertension, dyslipidemia, GERD, NASH, and pre-diabetes, along with recent thrombocytopenia. Family history includes migraines (son), diabetes (brother, son), and cancer (brother). Lab results were notable for high glucose and low platelets with high liver enzymes, reticulocyte count, kappa free light chain and C-reactive protein. B12 and TSH were normal. Prior labs revealed elevated anticardiolipin antibodies. MRI revealed “prominent sulci and ventricles” along with “mild chronic small vessel ischemic changes.” Emotionally she spoke of sadness and endorsed significant anxiety symptoms with worries around family stressors and fears of COVID-19 infection. Psychiatric history includes Major Depression and Seasonal Affective disorders treated with psychotherapy and pharmacological treatment for the last 5-8 years. Family psychiatric history is notable for depression (siblings) along with Autism Spectrum and Anxiety disorders (son). Inspection of test results revealed average scores on measures of intellect, learning, memory, language, and higher-order executive functioning with lower scores on measures of attention, processing speed, and visuoperceptual reasoning. Physically she demonstrated bilateral fine motor dexterity slowing and grip weakness.

Conclusions:

This is the first comprehensive neuropsychological assessment of a patient with systemic scleroderma with corresponding neuroimaging. The profile and constellation of symptoms are consistent with the systemic variant of scleroderma often referenced by “CREST” [Calcinosis, Raynaud’s syndrome, Esophageal problems, Sclerodactyly, and Telangiectasia]. A review (Amaral et al 2013) noted a high frequency of “white matter lesions in asymptomatic SSc patients, suggesting smaller branches and perforating arteries involvement”, which may explain the documented subtle executive dysfunction and neuroimaging findings seen in our patient. Neuropsychologists should be aware of this complicated syndrome.