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Neurology Jan 2020To assess the evidence of a relationship between muscle MRI and disease severity in Duchenne muscular dystrophy (DMD).
OBJECTIVE
To assess the evidence of a relationship between muscle MRI and disease severity in Duchenne muscular dystrophy (DMD).
METHODS
We conducted a systematic review of studies that analyzed correlations between MRI measurements and motor function in patients with DMD. PubMed, Cochrane, Scopus, and Web of Science were searched using relevant keywords and inclusion/exclusion criteria (January 1, 1990-January 31, 2019). We evaluated article quality using the Joanna Briggs Institute scale. Information regarding the samples included, muscles evaluated, MRI protocols and motor function tests used was collected from each article. Correlations between MRI measurements and motor function were reported exhaustively.
RESULTS
Seventeen of 1,629 studies identified were included. Most patients included were ambulant with a mean age of 8.9 years. Most studies evaluated lower limb muscles. Moderate to excellent correlations were found between MRI measurements and motor function. The strongest correlations were found for quantitative MRI measurements such as fat fraction or mean T2. Correlations were stronger for lower leg muscles such as soleus. One longitudinal study reported that changes in soleus mean T2 were highly correlated with changes in motor function.
CONCLUSION
The findings of this systematic review showed that MRI measurements can be used as biomarkers of disease severity in ambulant patients with DMD. Guidelines are proposed to help clinicians choose the most appropriate MRI measurements and muscles to evaluate. Studies exploring upper limb muscles, other stages of the disease, and sensitivity of measurements to change are needed.
Topics: Humans; Magnetic Resonance Imaging; Male; Motor Activity; Muscle, Skeletal; Muscular Dystrophy, Duchenne
PubMed: 31892637
DOI: 10.1212/WNL.0000000000008811 -
Journal of Clinical Neuroscience :... Aug 2019We studied patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma and...
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma: Comparison of clinical features and risk factors suggestive of underlying lymphomas.
BACKGROUND
We studied patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) associated with or without lymphoma and measured risk factors suggestive of an underlying lymphoma and follow-up outcomes.
METHODS
CLIPPERS patients associated with or without lymphoma were included into this study. Clinical presentations were documented, risk factors suggestive of an underlying lymphoma were tested, and prognostic differences in terms of death were compared.
RESULTS
Ten patients had a diagnosis of CLIPPERS associated with lymphoma, with 6 B-cell non-Hodgkin lymphoma, 2 T-cell non-Hodgkin lymphoma and 2 Hodgkin lymphoma. Using multivariate logistic analysis, the following 3 independent risk factors were found to be related to a final diagnosis of lymphoma: hyperreflexia (HR 16.56; 95% CI 1.03-265.29; p = 0.032), elevated protein in CSF (HR 11.59; 95% CI 1.24-108.39; p = 0.047), and recurrences between 2 months and 1 year after treatment (HR 29.27; 95% CI 2.09-409.58; p = 0.012). The model calibration was satisfactory (p = 0.392 with the Hosmer-Lemeshow test), and the discrimination power was good (area under the receiver operating characteristic curve 0.921; p < 0.001, 95% CI 0.826-1.000). Patients with CLIPPERS associated with lymphoma had higher mortality rate and lymphoma was a significant predictor of total mortality (HR 0.040; 95% CI 0.006-0.262; p = 0.001).
CONCLUSIONS
Hyperreflexia, elevated protein in CSF and recurrences between 2 months and 1 year after treatment are risk factors suggesting an underlying lymphoma. Relapses during high-dose steroids maintenance therapy can be indicative of lymphoma, too. Patients having CLIPPERS associated with lymphoma have a worse prognosis than those without lymphoma.
Topics: Adult; Brain Diseases; Female; Humans; Inflammation; Lymphoma; Magnetic Resonance Imaging; Male; Middle Aged; Pons; Steroids
PubMed: 31088767
DOI: 10.1016/j.jocn.2019.04.022