Objective Benign multiple sclerosis (MS) is a debated clinical entity, due to the absence of objective biomarkers of such disease subtype. The aim of the present study is to identify clinical, cognitive and MRI features of MS patients with long disease duration and low physical disability level, who were never treated with disease-modifying drugs (DMDs). Materials and Methods MS patients (McDonald’s criteria 2010) with at least 10 years of disease duration, EDSS score ≤3.5 and never exposed to DMDs were identified among 520 MS cases currently followed at Verona University Hospital MS Center. Patients accepting to participate in the study underwent neurological examination, cognitive assessment (Rao’s battery and Stroop test) and brain MRI including 3D T1, FLAIR and DIR sequences. Other clinical variables were retrospectively collected at the time of the first visit at our Center and prospectively updated thereafter. Results Thirty-five patients fulfilling inclusion criteria were identified. Twenty-one (60%) were females, current mean age was 51 (± 10) years, with mean age at onset 30 (± 9) years, median disease duration 18 (10-64) years, median EDSS score at last follow-up (2013 or 2014) 1.0 (0-3), and relapsing-remitting clinical course in all cases. Fifty-per-cent of cases had sensory symptoms at onset, 72% recovered completely after the first clinical episode, 79% were free from relapses during the first and second year from onset, and 11% had no clinical relapses during the disease course. Up to now fourteen patients have completed neuropsychological evaluation: 8 resulted normal, 4 cognitively impaired (≥3 failed tests), and 2 had borderline results (1-2 failed tests). Of the 9 subjects who performed the MRI protocol, all had multiple disseminated T2 lesions in brain white matter, while 7 (78%) had at least one intracortical lesion. Six patients underwent both neuropsychological and MRI assessment: cortical lesions were detected in 3 cases with cognitive impairment and in 2 cases without; one subject had neither cognitive impairment nor cortical lesions. Discussion and Conclusions Sensory symptoms at onset, full recovery after the first clinical event, and absence of relapses during the first and second year from onset may be considered clinical predictors of a benign disease course. MS patients with low physical disability after 18 years from onset seem to maintain preserved cognitive functions, despite the presence of cortical involvement. High efficiency of CNS compensatory mechanisms, neuronal plasticity, and limited extension of cortical lesions may be hypothesized as possible explanations of our findings
Long-term clinical, cognitive and MRI characteristics of patients with untreated benign multiple sclerosis: a natural history study
Forlivesi, Stefano;GAJOFATTO, Alberto;CALABRESE, Massimiliano;MONACO, Salvatore;BENEDETTI, MARIADONATA
2014-01-01
Abstract
Objective Benign multiple sclerosis (MS) is a debated clinical entity, due to the absence of objective biomarkers of such disease subtype. The aim of the present study is to identify clinical, cognitive and MRI features of MS patients with long disease duration and low physical disability level, who were never treated with disease-modifying drugs (DMDs). Materials and Methods MS patients (McDonald’s criteria 2010) with at least 10 years of disease duration, EDSS score ≤3.5 and never exposed to DMDs were identified among 520 MS cases currently followed at Verona University Hospital MS Center. Patients accepting to participate in the study underwent neurological examination, cognitive assessment (Rao’s battery and Stroop test) and brain MRI including 3D T1, FLAIR and DIR sequences. Other clinical variables were retrospectively collected at the time of the first visit at our Center and prospectively updated thereafter. Results Thirty-five patients fulfilling inclusion criteria were identified. Twenty-one (60%) were females, current mean age was 51 (± 10) years, with mean age at onset 30 (± 9) years, median disease duration 18 (10-64) years, median EDSS score at last follow-up (2013 or 2014) 1.0 (0-3), and relapsing-remitting clinical course in all cases. Fifty-per-cent of cases had sensory symptoms at onset, 72% recovered completely after the first clinical episode, 79% were free from relapses during the first and second year from onset, and 11% had no clinical relapses during the disease course. Up to now fourteen patients have completed neuropsychological evaluation: 8 resulted normal, 4 cognitively impaired (≥3 failed tests), and 2 had borderline results (1-2 failed tests). Of the 9 subjects who performed the MRI protocol, all had multiple disseminated T2 lesions in brain white matter, while 7 (78%) had at least one intracortical lesion. Six patients underwent both neuropsychological and MRI assessment: cortical lesions were detected in 3 cases with cognitive impairment and in 2 cases without; one subject had neither cognitive impairment nor cortical lesions. Discussion and Conclusions Sensory symptoms at onset, full recovery after the first clinical event, and absence of relapses during the first and second year from onset may be considered clinical predictors of a benign disease course. MS patients with low physical disability after 18 years from onset seem to maintain preserved cognitive functions, despite the presence of cortical involvement. High efficiency of CNS compensatory mechanisms, neuronal plasticity, and limited extension of cortical lesions may be hypothesized as possible explanations of our findings
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