Long-term clinical, MRI, and cognitive follow-up in a large cohort of pathologically confirmed, predominantly tumefactive multiple sclerosis

Background: Limited studies have described long-term outcomes in pathology confirmed multiple sclerosis (MS). Objectives: To describe long-term clinical–radiographic–cognitive outcomes in a prospectively followed cohort of patients with pathologically confirmed CNS demyelinating disease, consistent...

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Published in:Multiple sclerosis 2022-03, Vol.28 (3), p.441-452
Main Authors: Kalinowska-Lyszczarz, Alicja, Tillema, Jan-Mendelt, Tobin, W Oliver, Guo, Yong, Fitz-Gibbon, Patrick D, Weigand, Stephen D, Giraldo-Chica, Monica, Port, John D, Lucchinetti, Claudia F
Format: Article
Language:English
Subjects:
Age
Biopsy
Brain - pathology
Cognition
Cognitive ability
Demyelinating diseases
Demyelinating Diseases - diagnostic imaging
Demyelinating Diseases - pathology
Demyelination
Executive function
Follow-Up Studies
Humans
Lesions
Magnetic Resonance Imaging
Multiple Sclerosis
Patients
Short term memory
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Summary:Background: Limited studies have described long-term outcomes in pathology confirmed multiple sclerosis (MS). Objectives: To describe long-term clinical–radiographic–cognitive outcomes in a prospectively followed cohort of patients with pathologically confirmed CNS demyelinating disease, consistent with MS. Methods: Subjects underwent clinical assessment, standardized 3T-MRI brain, and cognitive battery. Results: Seventy-five patients were included. Biopsied lesion size was ⩾ 2 cm in 62/75. At follow-up, median duration since biopsy was 11 years. Median EDSS was 3 and lesion burden was large (median 10 cm3). At follow-up, 57/75 met MS criteria, 17/75 had clinically isolated syndrome, and 1 radiographic changes only. Disability scores were comparable to a prevalence cohort in Olmsted County (p < 0.001, n = 218). Cognitive outcomes below age-normed standards included psychomotor, attention, working memory, and executive function domains. Total lesion volume and index lesion-related severity correlated with EDSS and cognitive performance. Volumetric cortical/subcortical GM correlated less than lesion metrics to cognitive outcomes. Conclusion: Despite early aggressive course in pathologically confirmed MS, its long-term course was comparable to typical MS in our study. Cognitive impairment in this group seemed to correlate strongest to index lesion severity and total lesion volume. It remains to be established how the aggressive nature of the lesion, biopsy, and treatment affect clinical/cognitive outcomes.
ISSN:1352-4585
1477-0970
DOI:10.1177/13524585211024162