The modified rankin scale to assess disability in myasthenia gravis: Comparing with other tools

Introduction: We explored the modified Rankin scale (mRS) as a tool to quantitate disability in myasthenia gravis (MG). Our aim was to correlate patients' perception of their disability with that of the care provider and determine its relationship with other MG‐related scores. Methods: We evaluated 107 MG patients at 2 neurological centers. Patients were assessed over the telephone before and after clinic visits using the 15‐item Myasthenia Gravis Quality‐of‐Life index (MG‐QOL15) and mRS. At the clinic, patients were assessed using the MG‐QOL15, MG Composite (MGC), and mRS. Results: The MG‐QOL15 correlated with the MGC, mRS, and assessors' scores of patients. Assessors' perception of disease burden was in line with that of the patients' scoring. MG‐QOL15 scores obtained over the telephone were consistent with those obtained in the clinic. Scores were generally higher in patients receiving steroids at >5 mg/day and in those receiving or seeking benefits. Conclusion: The MG‐QOL15 and mRS are useful for estimating disability in MG. Muscle Nerve 50 : 501–507, 2014     

Epileptic Activity Intrinsically Generated in the Human Cerebellum

Neoplastic or dysplastic neuronal tissue in the brain stem and cerebellum can become epileptogenic in pediatric patients. However, it is unknown whether such tissue may transform intrinsic properties of the human cerebellum, making it capable of generating epileptic population activity. We noninvasively detected epileptiform signals unaveraged in a pediatric patient with epilepsy due to a tumor in the middle cerebellar peduncle. Analysis of generators of the signals revealed that the cerebellum ipsilateral and contralateral to the tumor was the dominant interictal spike generator and could initiate ictal activity, suggesting that human cerebellum may become capable of intrinsically generating epileptic activity. ANN NEUROL 2020;88:418–422.     

Debugging Adaptive Deep Brain Stimulation for Parkinson's Disease

Deep brain stimulation (DBS) is a successful treatment for patients with Parkinson's disease. In adaptive DBS, stimulation is titrated according to feedback about clinical state and underlying pathophysiology. This contrasts with conventional stimulation, which is fixed and continuous. In acute trials, adaptive stimulation matches the efficacy of conventional stimulation while delivering about half the electrical energy. The latter means potentially fewer side-effects. The next step is to determine the long-term efficacy, efficiency, and side-effect profile of adaptive stimulation, and chronic trials are currently being considered by the medical devices industry. However, there are several different approaches to adaptive DBS, and several possible limitations have been highlighted. Here we review the findings to date to ascertain how and who to stimulate in chronic trials designed to establish the long-term utility of adaptive DBS. © 2020 International Parkinson and Movement Disorder Society