This week, Neurology News Network covered the results of a observational “real-world” study that deep brain stimulation may increase the risk of dementia and cognitive decline in patients with Parkinson disease who also have mild cognitive impairment, and a novel ALS diagnostic index that's been shown to reliably differentiate ALS from mimicking disorders at an early stage in the disease pathology. Additionally, the network discussed preliminary findings from the IGNITE-DMD trial (Transcript below.)

Jenna:
Welcome to Neurology News Network. I’m Jenna Payesko. Let’s get into the news from this week.

Deep brain stimulation may increase the risk of dementia and cognitive decline in patients with Parkinson disease who also have mild cognitive impairment. The results found that disease duration, but not age, at the time of DBS surgery was significantly related to the risk of developing dementia.

Ultimately, those treated with subthalamic DBS deteriorated by 1.6 points per year on the modified Mattis Dementia Rating Scale, and preoperative MCI correlated with conversion to dementia. The investigators concluded that the study provides long-term results of cognitive decline in DBS-treated patients with presurgical MCI, possibly predicting the conversion to dementia.

A novel ALS diagnostic index has been shown to reliably differentiate ALS from mimicking disorders at an early stage in the disease pathology, according to new research.

The prospective, multicenter study ultimately found that the ALSDI, which includes clinical phenotyping, conventional peripheral neurophysiology, and transcranial magnetic stimulation measures, dependably distinguished ALS from other neuromuscular disorders. The reference standard was the Awaji criteria, which was applied to all patients at recruitment.

Preliminary findings from the IGNITE-DMD trial have shown positive signs for Solid Biosciences’s microdystrophin gene transfer therapy, SGT-001, which have led to the company’s desire to dose escalate as planned and as soon as possible, Solid announced.

The phase 1/2 dose-ascending trial is evaluating the safety and efficacy of the gene therapy in Duchenne muscular dystrophy. The initial 3-month biopsy data from the first 3 patients dosed showed levels of micro-dystrophin protein expression. Solid is currently “engaging with the appropriate parties” to move things forward.

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