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Background and Objective:The prevalence and role of the motor band sign (MBS) remain unclear in motor neuron disease. We report the frequency of MBS in amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS), its correlation with clinical upper motor neuron (UMN) signs, and prognostic value in ALS.Methods:We conducted a retrospective study of ALS, PLS, and controls with retrievable MRI between 2010 and 2018. We compared the frequencies of MBS across the three groups, and studied correlation between susceptibility-weighted MRI measurements in primary motor cortices and contralateral UMN features. Clinical outcomes were compared between ALS with and without MBS.Results:Thirteen ALS, 5 PLS, and 10 controls were included (median age 60 years, IQR 54–66 years; 14/28 males). MBS was present in 9/13 (69.2%, 95% CI 38.9–89.6%) and 4/5 (80.0%, 95% CI 29.9–99.0%) of ALS and PLS, respectively, and none in controls. 2/13 (15.4%, 95% CI 2.7–46.3%) ALS and 3/5 (60.0%, 95% CI 17.0–92.7%) PLS had MBS in the absence of corticospinal T2/FLAIR hyperintensity sign. Susceptibility measurements in left motor cortices had a significantly positive correlation with contralateral UMN signs in ALS (τb = 0.628, p = 0.03). Similar but nonsignificant trends was observed for right motor cortices in ALS (τb = 0.516, p = 0.07). There were no significant differences in mRS at last follow-up, mortality, or time from symptom onset to last follow-up between ALS patients with and without MBS.Conclusions:We provide limited evidence that MBS and susceptibility quantification measurements in motor cortices may serve as surrogate markers of UMN involvement in motor neuron disease.

Background: With the advent of the 2016 WHO classification of tumours, prognostically distinct subclasses of glioma have been revealed. A subset of gliomas which harbor the isocitrate dehydrogenase (IDH) mutation have a survival advantage. 2-Hydroxyglutarate (2-HG) is a byproduct of faulty IDH metabolism in IDH mutants making it an ideal tumour biomarker. Since pre-operative detection of this metabolite using magnetic resonance spectroscopy (MRS) may yield valuable information for the neurosurgeon, we undertook the first Canadian utility study to detect 2-HG via MRS. Methods: We will recruit 150 patients presenting with a newly suspected glioma. All patients will undergo MRS scans for 2-HG pre-operatively and the neuropathologist will determine IDH status post-operatively based on immunohistochemistry and DNA sequencing. Pre-operative detection of 2-HG will be compared to post-operative IDH status. Results: To date, of 34 eligible subjects, 29 have glioma determined by pathology. Seven of these were IDH-mutant positive by pathology, of which 3 were detected by MRS. One glioma positive for 2-HG on MRS turned out to be IDH mutant negative on pathology. Conclusions: Prospective detection of 2-HG via MRS is feasible in the clinical setting. Additional subjects as well as refinement of our MRS protocol may yield higher sensitivity and specificity of this novel and clinically relevant diagnostic tool.